Clinical features of touretts and two types of tics:
1. Motor tics (multiple) - jerking, shrugging, grimace, blinking
2. phonics (at least 1 kind) - grunting, sniffing, clearing throat, copralacia, repetition of words
Treatment of SAH
1. Consult neurosurgery. Berry aneurysm treat surgically
2. Therapy dec risk of rebleeds and vasospasms
-bed rest and quiet
-stool softeners to decreased straining (will otherwise inc ICP and inc rebleed risk)
-IV fluids for hydration
Myasthenia gracis is liminted (often) to _____ in elderly
Age of M v. F?
EOM (extraocular muscles)
peaks- in females at 20-30YO
peaks-in males 50-70
Huntingtons chorea characteristics
autodomal dominant chromosome 4; CAG trinucelotide repeat:
3. CAG repeat
4. Cuarenta (40YO)
5. Chromosome 4
-progressively worsens by generation
-mutation on chrom 4 expanded repeat CAG 0 loss of GABAergic in striatum (dec negative feedback and thus lack of inhibition
Abrupt onset of ASCENDING paralysis and weakness in all 4 limbs progressing to repiratory/facial/bulbar muscles
2. weakness mild-severe
3. weaknesss distal to central
4. Painful extremities but NO sensory loss
GB syndrome (starts low and GOES HIGH)
What is a TIA?
neurological deficit that lasts from a few minutes to NO more than 24 hours (~30 min = normal)
-doesnt cause permanent infarction
Autopsy of AD lesions show:
-quantity of senile plaque (age specific) focal collections of dilated neuritic processes surrounding amyloid core (b- protein amyloid)
-quantity of neurofibrillary tangles *agespecific
-neurofilaments in cytoplasm of neurons - neuronal degeneration
NF type I = AD; care au lait spots, neurofibromas, CNS tumors, glioma and meningiomas with MR, short height, macrocephalus
NF type II = AD/chromosome 22, bilateral acoustic schwannomas/multiple meningiomas/cafeaulait spots
Tuberosclerosis (TS) = AD/cog impairment, epilepsy, skin lesions, angifibromas, retinal hamatomas, renal angiomylipo, rhabdomyolipioma of heart
Sturge-weber syndrome = aquired; capillary angiomatosis of PIA mater. Facial vascular NEVI (port urine stain)
Generalized treatment of seizures
standard antiepileptic drug used to provide control in 70% of patients
-if partial (simple/complex) or grand mal use phenytoin/carb
-if absense = ethosuxamide/valproic acid
If persists - increase dose of first antiepitleptic until signs of toxicity appears then add the second
Continue 2 years as controlled then TAPER down
Differential diagnosis of ring-enhancing brain lesion
1. Metastatic cancer
2. Brain abscess
3. Glioblastoma multiforme
Bilateral fixed, dilated pupil caused by: ____ ___
Unilateral fixed; dilated pupils caused by:
Pinpoint pupils caused by:
Herniation with CN3 compression
Treatment for MG?
What drugs exacerbate them?
Treatment: pyridostigmine/edrophonium - ACHEi
-thymectomy - symptom benefit and complete remission +/- thymoma
-immunosuppressed = corticosteroids/aza/cyclosporins
AB- aminoglycosides (GNAT); tetrcyclines; BB; antiarrhythmics, quinidine, procainamide, lidocaine
forgetfulness v. dementia
Some degree of memory loss is accepted as a normal part of aging. May be difficult to distinguish this condition from TRUE dementia
Benign forgetfullness doesnt adversly affect ADL but can be a risk factr for progressive dementia such as AD
What infections can cause dementia?
HIV infections (AIDS related dementia)
cryptococcal injection of CNS
Creutzfeldt-jakob disease (spongiform)
PML from that of lysosomal d/o
Levodopa (portion of SINEMET) has what action:
aspect of sinemet that shows an "on-off" phenomenon over daily coarse leading to fluctuation of symptoms
-dose dependent response
Risk factors for alziemers dementia
Family history (especiall for early onset AD)
Etiology: chromosome 14, 19, 21 linked to AD
When are patients screened with carotid duplex?
