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Spring Quarter Patho Exam 2 (Neuro)

Terms in this set (367)

What is the vestibulo-ocular reflex?
An inhibitory signal to the extra ocular muscles on one side with an excitatory signal to the muscles on the other side in order to provide best vision and stabilization of the line of sight in space during head motion
What is the most important component of evaluation of patients with vertigo?
History - description of dizziness, associated neurologic factors, duration

ex) spinning = vertigo
unstable/off balance = disequilibrium
oscillopsia = cannot focus, eyes bouncing
Why is duration of vertigo an important piece of information?
Can be a guiding factor in diagnosis, ex)
Seconds = BPPV
Minutes = vertebrobasilar insufficiency, migraine
Hours = end-lymphatic hydrops (Menieres)
Days = labyrinthine injury
What is Tulios phenomenon?
Dizziness due to loud sound
What is nystagmus?
Involuntary movement of the eyes in an up/down, back/forth motion. Interferes with head control.
What testing should be considered in a dizzy patient?
Special PE: Dix-Hallpike, Romberg, Fukuda
Labs: ANA, RA, sedimentation rate, CBC, TSH
Imaging: MRI brain with gadolinium, CT temporal bone without contrast
What is benign paroxysmal positional vertigo?
Recurrent peripheral vertigo resulting from positional changes due to dislodged calcium carbonate crystals in the semicircular canals (95% posterior canal)
*Lasts less than one minute per episode
Note - no hearing loss in BPPV
What are causes of BPPV?
Idiopathic
Post-traumatic
Viral causes
How do you diagnose BPPV?
History
Dix-Hallpike maneuver - diagnostic and therapeutic
How do you treat BPPV?
Epley maneuver
Vestibular rehabilitation therapy
Surgery (singular neurectomy, posterior semicircular canal occlusion)
What is superior canal dehiscence syndrome?
Chronic transient vertigo induced by recumbency, valsalva, loud sound, changes in middle ear pressure
+/- sensorineural hearing loss
How do you diagnose superior canal dehiscence syndrome?
History
Can elicit nystagmus with tragal compression
CT of temporal bone showing thinning/loss of bone over semicircular canal
How do you treat superior canal dehiscence syndrome?
Tx often not needed
Superior semicircular canal ablation
Superior semicircular canal resurfacing
What symptoms are classic to Meniere's disease?
Paroxysmal attacks of vertigo
Fluctuating hearing loss
Low pitch tinnitus
Aural pressure
Meniere's syndrome is characterized by symptoms that _____:
a) last less than 1 minute in duration
b) occur together
c) fluctuate
d) both b & c
D) both b & c
What causes Meniere's disease?
Etiology unknown, theorized to be related to:
Overproduction of endolymph/under resorption
Autoimmunity
Viral injury
Genetic defect
How do you diagnose Meniere's disease?
No labs/imaging that can diagnose - strictly based on history/sxs

Can use MRI/audiogram to rule out neuroma, lab tests (TPPA, ESR, ANA, CMP/CBC, TSH) - rule out other causes
How do you treat Meniere's disease?
Low salt diet <2000mg/day
Diuretics (thiazides, potassium sparing)
Vestibular suppressants (meclizine, valium)

Second-line:
Antihistamines (theorized d/t allergy)
Desensitization therapy
Oral steroids
Meniett device

Surgery: intratympanic dexamethasone, end-lymphatic shunt, intratympanic gentamicin, labyrinthectomy, vestibular neurectomy
What can cause labyrinthine injury?
Vestibular neuronitis
Vascular injury
Labyrinthine concussion
What are symptoms of labyrinthine injury?
Acute spinning vertigo
Nausea
Blurred vision
Gradual resolution of sxs over days to weeks
How do you diagnose labyrinthine injury?
Neurologic/otologic exam
Audiogram
MRI
Labs (autoimmune workup)
How do you treat labyrinthine injury?
Symptom relief tx
Vestibular rehabilitation
Vestibular suppressants
Antiemetics
What causes vestibular migraines?
Abnormal discharges within the cells of the brain causing constriction of the walls of the blood vessels
What are symptoms of vestibular migraines?
Dizziness
Motion intolerance
Spatial disorientation
Diminished eye focus/photosensitivity
Sound sensitivity/tinnitus
Ear fullness
+/- headache
How do you treat vestibular migraines?
Migraine prophylaxis - avoid triggers
Beta blockers
Anticonvulsants
Calcium channel blockers
Tricyclic antidepressants
Vestibular rehabilitation
What is the vestibular migraine diet?
Encouraged to avoid:
Caffeine
Chocolate, nuts
Alcohol (beers and red wine especially)
Aged cheese
Processed meats
What are other causes of vertigo/dizziness?
Acoustic neuroma/Vestibular Schwannoma (benign tumor)
Brain tumors
Otosclerosis
Autoimmune inner ear disease
Perilymph fistula
Vertebra-basilar insufficiency
What is the medical definition of confusion?
Disturbed orientation in regard to time, place or person, sometimes accompanied by disordered consciousness
What history questions are important in the assessment of a confused patient?
Acute vs chronic?
Rapid vs gradual onset?
Prior episodes?
Baseline status?
Accompanying symptoms and provoking factors?
Any recent medication changes?
What is the AVPU scale for levels of consciousness?
Alert
Verbal
Pain
Unresponsive
What labs should be evaluated in a confused patient?
CMP (assess electrolytes, BG, renal fx, liver fx)
CBC (rule out anemia, infection)
UA (rule out infection/decreased renal function)
Toxicology
What are potential causes of high-risk acute confusion?
Hypoxemia
Shock
Electrolyte imbalance (hyponatremia)
Toxic ingestion
Trauma (head injury, concussion)
CNS infection (meningitis, encephalitis)
Encephalopathy
Intoxication
Seizure (post-ictal state)
Neoplasm
What is encephalopathy?
Broad term used to describe abnormal brain function
What are symptoms of encephalopathy?
Altered mental status and/or confusion/delirium
What are symptoms of viral/infectious encephalitis? How do you treat?
Headache
Fever
Nuchal rigidity
Myalgia
Seizures
Confusion

Supportive tx
What are symptoms of Wernike encephalopathy? How do you treat?
Ophthalmoplegia
Ataxia
Confusion
Coma

Tx w/ thiamine (vitamin B1)
Note Wernike encephalopathy is caused by vitamin B1 deficiency from malnutrition/alcoholism
What are symptoms of hepatic encephalopathy? How do you treat?
Sweet breath odor
Confusion
Asterixis
Ascites
Coma

Tx - lactulose, antibiotics
What are symptoms of metabolic encephalopathy? How do you treat?
Altered level of consciousness
Confusion
Seizures
Coma

Tx - correct underlying cause
What are symptoms of hypoxic-ischemic encephalopathy? How do you treat?
Altered level of consciousness
Confusion
Coma

Tx - correct hypoxemia, often irreversible
What are symptoms of Hashimoto encephalopathy? How do you treat?
Altered level of consciousness
Confusion
Seizures
Psychosis

Tx - corticosteroids
What are symptoms of Lyme encephalopathy? How do you treat?
Memory loss
Inability to concentrate
Headache
Depression

Tx - doxycycline
What are symptoms of chronic traumatic encephalopathy? How do you treat?
Cognitive impairment
Headache
Personality changes

Tx - irreversible, dx is usually by autopsy
What are symptoms of Creutzfeld-Jakob disease? How do you treat?
Ataxia
Memory loss
Dementia

No tx - caused by a prion but symptom onset may take years
What is the medical definition for delirium? What is the hallmark of delirium?
State of acute confusion that fluctuates over hours or days

Hallmark - deficit of attention with variable loss of memory, executive function, visuospatial tasks and language
*Note: delirium is usually reversible if you can find the cause
What symptoms are associated with delirium?
Altered sleep-wake cycles
Perceptual disturbances such as hallucinations/delusions
Affect change
Autonomic findings such as HR and BP instability
What are the two subtypes of delirium?
Hyperactive - involves hallucinations, agitation and hyperarousal; may be accompanied w/ life-threatening autonomic instability

Hyopactive - withdrawn, quiet, apathetic and psychomotor slowing
What are risk factors for delirium?
Older age >65yo
Baseline cognitive dysfunction such as dementia
Sensory deprivation such as hearing/visual impairment
Immobility
Malnutrition
Other underlying medical/neurological illnesses
What are common causes of delirium?
Infection (UTI)
Prescription medications
Drug abuse and/or alcohol
Toxic exposure (CO, pesticides, ethylene glycol)
Metabolic conditions
Electrolyte imbalance
Hypoxemia
Liver failure/hepatic encephalopathy
Other encephalopathies
Heart failure
Dehydration/malnutrition
Anemia
What diagnostics should be considered when evaluating a patient with delirium?
*Thorough history and PE
FSBS (finger stick blood sugar)
CBC
CMP
UA
Toxicology
ABG
CT/MRI
Lumbar puncture
EEG
If systemic infection suspected - CXR, ECG, lactate, blood cultures
How do you treat delirium?
Treat underlying condition
Supportive care
What is the definition of dementia?
Decline in cognitive functioning that interferes with the individual's ability to function independently - it is an acquired syndrome, not a specific disease
What are the two broad categories of dementia?
Neurodegenerative or irreversible
Non-neurodegenerative or potentially reversible
What are examples of neurodegenerative dementias?
Alzheimer disease
Dementia with Lewy bodies, Parkinson disease dementia
Multiple system atrophy
Non-parkinsonian movement disorders (Huntington)
Alcoholic cognitive impairment/dementia
Chronic traumatic encephalopathy
Prion disease
Motor neuron disease (ALS)
What are examples of non-neurodegenerative dementias?
Vascular dementia
Normal pressure hydrocephalus
Metabolic (Cushing's, hypothyroidism, malnutrition)
Depression, bipolar disorder
Neoplasia
Infection (syphilis, HIV, Lyme)
Toxins
Vasculitis
How common is Alzheimer disease?
Most common type of neurodegenerative dementia
Affects 5-6% of those >65yo
Affects up to 30% of those >85yo
Mean survival 10-12 years after onset
What are symptoms of Alzheimer disease?
Slowly progressive memory decline
Forgetfulness
Irritability
Apathy
Withdrawn mood
How do you diagnose Alzheimer disease?
Thorough evaluation to rule out other causes
Cognitive testing
MRI/PET scan - may show temporal lobe atrophy
CSF - shows biomarkers of increased beta-amyloid proteins/tau proteins
There are no disease-modifying medications to treat Alzheimer disease. What medications can be used temporarily to improve cognitive/behavioral function?
Donepazil
Rivastigmine
Galantamine
Memantine
What are considered protective factors to prevent Alzheimer disease?
Mediterranean diet
Aerobic exercise
Socially and cognitively stimulating activities
What are the most commonly seen peripheral nerve disorders?
Guillain-Barre syndrome
Peripheral neuropathy
Complex regional pain syndrome
What is Guillain-Barre syndrome?
An autoimmune disease in which the peripheral nerves become inflamed. Results in acute, rapidly evolving, ascending motor paralysis - evolves over a few hours to a few days

