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265 Cards in this Set

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What is MG?
an autoimmune disorder caused by antibodies directed against postsynaptic nicotinic acetylcholine receptors
best intial test for MG
Ach receptor Ab
sensitive test for MG
Fatigued muscle weakness plus +Ach receptor Ab test
most sensitive test for MG
Edrophonium (Tensilon) test
most accurate test for MG
EMG (decremental dec in muscle fiber content on repetitive nerve
stimulation
txmt for MG
Anticholinesterase (pyridostigmine)
if primary txmt for MG fails what is the next txmt option?
Immunosuppressive tx with glucocorticoids
txmt for acute MG crisis
Plasmapheresis and IVIG
What is Gullian-Barre Syndrome (GBS)?
Acute severe polyradiculopathy w/autoimmune destruction of myelin.
Body’s immune system attacks self antigens (molecular mimicry)
pattern of sensory distribution loss with GBS
Pain, tingling dysthesia. Loss of large sensory fibers -> loss of reflexes and proprioception
best intial test for GBS
LP
-inc protien
-no cell inc 48 hr after sx
most accurate test for GBS
EMG, demylination of peripheral nerves
txmt for GBS
IVIG or plasmapheresis (equally effective)
Acute abortive tx for migraine? If contraindicated, alternative tx?
triptains
-sumatriptan
-rizatriptan
prefered over ergotamine bc less se

avoid in pt with cvd
-->before give to pt must check for pregnancy
Prophylactic tx for migraine when to start & what tx?
for migraine when >3x/mo.

Tx: propanolol, timolol, metoprolol,TCA (amitryptyline), toprimate, valproic acid
Pt w/ severe, infrequent migraine unresponsive to any tx?
Opioid analgesics
Initial tx for tension H/A, what if refractory?
Relaxation & NSAIDS, if refractory: muscle relaxants
Prophylactic tx for cluster H/A?
Predinisone, Li, ergotamine, methylsergide & verapamil

also
valproic acid, topiramate, indomethacine
Most effective tx for acute cluster H/A, alternative?
100% oxygen, alternative: sumatriptan
Central vertigo
a)onset ?
b)assoc w/ tinitis,HL ?
c) assoc w/diplopia, blindness, dysarthria, weakness ?
d)nystagmus
Central vertigo
a)onset gradual
b)tinitis,HL absent
c)diplopia, blindness, dysarthria, weakness present
d)pure nystagmus, vertical, does not
nerve associated with pain created in cluster HA
trigeminal nerve V1 & V2

other possible moa for cluster HA:
-Histamine & mast cell release
-extracranial vasodilation
-dysfxn of CN5, ANS, hypothalamus
Peripheral vertigo
a)onset?
b)assoc w/ tinitis,HL
c)assoc w/ diplopia, blindness, dysarthria, weakness
d)assoc w/ nystagmus
Peripheral vertigo
a)usually sudden onset
b)tinitis,HL present
c)diplopia, blindness, dysarthria, weakness
absent
d)nystagmus - mixed,horizontal,suppress w/fixation,unidirectional
Triad of Meniere’s disease?

txmt?
Tinitis, hearing loss (ear fullness/aural fullness), episodic vertigo (1-8 hr)

txmt:
low-sodimum diet, restrict caffine and tobacco

drug txmt: dieuretics
Two most common causes of Meniere’s dis?
Syphilis & head trauma
Peripheral vertigo associated w/ blunt ear trauma, air flight, scuba diving?
Perilymphatic fistula
Symptomatic tx for peripheral vertigo? If severe?
Meclizine (antihistamine and antiemetic)

If severe: diazepam (benzodiazepine)
Medical tx for Meniere’s dis? If fails?
salt diet & diuretic. If fails: surgical decompression
sudden onset of severe vertigo lasting for several days w/hearing losss & tinnitus

-frequently follows upper respiratory tract infection, sore throat, ear infection
labyrinthitis
possible causes of central vertigo
-posterior fossa tumor
-MS
-medication, ex: gentamicin assoc with ataxia and vertigo

aminoglycosides assoc with vestibulotoxic damage to motion sensitive hair cells in inner ear
Symptomatic tx for vertigo secondary to labyrinthitis? If severe?
Meclizine. If severe: diazepam
Examples of reversible causes of dementia?
Hypothyroidism, Vit B12 def, Hep/uremic encephalopathy, CNS vasculitis,
Syphilis, Brain abscess, Brain tumor, Meds (anticholinergic), Sleep apnea,
Trauma, Subdural hematoma, NPH, Depression
Examples of irreversible causes of dementia?
Progressive multifocal leukoencephalopathy, Alzheimer’s dis, Dementia w/
Lewy bodies, Frontotemporal degeneration (Pick’s dis), Vascular dementia
multiinfarct, Binswanger dis), Creutzfeldt Jakob dis
Early presentation of Pick’s disease?
also called Frontotemporal Dementia

personality change
compulsive behavior
impaired memory later

NO VISUAL HALLUCINATIONS
triad of normal pressure hydrocephalus
dementia - memory loss w/o focal deficiets
gait apraxia
urinary incontinence

dont aways see inc ICP or HA
Presentation of Creutzfeldt-Jakob dis?
Dementia & myoclonus jerking(aggressive/rapid onset wks-mo)
seen in pt 50s-70s

abn protease-resistant prion protien accumulate in brain
Triggers that exacerbate MS?
Infection, trauma, post pregnancy (2- 3 mo after)
Most accurate and best intial test for MS?
Brain MRI (inc T2 density, dec T1 density) Gandolinium enhance lesions till 2-6 wks after exacerbation
If MRI is nonconfirmatory but MS suspicion still high, what test?
CSF (mild pleocytosis <50 cells, inc IgG oligoclonal band 70-90%pts)
Tx for relapsing-remitting dis of MS?
Disease modifying agents: IFN B1b(Betaseron)
IFN A1a (Avonex/Rebif)

