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283 Cards in this Set

  • Front
  • Back
Basal ganglia consists of?
caudate nucleus
globus pallidus
subthalamic nucleus
substantial nigra
striaum consists of?
caudate and putamen
What is the key deficiency in Parkinson's, as well as the rate-limiting step in creation of Homovanillic acid?
Tryosine hydroxylase
What is homovinillic acid?
a major catecholamine metabolite. It is associated with dopamine levels in the brain.cerebrospinal fluid levels of HVA are measured as a marker of metabolic stress caused by 2-deoxy-D-glucose.[1] HVA presence supports a diagnosis of neuroblastoma and malignant pheochromocytoma.
What do the locus cerulei make and where are they located?
norepinepherine, pons
What crainial nerves come of of the cerebellopontine angle?
Are lewy bodys intra or extra cytoplasmic, and what do you stain for to see them?
intra, alpha-synuclein
What two diseases have Lewy bodies?
Parkinson's and frontotemporal dementia
What three structures get damaged in Parkinson's?
Substantia niagra, Locus cerulei, and CN X motor nucleus
What two genetic mutations can cause early parkinson's?
"Parkin" on chromosomes 4 and 6, or synuclein on chromosome 4.
What causes Gaucher disease (Ashkenazi Jew most common, with brain, spleen and heart involvement)?
Glucocerebroside deficiency.
What toxin exposures can look like Parkinsons and why do they look that way?
Manganese, rotenone (rat poison), MPTP (recreational drug). They fry mitochondria.
Parkinson treatments
-L-dopa with carbadopa
-COMT inhibitors (make it so less dopamine is needed)
MAO-B inhibitors such as selegeline, rasagiline. These scavange free radicals and inhibit dopamine breakdown.
Where is a deep brain stimulator placed for a parkinson's patient, and what symptoms does it not fix?
subthalamic nucleus. Mood, cognition, or gait
Cardinal parkinson's features, including hz frequency of tremor?
asymmetric 3-5 hz resting tremor
cogwheel rigidity
What movement is always preserved in Parkinson's?
What is "dopamine dysregulation syndrome" in Parkinson's?
compulsive and frontally disinhibited behavior
Atheosis definition and cause
writhing movements since birth due to cerabellar injury, caused by RH incompatability. NO cognitive deficits
Chorea treatment
Tetrabenzine, which is a dopamine depleter. Careful, because it precipitates depression.
Huntington's early warning sign, classic brain scan finding
abnl saccades, "bat-wing" ventricles
Key lab markers for Huntington's?
- low GABA in CSF

- Chromosome 4 CAG repeats (N>36 disease, >60, shows up as juvenile Huntingtons)
Pathogenesis of Huntington's?
apoptosis in the striatum due to unhealthy interaction of NMDA and glutamate. It's a non-inflammatory process.
What causes Sydenham Chroria? What are the common symptoms?
rheumatic fever with possible OCD, and choreoform movements. May recur.
Cause of Hemiballismus? Move when asleep, or not?
stroke in contralateral subthalamic nucleus. These guys will be still while sleeping.
Wilson's characteristics? Brain damage location?
15-45 onset
wing-beating tremor
cirrhosis, Kayser-Fleisher rings, parkinsonism, dysarthria, dementia.

