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314 Cards in this Set

  • Front
  • Back

Nonspecific signs/symptoms of increased ICP

Headaches (on awakening, dependent on head position, progressive)

AM nausea/vomiting

Papilledema (swollen optic disc)

Seizures

Diagnostic test for CNS neoplasm
MRI w/ and w/o gadolinium

Brain biopsy is only definitive way to dx

Ways to control elevated ICP

Steroids
Mannitol
Hypernatremia


Hyperventilation


Differential dx of ring-enhancing brain lesion

Mets
GBM
Lymphoma



Brain abscess


Toxoplasmosis


Brain tumor in severely immunocompromised patient? (e.g. transplant recipient, AIDS patient)

Primary lymphoma!

PATHOGNOMONIC for neurofibromatosis II

BILATERAL schwannomas

(otherwise is unilateral)

CSF reveals malignant cells, elevated protein and lymphocytes, decreased glucose

+ focal neurologic deficits

Meningeal carcinomatosis (cancer metastasizes to meninges via blood)

Tx w/ intrathecal chemo

Hearing loss, tinnitis, loss of balance, nystagmus, motor/sensory deficits of face



imaging shows mass

Schwannoma (cerebellopontine angle)

ICP is determined by

Volume of brain
Volume of blood
Volume of CSF

Secondary insults to brain s/p trauma

Hypotension
Hypoxia
Hypercapnia (causes vasodilation --> increased ICP)
Increased ICP
Intracranial mass effect (subdural or epidural hematoma)
Anemia

Normal ICP
5-15 mm H2O

ICP>20 is worrisome
Effects of increased ICP

increase in cerebral perfusion pressure ---> hypoperfusion --> loss of autoregulation --> cerebral vasodilation --> vasogenic edema --> increased ICP

Systemic BP becomes sole determinant of cerebral blood flow

Transtentorial herniation may result

Bilateral fixed and dilated pupils suggest
Increased ICP

What is target MAP in the context of ICP

MAP > 80 mmHg

(recall normal ICP <20 mm Hg)

So 80 mmHg keeps cerebral perfusion pressure >50 mm Hg (normal)

Sgns of basilar skull fracture

Raccoon eyes - periorbital ecchymoses

Battle's sign - postauricular ecchymoses

Hemotympanum

CSF rhinorrhea/otorrhea

Cushing triad

hypertension


bradycardia


respiratory irregularity

Risk factors for subdural hematoma
Brain atrophy (EtOH, elderly)

Pts undergoing anticoagulation
Signs of subdural hematoma
Headache
Decreased level of consciousness (mass effect)
Cortical dysfx
Mechanism of myasthenia gravis

Autoantibodies against nicotinic acetylcholine receptors of NM junction -->



reduced postsynaptic response to acetylcholine

Proximal, asymmetric mm weakness with preservation of sensation and reflexes, worse with exercise
Myasthenia gravis

Dx test for myasthenia gravis

Acetylcholine receptor antibody test

EMG shows decremental response to repetitive stimulation of motor neurons

Edrophonium (Tensilon) test - shows sx IMPROVEMENT w/ anticholinesterase meds

Myasthenia gravis associated with?
Thymoma

Tx for myasthenia gravis

AChE inhibitors
Thymectomy
Corticosteroids; azathioprine, cyclosporine
Plasmapheresis (removes Ab to acetylcholine receptors)
IV immunoglobulin therapy for acute exacerbation

Myasthenic crisis

Diaphragm and intercostal fatigue



--> acute respiratory failure

Low threshold for intubation

Lambert-Eaton is associated with?

SMALL cell lung cancer

Lambert Eaton mechanism

Autoantibodies against presynaptic calcium channels

Proximal muscle weakness, hyporeflexia, symptoms improved with repeated use

Lambert Eaton syndrome...



"ya just gotta warm up for a bit" :-(

Inheritance of Duchenne MD?

X linked recessive

Mutation of dystrophin gene --> mm cell structure

Progressive, symmetric mm weakness starting in childhood with pelvic girdle affected

Duchenne MD

Signs/symptoms of Duchenne?

Gower's manuever (hands to get up)

Enlarged calf muscles - pseudohypertrophy as fat replaces mm

Wheelchair confinement, resp failure, death in 30s


Causes of syringomyelia

Cranial base malformation (Arnold-Chiari)

Intramedullary tumors

Traumatic necrosis of spinal cord

Bilateral loss of pain and temp sensation in caplike distribution

Muscle atrophy of hands and thoracic scoliosis

Preservation of touch

Syringomyelia (collection of fluid in spinal cord --> central cavitation of cervical cord)

Tx for syringomyelia
Syringosubarachnoid shunt (surgical)
Diagnosis of Duchenne's MD
Serum creatine phosphokinase

DNA testing

Contralateral loss of pain and temperature

Ipsilateral hemiparesis

Ipsilateral loss of position/vibration

Brown-Sequard (spinal cord hemisection)

Tracts affected in syringomyelia

Lateral spinothalamic tracts

Tracts affected in Brown-Sequard

Spinothalamic
Corticospinal
Dorsal columns

Lower extremity weakness or plegia
Back pain
Sensory deficits below level of lesion
Sphincter disturbance (urinary retention)