1. Carotid bruit
3. Coronary artery dz
-measures degree of stenosis if present
Diagnosis of MS
1. Fatigue - most common complaint
2. Motor symptoms - weakness, spasticity (UMN) involved
3. Visual disturbances - optic neuritis (loss of mononuclear vision) pain of eye mvmt, central scotoma, decreased pupillary light reflex
-internuclear ophthalmoplegia - lesion to MLF adduction defect and horizontal nystagmus of abduction.
4. cerebellar inovlvement-ataxia, intention trmor, dysarthia
5. bladder control - decreased with neuropathic pain
If the patient presents to ED with findings suggestive of an acute stroke, order:
1. Non contrast (CT scan) of brain
2. EKG, CXR
3. CBC, platelets
7. Bilateral carotid US
Duchenne's myotonic dystrophy
-dystrophen gene mutaiton: proteins in muscle cells that die and increase fat
Features = weaknes of muscles - symmetric and starts in childhood
-primary pelvic girdle - GOWERS
-enlarged calfs due to pseudohypertrophy
Dx: serum creatinine PK increases; DNA testing > muscle biopsy
Two major categories of hemorrhagic stroke?
ICH - intracranial into parenchyma
SAH - bleeding into CSF; outside brain parenchyma
Which medications cause parkonsonian like side effects
HIGH potency neuroleptics or antipsychotics
-chlopromazine, haloperidol (haldol), perphenazine
What is a lacunar stroke?
Small vessel thrombotic disease
-20% of all strokes affecting:
1. Basal ganglia causing parkinsonian symptoms
2. Thalamus-gateway to brain
3. Internal capsule-pathway from thalamus to the cortex and cortex to the SC thus can affect motor OUTput
Symptoms of TIA from carotids
1. Temporary loss of speech
2. Paralysis of contralateral extremity
3. Clumsiness of limb
4. Amarosis fugax (ex of TIA): Transient curtain like loss of sight, of ipsilateral eye d/t microemboli to the retina
What is the third most common cause of death in the USA?
Most common type?
-ischemic stroke (85%) of cases
-hemorrhagic is 15% of cases
-severe depression may cause dec in cognitive function that is difficult to distinguish clinically from AD but is responsive to antidepressant therapy
How might GB be treated?
1. Carefully monitor pulmonary function
-mechanical ventilation may be necessasry
2. Administer IV immunoglobulin if patient significant weakness. Plasmaphoresis can decrease disease
3. Do not administer steroids - harmful
Treatment of ISCHEMIC stroke =
1. Acute: airway protection, O2, IV fluids
-choose broad efficacy and safety treatment
-choose tPA (thrombolytics) if administered within 3 hrs of the onset of an acute ischemic stroke
-do not admin tPA if unknown time, >3 hr passed or patients has HTN, bleeding d/o, anticoagulated, or recent trauma/surgery
-must keep BP < =185/110 mm Hg
How do ischemic strokes appear on CT? How do hemorrhagic strokes appear?
Ischemic - appears dark and takes 24-48hr to se infarct
Hemorrhagic - appears white
what symptom has a better prognosis in patients dx with parkinsons?
List one surgical intervention
tremor with bradykinesia
DBS if pts do not respons to treatment like SINEMET or those dx <40
Presense of UMN and LMN signs, no sensory involvement. Starts at 50-70 years old with a prognosis of 80% within a 5 year period
ALS or Lou Gehrigs disease - only 10% familial but the remainder is sporatic
Thrombotic stroke (causing ischemia)?
Atherosclerotic lesions in lage arteries of the neck (CAD) or in medium sized arteries in the brain - especially MCA
Inflammatory demyelinating polyneuropathy that primarily affects motor nerves
Preceded by a virl/mycoplasmal infection of the upper GI = campylobacter jejuni, CMV, hepatits, HIV
Hodgkins, SLE, post surgically, HIV
Variants of MS
1. Clinically silent
2. Secondary progressive
3. Relapsing remitting
4. Primary progressive
1. Substantia nigra (loss of DAminergic containing herons-nerve cells that are located in the pigmented substantia and locus ceruleus of midbrain)
2. 50 YO
3. Pill-rolling tremor at rest, gets worse with stress, goes away with intention; bradykinesia, slowness of mvmt, cogwheel rigidity; poor postural reflexes, shuffling steps; expresionless faces
Key neuronal finding in brains of patints with parkinson's disease?