Globus Palladus and putamen (lenticular nucleii)
What is another name for cortcospinal tract, and where does it cross?
Pyramidal, crosses in Medulla
What physical sign will you see if the lateral ventricle swells for some reason?
contralateral leg paralysis, due to pressure on the motor strip
Frontal gaze center drives eyes with way if damaged?
ipsilateral (looks to the lesion)
Where do most partial complex seizures originate?
Mesial surface of the brain (medial inferior surface)
What part of the brain is hit first and ultimately most severely in Alzheimers?
Entorhinal Cortex (mesotemporal)
What goes through the cerebelo-pontine angle?
What happens if you get a PCA aneurism?
3rd nerve damage
Where does one place the deep brain stimulator for depression cure?
Broadman area 25
What is the planum temporale?
Language center in dominant hemisphere
How much does a single head CT scan increase lifetime cancer risk?
What's an MRI T1 view good for?
Looking at anatomical structure
What's an MRI T2 view good for?
looking for pathology (edema fluid will be white)
What's an MRI DWI view good for?
spotting infarctions
What's the most likely cause of a brain infarction in the young? The old?
Cocaine, chronic HTN
What's toxoplasmosis look like on imaging?
multiple rings with large dark centers. Think AIDS.
What's cisticercosis look like on imaging?
multiple rings with tiny bright centers. Accompanied by seizures.
What's TB look like on brain imaging?
ring-enhancing lesions
What percentage of schizophrenics have cerebral atrophy greater than expected for their age?
What is Pittsburgh Compound B?
Binds to amyloid, tests for Alzheimers disease.
What does DTI do?
measures abnormal water molecule motion. Great for looking at white matter tracks.
What is the Wada test?
Infusing a barbiturate into the carotid artery to see which brain hemisphere is dominant. Will cause aphasia when you've found the dominant side. Dude.
What electrolyte problem can SSRI's cause?
*always on test* What causes Alexia without Agraphia?
occlusion of the PCA. Splenium and posterior left hemisphere damaged.
What is Gerstmann's Syndrome?
finger agnosia, left/right disorientation, agraphia, acalculia
What does normal aging cause? (whole body, looking for 5 things)
-decreased muscle strength
-loss of vibratory sensation
-impaired balance
-cog decline in memory of recent things, names, attention span, and learning
-EEg background slowing
What cognitive ability stays normal throughout normal aging processes?
What chromosome controls APO E? Where is APO E synthesized?
19, liver and brain
What are two diagnostic ways to check for Alzheimer's?
-CSF, low amaloid beta-peptide and elevated tau protein

-Amyloid imaging using Pittsburg Compound B
What drug delays progression of Alzheimer's?
What one physical symptom tends to go along with Alzheimer's?
Visuospacial Deficit
Which chromosome codes for APP, the amyloid precursor protein? What does that protein do?
21. It spits into either alpha secretase (good, cleanly metabolized products) or beta secretase (bad, leads to amyloid beta peptide that sticks around forever).
What parts of the brain accumulate the most plaques?
hippocampus and cerebral cortex association areas
Neurofibullary tangles are in or outside of the nerve cells? Do they correlate with all-comer dementias better or worse than plaques?
Inside, better
What's the absolute best anotomic finding for correlating with dementia symptoms?
Loss of synapses.
What percent of early onset (before age 65) AD is familial?
Which chromosome codes for the gene Presinillin 2? What's Presinillin 2?
Chromosome 1. Gene that codes for early demential. Insanely rare.
Which chromosome codes for the gene Presinillin 1? What's Presinillin 1?
Chromosome 14. Correlated to increased risk for early dementia. Rare.
What gene codes for Amyloid Precursor protein?
21 (think Down's, since Amyloid buildup = Alzheimer's, and those guys have an increased risk of Alzheimer's)
Where is acetycholine made?
Basal Nucleus of Meinhert
Where does edrophonium act? What does it do?
Neuromuscular junction, increases available ACH
What makes phyostigmine different from edrophonium and pyridostigmine?
Crosses blood brain barrier, increases ACh availability there too.
How do insecticides and nerve gasses act?
increase ACh in neuromuscular junction and in CNS
what's the mechanism of action for memantadine? What level of dementia is it used for?
blocks excitatory neurotransmission and toxic effects of NMDA agonists (such as glutamate). For moderate or severe dementia.
What are the best antipsychotics for geriatric population?
Olanzapine and Risperidone. More effective than the others, but overall antipsychotics tend to show "no benefit compared to placebo"
What is Capgras syndrome?
familiar person is replaced by an imposter
What sleep disturbance happens in Lewy Body dementia patients?
REM sleep behavior disorder
What happens when you give a Lewy Body dementia patient antipsychotics?
They get WORSE.
What regions of the brain will collect Lewy bodies in an LBD patient?
Cortex, basal ganglia
What stain is used to find Lewy bodies?
alpha synuclein
Do Lewy bodies accumulate intraneuron, or extraneuron?
Can frontotemporal patients copy a figure? Can they draw that figure from recall memory?
Yes, no.
What's the mean age of onset for frontotemporal dementia?
53 (younger than Alzheimer's)
Where is the tau gene located (associated with FTD)
Chromosome 17
What percentage of frontotemporal cases are familial?
What stain do you do for Pick's disease (a specific kind of FTD)
silver stain, looking for intraneuronal inclusions
What disease does a subtype of Frontotemporal mimic (up to 10%)?
What type of headache is almost never the right answer on the boards?
Tension headache!
What percentage of people get migraines? What's the male/female ratio?
12%, 3:1
Migraine can be diagnosed with the following sx's (two of one set, one of another set)
two of:
-worsened by movement
-moderate or severe pain