Transverse myelitis (often after viral infection)

Causes of Horner's syndrome (ipsilateral ptosis, miosis, anhydrosis)

Idiopathic (most common)
Pancoast tumor (superior sulcus)
Internal carotid dissection
Brainstem stroke
Cervical spine injury
Asymmetric muscle weakness, absent DTR, atrophic/flaccid muscles, normal sensation
Poliomyelitis
Aphasia is more common when which hemisphere is involved?
Left
Visual spatial deficits are more common when what hemisphere is involved?
Right
Crossed hemiplegia (ipsilateral face and contralateral body) can be localized where?
Brainstem (corticospinal tract, dorsal columns, spinothalamic tract cross but CN do not)
Spinal cord injury - how to localize?

Level of lesion corresponds to sensory level (pinprick felt above level but not below)

Localize this lesion:

Weakness, atrophy, sensory deficits in dermatomal pattern, +/- fasciculations and diminished DTRs

Roots (radiculopathy) or
Peripheral nerves

Menier disease

Vertigo
Tinnitus
Hearing loss

Differences between central and peripheral vertigo:

Central: gradual onset, mild intensity, associated neurologic findings, mild nystagmus (multidirectional and vertical)

Peripheral: Sudden onset, severe intensity, rapidly refractory, unilateral vertical nystagmus
Tilt-table testing used for
Dx neurocardiogenic syncope (if episodes are recurrent, unexplained, no structural heart disease)

Causes of epilepsy

4 M's:
Metabolic (Hyponatremia, uremia)
Mass lesions
Missing drugs (withdrawal, noncompliance)
Misc (pseudoseizures, eclampsia, hypertension)

4 I's:
Intoxications
Infections
Ischemia
Increased ICP

Features of simple partial seizures
Consciousness intact
May involve transient unilateral clonic-tonic movements
Features of complex partial seizures
Consciousness impaired
Postictal confusion
Automatisms
Olfactory/gustatory hallucinations

Impaired consciousness, no loss of postural tone or continence, no postictal confusion

Absence seizures

Labs to order in unfamiliar seizing pt

Serum glu, Ca, sodium, BUN

Drugs of choice for generalized tonic-clonic and partial seizures

Phenytoin, carbamazepine

Phenobarbital, valproate, primidone

Drugs of choice for petit mal (absence) seizures

Ethosuximide
Valproate

ALS affects what areas?

UMN/LMN:
Anterior horn cells
Corticospinal tracts
Corticobulbar tracts

Progressive muscle weakness first noted in legs/arms, no associated pain, muscle atrophy

Exam findings: Muscle cramps and spasticity, fasciculations, impaired speech/swallowing, wt loss/fatigue, DOE/orthopnea

ALS

Normal and unaffected even in late stages of ALS

Bowel/bladder control
Sensation
Cognitive fx
EOM

Seen on EMG in myopathy

No electrical activity at rest, but amplitude DECREASES with muscle conraction

EMG findings for MS, GBS

Decreased nerve conduction velocity

Riluzole MOA?

Glutamate-blocking agent used to delay death in ALS (3-5 mo)

Receptive aphasia (fluent)



Impaired comprehension of written or spoken language

Wernicke

Broca aphasia

Expressive aphasia (nonfluent)



Speech is slow, requires effort

Where is lesion?

Aphasia + fluent speech

Posterior to central sulcus (W's)

Where is lesion?

Aphasia + nonfluent speech

Anterior to central sulcus (B's)

Brief, frequent attacks of severe lancinating facial pain

No motor or sensory paralysis

THIS IS TRIGEMINAL NEURALGIA

Jaw, lips, gums, maxillary

Drug of choice for trigeminal neuralgia

Carbamazepine
Baclofen
Phenytoin

Spont resolution in 85% cases

Localize lesion:

Weakness is observed only in the lower face and tongue

EOM, upper facial, pharyngeal, and jaw muscles are spared

Corticobulbar (unilateral)

Localize lesion:

dysarthria, dysphagia, dysphonia



emotional lability



bilateral facial weakness and a brisk jaw jerk.

Pseudobulbar palsy (bilateral corticobulbar tracts)

Localize lesion:

Homonymous visual field defect

Cortical/subcortical

Difference in presentation, LMN vs. myopathy

LMN = distal weakness

Myopathy = proximal weakness

Symmetric weakness of pelvic or shoulder girdle muscles
Myopathy

weakness of the lower two-thirds of the face with preservation of the upper third suggests?

UMN lesion

failure of vertical gaze
dysarthria
dysphagia
extrapyramidal rigidity
gait ataxia
dementia

Progressive supranuclear palsy

In PSP what lobe abnls predominate

Frontal lobe

Pathology of PSP

Atrophy of dorsal midbrain, GP, STN

Argyrophilic round intraneuronal inclusions

Pick bodies (frontotemporal dementia)

Rapidly progressive dementia with pyramidal signs, myoclonus, cerebellar or extrapyramidal signs

Periodic sharp waves in EEG

CJD

Presence of protein 14-3-3 in CSF

CJD
HIV associated dementia shows what on MRI

Cortical, subcortical atrophy

Neuropathy (paresthesias, sensory ataxia, visual loss, orthostatic hypotension), memory loss

Anemia, sore tongue

Dementia due to Vit B12 deficiency

Red desaturation can be seen early in what problems?