2 subtypes: demyelination resulting in interference of nerve conduction OR direct attack on axon that blocks nerve conduction
Who commonly presents with Guillain-Barre syndrome?
5000-6000 cases per year
Men > women
Adults at higher risk than children but can affect any age
What causes Guillain-Barre syndrome?
Typically triggered by an infectious event - onset of symptoms 1-3 weeks post-infection

*Campylobacter jejuni
Cytomegalovirus
Epstein-Barr virus
HIV
Hepatitis E
Zika
Mycoplasma pneumoniae
COVID-19
What are the cardinal clinical features of Guillain-Barre syndrome?
Progressive ascending symmetric muscle weakness with absent or depressed deep tendon reflexes
Weakness typically begins in legs
Can vary from mild difficulty walking to near complete paralysis involving all extremities/facial/respiratory and bulbar muscles
What are signs/symptoms of Guillain-Barre syndrome?
Back and extremity pain during acute phase
Paresthesias in hands and feet
Facial nerve palsies
Oropharyngeal weakness
Autonomic dysfunction (Brady/tachycardia, hyponatremia, GI/GU sxs, reversible cardiomyopathy)
How do you diagnose Guillain-Barre syndrome?
Confirmed by CSF and electrodiagnostic studies
CSF will usually show albuminocytologic dissociation (high protein levels, WBC count <5)
Electomyography (EMG) show evidence of acute polyneuropathy
Spinal MRI may show thickening of spinal nerve roots
How do you treat Guillain-Barre syndrome?
Supportive care - almost all require hospitalization
Respiratory failure requires intubation/mechanical ventilation
Management of autonomic dysfunction/CV involvement
Pain management
Bladder/bowel care
DVT prevention (because they are immobile)

Plasmapheresis or IVIG can help to shorten recovery
May need PT/rehab after recovery
Most patients fully recover
What causes peripheral neuropathy?
Injury to small or large-diameter nerves
What are common early symptoms of peripheral neuropathy? Later stages?
Distal numbness
Tingling
Pain
Burning sensation

Later: proximal numbness, distal weakness, atrophy
What commonly causes peripheral neuropathy?
Diabetes mellitus
Nerve compression or injury
Alcohol use
Toxin exposure
Hereditary diseases
Nutritional deficiencies
How do you diagnose peripheral neuropathy?
Hx and PE - thorough neurological exam, monofilament test, assess muscle bulk/tone
CBC
CMP w/ fasting BG
TSH
Vitamin B12
Serum protein electrophoresis w/ immunofixation
Imaging not often helpful in dx
How do you treat peripheral neuropathy?
Non-pharmacologic:
Good foot hygiene and wearing appropriate footwear
Physical therapy, gait training

Pharmacologic:
Gabapentinoids (gabapentin, pregabalin)
Antidepressants (amitriptyline, nortriptyline, venlafaxine, duloxetine, bupropion)
Topical medications (capsaicin, lidocaine)
Opioids generally not recommended
What is Erb's palsy (Erb-Duchene Palsy)?
Obstetric brachial plexus palsy - injury at birth to the upper brachial plexus (C5 and C6 nerve roots) resulting in loss of lateral rotators of the shoulder, arm flexors and hand extensor muscles

Arm hangs by the side and is rotated medially
Forearm is extended and pronated, wrist is flexed
What is Klumpke's palsy?
Low brachial plexus injury, typically results from stretching/hyperextension of the arm (can also occur from birth) - loss of C8 and T1 enervation which results in motor deficits in the hand "claw hand"

Loss of sensation along the C8 dermatome
May rarely also have ptosis
What is Bell's palsy?
Inflammation or viral infection of the facial nerve that causes one-sided or bilateral weakness/paralysis

*often d/t HSV-1, varicella-zoster virus, CMV, EBV, adenovirus, mumps, coxsackie, influenza B)
What are risk factors for Bell's palsy (cranial nerve VII)?
Pregnancy
Hypothyroidism
Diabetes
What are signs/symptoms of Bell's palsy?
Sudden onset (a few hours to 1-2 days) of facial weakness
Unilateral paralysis of facial muscles
Eyebrow sagging with inability to close the eye on the affected side
Disappearance of nasolabial fold
Drooping of the affected corner of the mouth

+/- Pain behind ear preceding paralysis, loss of taste in anterior tongue, altered secretions of lacrimal/salivary glands, abnormally sensitive hearing
How do you diagnose Bell's palsy?
Primarily clinical dx
Thorough cranial nerve evaluation
Labs/imaging generally not indicated
What is the prognosis for Bell's palsy?
~80% of patients recover within a few weeks or months - can take up to 6 months to fully recover (rarely patients will have permanent facial weakness)

May have recurrent attacks
How do you differentiate between Bell's palsy and stroke?
Peripheral vs central lesions
Bell's palsy affects the brow and forehead, asymmetrical elevation of eyebrows

Stroke is a central lesion, spares the forehead muscles on the affected side d/t bilateral central innervation of this area - symmetric elevation of the forehead and eyebrows
How do you treat Bell's palsy?
Symptomatic tx - eye patch to keep eye closed, artificial tears, massage weakened muscles around eyes

Glucocorticoids (shorten recovery time) prednisone
Antivirals (since often d/t herpes simplex - valacyclovir)
What is complex regional pain syndrome?
A chronic neurologic condition resulting from a traumatic injury that involves a body region, most often the distal limbs - pathogenesis is unknown
What are the two subtypes of complex regional pain syndrome?
CRPS type 1 - associated w/ absence of major nerve injury
CRPS type 2 - associated w/ presence of major nerve injury
What are risk factors for complex regional pain syndrome?
Females > males
Upper extremity injury
High-energy trauma
What are signs/symptoms of complex regional pain syndrome?
Burning, stinging or tearing pain
Continuous (spontaneous and/or evoked)
Regional (does not follow a specific dermatome)
Predominantly distal
Pain seems out of proportion in time/intensity to any known injury
Variably progressive over time, often worse at night
Sensory changes (light touch can cause pain)
Motor changes (extremity movement limited, may have tremor, decreased strength)
Differences in skin temperature/color/sweat/edema compared to unaffected side
Increased hair growth
Increased or decreased hair growth
Contraction and fibrosis of joints/fascia
Skin atrophy
What are the Budapest Consensus Criteria for Clinical Diagnosis of CRPS?
Continuing pain, which is disproportionate to any inciting event AND:
At least one symptom in 3 of the following 4 categories:

Sensory - increased sensitivity (hyperesthesia) or pain from a stimulus that typically does not cause pain (allodynia)

Vasomotor - reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry

Sudomotor/edema - reports of edema, heat intolerance, sweating changes, and/or sweating asymmetry

Motor/trophic - reports of decreased ROM and/or motor dysfunction (tremor, weakness, dystonia) and/or trophic changes (hair, skin, nails)
How do you treat CRPS?
Patient education about disease
PT & OT
Psychosocial assessment
Symptomatic pain management:
NSAIDs
Adjunctive meds for neuropathic pain such as gabapentin, pregabalin or a TCA
Bisphosphonates if they have abnormal uptake on bone scan
Topical lidocaine cream or capsaicin cream
Opioid use controversial and generally discouraged
What is a seizure?
Transient signs or symptoms resulting from abnormal excessive or hypersynchronous neuronal activity in the brain - can range from dramatic convulsive activity to experiential phenomena not readily discernible by an observer
What causes seizures?
Caused by paroxysmal discharges from groups of neurons in the brain (arise from excessive excitation or loss of inhibition of neurons, resulting in malfunction in the ion channels)

Can be acute symptomatic seizure - d/t systemic insult
Can be unprovoked seizure - unknown etiology likely d/t underlying brain lesion/nervous system disorder
What is a focal onset seizure?
A seizure that involves only a single area of the brain - typically one lobe of one hemisphere but can evolve to generalized tonic-clonic seizures

Further classified by level of awareness (intact awareness or impaired awareness)
Further classified by onset (motor or non-motor)
What is a generalized onset seizure?
May start in a specific point in the brain, but rapidly involves both cerebral hemispheres - always have some level of impaired awareness

Further classified by onset (motor or non-motor)
What are the different types of motor activity that can occur during seizures?
Tonic - sudden stiffness/tension in muscles of arms/legs/trunk and is most likely to happen during sleep

Clonic - sustained rhythmic jerking, may be focal or generalized

Myoclonic - brief shock-like jerks of a muscle or group of muscles

Atonic - sudden loss of muscle strength (drop seizures)

Hyperkinetic - irregular sequential ballistic movements of proximal limb or axial muscles, such as pedaling, pelvic thrusting, thrashing or rocking movements

Epileptic spasms - brief episodes of arm, leg, head flexion or extension (seen most commonly in children <2yo)
What are tonic-clonic seizures? (previously called grand-mal seizures)
Seizures characterized by generalized body stiffness/rigidity, arched back, and jerking movements of the trunk, extremities and head; associated with incontinence, frothing at mouth, tongue biting, eye blinking and cyanosis

Followed by post-octal phase
Most common type seen in metabolic derangements, drug withdrawal and head trauma
Where can you look for cyanosis in darker-skinned patients?
Lips
Tongue
Conjunctiva
Nail beds
What are different types of seizures manifestations associated with focal seizures?
Focal motor seizures - jerking movements of the face, one foot, one arm or another body part