one of:
-nausea or vomiting
-photophobia and phonophobia
What percentage of migrainers get auras?
What are some prodromal migraine symptoms?
hyper- or hypo- activity, food cravings, irritability, repetative yawning (!), depression, heightened sensory perception, fluid retention
What's an aura?
-scotomata, photopsia, visual distortions/hallucinations(!), fortification spectra, paresthesia, unpleasant smells, aphasia (!)
What's the least common form of aura?
motor weakness and aphasia
What two subtypes of hemiplegic migraine (rare but testable hemipolegic aphasia in young adult) are there?
What are the three subtypes of familial hemiplegic migrainers? Genes? *yes you must memorize this drivel*
FMH 1 - Chromosome 19p13 mutation is CACNA1A gene

FMH 2 - 1q12 mutation in ATP1A2 gene

FMH 3 - 2q24 mutation in SCN1A gene
What is the pathophysiology of an aura? Headache?
aura: spreading neuronal depression

headache: "neurovascular" (new theories say more neuronal than vascular)
What's the first line migraine prophy drug class? 2nd line?
anticonvulsants, then low-dose TCA's.
What's the definition of chronic migraine? Only FDA approved treatment?
migraine occurs more than 15 days per month x3 months. Botox.
Do cluster headache patients want to lie down?
No, makes it worse. They pace.
What physical manifestations can you see with cluster headaches?
ipsilateral Horner's syndrome (autonomic features)
What triggers cluster headaches?
Alcohol, nitroglycerine
Do cluster headaches disturb sleep?
Yup, classically are awakened from dreams.
How long do cluster headache cycles last and when do they occur?
4-12 weeks, fall and spring.
TREATMENTS for cluster headache ?
sumatriptan INJECTION only
PREVENTION for cluster headaches?
verapamil (treatment of choice)
prednisone (often used as a bridge before verapamil takes affect)
How are the paroxysmal hemicranias different from cluster headaches (chronic and episodic)?
- shorter (10 min max)
- more frequent attacks
- F>M
-responds to ONLY indomethacin

