Clinical Neurology For Psychiatrists (Kaufman Course)

Front 1

Basal ganglia consists of?

Back 1

caudate nucleus
putamen
globus pallidus
subthalamic nucleus
substantial nigra

Front 2

striaum consists of?

Back 2

caudate and putamen

Front 3

What is the key deficiency in Parkinson's, as well as the rate-limiting step in creation of Homovanillic acid?

Back 3

Tryosine hydroxylase

Front 4

What is homovinillic acid?

Back 4

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.

Front 5

What do the locus cerulei make and where are they located?

Back 5

norepinepherine, pons

Front 6

What crainial nerves come of of the cerebellopontine angle?

Back 6

5,7,8

Front 7

Are lewy bodys intra or extra cytoplasmic, and what do you stain for to see them?

Back 7

intra, alpha-synuclein

Front 8

What two diseases have Lewy bodies?

Back 8

Parkinson's and frontotemporal dementia

Front 9

What three structures get damaged in Parkinson's?

Back 9

Substantia niagra, Locus cerulei, and CN X motor nucleus

Front 10

What two genetic mutations can cause early parkinson's?

Back 10

"Parkin" on chromosomes 4 and 6, or synuclein on chromosome 4.

Front 11

What causes Gaucher disease (Ashkenazi Jew most common, with brain, spleen and heart involvement)?

Back 11

Glucocerebroside deficiency.

Front 12

What toxin exposures can look like Parkinsons and why do they look that way?

Back 12

Manganese, rotenone (rat poison), MPTP (recreational drug). They fry mitochondria.

Front 13

Parkinson treatments

Back 13

-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.

Front 14

Where is a deep brain stimulator placed for a parkinson's patient, and what symptoms does it not fix?

Back 14

subthalamic nucleus. Mood, cognition, or gait

Front 15

Cardinal parkinson's features, including hz frequency of tremor?

Back 15

asymmetric 3-5 hz resting tremor
cogwheel rigidity
bradykinesia

Front 16

What movement is always preserved in Parkinson's?

Back 16

occular

Front 17

What is "dopamine dysregulation syndrome" in Parkinson's?

Back 17

compulsive and frontally disinhibited behavior

Front 18

Atheosis definition and cause

Back 18

writhing movements since birth due to cerabellar injury, caused by RH incompatability. NO cognitive deficits

Front 19

Chorea treatment

Back 19

Tetrabenzine, which is a dopamine depleter. Careful, because it precipitates depression.

Front 20

Huntington's early warning sign, classic brain scan finding

Back 20

abnl saccades, "bat-wing" ventricles

Front 21

Key lab markers for Huntington's?

Back 21

- low GABA in CSF

- Chromosome 4 CAG repeats (N>36 disease, >60, shows up as juvenile Huntingtons)

Front 22

Pathogenesis of Huntington's?

Back 22

apoptosis in the striatum due to unhealthy interaction of NMDA and glutamate. It's a non-inflammatory process.

Front 23

What causes Sydenham Chroria? What are the common symptoms?

Back 23

rheumatic fever with possible OCD, and choreoform movements. May recur.

Front 24

Cause of Hemiballismus? Move when asleep, or not?

Back 24

stroke in contralateral subthalamic nucleus. These guys will be still while sleeping.

Front 25

Wilson's characteristics? Brain damage location?

Back 25

15-45 onset
wing-beating tremor
cirrhosis, Kayser-Fleisher rings, parkinsonism, dysarthria, dementia.

Globus Palladus and putamen (lenticular nucleii)

Front 26

What is another name for cortcospinal tract, and where does it cross?

Back 26

Pyramidal, crosses in Medulla

Front 27

What physical sign will you see if the lateral ventricle swells for some reason?

Back 27

contralateral leg paralysis, due to pressure on the motor strip

Front 28

Frontal gaze center drives eyes with way if damaged?

Back 28

ipsilateral (looks to the lesion)

Front 29

Where do most partial complex seizures originate?

Back 29

Mesial surface of the brain (medial inferior surface)

Front 30

What part of the brain is hit first and ultimately most severely in Alzheimers?

Back 30

Entorhinal Cortex (mesotemporal)

Front 31

What goes through the cerebelo-pontine angle?

Back 31

CN's V, VII, VIII

Front 32

What happens if you get a PCA aneurism?

Back 32

3rd nerve damage

Front 33

Where does one place the deep brain stimulator for depression cure?

Back 33

Broadman area 25

Front 34

What is the planum temporale?

Back 34

Language center in dominant hemisphere

Front 35

How much does a single head CT scan increase lifetime cancer risk?

Back 35

0.07%

Front 36

What's an MRI T1 view good for?

Back 36

Looking at anatomical structure

Front 37

What's an MRI T2 view good for?