Early optic nerve problems



(e.g. optic neuritis/Devic dx)

Pupillary constrictor (miosis) is innervated by?

PARA-sympathetic nerve fibers

(of CN III)

Pupillary dilator (mydriasis) is innervated by?

Cervical sYmpathetic system

Sympathetic system pathway

First order: Ipsilateral posterolateral hypothalamus to intermediolateral cell columns (C8-T1)

Second order: synapse in suprior cervical ganglion (pre-ganglionic)

Third order: Travel along internal carotid artery into cavernous sinus --> pupillodilator muscle

First sign of CN III compression = Dilated pupil

Why?



Parasympathetic fibers on outside, motor fibers on inside

Ilsilateral optic disc atrophy due to compression by space-occupying lesion in frontal lobe and papilledema in contralateral optic disc due to increased ICP

Foster-Kennedy syndrome

Painful visual loss, worse w. exercise/hot baths


Retro-orbital pain



Loss of color discrimination


Central scotoma

Optic neuritis (IV methyprednisolone)

Asymptomatic; enlarged blind spot with initially normal visual acuity



glistening hyaline bodies in fundi

Drusen or pseudopapilledema

Morning headache, ataxia, transient visual obscuration, central acuity spared, no color loss, enlarged blind spot, bilateral disc hyperemia

Increased intracranial pressure

Sudden painless visual loss in pt >50 yo, associated with HTN, DM

Ischemia

Destruction of abducens nucleus in brainstem

Complete ipsilateral conjugate gaze palsy (connection to contralateral third nerve through MLF)

Voluntary saccades arise from?

Frontal eye field and superior colliculus contralateral to direction of gaze

Child between 2-7 yo following viral infection presents with acute onset limb, gait ataxia, dysarthria

Postinfectious cerebellitis

Acute/subacute onset of truncal, gait, limb ataxia


Dysarthria



Ocular dysmetria or nystagmus


(no opthalmoplegia)

Paraneoplastic cerebellar degeneration



(GYN or SMALL cell lung cancer --> MRI is often nl, assoc. with autoantibodies)

Progressive ataxia in childhood with loss of reflexes



Spasticity and extensor plantar responses



Impaired vibration and position sense

Friedrich's ataxia (AR)

(affects Arms > Legs)

Brief episodes of ataxia, vertigo, nausea, vomiting caused by mutations of?

Inherited episodic ataxia (EA-1): voltage gated K channel

EA-2: voltage gated calcium channel --> longer attacks w/ interictal nystagmus and progressive irreversible ataxia later in disease

Insidious onset of progressive gait impairment, dysarthria in early adult life with mild-mod cognitive decline later on

Spinocerebellar ataxias (CAG expansion)

Autosomal dominant

Triad of ATAXIA, AREFLEXIA, OPHTHALMOPLEGIA in postinfectious autoimmune process

Miller Fisher syndrome --> ataxia due to proprioceptive loss



(not cerebellar dysfx, no nystagmus)

Cerebellar vermal lesions assoc with?

Truncal and gait ataxia

Cerebellar hemispheric lesions assoc with?

Ipsilateral limb ataxia

Affected leg is stiff and does not flex at hip, knee, or ankle

Leg is circumducted (scrape floor with toes)

Arm held in flexion and adduction, does not swing freely

Hemiparetic gait
Spastic gait with increased adductor tone and legs tend to cross during walking
Scissoring gait (paraparetic/bilateral hemiparesis)

headache in a young obese woman, worse in AM, worse when recumbent, accompanied by pulsatile tinnitus and blurring precipitated by valsalva maneuver

Idiopathic intracranial HTN

(pseudotumor cerebri --> impaired resorption of CSF)

Broca's aphasia: where is the lesion

Posterior part of inferior frontal gyrus in dominant (left) hemisphere

MCA territory

Broca's aphasia - deficits?
Impaired fluency
Impaired repetition
Normal comprehension
Huntington disease triad

Chorea


behavioral change/personality disorder


dementia

Diagnosis of Huntington

Family h/x
Clinical signs
>40 CAG repeats


CAUDATE ATROPHY

Pathology in Huntington's disease

Destruction of caudate and putamen (striatal and nigral GABAergic neurons)

Loss of neurons in cerebral cortex

Personality change, speech disturbance, inattentiveness, extrapyramidal signs, progressive dementia

Pick dementia

Rapidly progressive dementia with pyramidal signs, myoclonus, cerebellar or extrapyramidal signs, periodic sharp waves in EEG

CJD
CSF findings on CJD

Normal but presence of 14-3-3- protein (sensitive and specific)

