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Aphasia
Inability to express and/or understand written and/or verbal language
Acute—stroke or head injury
Insidious—brain tumor or degenerative disease
Broca's—good comprehension, but difficulty with language motor ability
Wernicke's—good language motor ability, but difficulty with comprehension
Treat underlying cause, then speech therapy and assistive devices
Athetosis
Slow, irregular, twisting, snake-like movements
Upper extremities—especially hands and fingers
Involuntary
Ataxia
Defective muscular coordination
Especially manifested when voluntary muscle movements are attempted
Interferes with proprioception
Chorea
Neurologic condition marked by involuntary muscle twisting of the limbs or facial muscles
Acute (Sydenham's)—childhood or pregnancy, involuntary contractions of muscles of the trunk and extremities; anxiety, impairment of memory and sometimes speech
Chronic—Huntington
Coma
State of being unarousable and unable to respond to external events or inner needs
Reflex movements and posturing may be present
Complication of serious CNS disorders
Can result from seizures, hypothermia, metabolic disturbances, or structural lesions
Rated using the Glasgow Coma Scale—Measuring eye opening, verbal response, and motor response capabilities
Treat with IV dextrose, naloxone, and thiamine
Diplopia
Double vision
Seen in myasthenia gravis
Doll's eyes
Vestibulo-ocular reflex
A patient's eyes move conjugately away from the direction in which their head is turned = brainstem is intact
Dysarthria
Speech that is characteristically slurred, slow, and difficult to produce/understand
Gets progressively worse with use
Can occur as a developmental disability
May be a sign of a neuromuscular disorder
May be caused by a stroke, brain injury, or brain tumor
Intensive speech therapy
Dyskinesia
Slow, rhythmical, automatic, stereotyped movements
Generalized or in a single muscle group
Dystonia
Impaired muscle tone
Nystagmus
Rapid, rhythmic, repetitious, involuntary eye movements
Pendular nystagmus—right eye is exotropic and vertically deviated—multiple sclerosis
Paresthesia
Numbness, tingling, pricking, burning, or creeping sensation felt in the skin
Ptosis
Downward displacement
Tics
Spasmodic muscle contraction
Most commonly involving the face, head, neck or shoulder
May be tonic or clonic
Often involuntary
Rotatoire—spasmodic toricollis; head and neck forcibly rotated or turned from one side to another
Tic douloureux—degenerative or pressure on the trigeminal nerve, resulting in neuralgia
HA General Information
Women 3x more than men
Most troublesome from late teens to early 50s
"Red flag" headaches—the "worst headache of their life", with papilledema, with altered mental status, with focal neurological findings, with meningeal signs, in patients with AIDS
One of the longest lists of DDx in medicine
HA pain is produced by nociceptors, injury to CNS or PNS, or displacement of pain sensitive structures
The key to DX is patient history
Key questions—time, character, causes, response, intervals
Acute headaches—Temporal arteritis, intracranial tumor, subarachnoid hemorrhage, meningitis, subdural hematoma, primary angle-closure glaucoma, idiopathic intracranial hypertension (pseudotumor cerebri), carbon monoxide (CO) poisoning
Cluster HA
Brief attacks of unilateral excruciating periorbital pain, typically occurring at night
Occurring in series over weeks or months
Episodic—80 to 90% of cases; bouts of 6-12 weeks, every 1 or 2 years
Chronic—20 to 10% of cases
Young adult males, often smokers
Nausea/vomiting are uncommon
Presentation—Red eye, rhinorrhea, photophobia, phonophobia, lid edema, sweating
Precipitating factors—alcohol, naps, stress, missed meals, vasodilating drugs
Patients often pace
Preventative TX—Ca channel blockers, lithium, steroids, valproate
Abortive TX—oxygen, "triptans", intranasal lidocaine
Giant cell (temporal) arteritis
Unilateral headache—dull onset, intensifies to burning and moderate to severe jabbing pain localized over the temporal arteries
Females > 60 years of age
Most common form of vasculitis
Can cause ischemic heart disease, stroke or optic neuropathy
Presentation—myalgia, malaise, weight loss, nausea, fatigue, intermittent jaw claudication, fever, cough, polymyalgia rheumatica
Scalp/temporal artery tenderness is prominent
Visual disturbances or changes
Temporal artery abnormalities
Definitive DX requires biopsy of temporal artery (60-90% sensitive, 100% specific)
Blood tests—ESR, CRP, Factor VII are all elevated
TX—prednisone; excellent response to steroids (most patients are disease free by 4 years)
Ultrasound may be helpful for DX
Intracranial tumor
Headache due to displacement of vascular structures and other pain-sensitive tissues, increased intracranial pressure
Presentation—nausea/vomiting, occipital pain, 30% have headache
Symptoms depend on growth rate and location of tumor
History of IV drug abuse
Headache is lateralized, steady, dull or aching
Headache is worsened by exertion or postural change
Behavioral changes, mental status changes, seizures, focal neurological deficits
CT scan, MRI
Children (75% infratentorial), adults (75% supratentorial)
Medication overuse withdrawal
Patient symptomatically treats headache with good results
Use of medication increases over time
Headaches then develop from medication withdrawal
First noted with ergotamine
Typically results from overuse of OTC analgesics
Drug medication history is essential for DX
Difficult to treat, must remove causative medication and avoid substitution
Lifestyle modification TX—limit caffeine intake, regular exercise, mealtimes, and sleep schedule
Preventative TX—low-dose tricyclic antidepressants, gabapentin, topiramate
Migraine
Women in their late teens to early 50s, can occur in children/elderly
Family history
Very similar to tension-type headache
Recurrent, episodic headaches with moderate to severe pain
Aggravated by stress, depression/anxiety, menstruation, menopause, head or neck trauma, wine, cheese, vasodilators, infection, routine physical activity
Alleviated by relaxation in dark room and sleep
Presentation—nausea/vomiting, photophobia, phonophobia are common
Classic type—unilateral, pulsating, aura
Typical type—unilateral or bilateral, pulsating or steady, no aura
Aura—alterations in vision or sensation, light-headedness and photophopsia, scotoma
Acute TX—Aspirin, Tylenol, ibuprofen, naproxen, prescription NSAIDs, Midrin, triptans, ergotamine
Prophylactic first-line TX—Beta-blockers, SSRIs, Amitriptyline
Prophylactic second-line TX—Topiramate, sodium valproate, calcium channel blockers, botox
Non-drug TX—physical fitness, acupuncture, psychological therapy, relaxation, stress reduction, coping strategies, biofeedback
Pseudotumor cerebri
Obese females during reproductive years
Presentation—Headache, visual field loss, reduced visual acuity, bilateral blindness, diplopia, "whooshing" tinnitus, papilledema
Ultrasound of optic nerve
MRI or CT scan of head
Elevated opening pressure with LP
Normal CSF chemistry & cellarity