glatiramer acetate
->long term txmt of MS to modulate T-cell mediated autoimmunity to myelin basic protien
Tx for secondary progressive dis of MS?
IFN B1b & mitoxantrone
Tx for acute exacerbation of MS?
3 days of intense IV steroids-> oral steroids (taper over 4 wks) If
severe & steroid unresponsive: plasma exchange
Tx for MS pt w/ spasticity?
baclofen
Tx for MS pt w/ nocturnal spasticity?
tizandine, diazepam
Tx for MS pt w/ bladder hyperactivity / urinary incontinence?
oxybutynin

n anticholinergic medication used to relieve urinary and bladder difficulties, including frequent urination and inability to control urination (urge incontinence), by decreasing muscle spasms of the bladder
Tx for MS pt w/ Urinary retention?
bethanechol
Tx for MS pt w/ fatigue?
amantadine or fluoxetine
Parkinsonism + vertical gaze palsy?
Supranuclear palsy
Parkinsonism + prominent ataxia?
Olivopontocerebellar atrophy
Parkinsonism + prominent orthostatic hypotension?

txmt?
Shy Dragger syndrome parkinson like disease
assoc w/orthostatic hypotension,impotence, incontinece, bulbar dysfxn, laryngeal stridor
-maybe fatal

txmt:
fludrocortisone
salt supplement
alpha adrenergic agonist
Parkinson pt w/ intact functional status (less bradykinesia) < 60 yrs,
initial tx?
Anticholinergic meds
-trihexyphenydl, blocks acetylcholine stimulation of basal ganglia

(in excess or if give to pt > 60 yr, causes dry skin, dry mouth, urinary retention, flush, vision change, confusion)

-benzotropine->improves tremor and rigidity
Parkinson pt w/ intact functional status (less bradykinesia) < 60 yrs,
initial tx?
trihexyphenydl

dopamine agonist: ropinirole, pramipexole
What drug used to treat parkinson disease in its mild state can cause acute glaucoma?
trihexyphenidyl
for late complications of carbidopa/levodopa (response fluctuations)?
with Sustained release form of carbidopa/levodopa may add dopamine agonist,
selegiline or COMT inhibitors (entacapone) or restriction of protein meal to night

COMT - inc availabilty of levadopa ot brain & may dec motor fluctuation

can also add these after being on carbadopa/levadopa for long time (late se) which can cause on/off phenenomnon
only drug that can arrest progression of Parkinson dis?
Selegiline (mao-B inhibitor), may be neuroprotective and may dec the levels of levadopa needed
Surgical TX for Parkinson pt, when? Procedure?
Surgery for who cant tolerate or respond adequately to medical tx.
Procedures: pallidotomy & thalamotomy
Test of choice for diagnosing epilepsy?
Electroencephalogram
Tx of status epilepticus?
1. Secure ABC
2. tx reversible causes
3. lorazepam or diazepam (potentiate GABA
recept)

still seizing
4. phenytoin or fosphenytoin (inhibit Na+ dependent AP)

still seizing
5. phenobarbitol

still seizing
6. phenobarbitol

still seizing
7. midazolam or propofol
When are first time seizures treated with long-term anticonvulsants?
If Pt has abnormal neurologic exam, presented w/ status epilepticus, has
strong family hx of seizures, or has abnormal EEG
first line tx for generalized tonic clonic seizure?

If not a choice?
Valproic acid (inc availability of GABA), phenytoin, fospheytoin

If not a choice, pick
lamotrigine (dec glutamate release) or topiramate, used as adjunctive therapy
First line tx for absence seizures? If not a choice?
Ethosuximide. Valproic acid
First line tx for partial seizures (complete/partial)? Acceptable
alternatives?
Carbamazepine, phenobabital & phenytoin. Valproic acid & lamotrigine

in children, first line: phenobabital
Tx of choice for myoclonic and atonic seizures?
Valproic acid
CNS SE of phenytoin?
Diplopia, dizziness & ataxia
Systemic SE of phenytoin?
Gum hyperplasia, lymphadenopathy, hirusitism, rash
SE of phenobarbitol?
Sedation, ataxia, rash
SE of valproic acid?
Ataxia, tremor, hepatotoxicity, thrombocytopenia, GI irritation,
hyponatremia
SE of lamotrigine?
Diplopia, ataxia, rash, Steven-Johnson syndrome
type of dementia associated with visual hallucintions early in the disease
dementia with Lewy bodies (DLB)
Location of lesion
-blurred vision and pain in the right eye
- decreased visual acuity in the right eye that does not correct with pinhole testing
-a relative afferent pupillary defect (RAPD) on the right
-testing of the right visual field shows a small central scotoma.
Decreased visual acuity that does not correct with pinhole testing, an RAPD, and a central scotoma is characteristic of optic nerve disease.
Location of lesion
a bitemporal heteronymous hemianopia
lesion affecting the optic chiasm
Location of lesion
macular sparing
occipital cortex
diseases associated with cherry-red spots in the macula in a child
Niemann-Pick disease, Gaucher disease, and Tay-Sachs

Niemann-Pick disease
->an autosomal recessive disorder caused by sphingomyelinase deficiency
an autosomal recessive disorder caused by galactosylceramide §-galactosidase deficiency
Krabbe disease
Disease associated with children and classical ophthalmologic finding is clouding of the cornea
Hurler syndrome
What test to order for pt with:
-distal symmetric paresthesias and dysesthesias, with preserved muscle strength
-smokes cigarettes
-electrodiagnostic study indicates that this is likely a sensory neuronopathy
Order anti-Hu antibodies test

sensory neuronopathies is a paraneoplastic disorder, in particular small cell lung cancer

- generally associated with positive anti-Hu antibodies (antineuronal antibodies)

-also be associated with paraneoplastic encephalomyelitis, ataxia, and autonomic neuropathy.
Test used to detect multifocal motor neuropathy with conduction block
Anti-GM1
What skull structure does the 7th and 8th cranial never exit from?
The seventh (facial) and eighth (vestibulocochlear) nerves both course through the internal auditory meatus
CSF findings:
150 red blood cells, xanthochromic fluid, protein 55 (slightly increased), 15 white blood cells (90% lymphocytes), and normal glucose.