Focal sensory seizures - may cause hearing problems, distorted olfactory sense or hallucinations

Focal autonomic seizures - can cause changes in HR/rhythm, BP and bowel/bladder function

Focal psychic seizures - can trigger emotions or memories causing fear, anxiety or a sense of deja vu
What is an absence seizure?
Characterized by a brief (4-20 seconds) loss of environmental awareness without loss of body tone, sometimes accompanied by simple automatisms - may have dozens of seizures per day

Age of onset 4-10 years old
Can be provoked by hyperventilation or flashing lights
What are the phases of a seizure?
Aura - feelings prior to a seizure which can manifest as music, swirling colors, a memory, a sense of impending doom, a smell or taste, nausea, or an intense sensation of deja vu

Ictal phase - time from onset of first sxs, including an aura, until end of seizure activity

Post-ictal phase - the time from end of seizure until return to baseline - initially unresponsive/limp, typically lasts 5-30 min, often feel profoundly fatigued after recovery
What are possible causes of acute symptomatic seizures?
Stroke/intracranial hemorrhage
Head trauma/TBI
Tumor
Eclampsia
Hypoxic-ischemic brain injury
Underlying/undiagnosed epilepsy
Infections (meningitis, encephalitis, brain abscess)
Vasculitis
Drug intoxication
Withdrawal states (alcohol, benzos)
Hypoglycemia, hyperglycemia
Hyponatremia
Hypocalcemia
Hypomagnesemia
Uremia
Hyperthyroidism
What are the key history points for evaluation of a first seizure?
Description of the aura
Duration of seizure
Nature of motor activity (general, local, tonic-clonic)
Loss of awareness (immediate or delayed)
Duration of post-ictal phase and course of return to baseline
Triggers/precipitating factors
Current medications/recent changes
Alcohol/drug use
What diagnostics should be ordered when evaluating a patient presenting with first seizure?
Rapid POC glucose
CBC
CMP
UA
Toxicology
hCG
Consider lactate
CT or MRI (MRI preferred)
ECG
Electroencephalogram
Lumbar puncture if infection suspected
How common is epilepsy?
Affects 50 million people worldwide
More common in low/middle income countries
Peaks in young age (0-1) and elderly (>60)
Can happen at any age
What criteria are required for diagnosis of epilepsy?
Meeting at least one of the following criteria:

At least 2 unprovoked seizures occurring more than 24 hours apart

One unprovoked seizure with a probability of further seizures occurring over the next 10 years (stroke, neoplasm, CNS infection, TBI)
Epilepsy syndromes are classified in two ways, what are they? (think age)
Epilepsy syndromes in the neonatal period
Epilepsy syndromes of childhood
What is status epilepticus?
5 minutes or more of:
Continuous seizure activity OR
Recurrent seizure activity without return to baseline

*medical emergency requiring prompt intervention - assess ABCs, trauma assessment, finger stick blood sugar
What are potential causes of acute status epilepticus?
Metabolic (hypoglycemia, electrolyte abnormality, renal failure)
Sepsis
CNS infection (meningitis, encephalitis, abscess)
Stroke (ischemic, ICH, SAH)
TBI
Drugs (medication non-compliance, intoxication, withdrawal from benzos/barbiturates/ETOH/sedatives)
Cardiac arrest, hypoxia
Hypertensive encephalopathy (or other encephalopathies)
Autoimmune encephalitis (paraneoplastic)
What re potential causes of chronic status epilepticus?
Epilepsy - breakthrough seizures
Chronic ETOH abuse
CNS tumors
Remote CNS pathology (stroke, abscess)
How do you manage patients with status epilepticus?
Protect from injury (but do not restrain)
Do not attempt to put anything in the mouth
Immediate seizure control tx (IV or IO access) - benzos, may be given bucally or rectally if no IV/IO available
What is included in follow-up care during the post-ictal phase in a patient with status epilepticus?
Positioning and airway control
Labs, imaging, etc to identify cause
Longer-acting anti-seizure medications as indicated
Intubation and continuous EEG monitoring may be required
Consult neurology
Who commonly presents with febrile seizures?
Most common between 6 months and 5 years of age
Note: this is a diagnosis of exclusion
What causes febrile seizures?
Viral illness
Pneumonia, URI/bronchitis, UTI, intra-abdominal infection, pharyngitis, otitis media

They result from an increase in body core temperature - high fevers increase neuronal excitability and lower seizure threshold; most common with temperatures >102.2F
Often associated with genetic susceptibilities/fam hx, certain immunizations (DTwP, MMR)
What is a simple febrile seizure?
Generalized tonic-clonic seizure < 15 min duratio
Usually have resolved before arrival to hospital and most children return to baseline quickly
*recurrence does not happen in 24 hours
What is a complex febrile seizure?
May be focal onset, prolonged or recurrent within 24hrs
The majority of children who develop complex febrile seizures do so with their first seizure
What are differential dx for febrile seizures?
Meningitis
Encephalitis
Septicemia
Heady injury
Toxic ingestion/exposure
Metabolic emergency
Febrile seizures
Shaking chills

Less likely: early manifestation of epilepsy, seizure mimic
What history points are important in evaluating a febrile seizure?
Prior seizures
Family hx of seizures
Description of seizure activity
Duration of seizure activity
Description and duration of post-ictal phase
Recent illness or abx use
Recent immunizations
Pt temperature at home
In simple febrile seizures, CBC/CMP are not of much value but they are indicated in complex febrile seizures to check for what?
Hyponatremia and other electrolyte abnormalities
When should you order imaging in the case of a patient with a febrile seizure?
Suspicion of ICH or tumor
Status epilpeticus
Failure to return to baseline in a reasonable period of time
Children with abnormally large heads
Persistently abnormal neurologic exam
Signs/symptoms of increased intracranial pressure

Chest x-ray if pneumonia suspected fever source
When should you perform an LP (lumbar puncture) and CSF examination in patients with febrile seizures?
Based on clinical exam - simple febrile seizures in otherwise well-appearing children who have returned to baseline generally do not require an LP

Who should be considered:
*Patients with other signs/symptoms of meningitis: altered LOC, nuchal rigidity, petechial rash, positive Kernig or Brudzinski sign
*Patients who have not received HiB or S. pneumonia immunization
*Patients who have recently been treated with antibiotics
How do you treat acute febrile seizures?
Most are self-limiting
Protect from injury
ABCs
IV lorazepam or diazepam (note: intranasal benzos not recommended)
Antipyretics
Hydration
Cooling measures
Treat source of fever
When should you refer a patient with a seizure to neurology? What will be included in initial work-up by the neurologist?
All patients suffering a first seizure should be referred
Initial work-up: EEG, seizure protocol MRI, risk-stratification, coordination of anti-seizure drug therapy

Patients with known seizure disorders who:
Are active alcoholics with withdrawal seizures
Patients with intractable seizures despite medication
What are differential dx for stroke-like symptoms?
Ischemic stroke
Hemorrhagic stroke
TIA
Intracranial mass lesion
Intoxication/overdose
Migraine
Hypoglycemia
Syncope
Vertigo
Metabolic/hypertensive encephalopathy
Seizure
Conversion reaction
What is the medical definition of stroke?
The sudden occlusion or rupture of cerebral arteries or veins resulting in focal cerebral damage and clinical neurologic deficits that persist for longer than 24hrs

*Can be ischemic or hemorrhagic
How common are strokes?
~795,000 new or recurrent strokes every year
~6.6 million Americans >20yo have had a stroke
Females > males
Age 55-75yo
Black Americans have double the rate of first stroke, 2x as likely to die from stroke

5th leading cause of death
Leading cause of adult disability
What are non-modifiable risk factors for stroke?
Increasing age
Gender
Race/ethnicity
Heredity/familial factors
What are modifiable risks factors for stroke?
Hypertension
Cardiac disease
Tobacco use (including vaping)
High fat/cholesterol diet
Prior TIA
Carotid stenosis
Diabetes (2x higher risk in women)

Not as well-documented but may be risk factors for stroke:
Physical inactivity
High weight status
Pregnancy/OCP use
Hormone replacement
Alcohol/drug abuse
Dietary factors
Insulin resistance
Hypercoagulable state
Hyperhomocysteinemia
Migraine headaches
Socioeconomic factors
What is the etiology of ischemic stroke?
Occlusion of a cerebral artery by a clot, may be embolic (clot came from somewhere else - typically cardiogenic) or thrombotic (formed in the brain) - accounts for 85% of strokes
What commonly causes cardioembolic stroke?
Atrial fibrillation - thrombus forms in heart
Atherosclerotic disease - thrombus forms elsewhere

Piece of thrombus or plaque debris breaks away becoming an embolus which then migrates and causes occlusion of a cerebral vessel leading to ischemia
What is carotid dissection? How does it cause stroke?
A tear in the intima of the carotid artery, blood dissects between the layers of the arterial wall and can release emboli and compress outflow to branching arteries leading to ischemia

*Can occur spontaneously or result from trauma to neck
What are intracranial dissections?
Rare - tear in the intima of an artery of the brain leading to ischemic stroke - can also result in SAH or other ICB

10-25% of strokes in patients <50yo
What is ischemic penumbra?
Vessels nearby a blocked vessel will immediately start to sprout new capillaries to try and salvage tissue and maintain blood supply - tissue may remain viable for several hours
Strokes are classified based on where they occur, what are the two areas they can occur?
Cortical regions (frontal, parietal, temporal and occipital lobes)

Subcortical i.e. below cortex (internal capsule, thalamus, basal ganglia, brainstem, cerebellum)
What are signs/symptoms of ischemic stroke?
New/sudden onset of:
Weakness (extremities, facial muscles)
Numbness
Vision changes (vision loss/diplopia)
Dizziness, vertigo, loss of balance
Aphasia (receptive or expressive)
Agnosia (impaired sensory)
Apraxia (impaired motor planning)