Most patients may have good visual outcome
TX—weight control, prednisone for visual complications, surgery (ventriculoperitoneal shunt, optic nerve sheath fenestration)
Subarachnoid hemorrhage
"Worst headache of my life"
Excruciating, exploding or bursting pain
Sudden onset, reaches crescendo in seconds
Localized anywhere on head, neck or back
Nausea and vomiting prominent
Presentation—nuchal rigidity (most common finding), altered mental status or coma, systolic BP > 200
Hemorrhage is usually from aneurysm rupture or arteriovenous malformation
Admit and refer for neuro consult
Do not drop diastolic BP below 100
Phenytoin to help prevent seizures
LP if imaging is negative
Tension-Type
Most common headache, "normal headache"
Presentation—skeletal muscle overcontraction, mild to moderate pain, variable location, often bilateral, "vice-like" or "tight-band," NO nausea, prodrome or photophobia
Aggravated by stress, depression, resentment, repressed hostility
Symptomatic TX—Aspirin, Tylenol, NSAIDs
Stress management—Biofeedback, stress reduction, posture correction
Non-drug TX—Exercise, massage, stress-coping, acupuncture
Chronic TTH—greater than 15 days per month, can result in substantial disability and work absence, > 50% of patients have sleep disturbances and mood disorders
Chronic TTH abortive TX—NSAIDs, Fioricet
Chronic TTH preventative TX—muscle relaxants, antidepressants, Amitriptyline (prophylactic drug of choice); consider a course of naproxen to break the cycle and stop analgesic overuse
Trigeminal neuralgia
Brief episodes of stabbing facial pain
Second and third division of the trigeminal nerve
Stimulated by wind, chewing, smiling, light skin touch, washing face, or brushing teeth
No clinically evident neurological deficit
Women over the age of 40
Patient presents with history of seeking dental care due to pain in the mouth
MRI is recommended for all patients
Hutchinson's sign—indicates herpes zoster involvement and doubles the risk for ocular complications
If diagnosed in a younger patient, multiple sclerosis should be ruled out
Weight loss can result due to painful chewing/swallowing
Severe pain unresponsive to treatment can lead to depression and/or suicide
Pain management TX—carbamazepine, oxacarbazepine, baclofen, gabapentin, muscle relaxers, NSAIDs
Seziure General Information
Seizure—a transient disturbance of cerebral function due to an abnormal neuronal discharge in the brain
Prodrome—mood or behavioral changes, myoclonic jerking, HA, lethargy, palpitations, epigastric sensations that may precede an attack
Aura—movement or odd sensation immediately before the before the attack, signals the beginning of the seizure
Ictus—the attack or seizure
Postictal period—period immediately following the attack
Todd's paralysis—transient hemiparesis occurring during the postictal period that may last minutes to hours
Etiology—congenital abnormalities, perinatal injuries, metabolic disorders, trauma, tumors and other space-occupying lesions, vascular disease, degenerative disease, infectious disease
Triggers—flashing lights, loud noises, fever, physical exhaustion, malnutrition, menses, injury, drugs, stress
Path—alteration in membrane potential that makes certain neurons abnormally hyperactive/hypersensitive to changes in their environment
DX—define the type of seizure, determine triggers, look for residual brain disease or dysfunction, perform general physical and neuro exam, UA, Blood work, TDM, EEG, CT scan of head, MRI of brain
TX during a seizure—maintain airway, protect from injury, record the attack
Medication TX—anticonvulsants, can discontinue gradually if seizure-free for 2 years
Surgical TX—anterior callosotomy (palliative), lobectomy/lesionectomy (curative), vagal nerve stimulation (partial-onset)
Solitary seizure—EEG within 24 hours, prophylactic drug TX not necessary
Alcohol withdrawal—generalized tonic-clonic within 48 hours, hospitalize for observation, treat with benzodiazepines
Febrile
Simple febrile seizure—most common, 15 minutes or less, high fever, normal neuro/physical exam, generalized tonic-clonic activity with no focal component
Complex febrile seizure—least common, greater than 15 minutes, consider CNS infection
Children aged 3 months to 5 years
Etiology—URI, OM, roseola, UTI, gastroenteritis, immunizations
Viruses (most to least common)—influenza, adenovirus, parainfluenza, RSV, rotavirus
Seizure usually manifests in first hours of acute infection
Immunizations—DTP, MMR
Family history of febrile seizure in primary relative is most consistently identified risk factor
Check for level of consciousness, rash, cranial sutures, meningeal signs
Meningeal signs—nuchal rigidity, Brudzinski sign, Kernig's sign
DX—age, fever, seizure are all essential
Usually self-limiting
No TX necessary for simple febrile seizure
If persistent seizure—ABC, diazepam
Parent education is key
Complications—biting oneself, breathing fluid into lungs, injury from falling
Absence (petit mal)
Consciousness impaired briefly
Begin in childhood and frequently cease by age 20
Presentation—brief (2-10 seconds), staring spells, abrupt, patient unaware of surroundings
EEG—bursts of bilateral synchronous/symmetric 3-Hz spike and wave activity
Atypical absence
Presentation—more gradual onset/termination, myoclonic jerks/automatisms with the staring spell
MRI or EEG
CBC, blood glucose, liver/kidney function, serology
Myoclonic
Presentation—extremely brief, single or multiple jerks of one or more muscle group
MRI or EEG
CBC, blood glucose, liver/kidney function, serology
Tonic-clonic (grand mal)
Sudden loss of consciousness followed by muscle rigidity
Tonic phase—individual falls, opening of the mouth & eyes, extension of the legs, adduction of the arms, tongue biting, high-pitched cry, arrested respiration and cyanosis, bowel/bladder incontinence, last abouts 10-15 seconds
Clonic phase—violent/rhythmic contractions, eyes roll, face grimaces, pulse accelerates, salvation increases, sweats excessively (diaphoresis), lasts 1-2 minutes with deep inspiration at the end
Terminal or postictal phase—disorientation/confusion, deep coma-like state, headache, drowsiness, nausea, muscle soreness, amnesia
MRI or EEG
CBC, blood glucose, liver/kidney function, serology
Atonic
"Drop attacks"
Sudden/complete loss of muscle tone
Falls and injuries are common
Complex Partial
Onset—loss/alteration of consciousness, lip-smacking, repetitive/semi-purposeful movements, aggressive behavior
Often lasts several minutes
Postictal—drowsiness, confusion
May be preceded, accompanied or followed by dysphasia, déjà vu, affective disturbances, illusions or structured hallucinations
Simple Partial
No loss of consciousness
Presentation—spreading or "marching" paresthesia, buzzing sounds, flashing lights, pupil dilation, diaphoresis, flushing, respiratory changes, convulsive jerking
Pseudoseizures
Usually no tonic phase
Consciousness may be normal or "lost"
Goal-directed behaviors such as shouting/swearing are present
Asynchronous thrashing of the limbs which increases with restraints