Lewy bodies (hyaline inclusion body)
Causes of hemorrhagic stroke
#1 HTN (sudden elevation in BP)
-50% of all hemorrhagic strokes
-ruptures vessels within the brain parenchyma
-chronic HTN causing degeneration of small arteries leading to microaneursm which can rupture easily
Two most important indcations for ordering an MRI to rule out an intracranial mass?
1. New onset of seizures
2. New and persistent/progressive headache
When is an ECG ordered in patients with stroke (+/-)?
to rule out acute MI as cause of embolic strokes by AFIBB or an MI
2 extraparenchymal brain tumors
1. Meningiomas (capable of resection)
a. high recurrance rate
b. benign but can cause significant compression of tissue
c. 40-50YO F>M; attach to dura; extrapyramidal
a. Benign tumors with no malignant potential
b. almost always unilateral if bilateral this is pathopneumonic for neurofibromatosis type II
Hemisection of the SC = diagnosis
Contralateral loss of pain
Ipsilateral loss of moto (LMN at site) UMN becomes slow
Ipsilateral loss of sensation
ex: Stab woulds
-movement at level would have HYPOreflext ipsilateral and hyper reflexia would be seen
What is the definitive test for identifying stenosis of the head and neck?
#1 senosis of head and neck
#2 aneurysms (looks at carotids, VB arteries, COW, ACA, MCA, PCA
What is the diagnosis of GB syndrome:
1. CSF analysis - elevated protein and cell count
2. Electrodiagnostic studies - decreased motor nerve conduction velocity
3. CLinical findings - ascening motor weakness with normal sensation preceeding infection of c. jejuni, CMV, HIV, hepatitis
3rd and 4th most common cause of death in the USA
1. Stroke = #3
-prevalence increases with age (10-15%) of individuls >65YO
-15-30% of individuals >80 YO
treatment used to:
Ldopa + carbidopa that ameliorates all symptoms of parkinson's disease
1. Dyskinesis (involuntary mvmt post 5-7 year use of Rx) - thus want to delay therapy for as late as possible
2. N/V, anorexia, HTN
Demyelination of CNS with multifocal zones of demyelination involving the white matter of the brain and SC without gray matter involvement
-common to women
-more likely at high latitudes and less at the equater
-involves pyramidal, cerebellar, MLF, optic nerve, posterior columns
Diagnosis of huntingtons and treatment:
What other disorder affects young patients with movement disorder?
MRI of head and caudate nuclei
DNA testing to confirm diagnosis - genetic counseling imp
No curative treatment - DA blockers help with psychosis and chorea with anxiolytic and SSRI.
WILSONS DISEASE PRESENTS WITH CHOREA!!! Cu2+ retainment
Stepwise deterioration of symptoms and neuro abnormalities are compatible with prior strokes and neuroimaging showing evidence of prior infarcts =
What is the most common cause of death after severe head injury?
What is normal ICP?
Increased ICP #1 cause of death
-normal 5-15 mmH20
-ICP > 20mmH2O is not good
-ICP is determined by volume of brain, volume of blood, volume of CSF
Diffuse axonal injury
Global damage to entire brain during impact
-may cause coma in patient
-CT scan doesnt show increased ICP but does show puntuate hemorrhages in the involved tracts
When should an LP not be performed in a patients with HIGH clinical suspicion of SAH?
IF CT scan reveals it, then look at the site of bleeding via cerebral angiogram
If papilledma is present, this indicates elevated ICP; thus this would lead to herniation and hemorrhaving if performed LP thus always perform opthalmologic exam
LE myasthenic syndrome
Associated with SMALL CELL LUNG CANCER
-autoAB against the presynaptic Ca2+ channels on NMJ.
-proximal muscle weakness and HYPOREFLEXIA
-symptoms improves with muscle stimulation
PEAK age (40-50)
-malignant and nonresectable glioma
-more indolent progression than fibrillary astrocytomas
-survival = 10-15 years post presentation
Childhood in onset, has ataxia, decreased sense of virbration, decreased proprioception, nystagms plus teleangiectasia
-increased cancer risk!
Dementia with Lewy Bodies
Both AD and parkinsons disease but progression may be more rapid than AD. Visual hallucinations predominate that include extrapyramidal features of functioning mental status
-treatment is similar to that for AD with neuroleptic selegiline can slow progression of dz.