"As the attacks get shorter, the name gets longer."
What percentage of brain tumor patients will complain of headache?
What are some warning signs that a headache could be a mass?
-increased pain with valsalva
-dull, non-throbbing
-nausea, vomiting
-neuro deficits
-sleep disturbances
What's a sentinel bleed?
Transient neuro symptoms that resolve during a subarachnoid hemorrhage before the serious symptoms occur.
Can you do an LP on a patient you suspect with a subarachnoid without getting a CT first?
NO, could have high ICP
what's a subhyloid hemorrhage?
blood pooling at the base of the retinal on eye exam. Means subarachnoid hemorrhage
What menstrual symptom tends to go along with pseudotumor cerebri? What MRI finding?
-irregular menses
-slit ventricles, empty sella
Which CN is usually affected first by pseudotumor cerebri? What opening pressure on LP is diagnostic?
VI, >250
What sized vessels does Giant Cell Arteritis attack? Who should we suspect it in?
medium sized arteries, to include coronaries. Suspect in elderly (esp women, ratio 3:1) with new or changing headaches Jaw claudication is pathopneumonic, systemic autoimmune sx's as well.
Cause of sudden, irreversible vision loss with retinal ischemia?
Giant Cell arteritis: ischemic optic neuropathy, central retinal artery occlusion and bilateral occipital lobe infarcts.
Trigeminal neuralgia is common in what age range? Worrisome in what age range?
Common in elderly, worrisome in the young because it may indicate a demyelinating disease
Treatments for trigeminal neuralgia:
When does Moro go away?
4 months
When does hand grasp reflex go away?
3 months
When does parachute response appear in children (hands out when fear falling)?
8-9 months
When do babies start to smile?
2 months (first two months are therefore brutal)
When can babies follow an object 180 degrees?
4 months
When do babies start reaching for objects?
4-5 months
When do babies sit? Stand?
6-11 months, 9-12 months
When do children display the "Cheerio sign" (pincer grasp ability)?
12 months
Do babies show a hand preference in the first year?
By what age can you expect baby's first steps?
9-17 months
When can you expect a kid to be able to hop?
4 years
When can you expect a kid to catch something?
5 years
At what age can a kid copy a vertical line?
2 years
At what age can a kid copy a circle?
2.5 years
At what age can a kid draw a plus sign?
3.5 years
At what age can a kid draw a square?
4.5 years
At what age can a kid draw a triangle?
5-6 years
Head circumference rule of thumb?
Go up by 5cm for each age milestone, starting wtih 35cm for an infant.

Birth 35cm
3 months 40cm
9 months 45cm
3 years 50cm
adult 55cm
Where do the calcifications appear in the brain of someone with a CSV infection?
around the ventricles
Where do the calcifications appear in the brain of someone with toxo?
everywhere "*T*otally all over the place."
What are MELAS and MERRF
Mitochondrial disorders
PKU and homocystenuria are what kind of disorders?
What kind of disorders are Rhett's and Lesch-Nyhan?
gray matter disorders
What kind of disorders are adrenoleukodystrophy (x-linked) and metachromatic dystrophy?
white matter diseases
PKU is caused by the absence of what enzyme?
phenylalanine hydroxylase
What does a homocystinuria patient look like?
Marfan's, lens dislocation, mental retardation and psychiatric illness.
What is Tay-Sach's disease?
European Jewish background, children are normal until 6 months, but then a deficiency in hexosaminidase-A causes listlessness, irritability, retardation, macrocephaly, *cherry red spot* blindness, seizures and spasticity.
What is Niemann-Pick disease?
Symptoms appear sometime within the first year of life. Deficiency in sphingomylinase causes retardation, regression (key symptom), seizures, hypotonia, jaundice and hepatosplenomegaly
What is Krabbe disease (also known as globoid cell leukodystrophy)?
a deficiency in beta-galactosidase leads to an accumulation of galactosylceramide in globoid cells, and leukodystrophy. Characterised by rapid onset of fisting, extension of legs, blindness, mental deterioration and death. POSTERIOR WHTIE MATTER IN BRAIN DETERIORATES, can see easily on MRI
Metachromatic leukodystrophy
Deficiency in arylsulfatase-A causes sufatides to accumulated in PNS, CNS and other organs. Appears between the ages of *1-26.* Symptoms include dementia, behavioral changes, schizophrenia-like sx's, ataxia, paraparesis and polyneuropathy. GLOBAL, CENTRAL demilination.
What does MELAS stand for?
Myopathy, Encephalopathy, Lactic Acidosis, Stroke
What does MERRF stand for?
Myoclonus, Epilepsy/encephalopathy, Ragged Red Fibers (in the muscle)
Gene for Rett syndrome?
X linked Xq28, can be seen in XXY boys, lethal en utero for XY boys
Age range for Rett syndrome apperance?
6-18 months
What's the inheritance pattern for Lesch-Nyhan syndrome?
X-linked recessive, child nl until 1st year
What's the deficient enzyme in Lesch-Nyhan syndrome?
Hypoxanthine phosphoribosyl Transferase
What's SSPE?
Subacute sclerosing panencephalitis. A late complication of a MEASLES infection. Might show elevated anti-measles titer in CSF
What is Adrenoleukodystrophy?
An X-linked disorder of lipid metabilism (long chain fatty acid metabolism) that appears between 3-10 years. Causes adrenal insufficiency, visual impairment (optic atrophy), dysarthria, abnl gait, slow mentation, apathy, hyperactivity, occasionally psychotic (on imaging white matter will be in the occipital lobes around ventricles
What substance abuse can look like a leukodystrophy syndrome?
Touluene abuse
Kleinfelter syndrome genetic anomaly? Physical features?
XXY. Tall long and lean. Sterile but with nl penis. Gynicomastia and sparse 2ndary sexual characteristics, MR in 25-30%.
Turner syndrom genetic anomaly? Physical features?
XO. Mental retardation in 18%. short stature, webbed neck. High palate, flat nose, wide mouth. no ovaries.
Fragile X syndrome genetic anomaly? Physical features?
Trinucleodide repeat disorder. MOST COMMON *INHERITED* MENTAL RETARDATION SYNDROME. Can be male or female. Look like prince Charles (long face, large ears, macro-orchidism in males)
XYY syndrome
Tall, tend to be prisoners, severe acne in puberty. Behavioral disorders in up to 50%, often with speech delay as a kid
XXX syndrome
some developmental delays, but otherwise no clinical symptoms. Sometimes have increased anxiety or depression.
William's syndrome kids look like what? Retardation? Special gift?
Elves, yes mild to moderate, musically gifted with perfect pitch and very sociable
Prader-Willi & Angelman syndromes have what gene problem?
AUTOSOMAL DOMINANT Micro-deletion or translocation on chromosome 15