FAST - face looks uneven, one arm hanging down, slurred speech, time - call 911 asap
What are signs/symptoms of cerebellar strokes?
Ataxia
Vertigo
Balance problems
Nystagmus
*Large infarcts can cause herniation and death
What are signs/symptoms of brainstem strokes?
Cranial nerve dysfunction
Ataxia
Weakness
Altered LOC
Crossed motor and sensory deficits
What is included in the initial assessment of a patient suspected to have a stroke?
ABCs
Rapid assessment of deficits (Cincinnati Stroke Scale)
Hx (time last seen with normal presentation)
Pmhx
STAT labs - finger stick blood sugar, CBC, CMP, troponin, coags, hCG, fox screen
EKG
Chest x-ray

Neuroimaging - CT, MRI (note ischemia often does not show up for 6 hours)
What is included in the Cincinnati Stroke Scale? What rating on the NIH stroke scale indicates severe stroke?
Facial droop - one side of face doesn't move as well as other
Arm drift - one arm does not move the same as the other
Abnormal speech

>21 on NIH scale indicates severe stroke
What are indications for the use of thrombolytic therapy (TPA - Alteplase) in treatment of ischemic stroke?
Age 18 or older
Dx of ischemic stroke w/ neurological deficit
Time of stroke symptom onset within 3 hours
No exclusion criteria present
What are exclusion criteria (i.e. contraindications) to the use of thrombolytic therapy in tx of ischemic stroke?
If they are improving on their own

Evidence of intracranial hemorrhage on CT scan
Active internal bleeding or trauma
Clinical presentation suggestive of SAH
Spinal surgery, head trauma, or prior stroke in last 3 months
Evidence of multi-lobar infarct on CT
Hx of IC hemorrhage, AVM, aneurysm, or neoplasm
Uncontrolled HTN (SBP >185 or DBP >100)
Uncompressible arterial puncture within past 7 days
Witnessed seizure at stroke onset
Elevated PTT/INR
When is endovascular therapy indicated for stroke treatment? (Merci Clot Retriever, Penumbra System)
When the stroke is moderate to large with a retrievable clot - goals are to reperfuse salvageable/viable brain tissue, minimize distal embolization, minimize repercussion injury/hemorrhagic transformation

*May be used in combo with IV thrombolytics
Once treatment has been initiated, what other diagnostics should be run on a patient with a stroke?
Check carotid arteries (ultrasound, MRI, angiogram)
Echocardiogram
Lipids, hemoglobin A1C
Cardiac monitoring for potential afib
Further labs/tests if suspect unusual cause
What measures should be implemented to prevent complications from stroke?
NPO until dysphasia/swallow screen passed
Prevent dehydration and malnutrition
Rehabilitation consult and inpatient therapy
DVT/PE prophylaxis (start day 1)
Antiplatelet medication by day 2 (non-tPA)
Anticoagulation if stroke d/t afib
Begin treating risk factors
What is a transient ischemic attack? (TIA)
A transient ischemic attack (TIA) is a stroke that lasts only a few minutes. It happens when the blood supply to part of the brain is briefly blocked.
What are signs/symptoms of TIA?
Sudden numbness or weakness in face, arm, leg on one side
Sudden confusion, difficulty speaking or understanding
Vision changes
Dizziness
Difficulty walking
What is the ABCD2 score for TIA?
Age >60 (1 pt)
Blood pressure >140/80 (1 pt)
Clinical (neurological deficits - 2 pts for hemiparesis, 1 pt for speech problem w/o weakness)
Duration (2 pts for >60min, 1 pt for 10-60min)
Diabetes (1 pt)

Admit all with score 5 or greater
How do you prevent stroke in patients who have had TIA?
Antiplatelets (ischemic)
Afib (anticoagulation CHADS2VASc score)
Blood pressure control
Lipids/statin/diet (LDL goal <70)
Smoking/alcohol cessation
Diabetes (A1C <7%)
Exercise, diet, weight management
Education on symptoms and when to call 911
If a patient has a carotid plaque, how do you treat?
Carotid endarterectomy/stent - consider if >50% occlusion
What is the proposed etiology of stroke in patients with COVID-19?
Thought to be due to hypercoagulability secondary to systemic inflammation and cytokine storm

*Clots have been seen to be growing while patients undergo clot retrieval procedure - abnormal - most have poor or fatal outcomes
What is hemorrhagic stroke?
Atraumatic bleeding from a weakened spot on a blood vessel such as an aneurysm or arteriovenous malformation (AVM) - accounts for 15% of adult strokes

Can be subarachnoid or intracerebral (intracerebral much more challenging d/t being deeper in brain)
What are signs/symptoms of subarachnoid hemorrhage?
Sudden onset of worst headache of the patient's life
Neck stiffness/rigidity
Altered LOC
Cranial nerve III palsy
Significant prehospital mortality
What are the different grades on the Fisher SAH (subarachnoid hemorrhage) scale? (based on CT findings)
1 - no SAH or IVH detected
2 - diffuse, thin (<1mm) SAH, no clots
3 - localized clots &/or layers of blood >1mm thick, no IVH
4 - SAH of any thickness with IVH or ICH present
How do you treat subarachnoid hemorrhage?
Endovascular treatment is definitive
Coil embolization or stent-assisted coil embolization

Surgical clipping of aneurysms or AVMs, evacuation of clot - more invasive, higher complication risk
How do you treat intracerebral hemorrhage?
Treatment is typically supportive
Reverse anticoagulants
Manage BP
Treat seizures as needed
Nimodipine or nicardipine to control vasospasm
Elevate head of bed (HOB)
CT angio or MR angio is indicated to look for AVMs or other vascular abnormalities
What are risk factors for intracerebral hemorrhage? (most difficult type of stroke to treat)
Hypertension
Anticoagulant use
How common are headaches?
Most common complaint (not just in neurology)
Third cause of disability ages 20-50yo
When gathering information on someone's headache, what specific questions should be asked?
Location
Type of pain
Accompanying symptoms - GI, sensoriphobia, ipsilateral dysautonomia
Lifetime headache hx
What are the two possibilities for clear primary HA? Secondary pattern?
Primary - either migraine or non-migraine (tension-type headache, trigeminal autonomic cephalgias such as cluster, related) - tension-type HA more common than migraine

Secondary - cause uncovered or no secondary cause found (trauma, vascular, non-vascular intracranial, substance-related, infectious, metabolic, HEENT, neck, psychiatric, neuralgias)
When should you pursue a thorough workup for a patient presenting with headache?
First or worst headache
Recent onset (weeks)
Gradually/continuously worsening headache
Unilateral without shift
Neurologic deficits (aphasia, hemiparesis, etc)
Background health (immunosuppression, cancer)
Very young or older age
Thunderclap (explosive headache: aneurysm rupture until proven otherwise)*
What should be included in your workup for someone presenting with a headache?
CT/MRI
Labs (especially ESR for inflammation)
LP
Consult neurology if unclear
What are signs/symptoms of cluster headaches?
Severe unilateral peri-orbital pain (1/4-3hrs)
Ptosis (droopy eyelid)
Lacrimation
Conjunctival injection
Rhinorrhea
Nasal congestion
Restlessness
How do you treat cluster headaches?
100% oxygen, SC sumatriptan > nasal spray, ergotamine

MRI to rule out a lesion
What are signs/symptoms of tension-type headache?
Dull, diffuse pain
Mild to moderate
Generally no accompanying symptoms

Benign - diagnosis of exclusion
What are signs/symptoms of intracranial neoplasms?
Nonspecific headache
Pain worse with decubitus or early AM
N/V
Pulsatile tinnitus ("Doppler in the ears")
Transient visual obscurations (vision blackout on physical strain)
Symptoms tend to linger or worsen
What are signs/symptoms of idiopathic intracranial hypertension (IIH)?
Similar to sxs d/t tumor except non-focal
Papilledema
What are risk factors for IIH?
High weight
Acne treatment (vitamin A)
How do you treat IIH?
Weight loss
Diuretics (acetazolamide)
What other differentials should be considered when patients present with headache?
Giant cell arteritis
Subarachnoid hemorrhage
Acute sinusitis (chronic is not considered a cause of HA)
How do you treat cervicogenic headache?
Local nerve blocks
Physical therapy
What are signs/symptoms of trigeminal neuralgia?
Lancinating pain in the face - distribution of the trigeminal branches
Triggered by touch, chewing
Can be primary or secondary (mass)
How do you treat trigeminal neuralgia?
Antiepileptic drugs
Focal radiation
Surgery
What are signs/symptoms of cervicogenic headache?
Pain arising in neck but radiating as hemicrania
Exacerbated by neck movements
Stiff neck
Grinding C-spine
Hx of arthritis, whiplash, surgery
Who is at risk for migraines?
Females 3x > males
High weight status
Hx of head injury
Medication overuse
Stressors
Sleep apnea
What comorbidities are often associated with migraine?
Epilepsy
Stroke
Depression
Anxiety
IBS
Celiac disease
What causes migraines?
The trigeminal nerve releases neuropeptides (including CGRP) causing dural inflammation and pain that is then transmitted by the trigeminal nerve
What causes an aura before a migraine?
An abnormal yet benign electric wave traveling slowly across the cortex causes the symptoms.