What is the significance of the finding of lymphocytes in the csf?
a subarachnoid hemorrhage.

Bleeding into the subarachnoid (CSF) space typically initiates an inflammatory response, one manifestation of which is a lymphocytic pleocytosis.

Xanthochromia is the result of breakdown of blood within the subarachnoid space. Its presence in a bloody CSF sample helps to distinguish intrathecal hemorrhage from a traumatic tap.
What is the normal opening pressure of csf?
6 to 15 cm H2O
What would be clinic ally contraindicated in the following pt?
-pt unresponsive
-temperature is 37°C
-heart rate 84 per minute
-respirations 10
blood pressure 152/84
-pt withdraws to noxious stimulation only
-right pupil is 10 mm and does not constrict to light
-left pupil is 5 mm and reacts normally
patient's clinical presentation suggests increased intracranial pressure (ICP) from a right hemispheric lesion. The “blown” right pupil suggests that herniation of the right hemisphere has compressed the right oculomotor nerve.

Performing an LP in this situation could be dangerous and could actually precipitate worsening herniation.
Ways to decrease icp
Raising the head of the bed
Intravenous administration of mannitol
Hyperventilation
Site of dysfxn in nervous system with following sx:
-gradually worsening weakness of the proximal arm and leg muscles symmetrically over several months
-neck flexors and extensors are found to be weak also
-no muscle pain or tenderness
Symmetric proximal weakness usually suggests a primary muscle problem, as does weakness of neck flexors and extensors.

The absence of muscle pain and tenderness does not argue against a primary muscle pathology.
Disease associated with chr 4 and CAG repeats?
huntington disease

HD mutation leads to abnormal cleavage of huntingtin protien which interfers with nuclear mechanism and cause cell death

autosomal dominant inheritance
Disease associated with
Ataxia- unsteady gait and limb ataxia
Dysarthria
Nystagmus

Pt can also have oviarian or breast cancer
The constellation of symptoms and their temporal evolution are most consistent with paraneoplastic cerebellar degeneration, a disorder associated with underlying gynecologic malignancy.
What area of the brain does alcohol effect for coordination?
Alcoholic cerebellar degeneration typically affects the vermis, and the characteristic manifestation is that of a gait ataxia.
MS pt complaining of uninary incontinence and urgency, what is the cause?
MS characteristically produces an upper motor neuron bladder or spastic bladder with increased frequency and urgency txt with oxybutynin (anticholinergic)
Pt with 3 months of intermittent urinary incontinence with atonic bladder, what is the cause of pt sx?
Atonic bladder implies an LMN lesion at the level of the conus medullaris, cauda equina, sacral plexus, or peripheral nerves.

It is characterized by overflow incontinence and increased capacity and compliance.

Diabetes is able to produce that type of lesion
Most common adult onset of Myotonic Dystrophy?

What is the inheritance pattern and gene involved
Myotonic dystrophy is a multisystem disorder that may also cause frontal balding, diabetes, and gastrointestinal symptoms.

It is inherited in an autosomal dominant fashion and is caused by a triplet repeat expansion in the DMPK gene. EMG typically shows myotonic discharges.
Name disease and how to test for it?

18 year old with:
-paranoid delusions
-slight tremor of both hands
-brownish discoloration of the cornea in the vicinity of the limbus
-Laboratory studies show a mild transaminitis
Wilson disease, an autosomal dominant disorder of copper metabolism that presents with neuropsychiatric symptoms as well as a movement disorder

Increased serum copper with decreased serum ceruloplasmin and increased 24-hour urinary copper
Dx?
woman with SLE develops weakness of her right finger and wrist extensors and pain on the right dorsum of her hand several months after being diagnosed with left carpal tunnel syndrome and right sciatic neuropathy
Dx: Mononeuropathy multiplex

patient's current symptoms are suggestive of a right radial neuropathy.

Multiple sequential mononeuropathies, each affecting a single peripheral nerve, are known as mononeuropathy multiplex.

Pain is a typical feature.

Patients with rheumatologic conditions are susceptible; vasculitis may be involved.
Presentation of occlusion of anterior cerebral artery?
Contralateral weakness & sensory loss in legs > upp ext.

Urinary incontinence, confusion, behavioral disturbances
Presentation of occlusion of middle cerebral artery? If dominant
hemisphere is involved? If non-dominant hemisphere is involved?
Contralateral hemiplegia face & arms > legs) , hemisensory loss, homonymous hemianopia w/
eyes towards cortical lesion.

Dominant: aphasia.