Prader-willi is if inherited from dad
Angelman is if inherited from mom
VCF (Velocardiofacial syndrome) is what? What psych diagnosis is it strongly associated with?
-*V*elopharyngeal dysfunction (cleft palate, very nasal voice)
-*C*ardiac abnormalities
-*F*acial abnormalities

Differences between NF1 and NF2? Chromosomes?
NF1 - "mostly everything's on the outside," chromosome 17

NF2 - "mostly everything's on the inside," chromosome 22
How can you tell the difference between NF1, NF2 and Tuberous sclerosis complex?
Tuberous sclerosis complex has symptoms IN and OUT, plus ash-leaf spots, shagreen patches, and angiofibromas. Also, nasty seizures and retardation. Chromosomes 9 and 16.
What is Ataxia-telangiectasia? What's the treatment?
big, visible blood vessels in eyes and abdomen. Deficiencies in IgA and IgE. Progressive cerebellar ataxia, leukemia, lymphoma, diabetes, delayed sexual development. Treatment: IVIG
What problems can a Sturge-weber patient have? What will the brain xray look like?
seizures, intellectual impairment, glaucoma. Brain xray will look like waves.
Neurologic causes of Austic symptoms ("Really Frustrating Autistic Toddler"):
Fragile X
Tuberous Sclerosis
Genetic Causes of Tall stature (XXY men kill homies):
XXY syndrome
Genetic causes of short stature ("Too Tiny Triple X"):
Trisomy 21
Turner's XO
XXX syndrome
Differences between NF1 and NF2?
NF1 - "mostly everything's on the outside," chromosome 17