Ex) flashes of light, bright lines, blinding; tingling/numbness; vertigo, double-vision, syncope
What are clinical features of migraine?
Intermittent - 1-4 per month on average
Location - unilateral
Character - throbbing
Intensity - moderate to severe (increases with exertion)
Duration - 4-72hrs
Associated sx - N/V, photophobia, phonophobia
How do you treat migraines?
Lifestyle changes (regular sleep/exercise/meals/stress management)
Address comorbidities
Prophylaxis (pure migraine meds, BP meds, antidepressants, antiepileptic, botulinum toxin)
Abortive therapy is individually tailored (acetaminophen, NSAIDs, migraine-specific [sumatriptan, lasmiditan, ubrogepant], isometheptene)
Rescue tx - IM or IV (fluids, injectable anti-emetics, NSAIDs)
Migraine-specific drugs treat pain but also treat what other symptoms? What is the MOA of -triptans and -gepants?
N/V, photophobia and phonophobia

Triptans: 5-HT agonists (inhibit CGRP release)
Gepants: block CGRP receptors
What is the main cause of chronic migraines? (note: chronic migraine = occurs at least 15 days per month for at least 3 months)
Medication-overuse - there is an aggravation parallel with increase in analgesic use

Provide education and encourage to reduce abortives use (i.e. limit analgesics, avoid opioids)
Are opioids used in headache care?
No - due to addictive nature and these are often a cause of medication-overuse headaches; not indicated for headache care
What is the definition of syncope?
Transient loss of consciousness due to inadequate blood flow to the brain
What are red flag signs/symptoms in a syncopal patient?
Chest pain
Palpitations preceding or associated w/ event
Difficulty breathing
Neurological deficits
What diagnostics should be ordered to help determine the cause of syncope?
CBC (r/o anemia, infection)
CMP (lytes, glucose, renal/hepatic abnormalities)
Serial troponin (r/o MI, PE)
Blood alcohol level
HCG (hypovolemia from bleeding/lyte imbalances in pregnancy)
Urinalysis (r/o dehydration, renal/hepatic dysfunction, infection)
Toxicology
Orthostatic vital signs (sensitive test for hypovolemia)
Chest xray
EKG
What is the most dangerous possible differential dx for syncope?
Arrhythmia or cardiac event - get an EKG, labs
What are differential dx for syncope?
Cardiac dysrhythmia
Vasovagal episode (neurally mediated)
Hypovolemia
Overdose of vasoactive med (beta-blocker)
Dehydration
What change in BP indicates orthostatic hypotension?
+20 in HR
-20 mm in systolic
-10 mm in diastolic
Who should be consulted prior to discharging a patient with syncope?
Cardiology - can also schedule outpatient appt for low-risk patients
Utilize outpatient cardiac monitoring
What is multiple sclerosis?
An autoimmune disease of the CNS characterized by chronic inflammation, demyelination, gliosis (plaques or scarring) and neuronal loss
How common is multiple sclerosis?
The most common permanently disabling disorder of the CNS system in young adults
Clinical disease becomes evident between age 15-45yo
Females > males
African Americans > Hispanics/Asians

>900,000 individuals in the US - more prominent in the north
What causes multiple sclerosis?
Exact mechanism not understood - classic pathological hallmark of MS is demyelination (+plaque formation and inflammation in cerebral cortex)

T-cells, B-cells and macrophages interact with substances on blood vessel walls permitting them to cross the damaged blood-brain barrier, allowing the release of inflammatory mediators and immunoglobulins that target myelin nerve sheath; macrophages release NO and oxygen free radicals resulting in damage to axon
What are risk factors for multiple sclerosis?
Smoking
Vitamin D deficiency
Family hx
Place of birth/residence
What is associated with decreased risk of multiple sclerosis?
Increased exposure to sunlight and higher 25-hydroxyvitamin D levels
What are the types of multiple sclerosis?
Relapsing remitting (90% of cases) - discrete attacks that evolve over days to weeks, followed by some degree of recovery over weeks to months - no worsening of neurologic function between attacks

Secondary progressive (develops in 50% of patients with relapsing remitting MS) - initial relapsing remitting disease, followed by gradual neurologic deterioration not associated with acute attacks

Primary progressive & progressive relapsing - characterized by steady functional decline from disease onset - primary progressive more common than progressive relapsing
What are signs/symptoms of multiple sclerosis?
Abrupt or insidious onset of:

Motor weakness (paraparesis, paraplegia, isolated upper extremity weakness)
Sensory disturbances (paresthesias, hyperesthesia)
Optic neuritis (monocular visual impairment w/ pain)
Lhermitte sign (electrical sensation down spine on neck flexion)
Fatigue
Impaired coordination (gait imbalance, slurred speech)
Spasticity (affects majority of patients)
Pain - HA, back pain, muscle spasms, trigeminal neuralgia
Depression
Sexual dysfunction
Bladder urgency/retention
Bowel dysfunction
What are differential dx for multiple sclerosis?
Huntington disease
Progressive multifocal leukoencephalopathy
Behcet syndrome
Sarcoidosis
SLE
Herniated disk
CVA
DM
HTN
Migraine
Vasculitis
Medication/illicit drug use
Nutritional deficiencies (folate, vitamin B12, vitamin E)
Psychiatric disorder
How do you diagnose multiple sclerosis?
Clinical - 2 episodes of neurologic deficits lasting at least 24 hours that are separated in time and space is considered diagnostic that occur in the absence of fever, infection or competing etiologies

MRI (CNS white matter lesion) - diagnostic standard

Visual, auditory and somatosensory evoked potentials
LP and CSF (not routinely recommended)
How do you treat acute exacerbations of multiple sclerosis?
Corticosteroids
If unresponsive to steroids - plasmapheresis
How do you manage multiple sclerosis long-term?
Disease-modifying therapy:
Interferon beta
Glatiramer
Fingolimod
Teriflunomide
Dimethyl fumarate
Natalizumab
Alemtuzumab
Ocrelizumab
Mitoxantrone

Symptom-specific tx
What is the prognosis of patients with multiple sclerosis?
Average lifespan is 25-35 years after dx
Most common cause of death is secondary complications such as immobility, chronic UTI, dysphagia, respiratory problems
What is myasthenia gravis?
An autoimmune disease involving the postsynaptic membrane in the neuromuscular junction (NMJ) - antibodies prevent acetylcholine from activating its receptors which reduces nerve impulse transmission causing weakness of the skeletal muscles - may be generalized or localized

(anti-acetylcholine receptor antibodies)
How common is myasthenia gravis?
Relatively rare - 8-10 cases per million people
Most common acquired disorder of neuromuscular transmission
Juvenile myasthenia gravis accounts for 10% of cases
Peaks around age 30 and again at age 50
More females in younger group, males in older group
What are signs/symptoms of myasthenia gravis?
Generalized weakness
Fatiguability
Ocular weakness - asymmetric (ptosis, diplopia)
Bulbar weakness (difficulty chewing, swallowing, loss of facial expression, inability to fully close eyelids)
Respiratory muscle weakness
What gland has been implicated in myasthenia gravis?
The thymus - it contains the elements required to initiate and sustain an autoimmune reaction (AChR autoantigen, AChR-specific T-cells, autoantibody-secreting plasma cells)

>80% of mysathenia gravis patients have thymic abnormalities such as hyperplasia and thymoma - thymectomy results in symptom improvement
What drugs can unmask or worsen myasthenia gravis?
Anesthetics
Aminoglycosides
Fluoroquinolones
Ketolides
Beta blockers
Procainamide
Quinidine
Anti-PD-1 monoclonal antibodies
Botulinum toxin
Chloroquine
Hydroxychloroquine
Magnesium
Penicillamine
Quinin
What other disorders often coexist with myasthenia gravis?
Thyroiditis
SLE
Rheumatoid arthritis
Thymoma (MG occurs in 1/3 of patients with thymoma)
How do you diagnose myasthenia gravis?
Thorough neurologic exam
Ice pack test - place an ice pack over the eyelid for 2 minutes - improvement of ptosis suggest neuromuscular disorder
Antibody tests
Single-fiber electromyography
CT scan of chest w/ contrast to look for thymoma
TSH, free T4
Forced vital capacity to determine respiratory muscle involvement
How do you classify myasthenia gravis?
Ocular vs generalized disease
Disease severity
Antibody type
Associated thymic pathology
How do you treat myasthenia gravis?
Acetylcholinesterase inhibition - pyridostigmine (primary drug)
Immunosuppressive therapy - prednisone or prednisolone/azathioprine
Thymectomy (if thymoma present)
What are signs/symptoms of myasthenic crisis? What can cause this?
Worsening muscle weakness
Respiratory failure
May involve respiratory muscles, bulbar muscles or both

Triggers - physical stressors, medications
How do you treat myasthenic crisis?
Admit to ICU
Respiratory support - intubation/ventilation
Plasmapheresis
IVIG
High-dose corticosteroids
Monitor and correct abnormal electrolytes
Correct anemia
What is the prognosis for patients with myasthenia gravis?
Near-normal life span for most patients
Risk factors for mortality include age >40, short hx of progressive disease, thymoma

Morbidity typically results from intermittent impairment of muscle strength which may lead to falls, aspiration pneumonia, and respiratory compromise (myasthenic crisis)
What organisms are typically responsible for bacterial meningitis?
Strep pneumo
N. meningitidis
Group B strep (S. agalactiae)
Listeria monocytogenes (vertical transmission, food poisoning)
Haemophilus influenzae (less common w/ vaccines)
E. coli
What are host risk factors for strep pneumonia or Neisseria meningitidis meningitis?
Children age <2
Elderly
CSF leak (following trauma/surgery)
Immune deficiency (especially defects in humoral immunity, asplenia, HIV with low CD4 counts)
Anatomical defects
What could lead to a staph-causing meningitis?
Trauma leading to staph on the skin getting into the meninges
Lumbar puncture done without thoroughly cleaning the skin
What are signs/symptoms of bacterial meningitis?
Triad: fever, headache, nuchal rigidity
Decreased level of consciousness
Seizures
Increased intracranial pressure (ICP)
What are risk factors for bacterial meningitis?
Age (babies at higher risk)
Group settings
Travel to sub-Saharan Africa
How do you diagnose bacterial meningitis?
Blood culture
CSF analysis (polymorphonuclear leukocytosis PMN, increased protein)
CSF pathogen panel
Neuroimaging
If a CT is deemed necessary on clinical grounds when suspecting meningitis, the emergency physician should perform tasks in what order?
1. Blood cultures
2. Steroids
3. Antibiotics
4. CT
5. LP
How do you treat bacterial meningitis?
Typical antibiotic of choice is vancomycin + ceftriaxone

Consider ampicillin if listeria likely cause
What commonly causes viral meningitis?
Enteroviruses
Varicella-zoster virus
Herpes simplex virus 2
Epstein-Barr virus
Arthropod-borne viruses
HIV
What are signs/symptoms of viral meningitis?
Triad: fever, headache, nuchal rigidity
Malaise
Myalgia
Anorexia
N/V
Abdominal pain
Diarrhea
How do you diagnose viral meningitis?
CSF analysis
Viral culture
Serologic studies
What are signs/symptoms of subacute meningitis?
Headache
Stiff neck
Low-grade fever
Lethargy ranging from days to several weeks
What commonly causes subacute meningitis?
M. tuberculosis
C. neoformans
H. capsulatum
C. immitis
T. pallidum (syphilis)
How do you diagnose subacute meningitis?
CSF analysis:
Elevated opening pressure
Lymphocytic pleocytosis
Elevated protein concentration
Decreased glucose concentration
How do CSF analyses differ between normal, bacterial infection, viral infection, and TB/fungal infections?
Normal: <5 WBC, 15-45 protein, 60% serum glucose (~40)