Postictally there are no changes in behavior or neurologic findings
No EEG changes
Status epilepticus
Continuing series of seizures without a period of recovery
Most frequent in first years of life
Second peak of frequency during puberty
Can occur with all types of seizures
Most often seen in patients with poor anticonvulsant drug regimen compliance
Can lead to permanent brain damage and death if ABC is not followed or the seizure is not stopped, from hypoxia, cardiac arrhythmias, lactic acidosis
Medical emergency, mortality rate of 20%
TX—IV benzodiazepine, IV phenytoin (dilantin)
If seizure continues give IV Phenobarbital
CNS Infection General Information
CSF analysis—tube 1 (fungal, bacterial, AFB cultures), tube 2 (chemistries, glucose, protein, serologies), tube 3 (cell count and differential), tube 4 (on ice for any viral cultures); collect 2 ml of CSF per tube
Criteria for CT before LP—immunocompromised, hx of CNS disease, new onset seizure, papilledema, abnormal level of consciousness, focal neurological defect
Most patients present with fever, HA, AMS or focal deficits
Clinical presentation depends on pathogenesis of spread of the infection to the CNS, virulence of the etiologic agent, area of CNS involvement
LP—positioning is everything, always get an opening and a closing pressure
Aseptic meningitis
Etiology—coxsackie virus, echovirus, infections near brain/spinal cord, fungi, mycobacteria, spirochete infection, TB, some cancers, some medications
~50% of cases are caused by coxsackie virus & echovirus, two members of the enterovirus family
Hand to mouth contact
Risk factors—health care worker, daycare, immunosuppression, exposure
Presentation—photophobia, N/V, chills, confusion, stiff neck, sore throat, drowsiness, HA, muscle pain, rash, fever
Lab—elevated protein, LP opening pressure, normal/low glucose
If caused by virus, self-limiting
Fungal/mycobacterial TX—high dose acyclovir
TX—antivirals, supportive care
Get vaccinated
Bacterial meningitis
Meningococcal meningitis—Neisseria meningitides, needs solid/moist environment, body temperature, only found in humans; most common cause in children, second most common cause in adults
Pneumococcal meningitis—Streptococcus pneumoniae; most common cause in adults, second most common cause in children
Neisseria meningitides has pili and lipopolysaccharide coatings, which make it possible to cross the BBB
Meningococcal risk factors—winter/spring, exposure, recent URI
Pneumococcal risk factors—alcohol, diabetes, trauma, ear infection, recent pneumonia, URI, spleen removal
Presentation—high fever, drowiness/confusion, N/V, photophobia, severe HA, neck stiffness, mental status change, fast heart rate
Complications—brain damage, hydrocephalus, deafness, MR, paralysis, shock
Early DX/TX extremely important to prevent serious illness or death
Pediatric TX—Dexamethasone + empirical ABX before culture, Ceftriaxone + Vancomycin after culture
Adult TX—Dexamethasone + targeted ABX (usually vancomycin + 3rd generation cephalosporin)
Get vaccinated—meningococcal currently recommended for college students, military recruits, travelers; pneumococcal currently recommended for children, everyone over the age of 55, people at high risk for pneumonia
Brain abscess
Occur when bacteria or fungi infect part of the brain
Usually spreads hematogenously
Most common source is a lung infection
Membrane forms around infected area and creates a mass
Membrane is supposed to protect, but often does more harm due to increased pressure on delicate tissue and blood vessel blockage
Risk factors—immunocompromised, chronic disease, corticosteroids or chemo, heart shunt, dental/intestinal infection
Presentation—aching neck, shoulders, back; changes in mental status, aphasia, fever/chills, HA, loss of coordination/muscle function, seizures, vision changes, vomiting, weakness
Neurological exam will usually show increased intracranial pressure and problems with brain function
Work-up—needle biopsy, CT scan before LP, MRI of head, blood cultures, CBC, EEG
Medical emergency—If untreated, fatal
TX—surgery to remove mass is gold standard
Medications—broad spectrum ABX, diuretics/steroids, amphotericin/diflucan for fungi
Medications should be used if several small abscesses are present, if the abscess is deep in the brain, if meningitis is a comorbid condition
Encephalitis
Inflammatory process of the brain with associated neurological dysfunction
Very important to obtain detailed H&P
Presentation—seizures, toxic appearance
CSF—could be normal or show elevated lymphocytes
TX—prevention, supportive
Prevention—vaccinations if traveling out of the country, DEET, avoid rural areas
Dementias General Information
Reversible—Drugs, delirium, depression; Emotions, endocrine disorders; Metabolic disturbances, medication-induced; Eye/ear impairments; Nutritional disorders, normal pressure hydrocephalus; Tumors, toxicity, trauma to head, thyroid disease; Infectious diseases; Alcohol, arteriosclerosis
Irreversible—Alzheimer, Lewy body dementia, Pick disease, Parkinson, head injury, Huntington disease, Jacob-Cruzefeld disease
Mini-Mental State Examination (MMSE), Functional Activities Questionnaire
Medications that may calm agitation without worsening dementia—Trazodone, Wellbutrin, Propranolol, cholinesterase inhibitors
Alzheimer disease
Progressive/fatal brain disease
Most common form of dementia
Women > 80 years of age
Unknown etiology, may have familial link (chromosome 14, 19, 21)
Hippocampus, temporal cortex, nucleus basalis of Meynert
Plaques, tangles, amyloid protein, decreased Ach & NE, choline acetyltransferase deficiency
Risk factors—old age, smoking, low folate levels, family history, twins, obesity, depression, lower education level, genetics (chromosome 14, autosomal dominant inheritence)
Presentation—subtle onset of memory loss followed by slowly progressive dementia, impaired language, visuospatial deficits, aimless wandering, delusions, loss of inhibition and aggression, disturbed sleep, rigidity, mutism, incontinence
Capgras syndrome—believing that a caregiver has been replaced by an imposter
Death usually occurs in 3 to 15 years due to malnutrition, secondary infections, pulmonary emboli, heart disease
Atrophy of cerebral cortex
Frontal release signs—palmomental reflex (twitching of ipsilateral chin upon stroking of thenar eminence), glabellar reflex (blinking with tapping on glabella), snout reflex (similar to primitive sucking reflex), grasp reflex
Lab/imaging—CT (diffuse cortical atrophy), MRI (hippocampus atrophy), PET (hypoperfusion in posterior temporal-parietal cortex), normal EEG
DX—insidious onset and progressive worsening of multiple cognitive deficits including: memory impairment, aphasia, apraxia, agnosia, disturbance in executive functioning
TX—no cure, symptomatic management, cholinesterase inhibitors (increase levels of Ach; Donepezil, Galantamine, Rivastemine), antioxidants (vitamin E, selegiline), Memantine, Gingko Biloba, Surgery
Dementia with Lewy bodies
Progressive dementing illness