Nondominant: neglect
Presentation of posterior cerebral artery? Involvement of penetrating
branches/CN III palsy?
Contralateral HH, visual hallucinations, agnosia. Weber synd
(w/contralat hemiplegia), Benedikt synd (contralat ataxia or athetosis)
Presentation of occlusion of major cerebellar artery?
Vertigo, Vomiting, nystagmus, ipsilat limb ataxia
What is the dx of pt who complains that there is another man’s arm in the bed with him after he had coronary artery bypass 2 days prior?
Right hemispheric stroke with neglect
Initial test of choice for cerebrovascular disease?
Non contrast head CT
Most sensitive test to detect blood in brain?
Non contrast head CT
Most accurate test for detecting cerebral ischemia?
Diffusion weighted MRI
Diagnostic workup for pt w/acute ischemic stroke?
Echo, carotid duplex, 24 hr holter, inherited coagulability
Tx for pt who present w/in 3 hrs of onset of stroke?
Tissue plasminogen activator (tPA)
contraindications for the use of tPA in stroke pt
-serious head injury in last 3 mo
-gi or gu hemorrhage w/in last 21 days
-surgery w/in last 14 days
-hx of intracranial bleed any time in pt life
-bp > 185/110
-current use of anticoag
-platelet < 100,000
-glucose > 400 or < 50 mg/dL
- PT> 15s
When is heparin given in acute ischemic stroke?
Inc risk of recurrent stroke (A fib, basilar art thrombosis, stroke in evolution)
First line tx for secondary prevention of ischemic stroke?
Aspirin or clopidogrel
If aspirin allergic or continue to have recurrent CVA on aspirin alone?
change to dipyridamole or clopidrogel
When to recommend carotid endarterectomy?
When occlusion >70% of arterial lumen & lesion is symptomatic
Most common site of spinal cord compression?
Thoracic cord (70%)level as spinal cord is narrowest at this point
Dx test of choice for SC compression? When contraindicated?
MRI of spine. If contraindicated: CT myelogram
type of stroke?
-gaze palsy
-deafness
-tinnitus
-ipsilateral facial weakness
Anterior Inferior Cerebellar Artery Ischemic Stroke
Tx of choice for herniated disc, epidural abscess, hematoma?
Surgical decompression
txmt for SC compression
High dose dexamethasone immediately. For radiosensitive tumors: RT. For
others: surgical decompression
prognosis of SC compression depends on what factor?
Functional status at time of presentation (80% who are initially able to ambulate -> retain function later)
E.g., of communicating syringomyelia?
Arnold Chiari
E.g., of non-communicating syringomyelia?
Spinal cord trauma
Most common site of syringomyelia?
Cervical cord level
Pattern of subacute combined degeneration (B12 related)
Distal paresthesia & weakness of ext followed by spastic paresis &
ataxia. Combined def of vibration & proprioception w/ pyramidal signs (plantar extension & hyperreflexia)
Pattern of ant spinal art infarction?
Acute onset of flaccid paralysis->evolves into spastic paresis over
days-wks. Loss of pain & temp (w/ sparing of vibration & position sense as
post column is supplied by post spinal art)
Intial txmnt for status epilepticus
iv Benzodiazepines (lorazepam or diazepam) which inc GABA receptor fxn
Subfalcine herniation results in what?
Bilateral leg weakness

Subfalcine herniation may result in compression of the anterior cerebral artery with leg weakness as the result.
What txmt may delay but not prevent the development of MS in a patient with optic neuritis?
Intravenous corticosteroids
Location of lesion?
-horizontal diplopia acutely
-normal right lateral gaze but difficulty with adduction of the right eye while looking to the left and nystagmus in the abducting left eye
Lesions of the MLF produce an INO. The clinical characteristics of a right INO include inability to adduct the right eye in left lateral gaze plus nystagmus of the abducting left eye.
most common causes of relative afferent papillary defect in the right eye as well as the bilateral facial weakness
Sarcoidosis is one of the most common causes of bilateral LMN facial weakness
Location of lesion?
sudden onset of numbness of his left leg, arm, and face, motor examination is normal
sudden onset of symptoms along with the patient's stroke risk factors, he most probably has had a pure sensory stroke.
The most likely lesion is in the contralateral thalamus, because the sensory pathways cross prior to synapsing in the thalamus
What syndrome or diseases could cause bilateral weakness and loss of pain and temperature sensation with preservation of joint position sense in both legs?
Anterior spinal artery syndrome usually results from infarction of the anterior spinal artery, causing ischemia to the anterior two-thirds of the spinal cord. Therefore dorsal columns are spared but weakness and loss of pain and temperature sensation result because of involvement of the ventral horns and spinothalamic tracts
What classically results in degeneration of the dorsal columns and corticospinal tracts

impaired position and vibration, gait ataxia, hyperreflexia , whereas pain and temperature are spared?
B12 deficiency
What is the dx and test need for child presents with new seizure and the Wood's lamp finding of hypomelanotic lesions?
patient meets the diagnostic criteria for tuberous sclerosis complex (TSC). A head CT or MRI may identify cortical tubers, subependymal giant cell astrocytomas, or other lesions.
Dx?
social, behavioral, and language abnormalities with onset before age three.
Autism
Dx?
social isolation and eccentric behavior
normal language
no gross nor fine motor delay
onset after age 3
Asperger `syndrome
pain provoked by normally innocuous stimuli
Allodynia
increased sensitivity to sensory stimuli
Hyperesthesia
abnormal spontaneous sensations
Paresthesias
decreased sensation
Hypesthesia
What is the symptom of autism but not of Asperger syndrome?
Abnormal language development
A diagnosis of autism requires a combination of social, behavioral, and language abnormalities with onset before age three. Asperger syndrome shares social isolation and eccentric behavior with autism. Language is normal in Asperger syndrome
Anisocoria
unequal pupil sizes
What syndrome is associated with unilateral miosis, ptosis, and (sometimes) ipsilateral facial anhidrosis and what part of the nervous system is effected?
Horner syndrome , can occur with neck injury causing carotid dissection and impaired sympathetic innervations
What effect on the eye does the Horner syndrome have?
Examine the pupils in the dark (turn the lights off and look at the pupils during the first 5 to 10 seconds). A dilation lag in the small pupil and anisocoria greater in darkness means a sympathetic defect in the smaller pupil and will help with the diagnosis.
What changes to csf and emg are associated with Guillain-Barré syndrome?
Albuminocytologic dissociation means high protein with almost no cells in the CSF, which is characteristic of this syndrome
nerve conduction studies and EMG to demonstrate slowing of conduction velocities, prolongation of F-wave latency, and possible conduction block.
What organism in HIV positive pt can cause subacute onset of a flaccid paraparesis, sacral pain, paresthesias, and sphincter dysfunction?
Cytomegalovirus (CMV) infection is the most common cause of polyradiculitis or cauda equina syndrome in an immunocompromised individual
an autoimmune disorder caused by antibodies directed against postsynaptic nicotinic acetylcholine receptors
myasthenia gravis (MG)
autoimmune disorder caused by antibodies directed against presynaptic voltage-gated calcium channels
Lambert-Eaton myasthenic syndrome
CN effected in this pt:

patient complains of difficulty chewing.
- found to have decreased strength of his muscles of mastication.
Trigeminal nerve cn 5
nerve innervates the muscles of facial expression
Facial nerve cn 7
nerve innervates some pharyngeal muscles, and the hypoglossal nerve moves the tongue.
the glossopharyngeal nerve cn 9
Signs of UMN or corticospinal tract dysfunction
hypertonia, spasticity, increased reflexes, and an extensor plantar response (Babinski sign).
When is the Glasgow Coma Scale (GCS) used?
a composite assessment of unresponsive patients based on their eye movements, motor function, and language ability—is typically used for patients after head trauma. It has prognostic value for head-injured patients and is easy for nonphysicians to use.
Dx and txmt:
age over 50, pain over the temporal arteries, jaw claudication, and an elevated ESR
temporal arteritis
Treatment with prednisone for several months must be initiated early, because involvement of the ophthalmic artery can lead to blindness if diagnosis and treatment are delayed.
Intracerebral hemorrhages caused by hypertension are most often found in what parts of the brain?
the basal ganglia, thalamus, pons, and cerebellum, in order of decreasing frequency.
What is the treatment of choice for obstructive sleep apnea?
Because obstructive sleep apnea is characterized by repetitive episodes of upper airway obstruction during sleep, treatment is often with CPAP, which helps maintain airway patency during sleep
Signs of lower motor neuron (LMN) dysfunction
hypotonia, decreased or absent reflexes, and a flexor plantar response (downgoing toe)
How does lyme disease present in cns?
acute bilateral facial nerve palsy and intermittent peripheral nerve symptoms for over 3 weeks. You find elevated Lyme titers in serum and CSF
How is Lyme disease treated?
intravenous ceftriaxone is the first choice.

The combination of oral amoxicillin and doxycycline is the most common treatment for uncomplicated Lyme disease
What is the pathology of Parkinson disease?
progressive death of dopaminergic neurons of the substantia nigra pars compacta (basal ganglia)

-loss of smell an early sign
Mutation associated with familial Parkinson disease?
mutations in the parkin and α-synuclein genes
4 cardinal features of Parkinson disease?
tremor, rigidity, bradykinesia, and postural instability
common feature of progressive supranuclear palsy (PSP), a neurodegenerative disorder that is also characterized by parkinsonian features
Impairment of vertical gaze

disorder characterized by parkinsonism, supranuclear impairment of eye movements (vertical gaze typically affected more prominently than horizontal gaze), and impaired postural reflexes.
What is the inheritance pattern of DMD and Becker muscular dystrophy?
mutations in the dystrophin gene, located on the X chromosome. The inheritance pattern is X-linked.
ischemic stroke involving the right side of the pons could lead to what patterns of weakness?
“Crossed signs” can occur with unilateral lesions in the pons if descending motor fibers heading for the ipsilateral facial nucleus are affected, with the descending fibers heading for the contralateral spinal cord.

With right pontine lesions, the right face and left body could be weak.
side effects of carbamazepine
hyponatremia, agranulocytosis, and the risk for Stevens-Johnson syndrome. Except for the hyponatremia, these side effects are rare.
Cause of subdural hematoma
Tearing of bridging veins
Cause of epidural hematoma
Laceration of the middle meningeal artery
cause of lobar hemorrhage in the elderly
Amyloid angiopathy
More common cause of subarachnoid hemorrhage
aneurysmal rupture
Brain tumor type that enhance in a bright and mainly homogeneous manner on MRI
Meningiomas
Brain tumors that show as ring enhanced lesions on MRI
tumors (particularly glioblastoma multiforme and metastatic lesions), brain abscesses, toxoplasmosis, granulomas, and active demyelinating lesions typically show ring enhancement after contrast administration
Which of the following distinguishes acute disseminated encephalomyelitis (ADEM) from multiple sclerosis (MS)?
Monophasic course

MS is characterized by multiple white matter lesions separated in space and time and is therefore not monophasic.

Oligoclonal bands in the CSF are more common in MS than in ADEM.

The pleocytosis of ADEM is lymphocytic.

Both MS and ADEM can produce multiple lesions on MRI.

ADEM is acquired and commonly occurs after viral infections or vaccinations.

A positive family history is more likely to be relevant for a patient with MS.
Symptoms of Small-fiber neuropathy
impaired temperature and pinprick sensation

large fiber neuropathy effect vibration, position and ankle reflexes
Physical signs of Parkinson disease
Asymmetric rest tremor, asymmetric rigidity, and poor postural reflex

The extrapyramidal features of idiopathic PD are typically asymmetric
disorder characterized by ataxia, ophthalmoplegia, and areflexia. It is considered a variant of the Guillain-Barré syndrome

how is it screened for?
Miller-Fisher syndrome (MFS)

associated with the finding of anti-GQ1b antibodies in the serum
Charactieristics of Alcoholic cerebellar degeneration
may be associated with a peripheral neuropathy and loss of joint position sense and deep tendon reflexes but should not produce ophthalmoplegia (unless associated with Wernicke encephalopathy, in which case confusion should also be present).
Charactieristics of chronic paroxysmal hemicranias
unilateral and can produce conjunctival injection.

common in women

Response to indomethacin

Episodes of chronic paroxysmal hemicrania typically last for 20 minutes rather than hours.
What is Apraxia and what lobe of brain affected?
inability to perform a learned motor task and it is typically caused by lesions in either the frontal or parietal lobe of the dominant hemisphere.
When is xanthochromia seen in csf?
Xanthochromia is the yellow discoloration of the supernatant (if blood has been present for a few hours) in a spun sample of CSF that characterizes the presence of blood due to a subarachnoid hemorrhage.
Type of image most sensitive to intracranial bleeds
susceptibility imaging or gradient-echo imaging is the most sensitive technique for determining the presence of intracranial hemorrhage
Type of imaging most useful to detect brain tumor
Contrast-enhanced T1 is more useful for detecting the presence of a brain tumor
Type of imaging best for screening for intracranial lesions
FLAIR imaging is the single best MRI technique for screening for most types of intracranial lesions.
Diseases most associated with REM sleep behavior disorder?
Synucleinopathies are associated with REM sleep behavior disorder. Examples of synucleinopathies are:
Parkinson disease,
Lewy body dementia,
multiple system atrophy.
Location for stroke with only right hemiplegia, no other symptoms associated with vision or sensation
pure motor stroke involving the right side.