Bacterial infxn: >500 WBC, PMNs >90%, >100 protein, <40 glucose (bacteria eat glucose)

Viral: 50-200 WBC, Lymphocytes, <100 protein, normal glucose

TB/fungal: 50-500 WBC, Lymphocytes, >100 protein, <40 glucose (also eat glucose)
What causes aseptic meningitis? (aka meningitis where organisms cannot be detected in CSF)
Syphillis
Leptospirosis
Lyme disease
Mycoplasma

Parasites: T. gondii, T. sodium, trichinella, N. fowleri
Drugs: NSAIDs, TMP-SMX, azathioprine
Autoimmune: sarcoidosis, SLE, Behcet's
Malignancy: lymphoma, leukemia, metastatic
Post-infectious
Post-vaccine
How do you treat meningitis?
Bacterial - antibiotics, corticosteroids

Viral - supportive care
What is encephalitis?
Inflammation of the brain itself (parenchyma)
What is the important distinguishing feature between encephalitis and meningitis?
Presence or absence of normal brain functioning
What viruses cause encephalitis?
Arboviruses
HSV-1 (most common cause)
Rabies
West Nile virus
Measles/mumps
HIV
What are signs/symptoms of encephalitis?
Alteration of consciousness (occurs in all patients)
Focal neurologic deficits
Seizures
Fever
Headache
Change of personality (hallucinations, bizarre behavior)
Dysphagia
How do you diagnose encephalitis?
MRI or CT
EEG (asymmetric sharp waves)
LP (usually similar to viral meningitis findings - elevated RBCs, elevated protein, normal glucose)
How do you treat encephalitis?
-supportive
-AED if needed
-acyclovir empirically pending result of CSF
What is a brain abscess?
A localized, walled-off collection of pus surrounded by a fibrous capsule within the brain parenchyma most commonly caused by bacteria

*Note: viruses do not cause brain abscess
What organisms commonly cause brain abscesses?
Otitis media or sinusitis
Gram-negative anaerobes (bactericides, prevotella, fusobacterium)
Dental infection
Trauma or neurosurgery (staph, strep)
Neutropenia
HIV (toxoplasma gondii, litsteria, nocardia, mycobacterium)
Endocarditis (staph aureus, strep viridians)
How do you diagnose brain abscess?
Head CT
How do you treat brain abscess?
Ceftriaxone + metronidazole (+/- vancomycin)

Steroids only if cerebral edema

Neurosurgery consult for possible drainage
What is cystocercosis?
Infection caused by pork tapeworm (Taenia solium) that can be found in the muscles, heart and eyes or found in the brain (neurocysticercosis NCC) that causes seizure, headache, swelling of the brain and death
Compare tetanus and botulism
Clostridium tetani (Tetanus) - Highly fatal neurologic disease that results in spastic muscle twitching and paralysis. A core vaccine in horses.

Clostridium botulinum (Botulism) - Rapidly progressie, fatal neurologic disease characterized by dysphagia and muscle fasiculations (the muscles can't twitch but they want to, often looks like a wave under the skin).
When should you consider doing a lumbar puncture (LP) without doing neuroimaging beforehand?
Age <60yo
Immunocompetent
No hx of CNS disease
No recent seizure
Normal sensorium and cognition
No papilledema
No focal neurological deficits
What are common causes of TBIs?
Falls (most common)
MVA (2nd most common - high mortality)
Struck by or against an object
Assaults
Unknown causes
Other
What are the 3 severities of TBI? How is it determined?
Determined by Glasgow Coma Scale (GCS)

80% of TBIs are mild (concussion, GCS 13-15)
10% moderate TBIs (GCS 9-12)
10% severe (GCS 8 and less)
What is the most common reason that children present with TBIs?
Falls age 0-4
Falls and struck by an object age 4-15
Abusive head trauma (leading cause of death in children <1yo)
Sports-related injuries (football and soccer most common)
What are potential consequences of TBI?
Bruising, bleeding
Tearing, swelling
Fracture
Seizures
Affects normal functioning and independence

80-90,000 pts left with permanent disability each year
How do you evaluate a TBI?
X-rays
CT
MRI
How do you prevent or limit secondary damage in patients with TBI?
Maintain oxygen supply to brain and control breathing
Prevent seizures (anti-epileptics - levetiracetam, phenytoin)
Prevent fever (antipyretics as soon as temp hits 99, remove excess clothing, turn on a fan)
Reduce swelling, inflammation and pressure in skull
Maintain blood pressure (you can double the chance of mortality by reducing blood pressure - reduces blood flow to brain)

Surgery if needed to remove blood clots or reduce pressure (remove large part of skull to allow brain to swell - put back in 3 months once brain has healed)
Differentiate between primary and secondary brain injury
Primary - injury that occurs at the time of impact and results in altered LOC

Secondary - focus of TBI management is prevention of secondary brain injury, follow-up injuries caused by complications such as hypoxia, ischemia, hypotension, hematoma expansion, cerebral edema, brain compression, intracranial HTN, seizure, fever
What are the parts of the Glasgow Coma Scale (GCS)? (the higher the score the better)
Eye opening - spontaneously (4pts), to speech (3pts), to pain (2pts), none (1pt)

Best verbal - oriented (5pts), confused (4pts), inappropriate words (3pts), in comprehensive sounds (2pts), none (1pt)

Best motor - follows commands (6pts), localizes to painful stimulus (5pts), withdraws from pain (4pts), flexion to pain [decorticate] (3pts), extension to pain [decerebrate] (2pts), none (1pt)

*motor predicts outcome
What treatment should be considered for patients with a GCS of 8 or below?
GCS of 8 - intubate (can't protect their own airway)
What does ABCDE stand for in prehospital care for TBI?
Airway
Breathing (ventilation and oxygenation)
Circulation
Disability
Exposure/environment
What are indications for head CT?
Loss of consciousness
Severe headache
Vomiting
Age >65 w/ brain injury
Drug/alcohol intoxication
GCS <14
Signs of basilar skull fracture
Neuro deficits
Amnesia
Seizure
Dangerous mechanism (auto hitting pedestrian)
Coagulopathy
What 3 views are taken in CT scans?
Sagittal
Axial
Coronal
What hematoma volume is associated with clinical decline and failed post-operative management in acute epidural hematoma?
Hematoma volume >30cm
MLS 5mm (midline shift)
need surgery


Regardless of initial GCS - often appear fine at first
When completing neuroimaging, what types of brain injuries are you looking for?
Subdural hematoma (crescent-shaped mass)
Epidural hematoma (lentiform mass)
Hemorrhagic cerebral contusion (salt and pepper appearance)
Open skull fracture (intracranial air)
What is a common symptom of acute epidural hematoma?
Pupillary abnormalities such as ipsilateral dilated pupil
When should you call neurosurgery for a patient with TBI?
Moderate-severe TBI
Mild TBI with other extra cranial injuries
Skull fracture
Signs of CSF leak with clear or serosanguinous fluids from ears or nares
Lateralizing signs on neurological exam (unequal pupils, focal weakness)
Cerebrovascular injury
C-spine injury
What are signs/symptoms of brain herniation?
Non-reactive pupil
Extensor posturing
Progressive decline in neurologic exam (decrease in GCS score >2 points)
Cushing's response: HTN, bradycardia, irregular respiration
Why do we put patients in transient hyperventilation with TBI?
It causes the vessels to vasoconstriction so it results in less brain swelling

*avoid prolonged hyperventilation - will lead to ischemia
What are the 4 domains of concussion symptoms?
Physical
Cognitive
Emotional
Sleep
What are signs/symptoms of concussion?
Headaches
Neck pain
N/V
Blurred/double/fuzzy vision
Photophobia
Dizziness
Concentration/memory problems
Irritability
Low energy
Seizures
Syncope
Behavioral changes
Slurred speech
What scoring systems can be used to assess concussion?
SCAT-2, SCAT-5
Glasgow Coma Score
What other patient education should be provided when a patient has a concussion?
Avoid alcohol, sleeping pills
No driving until approved by provider
Rest and avoid strenuous activity for at least 24 hours
Do not use aspirin or NSAIDs for pain - use paracetamol or codeine
Do not train or play sport until medically cleared
Concussions typically resolve within 3-4 weeks

Go to ED if you notice change in behavior, vomiting, dizziness, double vision, worsening headache, excessive drowsiness, slurred speech, seizure/convulsion, weakness/tingling of extremities, syncope, confusion
How long are patients with concussions required to sit out from sports after concussion?
Cannot return sooner than 7 days after diagnosis of concussion - recommended not to do any physical activity for at least 2 full symptom-free days after you have seen a physician (start off with light aerobic activity and build up to more strenuous/resistance training)
What are the 4 basic domains of child development?
Physical (gross & fine motor skills)
Cognitive (problem-solving)
Language (communication)
Social-emotional
What are common etiologies of developmental delay?
Down syndrome
Fragile X syndrome
Microcephaly
Hydrocephalus
Hemorrhage
Toxins (alcohol, smoking)
Intrauterine growth retardation
Hypoxic-ischemic encephalopathy
Hypoglycemia
Meningitis/encephalitis
Electrolyte imbalances
Head trauma
Severe malnutrition
When taking a pediatric Neuro history, what pieces of information about symptoms/signs are important?
Has it been static? (no change over time)
Progressive degeneration?
Intermittent/brief episodes (seizures, migraines)
Saltatory (bursts of symptoms follow by partial recovery)
What is the best initial diagnostic tool when evaluating a child?
Observation - watch behavior, interactions with parents
How do neurologic abnormalities present in adults vs in neonates/infants?
In adults - often produce asymmetric/localized findings