Progressive dementia with neuropathy (paresthesias, sensory ataxia, visual loss, orthostatic hypotension), memory loss, anemia, sore tongue
Vit B12 deficiency
50% theta slow wave activity
Stage 1 sleep
Sleep spindles and K complexes
Stage 2 sleep
20-50% delta wave activity
Stage 3 sleep
>50% delta activity
Stage 4 (slow wave sleep)
A typical night of sleep
4-6 cycles, lasting 90 min each (stage 1 absent after first sleep cycle)
REM sleep with age

Decreases from 20-25% per night to 15-20% per night

REM sleep over course of night
REM accounts for greater proportion of sleep as night progresses
Drug that suppress stages 3 and 4 of sleep
Benzos
Drug that suppress REM sleep
Antidepressants and alcohol
Restless leg syndrome tx

Dopaminergic agents e.g. ropinirole, pramipexole

Anticonvulsants, opioids, benzos

What can happen to restless leg syndrome symptoms with dopaminergic tx?

Augmentation: symptoms occur earlier in the day

Narcolepsy components:

Excessive daytime sleepiness with narcolepsy, cataplexy, sleep paralysis, hypnagogic hallucinations

Pathophysiology of narcolepsy/cataplexy

Loss of hypocretin secreting neurons in hypothalamus

Dx of narcolepsy

CSF with hypocretin <110

Multiple sleep latency test

Cataplexy tx with?

TCAs clomipramine
Sodium oxabate
SSRIs, atypical antidepressants

Foster Kennedy Syndrome

Ipsilateral anosmia
Ipsilateral optic atrophy
Contralateral papilledema
Tx for chorea

Neuroleptics



e.g. haloperidol (anti-dopaminergic)

Childhood onset of Parkinsonism with bradykinesia and rigidity, dystonia, myoclonus, seizures

Westphal variant of Huntington diease

Dystonia

Sustained muscle contractions

Etiology for dystonia

Abnl low basal ganglia output structures

Cardinal features of Parkinson disease

Tremor at rest
Rigidity
Hypokinesia
Postural instability

Sensory involvement in Parkinson disease

Loss of sense of smell
Pain
Speech disturbance
Autonomic disturbance
Depression

Early onset dementia
Delusions and hallucinations
Fluctuations in consciousness
Myoclonus

Dementia with Lewy bodies

Supranuclear downgaze palsy and square wave jerks, upright posture, frequent falls

Progressive supranuclear palsy
Levodopa is broken down peripherally by?

Gut: aromatic amino acid decarboxylase (carbidopa inhibits)

Peripheral: COMT (entacapone, tolcapone inhibit)

Dopaminergic agent requiring conversion

Levodopa

Dopamine agonists e.g. pramipexole, ropinirole, bromocriptine act directly

Symptomatic tx in Parkinson's
Selegiline, rasagiline (MAO-B inhibitors)

Anticholinergics (trihexyphenidyl, diphenhydramine)

Amantadine (NMDA receptor antagonist)
Reduces cardinal features of parkinson's + drug-induced dyskinesias
Amantadine
Agents causing drug-induced Parkinsonism

Antipsychotics, antiemetics e.g. metoclopramide, prochlorperazine

Arching spasms of back, neck, stereotypical repetitive movements of tongue, jaw
Tardive dyskinesia
Risk factors for tardive dyskinesia
Advanced age
female gender
Coexistent brain damage
Tx for tardive dyskinesia
Increased doses of dopamine receptor blockers
Dopamine depletion (reserpine, tetrabenazine)
Hemisection of spinal cord produces?

Ipsilateral hemiparesis
Ipsilateral loss of fine touch and vibration sensation
Contralateral loss of pain and temperature below level of lesion

Anterior cord syndrome
Bilateral spinothalamic tract (loss of pain and temp)

Bilateral weakness (corticospinal tract)

Preservation of dorsal columns (fine touch, proprioception, vibration)
Central cord syndrome

Bilateral loss of pain and temp sensation, weakness of upper extremities (motor fibers to legs are lateral and thus spared).

Preservation of fine touch

Complete loss of motor and sensory function of upper and lower extremities + resp difficulty

Preserved reflexes mediated by CNs

Loss of autonomic function
High cervical spine cord injury
Air in acute EDH suggests?
Fracture of sinuses or mastoid air cells
Initial evaluation for suspected epidural hematoma
Noncontrast CT (homogenous density, high density, convex)
Characteristics of delirium

Cognitive impairment
Impaired attention
Fluctuating course

When is a head CT indicated for concussion?

LOC!!!!
Persistent headache
Emesis
Age >60 years
Drugs/EtOH
Persistent anterograde amnesia
Soft tissue or bony injury above clavicles
Seizure

Post-concussion syndrome
Irritability
Depression
Insomnia
Subjective intellectual dysfx
Anxiety
Fatigue

Return to Play - Grade 2 concussion

Removal from game -->return after 1 wk

Return to play - Grade 3 concussion (+LOC)

ER evaluation --> 1 week if exam nl

2 wks if LOC prolonged

Atherosclerosis affects which brain vessels?

Carotid bifurcation



Major intracranial vessels



Vertebral artery

Lacunar strokes associated with?