Fluctuating cognitive impairment, visual hallucinations, features of parkinsonism, repeated falls
In some patients the first manifestation is delirium
Abnormal alpha-synuclein metabolism
Lewy bodies throughout the cortex, amygdala, cortex and substantia nigra
Presentation—recurrent visual hallucinations (vivid/colorful, mute animate objects), apathy, anxiety, delusions, parkinsonism, REM sleep behavior disorder, change in personality, impaired orientation/visuospatial functioning
Central feature that is essential for DX is progressive cognitive decline interfering with occupational or social functioning
Progresses to death, may have shorter survival time than Alzheimer's
TX—anticholinerasterase compounds, antidepressants, modify environment
Frontotemporal dementia (Pick disease)
Neurodegenerative condition characterized by disturbances in behavior or language
Behavioral symptoms predominate early
Autosomal dominant
Marked atrophy of the frontal and/or temporal lobes; frontal lobe (personality changes), temporal lobe (compulsive behavior, impaired memory)
Risk factors—family history, traumatic brain injury
Presentation—loss of social emotion, disinhibition, overactivity, apathy, deficit in executive functioning and working memory, poor attention and abstraction, progressive non-fluent aphasia
Neurological signs as disease progresses—repetitive motor behaviors, muscular rigidity
Obstinate imitation behavior
DX—neuropsychiatric assessment, neuropsychological testing, neuroimaging studies
Neuropsychological testing—memory somewhat spared, good orientation and recall of events, poor performance on recall-based tasks, reduction in spontaneous conversation, MMSE is unreliable for DX
Usually requires transition to long-term care facility, assistance with ADL
Estimated survival of 3-6 years from time of DX
TX—symptomatic, SSRIs can help behavioral issues
No anti-psychotics—they can exacerbate the condition
Normal-pressure hydrocephalus
Older adults > 60 years of age
Increased amount of CSF in the ventricles due to impaired CSF absorption
Underlying causes—intraventricular or subarachnoid hemorrhage, meningitis
Enlarged ventricular size with normal opening LP pressure
Common TRIAD—dementia, ataxia (feet "glued to the floor"), urinary incontinence
TX—ventricular shunting procedure, Acetazolamide
Parkinson disease
Men > 50 years of age
Most common neuro disorder
Idiopathic, associated with head injury, rarely genetic
Loss of dopaminergic neurons in substantia nigra, presence of "Lewy bodies"
Risk factors—elevated cholesterol, pesticide exposure, industrial plants
Presentation—asymmetric resting tremor (pill rolling) of upper extremity, bradykinesia, rigidity, gait difficulty (shuffling gait), mask-like facial expression
Cardinal Signs—pill rolling tremors, cogwheeling rigidity, bradykinesia
DX—2 out of the 3 cardinal signs must be present
Postural instability is 4th cardinal sign that presents later in disease
Lab—ceruloplasmin to rule-out Wilson's
TX—Levodopa/Carbidopa (tremors), Selegiline (neuroprotection), deep brain stimulation (last line tx for extreme cases)
Chronic Alcoholism
Wernicke's encephalopathy—ataxia, confustion, diplopia
Thiamine deficiency damages the thalamus, mamillary bodies, cerebellum, midbrain, peripheral nerves
Prompt administration of thiamine may reverse the disorder
Prolonged thiamine deficiency can lead to an irreversible dementia known as Korsakoff's syndrome
Patient is unable to recall new information
Recurrent head trauma
"Punch drunk"
Symptoms can be progressive, beginning late in the boxer's career, or even long after retirement
Severity of syndrome correlates with the length of boxing career
Initially note a personality change associated with social instability and sometimes paranoia/delusions
Later, memory loss progresses to full dementia associated with parkinsonian signs
Depression aka Pseudodementia
Particularly common in hospitalized or institutionalized elderly patients
Memory and language are usually intact, patient may feel confused & unable to accomplish routine tasks
Vegetative symptoms—insomnia, lack of energy, poor appetite, concern with bowel movements
Abrupt onset
Patients respond to treatment for depression—SSRIs, cognitive/behavior therapy
Vascular dementia
Men > 60 years of age
Results from vascular disease
Small artery occlusion resulting in multi-focal infarct areas of the brain tissue
Risk factors—old age, HTN, smoking, atrial fibrillation, DM, high cholesterol, obesity, sedentary life style
Presentation—impaired memory, impaired physical function, multiple mini-strokes
When compared with Alzheimer's, memory function is not as severe
CT/MRI will show multiple focal "lacunar infarctions"
TX—treat comorbidities, possible Cholinesterase inhibitors
Multi-infarct is multi-step process leading to depression and agitation
Cerebral infarction
People with clotting disorders or have undergone recent surgery at greater risk
Blood flow occluded to particular area of brain within an artery of the circle of Willis or smaller diameter artery
Risk factors—HTN, mitral stenosis, endocarditis, recent MI, prosthetic heart valve
Labs—CT, MRI, CBC, EKG
Speed is key, timing of treatment determines prognosis
TX—IvtPA (clot busters), ABC, control BP, finger stick blood sugar, endovascular excision of occlusion
Cerebral infarction (carotid circulation—anterior communicating/cerebral artery)
Has effects on the frontal lobe and basal ganglia
Frontal lobe infarction symptoms—aphasia (if on left brain), contralateral weakness (leg > arm), gaze preference, frontal release sign, abulia (lack of will to do anything), magnetic gait (without dorsi/plantar flexion), impaired judgement, overall personality change (if bilateral lobe affected)
Basal ganglia infarction symptoms—fine movement deficits, tremor, chorea
Cerebral infarction (carotid circulation—middle cerebral artery)
Has effects on frontal, parietal, temporal lobes and basal ganglia
Left parietal lobe infarction symptoms—aphasia, finger agnosia, right sensory loss, right-side weakness (face/arm > leg), right/left confusion, agraphesthesia (can't recognize written numbers/letters)
Right parietal lobe infarction symptoms—apraxia, left sensory loss, left side visual field neglect, left lower quadrant of vision loss, left-side weakness (face/arm > leg)
Temporal lobe infarction symptoms—aphasia (if on left), memory loss, deafness, contralateral upper quadrant vision loss
Cerebral infarction (vertebrobasilar circulation—posterior cerebral artery)
Has effects on the occipital lobe, thalamus, and brainstem
Occipital lobe infarction symptoms—major vision loss on contralateral side, may have hallucination
Thalamus infarction symptoms—loss of sensation on one side of body, ataxia, confusion, aphasia, sleep/wake cycle issues
Brainstem infarction symptoms—pinpoint pupils, decreased vertical eye movement, decreased level of consciousness
Cerebral infarction (vertebrobasilar circulation—vertebral artery)
Has effects on the brainstem
Brainstem infarction symptoms—decreased level of consciousness, dysphagia