Petit mal seizures are characterized by sudden _____ of mental activity. An episode is very short, but may occur _____ times per day. THere fore no associated signs of complex automatisms. This is confirmed by what type of test?
How might MS be diagnosed?
1. MRI - test of choice (most sensitive) and first diagnostic in the majority of cases abnormal in 90%.
2. CSF and LP analysis - although no labs are specific for MS (+) oligonal bands and IgG in 90%.
3. Evoked potentials suggest demyelination of ceratin areas by measuring the speed of nerve conduction within brain. Newly remyelinated nerves will conduct sensory impulses more slowly
How is delirium diagnosed?
1. Mental status examination, MMSE
2. Laboratory (chem panel, B12, thiamine)
3. LP (perform in any febrile deliruis patient unless contrindiation like cerebral edema - perform fundoscopy)
Epidural hematoma v. Subdural hematoma
EDH = rapid surgical decompression
SDH- surgical evaluation if acute; not always if chronic
Vertebrobasilar TIA symptoms:
1 Decrease perfusion to posterior fossa
2 Dizziness, DB vision, vertigo, numbness of ipsilateral face and contralateral limbs, dysarthria, horseness dysphasia, projetive vomiting, headaches, drop attacks.
Assessing the initial steps of COMA
1. ABCs - vitals
2. Stabilize cervial spine and asses the patients for signs of underlying causes of trauma
2. Assess level of consciousness using glascow coma scale
Rapid motor examination
Brainstem reflexes - round, symmetric, reactive (intact midbrain) whereas if assympetric (uncal herniation)
lab tests - CBC, Calcium, Bun/cr, ABG, CT/MRI
What is progressive supranuclear palsy
#1 = middle aged and elderly males (>50YO)
Degenerative condition involving the bstem, BG, cerebellum leading to bradykinesia, limb rigidity, cognitive decline, follows progressive course
NO resting tremor but postitive for opthalmplegia
Clinical features of a thrombotic stroke v. embolic stroke
Thrombotic = rapid/stepwise; awakens from sleep with neuro defects
embolic = rapid, mazimal defects initially
Site: pregang = central lesions/postgang = distal to SCG
features = anhydrosis, ptosis, MIOSIS
causes: idiopathic, pancoast tumor *UPPER LOBE); ICA dissection, brainstem stroke, neck trauma
Diagnosis of alzeimers dementia
-clinical diagnosis of exclusion
-CT scan or MRI showing diffuse cortical atrophy with enlargment of ventricles strengthens the Dx
ACE inhibitors have decreased levesl of ACh. Avoid anticholinergic treatment use DONEPOIL( > tacrine)
Clinical approach to dementia
1. Patient Hx
-ask patients and their family members about the nature of onset specfic eficits, physical symptoms, cormorbid conditions
-focus on through neurologic examinatino
-gait aalysis shed light on movement disorders with mass lesions and non pressure hydrocephalus.
3. LABS and imaging
-get CBC with DIFF to look at electrolyes, liver enzymes (asterixis abnormal gait hepatic encephalopathy) and diff to r/o infection. Get chem panel, thyroid, B12 for posterior column deficit folate, VDRL (for syphilis), HIV screen *viral load(, CT scan to look for SAH/ICH
What is the first imaging study that should be ordered if stroke is expected:
1. Ischemic stroke (85%)
2. Hemorrhagic stroke (15%)-identifies 95% of all SAH and all bleeds > 1cm; identifies abscess, tumor; subdural v. epidural hematoma
60 YO white male with worsening confusion and memory loss for three weeks. Muscle twitching and gait problems present but no fever headache or GU problems. Doesnt drink or smoke - normal pulse, pressure and temp. Poor grooming and disorientation noted. Nystagment and postivie extensor plantar response bilaterally on physical exam. Crit is 40%, WBC are 6k, platelets are 160k. Noncontract head CT = normal. EEG shows periodic sharp waves. Most likely dx:
Creutzfeldt Jakob syndrome - why?
1. Fatal neurodegenerative dz caused by PRIONS
2. Suspect in elderly patient with RAPIDLY progressive:
PERIODIC HIGH VOLTAGE COMPLEXES ON EEG.