(small vessels)

Occlusive disease of penetrating arteries in brain

Assoc with HTN, DM

Tx of ischemic stroke
tPA (within 3 hrs)
Antiplatelet drugs (aspirin, clopidogrel, dipyridamide)
Complications of subarachnoid hemorrhage (delayed)
Vasospasm
Acute hydrocepalus

Imaging type for detecting hyperacute ischemic injury caused by vasospasm

Diffusion-weighted MRI

Carotid dissection predisposing factors

Fibromuscular dysplasia



Ehlers-Danlos
Marfan

Carotid dissection often assoc with?
Ipsilateral ptosis + miosis (Horner's)

Headache

Recent head/neck injury
Drugs for absence seizures

valproate



lamotrigine



ethosuximide

Automatisms - e.g. bilateral cycling, swimming movements



+/- post-ictal period, appear to be awake but do not respond normally to environment

Complex partial seizure

Seizure with preservation of consciousness (focal), + aura

Simple partial seizure

Momentary lapses in awareness without automatisms (can have blinking or lip smacking), no aura
Absence seizures

Aura involving abd sensation, fear, unreality, deja-vu

Temporal lobe seizure

Aura involving electrical sensation, tingling, numbness

Parietal lobe
Aura involving visual changes
Occipital lobe seizures
Acute LOC without nausea, sweating, abd discomfort, regained consciousness quickly without confusion

Cardiogenic syncope

Causes of syncope

Cardiogenic
Exertional (cardiac outflow obstruction)
Vasovagal
Epileptic seizure
Orthostatic

Nonepileptic syncope w/u includes

EKG


Holter monitor


EEG


Orthostatics

Unilateral, periorbital/cheek pain
Onset in teens, 20s
Precipitated by fatigue/stress, diet, sunlight, hormones

Dull ache --> stabbing pain
+ Assoc symptoms

Migraine

Precipitous headache in temporal region, unilateral, pain + stiffness in neck, shoulders, back, pelvic girdle
Temporal arteritis (assoc with polymyalgia rheumatica)
Post-spinal headache characteristics
Better when lying down, can be assoc w/ n/v
Sudden pulsatile head pain involving entire head, before or after orgasm
Postcoital cephalgia
Headache in young obese woman with menstrual irregularities, + visual disturbances
Benign intracranial hypertension
Sudden orbital or eye pain + n/v beginning after use of anticholinergic meds
Acute glaucoma
Orbital or neck pain + Horner syndrome after trauma
Carotid dissection
Headache assoc with brain tumor
Typical tension or migraine headache occurring frequently, may wake pt from sleep

+ Focal abnls
Sudden onset headache with n/v/ stiff neck, fast decompensation, "worst headache"
Subarachnoid
Abortive therapy for migraine
Triptans
Ergotamine
Dihydroergotamine
Midrin: Isometheptene mucate (vasoconstrictor), dichloralphenazone (muscle relaxant), acetaminophen
Triptan mechanism
5HT-1D receptor agonists
Side effects of triptans
N/V
Numbness/tingling


CI if h/o CAD or HTN
Prophylactic tx for migraines
Anticonvulsants (topiramate)
Beta-blockers (propanolol)
Calcium channel blockers (verapamil)
Antidepressants
Chronic daily headache characteristics
Headache >15 days/month, >4 hrs/day
H/x of episodic migraines
Tx for chronic daily heacahe
Removal of acetaminophen or aspirin (analgesic rebound worsens headaches)

Tramadol, propoxyphene (analgesic) - bridging
Anticonvulsants, antidepressants, beta blockers and calcium channel blockers
Botulinum toxin
Botox is useful for what types of chronic daily headache?
Trigger points of head pain
Cervical pain or spasm
Posterior cortical dementia
Intellectual functioning lost --> behavior preserved

e.g. Alzheimer's (early involvement of recent memory, language dysfx, apraxia, agnosia)
The only "lower neurological fx" to be impaired in early Alzheimer disease
Olfaction
Imaging in AD shows?

Cortical atrophy (parietal, temporal, hippocampal)

Degeneration of cholinergic cells projecting from basal forebrain (nucleus basalis of Meynert) to cortex

Seen in?
Alzheimer's
Insidious decline in cognitive function in pts with cerebrovascular disease (e.g. HTN) --> diffuse subcortical white matter changes on imaging
Binswanger disease (vs. STEPWISE decline in multi-infarct)
Medical causes of dementia
Wernicke encephalopathy (B1)
Vit B12 deficiency
Hypothyroidism
HIV
Neurosyphilis
Normal press hydrocephalus
MS, Huntington, neoplastic
Dementia
Executive dysfx
Motor parkinsonism
Fluctuating cognition
Hallucinations
Sensitivity to neuroleptics
Sleep-related disorder
Diffuse Lewy Body dementia
Paresthesias in hands and feet
Loss of vibratory sense

+ pathological lesions in dorsal columns and lateral corticospinal tracts
Subacute combined degeneration (vit B12)