Wallenberg syndrome—loss of pain and temp on contralateral side of body and loss of sensation on ipsilateral side of face
Cerebral infarction (vertebrobasilar circulation—posterior inferior cerebellar artery)
Has effects on cerebellum and brainstem
Cerebellum infarction symptoms—wide-based gait, titubation (bobbing motion of head), ipsilateral dysmetria (undershoot/overshoot when trying to grab an object), ataxia, nystagmus, abnormal eye movements, decreased balance
Cerebral hemorrhage
Nimodipine and triple H therapy (fluid hypertension, hypervolemia, and hypotonicity of blood) to prevent vasospasm, ischemic stroke
CT/MRI/MRA/Cerebral angiogram without contrast are all needed for correct DX/TX
Subarachnoid hemorrhage TX—Clipping/Coiling
Intracranial hemorrhage TX—lower BP, elevate head above heart, if severe (craniotomy to release pressure)
Poor prognosis with 50% mortality rate, only 20% return to normal
No acute drug intervention, prevention is key
Cerebral hemorrhage (arteriovenous malformation)
Congenital defect in artery/vein of brain circulation
Results in ruptured aneurysm in the subarachnoid space if intravascular pressure is high enough
Accounts for ~5% of SAH
Cerebral hemorrhage (intercerebral hemorrhage)
Brain bleed within the brain tissue
Usually a result of trauma, can be caused by a ruptured aneurysm
Symptoms worsen progressively over time in the form of neuro deficits
May appear as ischemic stroke
Cerebral hemorrhage (intracranial aneurysm)
Ballooning of artery/vein inside the brain tissue that could result in rupture, causing bleeding in the brain
Risk factors—atherosclerosis, HTN, smoking, alcohol, drug abuse, long-term NSAID use
Cerebral hemorrhage (subarachnoid hemorrhage)
"Worst headache of my life"
80% caused by ruptured aneurysm
If symptoms present, it may be too late
Symptoms—meningeal irritation, cranial nerve deficits, confusion, LOC, coma, nausea/vomiting, sudden death
Can result in ischemic stroke
Chance of re-bleed is very high
Must observe for > 5 days
Lacunar infarction
Small artery disease
Elderly hypertensive and diabetic patients
If patient awakens with symptoms, more likely thrombotic
Capsular infarct symptoms—pure motor hemiplegia or hemiparesis
Dysarthria, pure sensory stroke, ataxic hemiplegia or hemiparesis with pyramidal tract signs
Multi-infarct dementia symptoms—sensory, motor, bulbar, and cerebellar signs
Status lacunatus symptoms—mild hemiparesis, ataxia, dysarthria, incontinence, and parkinsonian tremor
MRI, CT without contrast, Ultrasound, and Doppler Imaging
Medication TX—Aspirin, Ticlopidine, Clopidogrel, Thienopyridines
Clopidogrel side-effects—Rash, diarrhea, abdominal pain, dyspepsia, intracranial hemorrhage, GI bleeding
Control HTN
Transient ischemic attack (TIA)
Brief episode of neurologic dysfunction attributable to a focal disturbance of brain or retinal ischemia symptoms lasting < one hour and without imaging evidence of infarction
Hospital admission warranted
20% of TIA patients will have stroke within 90 days, 50% within the first 2 days
Risk factors—migraine, DM, smoking, alcohol, high cholesterol, poor diet, sedentary lifestyle, obesity, HTN, substance abuse, genetics
Presentation—weakness, clumsiness, total paralysis, ataxia of gait, trunk extremities, paresthesia, sensory loss, agnosia, vertigo, loss of vision in ipsilateral eye, diplopia, loss of vision of one or both homonymous visual fields, dysphagia, aphasia, dysarthria
DX—MRI is preferably imaging study, DX is based on history
Testing—Blood tests (CBC, electrolytes, serum glucose, creatinine level), CT, MRI, EKG, Echo, telemetry
Evaluation of carotid arteries—ultrasound, CT Arteriogram, MRA
ABCD Risk Score—Age, Blood pressure, Clinical features, Duration of symptoms
ABCD Score > 2 associated with increased risk of stroke within 90 days of TIA
Noncardioembolic TIA TX—antiplatelet drug therapy (Aspirin, Dipyridamole, Clopidogrel)
Cardioembolic TIA TX—long-term oral anticoagulation drug therapy, if contraindicated, Aspirin
Hospitalization—ABCD score >3, ABCD score 0-2 and unclear if outpatient workup can be completed < 2 days
Management of risk factors following a TIA—control HTN, smoking cessation, control diabetes
Benign essential (familial) tremor
Benign action tremor, usually of the head, chin, outstretched hands, occasionally of voice
Most common movement disorder
Characterized by a slowly progressive postural and/or kinetic tremor
Both sexes, prevalence increases with age
Usually begins in one upper extremity, head is involved most frequently
May be intermittent initially, emerging only during periods of emotion, resolves during sleep
Family history
Tone/reflexes are normal, parkinsonian features are absent, resolves when the body part relaxes
DDx—metabolic tremors (kidney/liver disease, B12 deficiency, hyperthyroidism), toxic tremors (caffeine, alcohol, nicotine, withdrawal, lead, arsenic)
Work-up—thyroid panel, toxicology screening, TDM tests, heavy metal screen, Ceruloplasmin (Wilson's), gluose (hypoglycemia)
Maintenance therapy TX—Propanolol, Primidone
Surgical TX—implanted brain stimulator (gold standard), thalamotomy (gold standard for severe cases)
Cerebral palsy
Developmental disorder of motor function
Not a single disease, but a group of conditions
Present after birth
Characterized by abnormal control of movement or posture
~15% of cases acquired through brain injury, infection, trauma after first month of life
Etiology—pre-maturity, congenital birth defects, intrapartum asphyxia, cerebral hemorrhage at birth, infection, anoxia at birth
Presentation—impaired movement, speech defects, MR, ocular defects, seizures, hearing impairment, seriously disabled
Early death is usually due to respiratory involvement
DX—delayed milestones, persistence of primitive reflexes, pathologic reflexes, failure to develop protective reflexes
MR is a common associated problem, seizure disorders, visual abnormalities
Most children who sit by 2 years old will walk
TX—physical therapy, speech therapy, orthopedics, botox (mainstay), Baclofen (muscle relaxor), Benzodiazepam (Valium), Valproic acid (anticonvulsant), Levodopa
Prevention—therapeutic hypothermia
Cerebral palsy (Spastic type)
Hyperirritability of muscles, arms flexed, legs internally rotated, difficulty bending into a sitting position, difficulty with head control, postural difficulty, may not have protective extension
Cerebral palsy (Spastic type—Spastic Quadriplegia)
Involves all four limbs (legs > arms)
Bilateral hemisphere involvement, severely impaired and MR, often have bulbar symptoms
Gait—fisting of hands, scissoring of lower limbs, probably unable to walk
25% of patients will require total care
Cerebral palsy (Spastic type—Spastic Diplegia)
Involves legs more than arms
Often associated with premature births, MR not as severe, impairment not as severe
Gait—contractures of hips, knees, and feet; have control of their upper extremities
Cerebral palsy (Spastic type—Spastic Hemiplegia)
Involvement of arm and leg on one side
Motor handicaps least likely to be disabling, intelligence is normal to dull