3. These are the top diagnostic clues. EEG pattern of periodic bi or tiphagic sharp wave complexes is typical for CJ syndrome. Majority is sporatic and not genetically inherited (80%)
CLinical characteristics of huntingtons
change in behavior - irritable/change in personality antisoail behavior, depression, OCD
decreased mentation-progressive dementia < 50YO (~90%)
GAIT-unsteady and irregular
What happens if cerebral perfusion pressure is compromised due to increased ICP?
1. Loss of autoregulation thus asodilation is evident in cerebrum
2. Vasogenic edema occurs as fluid is lost into extravascular space
3. Systemic BP becoes sole determinant of cerebral blood flow
Mimics coma because patients paralyzed although spares muscles required for respiration, blinking, vertical eye movement
-patients fully aware of surround and can feel pain
"Locked in syndrome" - infarctions/hemorrhage of ventral pons
Hypertension, bradycardia, and respiratory irregularity
CUSHINGS TRIAD - due to elevated ICP causing a low HR which causes peripheral vasocontriction and hence the HYPERTENSION.
CNS tumors capable of resection:
1. Meningiomas/pituitary adenomas/schwannomas
The most common primary CNS tumor (GLIOMAs - astrocytomas and oligodendrocytes) CANNOT be resected
What are the predisposing factors of lacunar strokes?
#1 HTN in 80-90%
#2 DM Type II
#3 Narrowing of lumen due to thickened vessel walls
Intraparenchymal brain tumor related to immunosupression and encountered especially in recipients of transplants organs and in patients with AIDS
Primary lymphomas survival < 2 years
How is tourettes syndrome treated?
if symptoms affecting patiets QOL then patient education is important
1. Clonidine - CNS a2 agrenergic acting centrally to decrease CNS by negative feedback
2. Pimozide - antipsychotic drug like chlorpromazine (HIGH POTENCY - causes tarditive dyskinesia)
What is status epilepticus?
Prolonged sustained periods of unconsciousness with persistant convulsive activity.
-Medical emergency = 20%
D/T poor anticonvulsive activity, alcohol withdrawal and intracranial infection/neoplasms
Treatment: ABC, IV DIAZEPAM, IV PHENYTOIN, 50mg DEX. If resistance - phenobarbitol
Symptomatic therapy for MS:
1. BACLOFEN for muscle spasms
2. CARBAMAZEPINE/GABAPENTIN for neuropathic pain
Prognosis of GB syndrome
signs of recovery within 1-3 weeks after onset. If >6weeks, chronic replapsing course more likely and prognosis is less favorable
5% die from respiratory failure, pnemonia, arhythmias
Treatment of acutely altered mental status
-Intact cerebrl cortex
Treatment underlying cause; then haloperidol
Arousal dependent upon = agitation/psychotic
Cognition dependent upon = delirium behavior
Sign of basilar skull fracture
1. Raccoon eyes - periorbital ecchymoses
2. Battle signs - post auricular eccymosis
4. CSF rhinorrhea/otorrhea
When should ASA be administered to stroke patients with an acute stroke
within 24 hrs onset
-no admin if thrombotic therapy, inc risk of ICH
How is ICH diagnosed?
CT Scan is 95% ICH
coagulation panel and platelets to evaluate bleeding diathesis
EMG and NERVE conduction studies?
EMG measures contractile properties of skeletal muscle
-LMN lesions fibrillations/fasciulations at rest
-myopathy - no electrical activity at rest, decreased amplitude with muscle contraction
NC studies = demyelination will slow nerve conduction velocity (MS/GB); repetitive stimulation causes fatigue (MG)
Treatment of strokes is ______. Once stroke has occurred, goal is to?
Prophylactic - prevent future strokes
1. control risk factors (HTN/HM/SMOKING/HYPERCHOL/OBESITY)
3. Surgery (carotid endarterectomy in patients with CAS)
Major complication in patients POST SAH?
#1 Vasospasm in 50% of patients
#2 rereupture of same vessel (30%)
#3 hydrocephalus; elevated ICP, use mannitol
#4 seizures (blood irritatnt to it)
#5 SIADH = hypovolemia with hyponatremia
Cancer that metastasizes to meninges via the bloodstream
-may cause focal deficits depends on the involved locations
-CSF = malignant cells, inc protein, inc lymph, dec glucose
Rx: intrathecal chemotherapy
Interferon therapy for MS:
1. Recombinant interferon B-1a, recombinant interferon B-1b, GLATIRAMER acetate have shwon a reduction in relapse rates 37%, 33%, 29%
2. Interferons can cause flulike symptoms
3. Interferon therapy started early in course of disease before disability becomes irreversible
Pupillary findings of patients with ICH?