Possible localization for this syndrome includes the left corona radiata,
left internal capsule, and the
left side of the base of the pons.
Location of stroke with:
right hemiplegia would also likely cause an aphasia.
Infarction of the motor cortex
Location of stroke with:
more prominent sensory than motor deficits.
Thalamic strokes
Location of stroke with:
hemiplegia would also be associated with eye movement abnormalities.
Infarction of the midbrain
Location of stroke with:
associated with ipsilateral ataxia, ipsilateral Horner syndrome, and ipsilateral facial sensory loss, with contralateral impairment of pain and temperature in the arm and leg, nystagmus, and vertigo

absence of weakness
lateral medullary (Wallenberg) syndrome

Weakness is absent in a Wallenberg syndrome because motor fibers travel more anteriorly within the medulla.
Type of MD associated with highest level of CK?
Duchenne muscular dystrophy is associated with a marked elevation of the serum CK level
Type of MD associated with normal levels of CK?
Myotonic dystrophy may be associated with a normal CK or only mild elevation
Condition associated with:
loss of all significant motor function except eye blinking or perhaps vertical eye movements, with preservation of awareness and cognitive function.
Locked-in syndrome, which generally occurs with large lesions in the base of the pons, such as infarcts from cardiac embolism or basilar artery stenosis,

A large pontine lesion will typically affect corticobulbar and corticospinal fibers bilaterally, but blinking and vertical eye movements are preserved because of intact midbrain function
What actions can have the effect of raising intracranial pressure?
Lowering the head of the bed, loading intravenous fluids, or a decrease in respiratory rate
What actions can have the effect of lowering intercranial pressure?
Mannitol is an osmotic diuretic which can be used to lower increased intracranial pressure, although the benefit may be transient.
Location for stroke with only right hemiplegia, no other symptoms associated with vision or sensation
pure motor stroke involving the right side.

Possible localization for this syndrome includes the left corona radiata,
left internal capsule, and the
left side of the base of the pons.
Location of stroke with:
right hemiplegia would also likely cause an aphasia.
Infarction of the motor cortex
Location of stroke with:
more prominent sensory than motor deficits.
Thalamic strokes
Location of stroke with:
hemiplegia would also be associated with eye movement abnormalities.
Infarction of the midbrain
Location of stroke with:
associated with ipsilateral facial sensory loss, with contralateral impairment of pain and temperature in the arm and leg, nystagmus, and vertigo

absence of weakness
lateral medullary (Wallenberg) syndrome

Weakness is absent in a Wallenberg syndrome because motor fibers travel more anteriorly within the medulla.
Type of MD associated with highest level of CK?
Duchenne muscular dystrophy is associated with a marked elevation of the serum CK level
Type of MD associated with normal levels of CK?
Myotonic dystrophy may be associated with a normal CK or only mild elevation
Condition associated with:
loss of all significant motor function except eye blinking or perhaps vertical eye movements, with preservation of awareness and cognitive function.
Locked-in syndrome, which generally occurs with large lesions in the base of the pons, such as infarcts from cardiac embolism or basilar artery stenosis,

A large pontine lesion will typically affect corticobulbar and corticospinal fibers bilaterally, but blinking and vertical eye movements are preserved because of intact midbrain function
What actions can have the effect of raising intracranial pressure?
Lowering the head of the bed, loading intravenous fluids, or a decrease in respiratory rate
What actions can have the effect of lowering intercranial pressure?
Mannitol is an osmotic diuretic which can be used to lower increased intracranial pressure, although the benefit may be transient.
Type of dementia:
vivid visual hallucinations at times, and his alertness has been fluctuating on a day-to-day basis. Examination demonstrates bradykinesia and rigidity in the extremities, without any dyskinesias
Dementia with Lewy bodies may be the second most common type of dementing illness after Alzheimer disease.

exquisite sensitivity to neuroleptic medications
Type of brain tumor associated with:
MRI with contrast shows an enhancing mass in the left frontal region, crossing the corpus callosum in a “butterfly” pattern with surrounding edema.
typical “butterfly” pattern is characteristics of glioblastoma multiforme.
Location of lesion:
Bitemporal visual field defects
optic chiasm, such as suprasellar tumors.
Location of lesion:
right homonymous hemianopia
Lesions in the left optic radiation
Location of lesion:
left homonymous hemianopia
lesions of the right occipital lobe
Location of lesion:
unilateral visual field defects.
Optic nerve lesions
Location of lesion:
paresthesias in the first and second digits of his right hand.

physical examination shows no weakness but a mild decrease in light touch over the thumb.

nerve conduction study to rule out carpal tunnel syndrome, and it turns out to be normal.

sharp, “electric” pain travelling from his neck to the right hand
Sensory symptoms in the thumb can be related to median neuropathies (as in a carpal tunnel syndrome) but also to higher lesions such as those seen in a C6 radiculopathy
What is anterior spinal artery syndrome (ASAS)?
When does it occur?
How is it DX?
anterior spinal artery syndrome (ASAS), a well-recognized complication of abdominal aortic surgery. It usually includes a dissociated sensory loss as the anterior two-thirds of the spinal cord is perfused by the ASA, while the posterior third (posterior columns) is perfused by posterior spinal arteries.

the corticospinal and spinothalamic tracts are affected in ASAS, but the posterior columns remain intact, with preservation of joint position sense.