Gait—contractures of hip, knee, and foot
Presents similar to someone who has had a stroke
Cerebral palsy (Extrapyramidal)
Uncontrollable writhing movements of opposing muscle groups
All four extremities, neck/face involvement
Voluntary movements are flailing
Difficulty staying upright and balanced
May lack protective extension
Defects of posture, involuntary movements, ataxia, and rigidity
Usually in newborns
Persistent asymmetric tonic neck reflex
Cerbral palsy (Ataxic type)
Affects balance and coordination
May walk with an unsteady gait with feet far apart
May have difficulty with motions that require precise coordination
Tremors
Rhythmic, oscillatory movements, best characterized by its relationship to voluntary movement
Often due to organic disease or emotional stress, disappear during sleep
Described as fine or coarse, rapid or slow, occurring at rest, holding a posture, or performing an activity
Classification—Static tremors (resting/postural), Action tremors, Intention tremors
DX—Hx, PE, lab work, action tremor is most common, with ET/EPT being most frequently diagnosed
History—visible symptom, previous handwriting samples, list of medications
Examination—observe patient while seated, lying down with the affected body part fully supported, and while walking; tremor frequency should be documented in all positions
If patient is under the age of 40, always suspect Wilson's disease
TX—depends on the underlying cause
Physiological tremor
Occurs in normal individuals
May be transient and occur in association with excessive physical exertion, fatigue, excitement or hunger
Not visible under ordinary circumstances
Enhanced physiologic tremor
Action tremor associated with catecholamine excess
Causes—anxiety/fear, drug toxicity, heavy metal poisoning, carbon monoxide, fever, hypoglycemia, alcohol withdrawal
May occur as a drug side-effect—epinephrine, caffeine, tricyclic antidepressants, lithium
Most common cause of postural and action tremors
Flapping tremor (Asterixis)
Coarse tremor of a muscle group
Brief, arrhythmic voluntary muscle contraction, usually bilateral, may be asymmetric
Wrist tremor with outstretched arms (bird-flapping)
Seen in hepatic coma & other diseases causing encephalopathy
Intention tremor
Occurs when voluntary motion is attempted
Increases in severity as the hand moves closer to its target
Causes—brainstem/cerebellar disease, drug toxicity, Wilson disease, MS, midbrain trauma/stroke
Rest tremor
Occurs at rest, absent/diminished with active movements
Causes—Parkinsonism, Wilson disease, heavy metal poisoning
Tremor quickly reappears as soon as the body part assumes a new resting posture and may therefore interfere with the use of utensils, handwriting, and other purposeful movements
Parkinsonian tremor
Rest tremor that is suppressed briefly during voluntary activity
Tourette syndrome
Autosomal dominant disorder that causes involuntary motor and vocal "tics"
Becomes more severe during puberty, stabilizes in adulthood
Affects how the basal ganglia uses its transmitter chemicals
Males; motor tic onset 5-7, vocal tic onset 7-9
Risk factors—strep infection, Mg deficiency, low birth weight, maternal life stress during pregnancy
Motor tics—involuntary, rapid, sudden movement (usually of muscles), most common manifestation, commonly involve the face
Vocal tics—involuntary, rapid, sudden vocalization (involves the larynx, tongue, sinuses, or mouth)
Simple motor tics—usually only one muscle group, sometimes mistaken for muscle spasms
Complex motor tics—more than one muscle group, similar to compulsions of OCD
Simple vocal tics—completely meaningless
Complex vocal tics—long and intricate
DX—based on H&P, no diagnostic test, onset before the age of 18, multiple motor tics and one or more vocal tic, occur many times a day or intermittently over a period of one year, causes marked distress or significant social/occupational impairment
Associated with ADHD, OCD, LD
Worst symptoms during the teen years
TX—behavior therapy, Clonidine (1st line med)
Huntington disease
Autosomal dominant disorder associated with cell loss in the basal ganglia and cortex
Incurable, adult-onset, symptoms don't usually occur until 30
Repeated CAG in DNA chain seen in chromosome 4
Gross atrophy of the caudate nucleus and putamen
Presentation—chorea, dementia, short-term memory loss, diminished verbal fluency, sleeping problems, depression, psychosis, OCD, personality changes, bradykinesia, unsteady gait, anorexia, weight loss, dysphagia, difficulty performing simple tasks
Dopamine excess
CT scan, genetic blood tests
Most patients survive 15-20 years after diagnosis
TX—no cure or prevention, symptomatic
Benzodiazepines/antidepressants (insomnia, anxiety, depression), antipsychotics (psychosis), antidepressants, Tetrabenazine (chorea)
Parkinsonism
Etiology—genetic mutations, drug-induced toxicity, metabolic disease, dopamine depletion
Presentation—rest tremor that is suppressed briefly during voluntary activity, rigidity, bradykinesia
TX—Levodopa, anticholinergic (resting tremor)
Surgical TX—Thalamotomy, Pallisotomy, deep brain stimulation
Restless leg syndrome
Elderly women
Autosomal dominant in young-onset RLS, although no genes have currently been found
Primary RLS—idiopathic, with a strong likelihood of family history
Secondary RLS risk factors—pregnancy, chronic renal failure, iron deficiency, diabetes, hypothyroidism, alcohol, caffeine, nicotine
Presentation—restlessness, sensory disturbances that lead to an irresistible urge to move the limbs, especially during relaxation, primarily at night, may interfere with sleep, partial or complete relief by movement
Severity and symptoms may worsen with age
TX—avoidance of caffeine, alcohol, nicotine, discontinue medications that exacerbate, increase iron intake, Dopamine agonists, Benzodiazepine (anticonvulsant), Levodopa, Klonopin, Clonidine
Exacerbating medications—SSRIs, neuroleptics, lithium, beta blockers, sedating antihistamines
Complications—insomnia, irritability, anxiety, depression, excessive daytime sleepiness
DX—exclusion of secondary causes, CBC, NCS, electromyography, polysomnography
Wilson's disease
Neurodegenerative disease of copper metabolism—autosomal recessive disorder
Mutations/deletions of the ATP7B protein encoded by chromosome 13
Loss of ability to export copper from the liver into the bile and to incorporate copper into hepatic ceruloplasmin
Results in excessive deposition of copper in the liver, brain, kidney, and cornea
Typical onset is 6-20 years of age
Usually presents as liver disease in adolescents
Usually presents as neuropsychiatric disease in young adults
Presentation—tremor, dystonia, incoordination, dysarthria, dysphagia, abnormal eye movements, spasticity, respiratory dyskinesia, behavioral/personality changes, emotional lability, irritable, anger, difficulty concentrating, osteoporosis, OA, joint hypermobility
DX—Kayser-Fleischer Rings, low serum ceruloplasmin level, high urinary copper level
Work-up—CT of head (putamen atrophy), MRI of brain (cortical atrophy/ventricular enlargement), liver biopsy (increased copper levels)