PINPOINT if in PONS
POORLY reactive if THALAMUS
DILATED if PUTAMEN
Causes of seizures
1. What are the 4 M's and 4 I's?
Metabolic - decreased Na2+, H2O intoxication, hypo.hyperglycemia, thyroid storm (thyrotoxicosis where elevated T3/T4 must be treated with either PTU or radioactive iodide therapy).
Mass lesions - brain mets, primary tumors
Missing drugs - noncomplicance/acute withdrawal from alcohol, benzo and barbs
Miscellaneous - pseudoseizure
Intoxication - cocaine/lithium/lidocaine/theophylline
Infection - septic shock
Ischemia - stroke/TIA
Increased ICP - d/t trauma
Partial seizures: Is consciousness impaired?
IF N - then simple partial and treat with phenytoin and carbamazepine
IF Y - then complex partial and treat with phenytoin and carbamazepine
NPH is suspected in any patient (normal pressure hydrocephalus) with
DEMENTIA, INCONTINENCE, POOR GAIT
CT will show ventricular enlargement
What are the secondary insults associated with head trauma?
1. hypotension (<90/60)
2. hypoxia (<60mmHg)
3. Hypercapnia (>50 mmHg)
4. Increased ICP
5. Intracranial mass effect - epidural hematoma, SD hematoma
6. anemia = blood loss in trauma
COUP v. CONTRCOUP injury
coup = injury at the site of injury
contracoup = injury at the site oppositeto point of impact
1. Most common primary CNS neoplasm (80%)
2. Exclusive in the cerebral hemisphere
-tumors infiltrate brain with indistinct boundary
-tend to spread along WM tracts and may cross the corpus callosum into opposite hemisphere
-glioblastoma multiforme is worst prognosis
What anticholinergics are used to treat the resting tremers associated with parkinsons
Which arteries are responsible for lacunar strokes?
Small branches off of the MCA that make up the COW and the basilar and verteral arteries. Once occluded, small infarcts result. These heal and form "lacunes"
Most common site of SAH?
Junction of the A communicating artery with ACA
Junction of the P communicating artery with ICA
Bifurcation of the MCA
Most important regulator of cerebral vessel dilation/contriction:
Increased PaCO2 because it results in vasodilation and subsequently causes cerebral vasodilation
Which is most sensitive for ischemic strokes (MRI or CT)?
MRI > CT because identifies all infarcts early in comp to CT however not preferred in ED because taes long time to perform and not good in unstable patients
COMA - general characteristics
Decreased level of consciousness to extent that patients unresponsive to stimuli:
1. Structural brain resins causing come = bilateral
2. Global brain dysfunction
3. Conversion d/o and maligering difficult to diagnose from COMA
In symptomatic pateints, what is the benefit of patients to endarterectomy of carotid?
Recommendation if asymptomatic?
> 70% if carotid stenosis
If asymptomatic, dec atherosclerotic risk factors and use of ASA are recommended, prevents embolic strokes with ASA and prevents lacunar strokes with HTN.
Hereditary causes of muscle weakness?
1. Mitochondial d/o - maternal inheritance (RED RAGGED FIBERS)
2. Glycogen storage d/o like mcardles dz or glycogenphosphorylase deficiency
3. DMD, Beckers MD, myotonic MD (CTG trinucleotide repeat)
Treatment for head trauma
2. Lower ICP
-reverse trendelenburg position, if spine cleared, elevate head of bed.
-intubate (+) hyperventilation is effective in decreased ICP. Hyperventilation causes decreased PaCO2 and decreases vasodilation
-maintain PaCO2 level at 30-35
Mortality ratio is HIGH (40-50%) @ 30 days
-saccular aneurysms occur at bifurcations of arteries at the COW
Normal cerebral perfusion pressure?
-how does pupil size indicate increased ICP?
In the ICU, what is the goal of managment?
-bilateral fixed and dilated pupils suggest diffusely increased ICP
-keep the mean arterial pressure > 80 and ICP <20. Ensures a cerebral perfusion pressure of at least 60 mmHg
Medical emergency that can occur in patients with MG?