Dx with Toe position sense
Location of stroke:
Pt cant name objects
Has reduced verbal fluency
Speaks softly
The anomia (impaired naming) and reduced verbal fluency indicate the presence of an aphasia (language disorder).

The reduced verbal fluency does suggest an anterior location, but the hypophonia is most suggestive of a subcortical (usually thalamic) syndrome.
What is the dx:

27-year-old African-American woman with diabetes
w/ subacute onset of bilateral facial weakness
On examination you find bilateral lower motor neuron facial palsy as well as tender erythematous nodules over both shins
The rash on her shins likely represents erythema nodosum. Sarcoidosis is one of the most common causes of bilateral lower motor neuron facial palsy, especially in the African-American population.
What vitamin deficiency assoc with ataxia?
Vitamin E deficiency may cause ataxia, myelopathy, and polyneuropathy
Vitamin deficiency assoc with dementia?
Vitamin B deficiency may cause subacute combined degeneration of the cord and dementia.
a language (not a speech) disorder
Aphasia
inability to carry out a learned motor task.
Apraxia
an inability to recognize objects through a sensory modality even when the primary sensory modality is unimpaired
Agnosia
describes an inability to write but can draw
Agraphia
dx?
-rectal tone absence
-urinary incontince
-motor and sesnory loss in extermities

txmt?
cauda equina
-acute spinal cord compression

txmt: surgical emergency
1st line agent for mixed seizure and 2nd line txmt for generalized tonic-clonic and partial seizure
lamotrigine
dx?
-seizure type that involves both cerebral hemispheres
-rarely causes loss of conciousness or postical confusion
Juvenile Myoclonic epilepsy
what 2 drugs can not be used together in the txmt of generalized tonic clonic seizures?
valproic acid can not be added with phenytoin bc both compete with plasma protien binding sites
dx?
-child with developmental delays
-walks on tip toes with scisored gait
-legs are hypertonic bilaterally w/ brisk patellar reflexes
-ankle clonus
-up going toes bilaterally
cerebral palsy
-most common type, pyramidal
-mental retardaton in 90% of pt
-nonprogressive
-spasticity, hyperreflexia, slow effortful voluntary mvmt, and impaired fine motor fxn
next step for pt?

pt w/ back pain after moving furniture
-pain is sharp, travels down leg and feels like electric shock
-releived by rest & aggrevated by walking
-straight leg raise replicates pain
-decreaesed reflexes in lower extremities
pt dx is herniated nucleus pulposus

>90% pt sx resolve w/in 6 wk

next step: conservative txmt:
-bedrest 1 to 2 days
-limit activities for next 2 to 3 wks
-restriction on lifting
-NSAIDS
-moist heat
-gentle exercise to promote joint mobility
cause of exagerated reponse of destrusor muscle to bethanechol?
upper motor neuron dysfxn most often associated with MS

presenting sx can be bladder spaticity adn urinary retention
elevated LP opening pressure
100 to 300 mm H2O
normal protien levels in csf
20 to 45 mg/dL

elevated protien
100 to 500 mg/dL
normal csf glucose levels
> 50 mg/dL
normal opening pressure for csf during LP
50 to 80 mm H2O
what is the approach to identifying and correcting the following sx:
-fluctuating and cloudy level of consciusness
-impaired orientation
-disordered thinking
these are sx associated with delirium. must identify underlying cause such as
-dehydration
-electrolye imbalance
infection
and polypharmacy
dx?
female child with normal development until age of 5 mo
-after which begin to decline developmentally
-language and coordination most affected
Rett's syndrome
-genetic neurodegenerative disease found almost exclusively in female pt
what is the first step in management of cluster HA
primary txmt:
-avoid exacerbating factors

if therapy indicated:
prophlactic therapy:
- use of predinisone, Li, CCB (verapamil)

abortive therapy:
oxygen & use of triptans
what test is used when suspecting CO poisoning?
check for increased anion gap metabolic acidosis

assoc with sx of loss of conciousness and difficulty walking

other sx include:
chest pain, dyspnea, HA, ataxia, confusion seizures, coma
type of glaucoma?
-inc intraocular pressure
-abn optic disk findings: enlarged optic nerve head cupping

-no red or painful eye
-normal looking pupil & cornea

txmt?
-
pt has chronic open-angled glaucoma

txmt:
pilocarpine - muscarinic agonist producing rapid miosis and contraction of ciliary muscles to increase drainage of aqueous humor and decreasing intraocular pressure

other txmt to dec IOP:
BB - timolol
carbonic anhydrase inhibitor
alpha-2 agonist-brimonidine
physical signs required to declare brain death
-core body temp of 90F or 32.2C
-absence of intoxication or poisoning
-absence of metabolic or endocrine derangemnts
-evidence of catastrophic cerebral event
-absence of brain stem reflexes such as doll's eyes (oculocephalic) and vestibulo-ocular reflex (response to caloric stimulation)
what test should be performed if inc ICP is suspected?
first stabilize pt then a CT scan of the head without contrast should be preformed
causes of seizures
V - vascular (stroke,av malformation)
I - infection (meningitis, abscess, encephalitis), fever, changed mental status, photophobia, nucal rigidity
T- trauma
A - auto immune (cns vasculitis) SLE, MS,+ANA
M - metabolic( hypo/hypernatermia, hypomagnesemia, hypoglycemia, hypoxia, drug od/wd)
I - idiopathic
N - neoplasm (metastatic)
S - psychiatric
partial sz txmt
1st line - carbamazipine or phenytoin