In infants - typically present as developmental abnormalities such as failure to do age-appropriate tasks
What is defined as macrocephaly and microcephaly in infants?
Macrocephaly = >95th percentile head circumference
Microcephaly = <5th percentile
What are signs of severe neurologic disease in infants?
Irritability
Persistent asymmetry of posture
Persistent extension of extremities
Constant turning of the head to one side
Marked extension of the head, neck and extremities (opisthotonus)
Severe flaccidity
Limited response to pain
Seizures
How do you assess for meningeal irritation (meningismus) in children?
Nuchal rigidity (resistance or pain to passive flexion of neck)
Kernig sign
Brudzinski sign (lift neck while child is laying down - knees coming up is positive)
What is cerebral palsy (CP)?
Chronic disorder involving damage to the motor areas of the brain resulting in impaired control of movement - depending on the area of the brain affected and the extent of the damage, children with CP can have disturbances in movement, sensation, perception, cognition, communication and behavior
How common is cerebral palsy?
2-3.6 per 1000 live birth

Etiology is unknown in a lot of cases
What are risk factors for cerebral palsy?
Small for gestational age
Preterm babies
Complicated labor/delivery
Microcephaly
Apgar score of <3 at 10 minutes or more
Severe proteinuria late in pregnancy
Maternal stroke
Intrauterine drug exposure
Low birth weight
Breech presentation
Traumatic delivery
Exposure to maternal infection
Fetal distress
Intrauterine infections
Neonatal seizures
10-15% of cerebral palsy cases are acquired after birth, what causes acquired CP?
Meningitis
Encephalitis
Head trauma
Non-accidental trauma (shaken baby syndrome)
Car accidents, falls
Near drowning
What are the 4 major types of CP?
Spastic CP - characterized by muscle stiffening/tightness

Athetoid CP - affects muscles that enable smooth, coordinated movement, resulting in movement which is involuntary and purposeless

Ataxic CP - affects balance and coordination

Atonic or flaccid CP
What history findings are concerning for CP?
Hypotonic muscle tone before age 6mo, then becoming hypertonic
Delayed developmental milestones
Inability to coordinate suck and swallow
Regurgitation through the nose
Difficulty with movement
Impaired speech language skills
Failure to achieve motor milestones
Skin findings including cafe au lait spots
Scoliosis or contractures
Diminished deep tendon reflexes
Increased muscle tone (>6mo)
Asymmetric movements
Preferred handedness before age 1-2
Abnormal head size
Difficulty feeding
Involuntary movements of lips, cheeks, tongue
What diagnostic studies should be done in the evaluation of a patient suspected to have CP?
CT and/or MRI
Chromosomal and metabolic studies to identify genetic disorders (especially single gene defects)
Lumbar puncture if sepsis is suspected
What are differential dx for cerebral palsy?
Sepsis from intrauterine infection
Fetal alcohol syndrome
Hydrocephalus
Tumors
Brain malformations
Tay-Sachs disease
Phenylketonuria
Spinal cord injury
Hypothyroidism
Severe TBI
How do you manage cerebral palsy?
Refer to development pediatrician and neurology

Antispasmodics, botulinum toxin
Management of CP requires multidisciplinary team
What red flag symptoms may indicate intracranial pathology in infants?
Full anterior fontanelle
Open metric and coronal sutures
Poor growth
Impaired upward gaze
Abnormal head growth
Shrill cry
Lethargy
Vomiting
What red flag symptoms may indicate intracranial pathology in children?
Headache described as severe, excruciating recent onset
Papilledema or abnormal eye movements (one or both eyes suddenly turn in)
Ataxia, hemiparesis, abnormal deep tendon reflexes
Cranial bruits
Personality changes
What is West Syndrome?
A specific type of seizure seen in the epilepsy syndrome that is characterized by infantile spasms, developmental regression and specific EEG changes
What are signs/symptoms of West Syndrome?
Brief, symmetric contractions of extremities/trunk/neck muscles
Happen in clusters
Occur just before sleep or upon waking
Onset is typically between 4-8mo
How do you diagnose West syndrome?
EEG (hypsarrthymia pattern - chaotic brain waves)
MRI is preferred test to determine etiology
How do you treat West syndrome?
ACTH, vigabatrin, ketogenic diet

Spasms usually resolve by mid-childhood but more than half of the children with infantile spasms will develop other types of seizures
What are risk factors for perinatal stroke?
Congenital heart disease
Infections
Placental disorders
When do perinatal strokes typically occur?
During later stages of pregnancy through 30 days of age
When do pediatric strokes typically occur?
Ages 1 month to 18 years
*very rare but it is one of the top 10 causes of death in childhood - often because it goes unrecognized for a long time
What percent of pediatric strokes are hemorrhagic vs ischemic?
50/50
How do you treat pediatric stroke?
Early imaging (CT/MRI) important
Referral to meds neuro and/or specialty center
Treatment is primarily supportive
Thrombolytics are being investigated
Surgical/endovascular treatments are used for hemorrhagic strokes
What is spina bifida?
Defective closure of the neural tube at end of week 4 of gestation resulting in anomalies of the lumbosacral vertebrae or spinal cord - can range from physical defects to total paralysis/incontinence
How can you prevent spina bifida?
Can be prevented by folic acid administration
Compare meningomyelocele and meningocele
Meningomyelocele - most severe form, spinal cord or nerve roots exposed on back in sac; leg paralysis and incontinence present - requires immediate surgical closure

Meningocele - rarest form, only meninges are exposed on back in cyst like sac - no hydrocephalus or neurologic deficits; surgically repaired
What are Arnold-Chiari malformations?
Cerebellum herniation thru foramen magnum - associated with hydrocephalus (due to obstruction of 4th ventricle)
What are signs/symptoms of Arnold-Chiari malformations?
Occipital headache that radiates upward, worsens with valsalva
Visual or balance problems
Muscle weakness may be present
How do you treat Arnold-Chiari malformations?
Pain management, surgical decompression
What causes microcephaly?
Chromosomal disorders
Infections
Toxic (FAS, phenylketonuria)
Prader-Willi syndrome
What causes hydrocephalus?
Obstruction or overproduction of CSF
What is hydrocephalus?
Slowly evolving accumulation of CSF over weeks to months
What are exam findings in patients with hydrocephalus?
Bradycardia
HTN
Altered respiration
Increased head circumference
Bulging anterior fontanelle
CN III or VI dysfunction
Papilledema
Frontal bossing
How do you treat hydrocephalus?
Temporary, symptomatic relief: loop diuretic, acetazolamide

Surgical intervention - remove obstructive lesion, ventriculo-peritoneal shunt
Who is commonly affected by Duchenne & Becker muscular dystrophy?
*Most common hereditary neuromuscular disease
More common in males (X-linked recessive)
Starting at 3 years, fully expressed by 5-6yo
What are signs/symptoms of Duchenne muscular dystrophy?
Progressive weakness - waddling gait, inability to rise from ground easily
Gower's sign - use hands to push up from floor to standing
Intellectual impairment (usually mild)
Proliferation of connective tissue in muscle (hypertrophy of calves)
How do you diagnose muscular dystrophy?
Muscle biopsy (shows degeneration and regeneration with connective tissue)
Absence of dystrophin
EMG to assess muscle weakness
Serum CPK levels will be markedly elevated
How do you treat muscular dystrophy?
Glucocorticoids
Newer therapies include gene therapy, creatine, aminoglycosides
What is the prognosis for Duchenne & Becker muscular dystrophy?
Wheelchair bound by age 10
Cardiomyopathy in 50-80%
Life expectancy ~18-20yo
What is neurofibromatosis?
Autosomal dominant genetic disorder that causes tumors (neurofibromas) to form on nerves

Type 1 - more common, referred to as peripheral type
Type 2 - referred to as central type - incurable disease, multiple intracranial and spinal tumors
What are physical exam findings of neurofibromatosis?
Cafe-au-lait spots (present at birth)
Neurofibromas form in late adolescence
Learning disability but minimal cognitive impairment
Short stature
Macrocephaly
What symptoms are suggestive of brain tumors?
Headache
N/V
Loss of balance
Speech problems
Sleepiness
Personality changes
Seizures
Increased head circumference in infants
Memory loss
Stroke symptoms
What are signs of brain tumors?
Focal neuro deficits (location depends on site of tumor)
Seizures
Increased intracranial pressure
Cerebral edema
Hydrocephalus
What should always be on the list of differential dx for all patients with CNS symptoms?
Tumor
Compare primary and secondary tumors
Primary = arises in the spot that you find it
Secondary = metastasis
What is a glioma?
Refers to all tumors that arise from glial tissues - can range from relatively benign to aggressively malignant and rapidly fatal
What are different types of gliomas?
Astrocytoma
Glioblastoma multiforme
Diffuse midline glioma
Ependymoma (in ventricles and spinal cord - common in kids)
Oligodendroglioma (usually in cerebrum, slow growing)
What type of glioma is the most common?
Astrocytoma - can occur anywhere in the brain or spinal cord

*more common in cerebrum in adults
What type of glioma is the most malignant?
Glioblastoma multiforme
How common are meningiomas?
Make up about 1/3 of all brain tumors - usually benign
Slow-growing and often grow quite large before causing symptoms
Most common in women 30-50yo
Who commonly presents with medulloblastomas?
Children
Boys > girls

*develop from embryonal or developing nerve cells that normally do not remain after birth
What is a Schwannoma?
Benign tumors that begin in Schwann cells which produce the myelin on cranial and peripheral nerves - 90% are vestibular schwannomas (acoustic neuroma) that cause hearing loss

Occurs mainly in adults, women > men
What other primary CNS tumors are there?
Craniopharyngioma (benign but can damage hypothalamus, compress optic chiasm)
Pineal region tumors (often cannot be removed)
Cerebral lymphoma (rare, mostly seen in immunocompromised patients)
How common are metastatic brain tumors in comparison to primary brain tumors?
Metastatic are 5x more common than primary