+ beefy tongue, premature whitening of hair, megaloblastic anemia
Nerve conduction studies in Vit B12 deficiency
Demyelination and denervation
Acute, unilateral loss of visual acuity or visual field + ocular pain in a young adult
Optic neuritis
Optic neuritis is assoc with?
Multiple sclerosis
Sjogren
Guillain Barre
HIV infection
Optic neuritis tx
IV corticosteroids (hasten recovery but have no effect on visual fx or recurrence)
Tx for optic neuritis dx in MS
Interferon beta 1a
Weakness + stiffness
Tingling/pins and needles, numbness, band around torso

Limb tremor, ataxia, scanning speech (cerebellar)

Optic neuritis and trigeminal neuralgia
Multiple sclerosis
Charcot's triad
Intention tremor
Dysarthria
Nystagmus
Severe, lancinating maxillary or mandibular pain - brief
Trigeminal neuralgia (carbamazepine)
Bright lesions on T2-weighted imaging, in corpus callosum and periventricular regions + inferior to tentorium
MS
Imaging technique to detect MS
T2 weighted images
FLAIR (fluid attenuated inversion recovery)
Decreased N-acetylaspartate measured by SPECT


+ CSF - IgG and oligoclonal bands
Tx for MS
IV steroids (do not decrease future attacks)

Immunomodulation
- Interferon b-1a
- interferon b-1b
- Glatiramer acetate (synthetic polypeptide of myelin basic protein)
Tx for MS patient with secondary progressive or worsening relapsing-remitting disease
IV mitoxantrone (antineoplastic immunomodulatory agent)
Oligoclonal bands are found in?
SLE
Neurosarcoidosis
SSPE
Subarachnoid hemorrhage
Syphilis
CNS lymphoma
Multiple neurologic symptoms 1-3 weeks post-infection or vaccination in young adult/children
Acute Disseminated Encephalomyelitis
Pathology of ADEM
Perivenular demyelination, relative sparing of axons

(peripheral subcortical cerebral white matter, thalami)
Distinguish between MS and ADEM
Post-infection
Early-onset ataxia
High lesion load on MRI
Involvement of deep gray matter (thalami)
Absence of oligoclonal bands
Tx for ADEM
Corticosteroids can shorten duration of disease
Mechanism of botulinum toxin
Binds presynaptic cholinergic receptors on motor nerve terminals --> inhibit acetylcholine release --> disrupted neurotransmission between nerve and end plate on muscle
Cancers that metastasize to brain
Lung
Breast
Kidney
Skin
Uterus
Non-neoplastic malformations involving neurons and glia near hypothalamus, involving neuroendocrine fx (precocious puberty or acromegaly); pts may experience paroxysms of laughter
Hypothalamic hamartomas

(gelastic seizures = paroxysmal laughter)
Pt with AIDS and tumor that can directly invade optic nerve
Primary lymphoma
Hepatic encephalopathy causes changes to what cells?
Increased astrocytes
HTN encephalopathy will show what CSF changes?
Mod increase in CSF protein (<100)
Neuro consequence of chronic renal failure
Peripheral neuropathy - symmetric, distal, mixed sensorimotor due to axonal degeneration

Improved with dialysis
Tx for restless leg syndrome

Gabapentin
Clonazepam
L-dopa
Dopamine agonists
Opiates

Type of visual field cut seen in vit B12 deficiency
Centrocecal scotoma
Cancers that metastasize to brain
Lung
Breast
Kidney
Skin
Uterus
Non-neoplastic malformations involving neurons and glia near hypothalamus, involving neuroendocrine fx (precocious puberty or acromegaly); pts may experience paroxysms of laughter
Hypothalamic hamartomas

(gelastic seizures = paroxysmal laughter)
Pt with AIDS and tumor that can directly invade optic nerve
Primary lymphoma
Hepatic encephalopathy causes changes to what cells?
Increased astrocytes
HTN encephalopathy will show what CSF changes?
Mod increase in CSF protein (<100)
Neuro consequence of chronic renal failure
Peripheral neuropathy - symmetric, distal, mixed sensorimotor due to axonal degeneration

Improved with dialysis
Tx for restless leg syndrome
Gabapentin
Clonazepam
L-dopa
Dopamine agonists
Opiates
Type of visual field cut seen in vit B12 deficiency
Centrocecal scotoma
Anemia, dermatitis, memory deficits in diet limited to corn
Pellagra
(nicotinic acid or tryptophan)
spinocerebellar degeneration, polyneuropathy, pigmentary retinopathy - deficiency in?
Vit E (early childhood)
obesity + hypersomnia + sleep apnea
Pickwickian syndrome (hypoxemia, pulm HTN)
EEG findings with hepatic encephalopathy
Triphasic waves
Normal background posterior-dominant rhythm on EEG
alpha 8-12 Hz
Gait d/o in NPH looks like?
Apraxic gait (like Parkinson gait)
NPH on CT/MRI
Enlarged temporal/frontal horns of lateral ventricles out of proportion to cortical atrophy; blunted frontal horns
Language disturbance in Alzheimer's
Decreased fluency
Dysnomia
Transcortical sensory aphasia