NF2 - "mostly everything's on the inside," chromosome 22
How can you tell the difference between the NF syndromes and Tuberous sclerosis complex?
Tuberous sclerosis complex has symptoms IN and OUT, plus ash-leaf spots, shagreen patches, and angiofibromas. Also, nasty seizures and retardation. Chromosomes 9 and 16.
What is Ataxia-telangiectasia? What's the treatment?
big, visible blood vessels in eyes and abdomen. Deficiencies in IgA and IgE. Progressive cerebellar ataxia, leukemia, lymphoma, diabetes, delayed sexual development. Treatment: IVIG
What problems can a Sturge-weber patient have? What will the brain xray look like?
seizures, intellectual impairment, glaucoma. Brain xray will look like waves.
Neurologic causes of Austic symptoms ("Really Frustrating Autistic Toddler"):
Fragile X
Tuberous Sclerosis
Genetic Causes of Tall stature ("XXY men kill homies"):
XXY syndrome
Genetic causes of short stature ("Too Tiny Triple X"):
Trisomy 21
Turner's XO
XXX syndrome
How you can tell weakness is a myopathy vs. a neuropathy.
mypoathies are proximal weakness, neuropathies are distal.
Genetic inheritance pattern of Duchenne's?
sex linked recessive
muscle membrane protein lost in
Is there MR in Duchenne's?
Can be, in 1/3 of cases. Also occasional cardiac involvement
What's the genetic inheritance pattern in Myotonic dystrophy? The specific gene problem?
autosomal dominant, trinucleotide repeats on #19
pathophysiology of Myotonic dystrophy?
abnormal membrane ion chanel permeability. Distal AND proximal limb weakness. Baldness and facial muscle atrophy. "EMG has "dive bomber" sound. Grip myotonia, can't let go. MR and very suspicious and argumentative. also with other system involvement.
consider mtDNA disorder when:
episodic confusion with acidosis
strokes in kids and young adults
progressive ocular muscle paresis
What else has a "dive bomber" sound on EMG besides Myotonic dystrophy?
Myasthenia Gravis
What symptoms are in botulism poisoning not found in Myasthenia Gravis?
DILATED pupils. Also constipation in kids.
What's the Eaton-Lambert syndrome associated with?
small cell lung cancer
What neurotransmitters does clostridium tetani interfere with?
GABA and glycine
Can you tell tetanus and strychnine poisoning apart, based on symptoms?
What is Werdig-Hoffman disease?
ALS in kids.