TX—life-long chelating agent (D-penicillamine is gold standard), low-copper diet (no shellfish, liver, mushrooms, broccoli, chocolate, nuts), liver transplant
Peripheral Neuropathies General Information
Sensory symptoms—loss of sensation (touch & joint proprioception in large fibers; pain & temperature in small fibers), disturbed sensation ("pins and needles" in large fibers; burning in small fibers)
Motor symptoms—muscle atrophy, weakness, and flaccidity; decreased reflexes
Nerve Conduction Studies (NCS)—performed to evaluate the physiological function of the peripheral nerves, sensory nerve studies, motor nerve studies, conduction velocity
Electromyography—an invasive procedure used to identify electrical activity in the muscle
Carpal tunnel compression syndrome
Pain, burning, tingling in the median nerve, initially bothersome at night, weakness of atrophy of the thenar eminence appears later, common in occupations that require repetitive wrist movements and in pregnancy, DM, and RA
Compression of the median nerve between the carpal ligament and other structures within the carpal tunnel
Palmar surface of thumb, index and long fingers, radial half of ring finger
Pain may radiate proximally into the forearm, shoulder, neck/chest
Phalen sign/Tinel sign/Carpal compression test—positive
TX—modify hand activities, splint wrist for 2-6 weeks, NSAIDs, corticosteroids, surgery
Diabetic neuropathy
Most common cause of neuropathy in the U.S., increases with age, uncommon in childhood
Peripheral nerve damage related to poor control of DM (more common in DM I)
Polyneuropathy—distal weakness and sensory loss (ataxia with large fibers; paresthesia with small fibers)
Autonomic neuropathy—pupil abnormalities, loss of sweating, orthostatic hypotension, resting tachycardia, gastroparesis, diarrhea, hypotonic dilated bladder, impotence
Diabetic amyotrophy—severe pain and weakness rapidly develop, weakness of quads/iliopsoas, loss of knee jerk, minimal sensory loss
Cranial nerve palsy—CN III, pain behind eye, followed by ptosis and extraocular paralysis within 24 hours, may occur with pupillary sparing, complete recover is the rule
TX—control DM, neuropathic pain (Carbamazepine, Gabapentin, Duloxetine), Capsaicin cream
Postherpetic neuralgia
Continued dermatomal pain persisting beyond 4 months from initial onset of lesions, due to inflammation and nerve structure damage after Herpes Zoster (Shingles)
Typically affects older individuals, increased prevalence with age
Presentation—burning, aching, itching, multiple dermatomes, severe paroxysm of stabbing or burning pain, allodynia, hyperesthesia, dysesthesia, anesthesia
TX—low-dose antidepressant (Amitriptyline, Nortriptyline), anticonvulsants (Carbamexpine, Gabapentin), Opioids (Clonidine), topical capsaicin cream, nerve blocks (older patients respond better)
Get vaccinated—Herpes Zoster vaccination
40% of patients remain totally refractory or unsatisfactorily relieved
Radial nerve compression palsy
"Saturday night palsy"
Weakness or paralysis of all muscles being supplied by the radial nerve
Etiology—crutches, arm hanging over back of chair, hand cuffs, tight watch straps
Presentation—pain at night and after exercise, deep aching in extensor mass of forearm radiating to wrist
Wrist drop in acute cases
No TX is necessary, complete recovery usually occurs within 2 months
Sciatic and common peroneal nerve compression palsy
Injury, trauma, rare
Sciatic—trauma to buttocks, hip or thigh due to any injury; most common cause is misplaced deep IM injections
Peroneal—compression or injury to head, neck of fibula; wearing tall boots or sitting with legs crossed
Presentation—foot drop, weakness or gait difficulty, paresthesia, tenderness at fibular head
Lab—lumbosacral spine x-ray, CT to rule-out spinal cord lesion, possibly EMG-NVC
Axon degeneration can occur
Complete recovery generally occurs within 2 months
Vitamin B12 deficiency
Form of nutritional polyneuropathy
Distal portion of nerves initially affected, segmental demyelination and axonal degeneration occur simultaneously
Vagus nerve and sympathetic trunk involvement in severe cases
Presentation—impotence, dizziness, disordered sweating, areflexia, "stocking/glove" sensory loss, hoarse voice and disturbance of swallowing (vagus nerve involvement), shiny skin on legs due to poor distal hair growth, painful soles of feet, evidence of liver failure, progressive distal weakness & sensory loss with painful, tingling paresthesia of the lower extremities
Labs—nutritional/alcohol history, CBC with elevated MVC, elevated liver function test, mildly reduced motor and sensory conduction velocities
Recovery may be slow and incomplete
TX—high calorie diet supplemented with Thiamine, Carbamazepine or Gabapentin for paresthesia
Amyotrophic lateral sclerosis
Most common progressive motor neuron disease causing muscle weakness, disability, and eventual death
Autosomal dominant inherited disease (10% of cases)
Males 50-70 years of age
Path—loss of motor neurons in anterior horn, brain stem, and motor cortex; loss of corticospinal fibers in the lateral and anterior columns of the spinal cord; atrophy of peripheral nerves, denervation atrophy of skeletal muscle
Risk factors—age, family history
UMN Presentation—hyperreflexia, spasticity, +Babinski's sign, jaw jerk
LMN Presentation—weakness, muscle atrophy, cramps, fasciculations
Asymmetric limb weakness in 80% of cases, dysarthria, dysphagia, increased risk of falling, hand weakness, foot drop, respiratory muscle weakness, axial muscle weakness
Frontotemporal executive dysfunction—changes in personality, impairment of judgment, development of obsessional behaviors
DX—must present with lower motor neuron involvement (2 limbs), upper motor neuron involvement (1 region), EMG findings of fibrillation, exclusion of other closely resembling disorders
Median survival of 3-5 years; longer term survival associated with younger age of onset, male gender, limb rather than bulbar symptom onset
TX—no cure, goal is to control symptoms and maintain quality of life, Riluzole (glutamate inhibitor), pressure ventilation, permanent ventilation, PEG tube placement
Progressive neuromuscular respiratory failure is the most common cause of death in ALS
Bell's palsy
Lower motor weakness of CN VII not caused by trauma or other definable etiology
Common with DM, pregnancy, Hispanics
Probable HSV, HZ, EBV, CMV
Presentation—prodrome consisting of ear pain or hyperacusis, progressive facial weakness, drooling, loss of taste, decreased tearing
Usually resolves on its own, can use Prednisone x7 days
Patients with poor outcome—HTN, DM, elderly
Complications—inability to close affected eye, lacerations of inner cheek/tongue, decreased muscle tone
Guillan-Barre syndrome
Acute inflammatory demyelinating polyneuropathy
Males aged 15-35 & 50-75
Etiology—URI, GI illness, HZ, mumps, measles, EBV, mycoplasma pneumonia, campylobacter jejuni enteritis, immunizations, trauma, surgery, Hodgkin's disease