Repetition intact
Type of dementia in Huntington's
Subcortical - impaired executive fx and concentration

no aphasia, apraxia, amnesia (classical cortical features)
Headache, dementia, psychosis, decreased consciousness, myopathic weakness, delay in relaxation phase of reflexes (hung up reflex), cerebellar ataxia
Hypothyroidism
Seizures, myoclonus, ataxia, supranuclear gaze disturbance, hypothalamic dysfx, dementia, pendular convergence movements of eyes w/ masticatory mm movement (oculomasticatory myorhythmia)
Whipple disease (T. whippelii)
Areas affected by progressive multifocal leukoencephalopathy (JC virus)

Subcortical white matter - occipital, parietal --> visual complaints, alien hand syndrome

lesions do not enhance

Which drugs enhance chorea in Huntington's disease?
Dopaminergic (L-dopa, bromocriptine, lisuride)
Trihexylphenidyl MOA
decreases acetylcholine transmission --> can be used to counteract parkisonian side effects for neuroleptic drugs
Blepharospasm, forceful jaw opening, lip retraction, neck contractions, tongue thrusting, idiopathic in 6th decade

Meige syndrome

Tx: botulinum

Tx for tremor that comes on with action, involves arms/head, diminished with EtOH
Beta-blockers
Primidone (anticonvulsant)
Side effect of metoclopramide hydrochloride (Reglan)
parkisonism, tardive dyskinesia
Lhermitte sign
Electrical sensation radiating down spine when neck is passively flexed (spinal cord disease, MS)
Tx to reduce freq of MS flares
Interferon beta-IB
Glatiramer (immunomodulator)

Glucocorticoids to tx acute flare
Developmental regression at 6 month, extensor posturing + rigidity, myoclonic seizures (defect in NAA metabolism --> elevated levels in brain), brain has spongiform appearance
Canavan disease
Symptomatic tx for MS
Baclofen (antispasmodic)
Tizanidine (a2-agonist, muscle relaxant)
Benzodiazepine
Bilateral optic neuritis + transverse myelitis (paraparesis, bladder/bowel dysfx, sensory deficit)
Neuromyelitis optica (more likely to develop relapsing-remitting MS)
Male children in first few months of life, + nystagmus, limb tremors, optic atrophy, chorea, seizures, gait ataxia

Demyelination with sudanophilic staining

Sudanophilic leukodystrophy (Pelizaeus-Merzbacher disease)

Intracranial calcifications that follow gyral pattern of cerebral crotex --> railroad tracks on XR
Sturge-Weber
Lateral ventricles with typical batwing conformation
Agenesis of corpus callosum
Children with scaly erythematous rash on face, episodic ataxia

Hartnup disease - deficiency in?

tryptophan (precursor for nicotinamide) and other neutral amnio acids

Polycystic liver disease
Polycystic kidney disease
Retinal angiomas (telangiectasia)
Cerebellar tumors
Hemangiomas, adenomas, hemangioblastomas
von Hippel-Lindau
Erythrocytosis with cerebellar signs
Microscopic hematuria
Hepatosplenomegaly
Von Hippel Lindau
Acanthocytes
Posterior column and spinocerebellar degeneration
Retinitis pigmentosa
Abetalipoproteinemia
Associated with Arnold-Chiari type 2 (downward displacement of cerebellar tonsils into foramen magnum)
Spina bifida
Retinal problem assoc with tuberous sclerosis
Retinal phakoma (gliomatous tumors)

CNS calcifications, ash-leaf spots, renal tumors, cardiac rhabdomyomas, seizure disorders
enlargement of the fourth ventricle; a partial or complete absence of the cerebellar vermis, the posterior midline area of cerebellar cortex responsible for coordination of the axial musculature; and cyst formation near the internal base of the skull.
Dandy-Walker malformation
Effect of EtOH on developing brain
impaired neuronal migration --> MR, learning disabilities, hyperactivity, microcephaly
proximal muscle weakness in cancer patient
Dermatomyositis (paraneoplastic) - but not assoc with CNS tumors
man with problem relaxing grip, hypersomnolence, premature baldness, testicular atrophy, cataracts
Myotonic dystrophy
EMG pattern for myotonic dystrophy

"Dive bomber pattern"

(repetitive discharges with minor conduction defect)

Signs of poor prognosis in ALS
Facial fasciculations
Diaphragmatic weakness
Most common site of CNS atrophy associated with chronic alcoholism
Superior vermis
organophosphate toxicity
Severe motor polyneuropathy (UMN and LMN)

anticholinesterase activity = headache, vomiting, abd cramps, sweating, wheezing
Toxic lead exposure

Brain edema --> herniation

Low levels = ataxia, tremor, bilateral NEUROPATHIES

Pyridostigmine, physostigmine, edrophonium are?
Anticholinesterase
Atropine is a?
Anticholinergic
Toxicity:

Personality change, tremor, ataxia in a paper/pulp/electrochemical plant
Mercury

Damage to cerebellum, calcarine cortex of occipital lobe (visual field constriction)
Toxicity: wrist and finger drops and mild sensory abnormalities in distribution of radial nerves
Lead toxicity