: (
What usually preceeds Guillain-Barre syndrome?
GI or respiratory tract infection, but could be any infection
What eye stuff does CN III enervate?
up, down, in, pupil and lids
What does CN VI enervate?
Out gaze!
If eyes deviate towards body hemiparesis are on the same side, where's the stroke?
contralateral pons
if eyes deviate opposite of hemiparesis, where's the stroke?
contralateral frontal lobe
What's the cause of a CN III palsy that is PAINFUL and spares the pupil?
Diabetes, pain is caused by an infarction.
What happens if the MLF is disrupted?
CNIII nucleus affected, ipsilateral eye can't adduct. Rest of CN II functions remain intact.
Most common cause of MLF being damaged?
Multiple Sclerosis
What spot damaged in the optic tracks will give you a bilat quadrantanopsia?
optic chiasm (usually pituitary adenoma)
Is it possible to get HIV related dementia and have good viral load counts/CD4 ct?
Distinctive characteristics of HIV dementia?
subcortical dementia, diffuse neurologic signs, extrapyramidal features, frontal release signs, gait impairment
Mechanism of AIDS related dementia?
probably cytokines, NOT primary neuronal infection
What does AIDS related dementia look like on imaging?
random, mostly symmetric subcortical white spots, not predominantly periventricular
What infection is most common infectious mass lesion in HIV pt's? Treatment?
Toxoplasmosis, treated with Pyrimethamine or sulfadiazine. Serum titers may be false negative.
What's the most common HIV associated FUNGAL CNS infection? Treatment?
Cryptococcus, amphotericin
Most common cause of blindness in HIV?
CMV retinitis
What ages are most likely to get meningitis? What populations are most at risk?
<5 and <60. Crowded populations: military barracks, college dorms, etc.
What viral meningitis can also have RBC's in it?
Herpes Simplex
Where would a primary CNS lymphoma (B cell tumors) be most likely to show up on an MRI?
Periventricular area
On SPECT what's the difference between lymphoma and Toxoplasmosis?
Lymphoma is "hot" and toxo is "cold."
If you can't tell by imaging if lesions are cancer or toxo, what should you do?
treat empirically for toxo before biopsying, it's safer
If a VDRL is given as negative in a test question, should you assume it's not syphilis?
NO! Negative in up to 25% of cases!
What's with Argyll-Robertson pupils?
They accomodate (to a close object) but don't react (to light).
Where do first order sensory neurons synapse?
dorsal root ganglion, talks to spinothalamic tract
Nocioceptor fibers are which kind (2 types)?
unmylinated C fibers and mylinated A-delta fibers.Memory aid: "A dull constant pain"
Where does descending pain modulation come from?
Periaqueductal gray and rafe nucleus (enkephalins and seratonin)
What is Dejerine-Roussy syndrome?
centrally caused pain 2ndary to thalamic infarction, presumably due to interruption to communication with the spinothalamic tract.
Is there a widely accepted cure for intention tremor?
what genetic disorder has a similar tremor to essential tremor?
Fragile X
Treatments for essential tremor:
propranolol, metoprolol, primidone, DB stimulator in thalamus(Ventralis intermedius nucleus)
Hallmarks of a psychogenic tremor.
-oscilates in 2-3 planes
-variable frequency, amplitude, location
-when bilat it's symmetrical ("coherent")
Corticospinal (pyramidal) tract does what? Is where? What's the major disease when it's lost?
DESCENDS in lateral columns of spinal cord. motor from upper motor neurons in brain. ALS (on cross section the spinal cord will look like it has "eyes")
What do fasiculus gracilis and F. cuneatus (posterior columns) do?
position, vibration and light touch.
What does the lateral spinothalamic tract carry? From which side of the body?
pain and temp from CONTRALATERAL body
What does the spinocerebellar tract carry? What does spinocerebellar ataxia look like?
feedback to cerebellum. High foot arch (pes cavus), decreased reflexes, loss of position and vibration sense.
What's Friedrich's ataxia?
Most common cause of inherited ataxia, it's a spinocerebellar ataxia. Caused by trinucleotide repeats, can be autosomal dominant or recessive.
Symptoms of tabes dorsalis?
lightening-like pains, position sense loss, high steppage gait, Argyl-Robertson pupil
What's "combined systems disease" and what are the symptoms?
b12 deficiency/pernicious anemia. Loss of lateral and posterior columns. Sometimes they give a vignette about eating raw fish?!
What does copper deficiency look like? What are common vignettes used on the boards for this?
spinal cord demylination with myopathy symptoms/spastic paraparesis. Usually caused by excessive Zinc intake, so vignettes present people who take folk remedies, lots of denture cream, etc.
What's a syrinx and what causes it?
loss of pain and temp sensation from damage of the spinothalamic tract fibers *where they cross.* Position sensation intact. Usually in fingers
What happens with an injury at C4?
paralyzed below the neck, APNEA, bladder, bowel, sexual impairment.
What happens with an injury at T4?
paralyzed below the nipples, bladder, bowel, sexual impairment.
What happens with an injury at T10?