2nd line - valproic acid
generalized sz txmt
first line - valproic acid

2nd line - lamotrigine
type of seizure
lose posture and tone, drop attack

txmt
atonic seizure

txmt: valproic acid
type of sz
keep tone, no conciousness, blank look, just blink

txmt
absence sz

ethoxisumide
type of sz
muscle jerking

txmt
myoclonic sz

valproic aicd
txmt of sz of unknown type
valproic acid
type of sz?
assoc with fear, deja vue, hallucination

txmt?
simple partial seizure

txmt:
carbamazepine
phenytoin
valproic acid
type of sz?
EEG typically show 10 Hz activicty and slow waves

txmt?
tonic clonic sz

txmt:
1st - protect airway
2nd - treat underlying cause if known
3rd - phenytoin, fosphenytoin or valproic acid ( all can be 1st line)

adjuctive therapy:
lamotrigine or topiramate
type of sz?
EEG shows 3 sec spike and wave discharge
absence (petit mal) seizure
moa of ethosuximide
affects thalamic neurons by working against calcium current

se:
drowsiness, nausea, vomitting, hyperactivisy or sleep disturbance
moa for phenytoin
inhibit Na dependent action potential
dx: tremor during purposeful mvmt, during when do finger to nose test
cerebellar disease, intential tremor
dx: tremor during rest, resolve with mvmt
parkinson tremor
dx: tremor worse with mvmt, family hx and no other complaint
essential tremor
pneumonic for parkinson disease
BRIT

bradykinesia
rigidity (cogwheel)
instability (postural)
tremor (resting)
what disease is assoc with positive myerson sign?
parkinson disease

+ myerson sign:
tap on forehead continue to blink and do not habituate and stop blinking
2nd causes of parkinson disease
metoclopramide (antiemetic)
MTPT
cobalt
CO
cyanide
haloperidol (typical antipsychotics)
chlorpromazine
to ways to cause parkinson on a molecular level
1. death of dopanergic neurons in substancia niagra (basal ganglia), loss of dopamine tone (as with antipsychotics)

2. increase amount of Ach, which inhibits dopamine action
txmt for response fluctuation for parkinson pt on carbadopa / levadopa?
goal is to inc dopamine so tx with:
dopamine agonist
-ropinirole or pramipexole

COMT inhibitor
-entacapone

MAO-B inhibitor
-selegiline
What drugs for parkinson should not be used to treat resting tremor in pt > 60 yr old and why?
anticholinergics
-benzotropine
-trihexyphenidyle

bc excess Ach can cause confusion, dry eye and mouth, urinary incontinence, visual changes (acute glaucoma) , constipation, dry skin

better to tx these pt with amantadine, act as anti-viral and anti-parkinson drug
dx?
slow progression of visual loss dx confirmed by visible drusen on dilated eye exam
dry or atropic form of age related macular degenraion

drusen are small, granular, subretinal deposits that are age related
dx?
abnormal growth of vessels from choroidal circulation into subretinal space

txmt?
-vessels leak causing collectoin of subretinal fluid, localized, exudative retinal detachment
wet or exudativce age related macular degeneration

acute onset (weeks to months)

txmt: laser photocoagulation (coaculates abn choroidal vessels & prevent progression) or
photodynamic therapy (thrombosis of damaged vessels & prevent progression)
dx?
-sudden loss of vision, painless, unilateral
-pale retina
-cherry red spot on fovea
-box car segmentation of blood in veins

txmt?
central retinal artery occulusion

txmt: like cva or tia
-lye pt flat and supply O2
-ocular message to unobsturct vessel

drug therapy:
acetazolamide may improve retinal perfusion
thrombolytics w/in 8 hrs of sx onset
how is central retinal artery occulusion dx?
carotid artery imaging
echocardiography
evaluate for thrombophilia
cause of central retinal artery occulusion?
due to atherosclerosic embolus from L carotid artery
dx?
rapid, painless loss of vision
-retinal hemorrhage, superficial along retinal vein
-cotton-wool spots
-edema on fundus,

txmt
central retinal vein occulusion

txmt:laser photocoagulation may help, results vary

note: hemmorage does not ususally cross horizontal raphee midline
what ophthalmic condition is associated with hx of Amaurosis fugax?
central retinal artery occulusion

Amaurosis fugax: unilateral loss of vision for few minutes
dx?
-sudden extreme pain in eye
-blurred vision
-n/v
-pupil dilated and nonreactive to light
-inc intraocular pressure
-hard red eye

txmt?
closed angle glaucoma

txmt:
emergency txmt needed, must dec iop with acetazolamide (carbonic anhydrase inhibitor to dec secretion of aqueous humor) then pilocarpine

Laser iridotomy is curative txmt
type of glucoma?
risk factors:
-older age
-asian ethnicity
-dilated pupils (prolonged time to dilate in dark areas)
-anterior uveitis
-lens dislocation
closed angle glaucoma
dx?
-age > 40
-diabetic
-require frequent changes in lens prescription
-cupping on optic disk
-asymptomatic

txmt?
open angled glaucoma

txmt? beta blockers (timolol & betaxolol)
txmt for closed angle glaucoma
medical emergency if not tx lead to blindness

-dec IOP with acetazolamide eye drops, carbonic anhydrase inhibitor, dec secretion of aqueous humor

then use pilocarpine
curative txmt for closed angle glaucoma
lasar iridotomy
-permanet connection between anterior and posterior chambers
txmt for open angle glaucoma
prevention, ocular exam every 3 - 5 yrs for pt > 40

tx w/ topical BB (timolol, betaxolol) to dec prod of aqueous humor
or
pilocarpine to inc aqueous outflow
dx?
vision loss usually monocular, transient like curtains falling down

fundscopy exam: whitened retina
Amaurosis fugax, emboli often at carotid birfurcation

dx with carodtid duplex

sign of upcoming stoke
drugs that can cause open or narrow angle glaucoma
anticholinergics
ipratroprium
TCA