+ abd pian, constipation, anemia, basophilic stippling, lead lines along gingival margin
Toxicity: Mucosal irritation, hemolysis, tonic-clonic seizures, polyneuropathy, psych changes
Arsenic
Toxicity: peripheral neuropathy, degeneration of posterior columns/dorsal roots assoc with rye ingestion
Ergot
Toxicity: Parkinsonism

MPTP
Manganese inhalation by miners

Toxicity: Confusion, headache, coma/posturing/seizures --> delayed neurologic deterioration with parkinsonian features 1-3 weeks after initial event

CO

Hypodensities in globus pallidum

Contralateral hemiparesis, retardation, seizures, glaucoma, intracranial calcifications assoc with leptomeningeal angiomatosis
Sturge-Weber (port wine stain)
Effects of HTN on retina

Segmental narrowing of arterioles
Nicking (arteriolar-venular compression)
Arteriolar straightening

Retinal microaneurysms are associated with?

DM

Osteomyelitis of the petrous pyramid leading to abducens and trigeminal nerve injury
Gradenigo syndrome (facial pain and diplopia)
Trauma to the face is most likely to injure which mm of the eye?
Superior oblique (CN 4) --> impaired intorsion of eye --> slight head tilt
Eye nerve most likely affected by varicella zoster

CN 4

Shares nerve sheath with V1 (herpes zoster spreads to face along trigeminal nerve)

MLF syndrome
weak adduction of affected eye
abduction nystagmus of contralateral eye
Jerky downward motion of both eyes with slow return

Ocular bobbing - pontine damage

slower than downbeat nystagmus
+ brief tonic interval before slow return

Transient loss of vision caused by disease of arteries - which artery?
Internal carotid (emboli)
Absence of thiamine in EtOHic leads to?

Periaqueductal and mamillary body lesions --> autonomic failure

Decremental response of muscles to repetitive stimulation of nerve at low frequency

Incremental response to repetitive stimulation at high frequency
Botulism
Bugs most responsible for meningitis in 6 mo old

H. flu
Strep neumo
N. meningitidis

<3 mo old
Group B strep
E. coli
Listeria

Signs of cauda equina compression
Loss of bowel and bladder control
Paraparesis/paraplegia
Deteriorating consciousness, autonomic disturbances (hypotension, hypothermia), ocular motor problems, gait difficulty in an alcoholic
Wernicke encephalopathy --> hemorrhagic necrosis
Acute severe fever, tachypnea, tachycardia, rigidity --> diffuse segmental muscle necrosis
Malignant hyperthermia
Fourth nerve palsy (superior rectus)
Difficulty looking down and medially (can't go down stairs)
Evidence of basilar skull fracture

Periorbital ecchymosis (raccoon eyes)
Ecchymosis over mastoid region (Battle sign)
Hemotympanum
CSF rhinorrhea/otorrhea

Person with head trauma has multiple punctate hemorrhage on MRI - reason?

Diffuse axonal injury - swelling in white matter, corpus callosum, upper brainstem.

Tx for trigeminal neuralgia
Carbamazepine
Phenytoin
Baclofen
Tx for incontinence in MS
Imipramine
Oxybutynin (anticholinergic)
Tx for spasticity
Baclofen
Tizanidine
Tx for status epilepticus

Lorazepam/Diazepam IV
+ phenytoin in conjunction to prevent relapse (IV)

Tx of glioblastoma multiforme

Complete gross resection + radiation therapy

Factors w. WORSE prognosis in MS ?? ???



2+ attacks per yr



Motor or cerebellar component



Older age onset (40+ yr old)



Residual motor or cerebellar fx 6 mo. s/p attack



Moderate disability within 5 yrs

GOOD prognostic indicators??

Initial presentation w/optic neuritis



Pure sensory sxs during attacks

transient monocular blindness

ICA/opthalamic a. stroke



(amaurosis fugax)

Hemiplegia (Leg >arm)


Confusion


Abulia


Incontinence


+Babinski

ACA stroke



(abulia = "loss of volition")

Aphasia



Homonymous hemianopia (w.macular sparing)



Thalamic Pain Syndrome (w. contralateral hemisensory dysfx)

PCA stroke

Dysarthria



Diploplia



Ipsilateral Horner syn



ipsilateral facial + contralateral limb numbness

Wallenberg Syndrome!



Vertebral artery (sometimes PICA)

Pinpoint pupils



Quadraplegia+sensory loss ("long tract signs")



CN abnormalities



Cerebellar dysfx

Basilar stroke

Diploplia



Nystagmus



Vertigo +/- N/V



Ipsilateral limb ataxia

Cerebellar stroke

Pure hemiplegia



Pure hemianesthesia



Ataxic hemiparesis



Dysarthria + Clumsy Hand


Lacunar stroke

Aphasia if dominant


Apraxia/neglect if non-dominant



Hemianesthesia


Hemiplegia (Arm or Face >>> leg)


Homonymous hemianopia

MCA

Stroke in MCA branch on dominant side (either inf or sup. branch) causing what?

Superior = expressive



Inferior = receptive

Presentation of



Pyridoxine toxicity (vB6 tox from TB tx)

Sensory Neuropathy

tingling/numbness hands and feet


areflexia