paralyzed below the belly button, bladder, bowel, sexual impairment.
What happens with an injury at S1-3?
leg extensor weakness, bladder, bowel, sexual impairment.
What medication was mom probably taking if the kid has a meningocele or a menigomyelocele?
valproate or carbamazapine
What genetic syndrome is associated with meningocele or menigomyelocele?
Arnold Chiari malformation
What's a Dandy Walker malformation?
cyst where cerebellum should be. No vermis! Also often with hydrocephalus and enlarged 4th ventricle.
What's Brown-Sequard syndrome? What are the symptoms?
lateral hemi-transection of the spinal cord. Ipsilateral paresis with hyeractive DTR's, and contralateral position, vibration, pain and temp sense loss
What's cauda equina syndrome? Symptoms?
Injury to L2-S5. pain in legs and low back, flaccid areflexic paraperesis, incontinence.
What's the mechanism of action for Baclofen?
GABA B agonist
What's an MRI for an MS patient look like?
Periventricular demylination/plaques (bright spots)
Depression is most common in what type of MS?
What's fingolimod?
An immunomodulator used for MS treatment.
What's mitroxantrone?
Immunomodulator for MS
What's the problem with using Natalizumab (Tysabri) for MS patients?
may allow development of PML!
Which brain region is most sensitive to a lithium level > than 3.5?
cerebellum (we don't know why)
What's pseudobulbar palsy?
emotional incontinence (after head injury)
Rapid dementia with myoclonus, sporatic not inherited
What's Variant CJD?
Genetically inherited (Brits) personality changes, sensory problems then cognitive changes in young adults (average age 27)
What is SSPE?
Subacute Sclerosing Panencephaliis, a reaction to measles, dementia and myoclonus with onset in childhood. DIAGNOSTIC: CSF contains anti-measles antibodies. 90% mortality.
Where does Wernicke-Korsakoff cause hemorrhaging?
lymbic system (mamillary bodies and periaqueductal gray)
Can anorexia cause Wernicke-Korsakoff syndrome?
Nope, almost never.
What vitamin deficiency does bariatric surgery commonly cause?
What's TGA (Transient Global Amnesia)? How can you tell it's not a fugue?
anteriograde amnesia that lasts only hours, PERSONAL INFORMATION IS RETAINED unlike in a fugue.
What do white striae on fingernails mean?
Arsenic poisoning.
What toxicity causes hair to fall out?
When interpreting an EEG, what seizure type has regular spikes and waves?
What's a normal EEG background frequency?
alpha 8-13 Hz
What medications will speed up EEG?
benzos, barbiturates
What medications will slow down an EEG?
Phenothiazines, lithium
What medications will cause spikes in an EEG?
Lithium, clozipine, TCAs
What meds lower the seizure threshold?
neuroleptics, TCAs, wellbutrin, benzo or barbiturate withdrawal
What's non-convulsant status epilepticus look like?
semi-purposeful behavior, intermittently responsive. R/o drug intoxication and fugue state.
Treatment of choice for complex seizures (either simple or complex?
oxcarbamaepine, carbamazapine, phenotoin, lamotrigine, topiramate
What seizures happen in sleep?
frontal lobe seizures. Sometimes ONLY happen during sleep. Weird looking, often easily mistaken for psychogenic.
if a prolactin level is neg, is that proof the pt has a pseudoseizure?
What two antiseizure meds are renally cleared?
gabapentin and levetiracetam
medications of choice for tonic-clonic seizures?
valproate, carbamazepine, phenytoin
How does one treat erythema multiforme after you've caused it with one of our anticonvulsants?
What can phenobarbital do to kids?
Cause hyperactivity!
Major carbamazepine side effects
diplopia, osteopenia, leukopenia, liver damage, neural tube defects, hyponatremia (though not as bad as oxcarbazepine)
LAbs you need to monitor for carbamazepine? Is it an inducer or an inhibitor of P450
lft's, CBC, platelets, sodium and levels monthly for 3 months, then at least quarterly. It's a p450 inducer.
Who should you not give carbamazepine to?
People of asian descent, since they're at higher risk for Stevens-Johnson syndrome.
What's a side effect of valproate for unborn infants?
lower IQ.
If a patient on valproate gets a tremor, what should you do? What if they get abdominal pain?
Check ammonia level. Check amylase level.
what two antiepileptic drugs can cause psychosis and aggression?
Keppra and zonisamide
What causes burst-suppression in an EEG?
post anoxic injury
what causes 3hz spike and wave in an EEG?
Absence seizure
What causes slow disorganized background in an EEG?
What causes periodic complexes in an EEG?
What kind of sleep causes sleep spindles and K complexes in an EEG?
Non-rem sleep
what causes 8-12hz occipital rhythm?
Nothing, that's normal
What anti epileptic drug can cause PCOS?
valproic acid
Are suicide rates the same amongst those with seizures and the nl population?
No! 4-5 times higher!
What may aggressive, violent behavior be a manifestation of?
frontal lobe seizures.
What's postictal psychosis?
Psychosis that shows up days after a psychosis, usually after a lucid period
What's interictal psychosis?
hallucinations and thought disorder with clear sensorium. Not temporally related to seizures. DURATION OF 6 MONTHS if not treated. NOT genetic.