Presentation—progressive symmetric muscle weakness usually starting in the proximal leg, paresthesia of hands/feet in 80%, absent/decreased DTR, severe lower back pain, tachycardia/bradycardia, HTN alternating with hypotension, urinary retention, loss of sweating, pulmonary dysfunction, papilledema, hearing loss, meningeal signs, vocal cord paralysis, mental status changes
DX—clinical history, elevated CSF proteins, normal CSF WBC count, nerve conduction study shows prolonged motor conduction findings indicative of demyelination
Complications—respiratory failure, DVT/pulmonary embolism, muscle atrophy, disability, death
Poor prognosis factors—older age, rapid onset, ventilatory support, preceding diarrheal illness
TX—mainly supportive, disease modifying (plasmaphoresis, IV immunoglobulin)
Multiple sclerosis
Autoimmune inflammatory demyelinating disease
Females of Northern European descent between age 20-45
Path—areas of demyelination in basal ganglia, medulla, cerebellum, optic nerves, spinal cord
Acute lesions—inflammatory mononuclear cells, presence of macrophages containing lipids
Relapsing remitting—most common type of MS, clearly defined exacerbations followed by periods of partial or complete recovery and remissions
Secondary progressive—second phase of relapsing remitting followed by steady decline in neurological function
Primary progressive—rare, slow but almost continuous decline in neurological function
Progressive relapsing—rare, progressive from onset but with clear exacerbations
Risk factors—genetics, optic neuritis
Presentation—numbness, tingling impairment of vibration and joint position sense, decreased pain and light touch perception, trigeminal neuralgia, back pain, muscle cramping due to spasticity, muscle weakness, bowel/bladder incontinence, dysarthria, ataxia, tremor, fatigue, dizziness, vertigo, depression, diplopia, optic neuritis, heat sensitivity, action tremor, positive Babinski's sign, impaired coordination/balance, impaired visual acuity, nystagmus
DX—LP with CSF analysis, WBC count slightly elevated, normal glucose, protein slightly elevated or normal, electrophoresis (oligoclonal bands), IgG elevated, MRI (diagnostic tool of choice)
ESR, ANA, RF titer to help identify the presence of a vasculitic disorder that may be mimicking MS
MRI—Ovoid appearing lesions in the periventricular region, corpus callosum, deep white matter structures, basal ganglia
Evoked potential testing—detecting clinically silent lesions, documenting an organic basis for vague complaints
Complications—increased risk of infection, sexual dysfunction, pressure ulcer, spasticity, depression
Average life expectancy is 5-7 years
TX—self-catheterization/fiber (urinary/bowel incontinence), SSRIs for depression, Corticosteroids (RRMS/PRMS), IV glucocorticoid + cyclophosphamide (SPMS), IV glucocorticoid (PPMS)
Disease modifying therapies—Avonex, Copaxone, Rebif, Betaseron
Myasthenia gravis
Autoimmune disorder of neuromuscular transmission
Females during infancy and 20-30 years of age, males > 50 years of age
Path—postsynaptic response to Ach is reduced, threshold for activation of muscle action potential not reached
Patients become symptomatic when Ach receptors are reduced to 30% of normal level
Presentation—fluctuating skeletal muscle weakness, often with muscle fatigue (hallmark), ptosis, diplopia, dysarthria, dysphagia, fatiguable chewing, isolated neck weakness, respiratory muscle weakness, and distal limb weakness
Active phase—most fluctuations, most severe
Stable second phase—symptoms stable but persistent
Third phase—remission may occur
Myasthenia crisis—brochospasm with wheezing, bronchorrhea, respiratory failure, diaphoresis and cyanosis; miosis + sludge syndrome (saliva, lacrimation, urinary incontinence, diarrhea, GI upset/hypermotility, emesis)
DX—icepack test, Tensilon challenge test, serologic testing, electrodiagnostic studies
Complication—respiratory failure, aspiration pneumonia, community-acquired pneumonia
Most patients on therapy have near normal lifespan, thymectomy results in complete remission in most patients
TX—symptomatic (Pyridostigmine), chronic immunomodulating (Prednisone, Azathioprine, Cyclosporine), rapid immunomodulating (myasthenia crisis—plasma exchange & IV immunoglobulin), surgical (thymectomy)
Cryptococcal Meningitis
Caused by the fungus Cryptococcus neoformans, which is commonly found in dirt and bird droppings
First invades the lungs and spreads to the covering of the brain/spinal cord, causing inflammation
Presentation—fatigue, fever, HA, nausea, memory loss, confusion, drowsiness, vomiting
If untreated, patients may lapse into coma and die
Herpes Viruses
The varicella zoster virus can infect the brain and produce encephalitis and myelitis
Commonly produces shingles, which is an eruption of blisters and intense pain along an area of skin supplied by an infected nerve
In AIDS patients, the viruses can disseminate to the entire body and be life-threatening
CMV
Encephalitis presentation—weakness in the arms and legs, problems with hearing and balance, altered mental status, dementia, peripheral neuropathy, coma, and retinal disease that may lead to blindness
Infection of the spinal cord and nerves can result in weakness in the lower limbs and some paralysis, severe lower back pain, and loss of bladder function
Neurosyphilis
May cause slow degeneration of the nerve cells and nerve fibers that carry sensory information to the brain
Presentation—weakness, diminished reflexes, unsteady gait, progressive joint degeneration, loss of coordination, episodes of intense pain and disturbed sensation, personality changes, dementia, deafness, visual impairment, impaired response to light
More frequent in men, onset is common during mid-life
PML
Progressive multifocal leukoencephalopathy caused by the JC virus, which travels to the brain, infects multiple sites, and destroys the cells that make myelin
Presentation—mental deterioration, vision loss, speech disturbances, ataxia, paralysis, brain lesions, coma; seizures may occur
Relentlessly progressive and death usually occurs within 6 months of initial symptoms
Toxoplasmosis
Toxoplasma encephalitis occurs in about 10% of untreated AIDS patients
Caused by the parasite Toxoplasma gondii, which is carried by cats, birds, and other animals and can be found in soil contaminated by cat feces and sometimes in raw or undercooked meat
Presentation—encephalitis, fever, severe HA that does not respond to treatment, weakness on one side of the body, seizures, lethargy, increased confusion, vision problems, dizziness, problems with speaking and walking, vomiting, and personality changes
Vacuolar Myelopathy
Causes the protective myelin sheath to pull away from nerve cells of the spinal cord, forming small holes called vacuoles in nerve fibers
Presentation—weak/stiff legs and unsteadiness when walking; walking becomes more difficult as the disease progresses and many patients eventually require a wheelchair; some patients also develop AIDS dementia
May affect up to 30% of untreated adults with AIDS and its incidence may be even higher in HIV-infected children