MG crisis is a 15% likelihood where the disaphragm and intercostals fatigue in respiratory failure
Treatment : PEEP with mechanical ventilation
Ataxia: gait instability, loss of balance, impaired limb coordination
1. Alcohol intox (anterior vermis of cerebellum)
2. Vitamin B12/thiamin B deficiency
3. Cerebellar infarct
4. Demyelinating disease (MS, GB, AIDS, tertiary syphilis)
5. Inherited causes
-freidrichs ataxia (frataxin gene - GAA trinucleotide repeat)
Causes of an acute confusional state (delirium)
P - postoperative state (compounded with treatment for pain)
D - dehydration and malnutrition
I - infection (sepsis, meningitis, encephalitis, UTI
M - meds/drug intoxs (TCAs, cortisteroidsm anticholinergic)
M - metals (heavy metal exposure)
W - withdrawal states form OH + benzos
I - inflammatory fevel
T - trauma burns
How are CNS neoplasms diagnosed?
MRI without GADOLINUM = #1
BRAIN BIOPSY = only definitive way to diagnose a brain tumor and determine the specific type
Ataxia, nystagmus, dec vibration sense and proprioception (GAA)
-autosomal recessive inheritance by youdn adulthood
Average time of death from onset of A.D.
1. Stage (early) = mild forgetflness; imparied ability to learn new; decreased erformance at work, decreased concentration; change in humor/change in personality
2. Intermediate = memory decline; denial may be present; visual disturbance of condition; getting lost in familiar place and difficult following direction
3. Later assistance needed with ADL; paranoid delusions and hallucination
4. Advanced disease - complete debilition and dependent of others (bowel and bladder)
What is VHL disease
-cavernosis hemagiomas of brain and brain stem
-cysts in organs
-increases the risk of RCC
Diagnosis of patient with primary seizure
1. EEG with neuroconsult
2. Anticonvulsant agent
3. Risk of recurrence if normal EEG
- Low 15% year 1
4. If abnormal - then risk is 41% in first year
General seizures: Are they convulsive?
If Y - TONIC CLONIC or MYOCLONIC then treat with phenytoin and carbamazepine
If N - then absense and treat with ethosuxamide and VALPROIC acid
What are the risk factors regarding ischemic stroke (cerebral infarction)?
4. Atrial fib
6. Family hx
7. TIA history
8. Carotid bruits
IF MCA is occluded, what symptoms would be expected?
Contralateral hemisensory hemiparesis of body
Asphasia if dominant hemisphere (BROCAS)
Apraxia, contralateral body neglect and confusion if nondominant hemi involved
Types of dementia
1. AD -- 66% of all cases of dementia
2. Vascular dementia = multi-infarct dementia; BINSWANGER DISEASE
3. Space occurping lesions = brain tumor, chronic SDH
4. NPH - triad of dementia, gait distubrance, incontinence (wacky, wobbly, wet)
5. Dementia with lewy body
6. Pick's disease = identical to AD but had pick bodies
Obsessive compulsive disorder
onset < 21YO
autosomal dominant inheritance pattern
not all have "TICS"
not all experience involuntary swearing
Most important risk factor for dementia
preservation of consciousness with deterioration of intellectual function
Treatment of coma if:
Identify and treat herniation
Thiamine b4 glucose
lower ICP if ICH
Treatment used to decrease ICP in patients with increased ICH/SAH risk?
Treatment used to decrease BP in patients with iCH/SAH?
Decrease ICP = mannitol (osmotic agent) - only if inc ICP
Decrease BP = nitroprusside if BPsys>160-180 or BPdys>105
Hemorrhagic stroke (ICH/SAH) clinical findings
1. Abrupt in onset of focal neuro deficit that worsens (30-90 min)
2. Altered level of consciousness, stupor, coma
3. Headache, vomiting
4. Signs of increased ICP
Origins of embolic stroke
1. Heart (most common) - embolism of mural thrombus, atrial fibb
2. Internal carotid artery (ICA)
4. Paradoxical = embolit that arises from blood clots in perhpheral vein that passes from RH to LH by ASD, VSD, PFO or pulmonary AV fistula and GOES to brain
Treatment of parkinson's disease
#1 = SINEMET!