*often present with multiple tumors in different areas of the brain (differentiating factor from primary tumors)
Where do most metastatic brain tumors arise from?
Breast
Liver
Lung
Skin (melanoma)
Colon
Kidney
Thyroid
How do you definitively diagnose brain tumors?
Biopsy
What are signs/symptoms that are suggestive of cranial nerve pathology?
Facial pain and numbness
What are causes of oculomotor nerve palsy?
Vascular ischemia
Trauma
Intracranial neoplasm
Hemorrhage
Congenital
Idiopathic
DM
HTN
What are signs/symptoms of oculomotor nerve palsy?
Ptosis d/t paralysis of the elevator palpebrae muscle
Ocular deviation (down and out position)
Diplopia
Pupil may be fixed and dilated d/t paralysis of sphincter pupillae
How do you treat oculomotor nerve palsy?
Conservative - for acute palsy and patients >50yo w/ hx of DM, HTN; diplopia can be treated with occluding the affected eye with an eye patch or opaque contacts
Ischemic third-nerve palsy patients often demonstrate improvement within 1 month and complete recovery in 3mo

Surgical tx - advised after 6 months in pupil-sparing acquired palsies, goal is to provide alignment of the eye in primary gaze and to provide binocular single vision
What are causes of trochlear nerve (IV) palsy?
Congenital - born without the nerve or the tendon of the superior oblique was abnormal

Acquired - associated w/ mild to moderate closed head injury or infarction often d/t diabetes
What are signs/symptoms of trochlear nerve (IV) palsy?
Impairs superior oblique muscle, causing paresis of vertical gaze (paralytic strabismus)
Diplopia
Subtle impairment may be evident on examination
What workup should be done to diagnose trochlear nerve palsy?
CT or MRI may be done to identify cause
LP may be warranted in patients with normal imaging and if meningeal process suspected
How do you treat trochlear nerve palsy?
Treat underlying etiology
Oculomotor exercises or prism glasses may help restore concordant vision
If palsy does not resolve - surgery may be needed
What are signs/symptoms of cranial nerve V neuralgia (trigeminal)?
Excruciating paroxysms of pain in lips, gums, cheek or chin
Sharp, sudden jabs
Pain can be provoked by speaking/chewing/smiling
Pain usually lasts from a few seconds to 1-2 minutes
Pain may be so intense that the patient winces ("tic")

No sensory loss
What is believed to be the source of trigeminal neuralgia in most patients?
Compression of the trigeminal nerve root by a blood vessel
How do you treat trigeminal neuralgia?
Carbamazepine
Iamotrigine
Phenytoin
Baclofen

Surgery if meds not sufficient
What are causes of cranial nerve VI (abducens) palsy?
Neoplasm and trauma
Stroke
Demyelinating lesions (MS)
Otitis media
Mastoiditis
Viral illness
What are signs/symptoms of abducens palsy?
Binocular horizontal diplopia (double vision when looking at objects side-by-side)
May have vision loss, HA, N/V, hearing loss
What is included in workup for abducens nerve palsy?
MRI
CBC
CMP
ESR/CRP
How do you treat abducens nerve palsy?
Tx underlying cause

Alternate patching (not proven to be effective in adults)
Prism therapy
Strabismus surgery
Botulism toxin

Most cases are self-limiting and don't require tx
What is Sydenham chorea (SC)?
A neurological disorder of childhood resulting from infection via group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever

An autoimmune attack on basal ganglia occurs weeks to months following group A strep infection
What are signs/symptoms of Sydenham chorea?
Characterized by rapid, irregular and aimless involuntary movements of the arms, legs, trunk and facial muscles
How do you treat Sydenham chorea?
No specific tx
For mildest form - bedrest during periods of active movements is sufficient
Monitor the heart
Sedative drugs may be used if movement interferes with rest
Antiepileptic medications are often prescribed (valproic acid)

Children who have SC should take penicillin over the course of the next 10 years to prevent additional manifestations of rheumatic fever
What is the prognosis of Sydenham chorea?
Most children recover completely, although a small number will continue to have disabling, persistent chorea despite tx

Duration of symptoms varies generally from 3-6 weeks but some will have sx for several months

Sometimes will have recurrent attack in next 1-2 years
When evaluating a tremor, what is the first thing you want to know?
Is it a rest tremor (occurs in a body part that is fully supported, relaxed) - diagnostic of Parkinsons

Or an action tremor (occurs with voluntary muscle contraction):
Kinetic tremor (occurs during any voluntary movement)
Intention tremor (characterized by crescendo increase in tremor as the affected body part approaches its target)
Task-specific kinetic tremor (occurs during specific tasks)
What is the most common cause of action tremor in adults?
Essential tremor (ET) - classically involves the hands and is brought out by arm movement and sustained antigravity postures, affecting common daily activities such as writing, drinking from a glass and handling eating utensils
How do you diagnose essential tremor?
Based on clinical features
Family hx?

4 features:
1) Isolated tremor consisting of bilateral upper limb action (kinetic and postural) tremor, w/o other motor abnormalities
2)At least 3 years in duration
3) With or without tremor in other locations (i.e. head, voice, lower limbs)
4) Absence of other neurologic signs such as dystonia, ataxia or Parkinsonism
What are treatable causes of action tremors that you want to rule out before diagnosing someone with an essential tremor?
Thyroid disease
Metabolic problems
Drug side effects (SSRIs)
Levels of chemicals that may cause tremor
How do you treat essential tremor?
Beta blockers
Mysoline, Topamax
Klonopin

Surgery - DBS, thalamotomy, MRI-guided UTZ
What is Huntington's disease?
An inherited progressive neurodegenerative disorder characterized by choreiform movements, psychiatric problems and dementia

Caused by a cytosine-adenine-guanine (CAG) trinucleotide repeat expansion in the huntingtin (HTT) gene on chromosome 4p and inherited in an autosomal-dominant pattern
What are signs/symptoms of Huntington's disease?
Movement abnormalities (chorea is key feature)
Chorea is characterized by brief, abrupt, involuntary movements involving the face, trunk and limbs
Progressive loss of voluntary motor control
Mood swings
Gradual loss of cognitive and motor skills

In later stages chorea also affects diaphragm, pharynx, larynx producing dysarthria, dysphagia and involuntary vocalizations
How do you diagnose Huntington's disease?
Diagnostic genetic testing indicated for symptomatic adults
How do you treat Huntington's disease?
No cure
Symptomatic/supportive treatment
What is the prognosis for Huntington's disease?
Life expectancy 10-20 years after diagnosis
What is Tourette syndrome (TS)?
A neurological disorder manifested by motor and phonic tics with onset during childhood - thought to result from a complex interaction between social and environmental factors and multiple genetic abnormalities (can inherit from both parents)

Male > females (4:1)
Tourette syndrome is often associated with other mental, behavioral or developmental disorders such as?
ADHD
OCD
ODD (oppositional defiant disorder)
CD (conduct disorder)
Anxiety
Depression
Autism spectrum disorder
Learning disabilities
Speech/language problems
Developmental delays
Intellectual disabilities
What are signs/symptoms of Tourette syndrome?
Sudden, brief intermittent movements or utterances
Involuntary
Simple or complex
Involuntary vocalizations including coprolalia (obscene language)
How do you diagnose Tourette syndrome?
Clinical - neuro exam usually normal and imaging often normal

Presence of both motor and vocal tics that occur several times a day, every day or intermittently for at least 1 year
Onset of tics before age 18
Tics not caused by medications, other substances or medical conditions
How do you treat Tourette syndrome?
Often require no treatment

Medications that block dopamine
Alpha-adrenergic agonists (clonidine, guanfacine)
Stimulant medications (methylphenidate, dextroamphetamine)
Antidepressants (SSRIs)

Behavioral treatments
What is the prognosis of Tourette syndrome?
Tic severity declines yearly during adolescence
By age 16, mostly resolved
Tics may persist into adulthood but gradually diminish in 50% of patients
What is Parkinsonism?
A generic term for the symptoms of tremor, stiffness and slowness of movements
What is Parkinson's disease?
Progressive neurodegenerative movement disorder caused by the death of cells that generate dopamine in the basal ganglia and substantia nigra
How common is Parkinson's disease?
Uncommon in people younger than 40yo
Incidence increases rapidly in people >60yo
Affects over 1 million people in North America
What are potential risk factors for Parkinson's disease?
Genetics
Hx of TBI
Toxin exposure (pesticides, solvents, metals, pollutants)
Males > females
Age >60
What are the 3 stages of compensation during the pre-symptomatic period of Parkinson's disease?
An early period during which the dopamine homeostatic compensatory mechanisms are capable of "masking" the disease

Increased activity of the basal ganglia output nuclei as striatal dopamine homeostasis breaks down

Increased intensity of compensation in structures outside of the basal ganglia as parkinsonian motor abnormalities emerge
How do you treat Parkinson's disease?
Levodopa + carbidopa
Selegiline
MAO inhibitors
Dopamine agonists
Apomorphine (Apokyn)
Speech therapy
Exercise
What 4 signs/symptoms characterize Parkinson's disease? Other features?
1) rest tremor
2) rigidity (cogwheel rigidity - resist you moving their extremities)
3) bradykinesia (dragging legs, shuffling steps)
4) postural instability (typically seen in later disease, falls)


Hypomimia (masked facial expression)
Decreased spontaneous eye blink rate
Speech impairment
Dysphagia
Blurred vision
Impaired upward gaze and convergence
Stooped posture, kyphosis
Shuffling, short-stepped gait, freezing
Cognitive dysfunction and dementia (late stage)
Psychosis and hallucinations
Mood disorders (anxiety, depression, apathy)
Sleep disturbances
Fatigue
Autonomic dysfunction, olfactory dysfunction, GI dysfunction
Pain and sensory disturbances
Dermatologic findings (seborrhea)
Rhinorrhea
When is apomorphine indicated?
as a "rescue" medication to help patient move during off periods

Note: patients experience motor fluctuations between periods of being "on" meaning that the patient enjoys a good response to medication and they can move better but they also have periods of being "off" in which they experience Parkinsonism symptoms
What are red flag symptoms/signs that may indicate that a patient does NOT have Parkinson's disease?
Rapid deterioration of gait
Absence of progression of motor symptoms/signs
Early bulbar dysfunction
Inspiratory respiratory dysfunction
Orthostatic hypotension
Severe urinary retention or urinary incontinence
Recurrent falls within 3 years of onset
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