-no cure for parkinsons
-delay disease progression
-use symptomatic treatment
#1 = carbidopa-levodopa (SINEMET)
What are the classes of ischemic strokes (85%)
1. Transient ischemic attack (TIA)
2. Reversible ischemic neurologic deficit is the same as TIA except the duration of symptoms lasts longer than 24 hours but resolves in < 2 weeks
3. Evolving stroke (worsening)
4. Completed stroke (maximal deficit has occured)
What is the cerebral perfusion pressure?
Mean arterial pressure minus ICP
CPP = MAP - ICP want to be <60
How is MG diagnosed?
1. AChR antibody test is the test of choice
-20% AB (-)
2. EMG decreases response to stimulation of motor nerves
3. CT scan of thorax to r/o thymoma (10-15% of MG patiets; thymus histologically abnormal in 75% of patients)
4. Edrophonium (tensilon test) - achE treatment causes elevation in smptoms
Most common brain mets:
(prostate CA, non-melanoma and esophageal CA will never met)
Treatment of ICH/SAH
1. ICU admission
2. ABC airway management
3. BP reduction - inc BP and inc ICP and can INC bleeding.
4. Hypotension can also lower blood flow so must slowly decrease BP
How is MS treated?
1. Short term therapy
2. Longterm therapy
ST: (1) high dose IV corticosteroids can shorten an acute attack; these do not change course of MS. (2) Theraprutic plasma exchange (TPE) for steroid refractory acute demyelinating attacks at 42%
-nonspecific immunosuppressive therapy like cyclophosphamide should be reserved for rapidly progressive disease
4 causes of ischemic stroke:
1. Embolic stroke
2. Thrombic stroke
3. Lacunar stroke
4. Nonvascular causes (decreases CO, anoxia - global ischemia and infarct)
First line agent for AD
Donepozil (newer AChEi than tacrine)
-advantages over tacrine include
-1 per day dosing with better and decreased side effects
-tacrine 4x/day another AChEi
-may slow dz progression
What is the diagnosis if patient history is unclear of seizure d/o
1. CBC, electrolytes, blood glucose, LFT, RTF and serum Ca2+ and UA
2. CTs of the head
3. MRI with then without GAD
4. LP and cultures
Poliovirus = myelitis
Ant horn cells and MN of the SC and BStem
-LMN involvment (HYPOREFLEXIA)
-asymmetric muscle weakness
-absent DTR, flaccid m and normal sensation
-+/- bulbar involvement (IX/X)
Abnormal pupillary light reflex- structural intracranial lesions like ____ or _____; drugs including (____,______) and anoxia can result in change.
morphine; atropine-like agents
Risk factors for CVA in young patients?
Sickle cell anemia
Hypercoaguability states (protein C/S deficiency, increases clotting)
Antiphospholipid AB syndrome
vasocontrictive drug use (cocaine, amphetamines-methamphedamine, ephedrine)
polycythemia vera (myeloproliferative dz)
What is binswangers disease?
vascular dementia with insidious onset due to diffuse subcortical white matter degeneration, mst commonly seen in patients with londstaning HTN and atherosclerosis
How is SAH diagnosed?
1. CT scan noncontrast - identifies hemorrhagic strokes
2. Lumbar puncture if CT scan unremarkable or (-) with high suspician. LP is diagnostic; if (+) for blood, then hallmark for SAH. If xanthochromia (yellow) in CSF - also diagnostic
3. Cerebral angiogram once diagnosed to detect site of bleeding of the SAH
What is "subclavian steal" syndrome?
Treatment is by surgical bypass
1. Stenosis of subclavian before the vertebral artery
-dec cerebral blood flow (stolen) from basilar system
-causes symptoms of vertebral basiliar arterial insufficiency
-claudication of upper arm BP in Larm<right arm, decrease pulses
Medical emergency involved with GB?
Rapidly progressive respiratory failure can occur within horus
-if suspect GB syndrome
-immediately admit patient to the hospital for monitoring
Cranial nerves affected by schwannoma
1. Start @ CN VIII, CNV and CNVII
-hearing loss is usually the first symptoms
-other symptoms may include tinnitis, loss of balance, nystagmus, and motor and sensory deficits involving the face
Ecclampsia v. pre-eclampsia and the treatment?
Ecclampsia = HTN in pregnancy with seizures
Treatment = MAGNESIUM infusion