Back 37

looking for pathology (edema fluid will be white)

Front 38

What's an MRI DWI view good for?

Back 38

spotting infarctions

Front 39

What's the most likely cause of a brain infarction in the young? The old?

Back 39

Cocaine, chronic HTN

Front 40

What's toxoplasmosis look like on imaging?

Back 40

multiple rings with large dark centers. Think AIDS.

Front 41

What's cisticercosis look like on imaging?

Back 41

multiple rings with tiny bright centers. Accompanied by seizures.

Front 42

What's TB look like on brain imaging?

Back 42

ring-enhancing lesions

Front 43

What percentage of schizophrenics have cerebral atrophy greater than expected for their age?

Back 43

20%

Front 44

What is Pittsburgh Compound B?

Back 44

Binds to amyloid, tests for Alzheimers disease.

Front 45

What does DTI do?

Back 45

measures abnormal water molecule motion. Great for looking at white matter tracks.

Front 46

What is the Wada test?

Back 46

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.

Front 47

What electrolyte problem can SSRI's cause?

Back 47

hyponatremia

Front 48

*always on test* What causes Alexia without Agraphia?

Back 48

occlusion of the PCA. Splenium and posterior left hemisphere damaged.

Front 49

What is Gerstmann's Syndrome?

Back 49

finger agnosia, left/right disorientation, agraphia, acalculia

Front 50

What does normal aging cause? (whole body, looking for 5 things)

Back 50

-decreased muscle strength
-loss of vibratory sensation
-impaired balance
-cog decline in memory of recent things, names, attention span, and learning
-EEg background slowing

Front 51

What cognitive ability stays normal throughout normal aging processes?

Back 51

KNOWLEDGE

Front 52

What chromosome controls APO E? Where is APO E synthesized?

Back 52

19, liver and brain

Front 53

What are two diagnostic ways to check for Alzheimer's?

Back 53

-CSF, low amaloid beta-peptide and elevated tau protein

-Amyloid imaging using Pittsburg Compound B

Front 54

What drug delays progression of Alzheimer's?

Back 54

Donepezil

Front 55

What one physical symptom tends to go along with Alzheimer's?

Back 55

Visuospacial Deficit

Front 56

Which chromosome codes for APP, the amyloid precursor protein? What does that protein do?

Back 56

21. It spits into either alpha secretase (good, cleanly metabolized products) or beta secretase (bad, leads to amyloid beta peptide that sticks around forever).

Front 57

What parts of the brain accumulate the most plaques?

Back 57

hippocampus and cerebral cortex association areas

Front 58

Neurofibullary tangles are in or outside of the nerve cells? Do they correlate with all-comer dementias better or worse than plaques?

Back 58

Inside, better

Front 59

What's the absolute best anotomic finding for correlating with dementia symptoms?

Back 59

Loss of synapses.

Front 60

What percent of early onset (before age 65) AD is familial?

Back 60

60%

Front 61

Which chromosome codes for the gene Presinillin 2? What's Presinillin 2?

Back 61

Chromosome 1. Gene that codes for early demential. Insanely rare.

Front 62

Which chromosome codes for the gene Presinillin 1? What's Presinillin 1?

Back 62

Chromosome 14. Correlated to increased risk for early dementia. Rare.

Front 63

What gene codes for Amyloid Precursor protein?

Back 63

21 (think Down's, since Amyloid buildup = Alzheimer's, and those guys have an increased risk of Alzheimer's)

Front 64

Where is acetycholine made?

Back 64

Basal Nucleus of Meinhert

Front 65

Where does edrophonium act? What does it do?

Back 65

Neuromuscular junction, increases available ACH

Front 66

What makes phyostigmine different from edrophonium and pyridostigmine?

Back 66

Crosses blood brain barrier, increases ACh availability there too.

Front 67

How do insecticides and nerve gasses act?

Back 67

increase ACh in neuromuscular junction and in CNS

Front 68

what's the mechanism of action for memantadine? What level of dementia is it used for?

Back 68

blocks excitatory neurotransmission and toxic effects of NMDA agonists (such as glutamate). For moderate or severe dementia.

Front 69

What are the best antipsychotics for geriatric population?

Back 69

Olanzapine and Risperidone. More effective than the others, but overall antipsychotics tend to show "no benefit compared to placebo"

Front 70

What is Capgras syndrome?

Back 70

familiar person is replaced by an imposter

Front 71

What sleep disturbance happens in Lewy Body dementia patients?

Back 71

REM sleep behavior disorder

Front 72

What happens when you give a Lewy Body dementia patient antipsychotics?

Back 72

They get WORSE.

Front 73

What regions of the brain will collect Lewy bodies in an LBD patient?

Back 73

Cortex, basal ganglia

Front 74

What stain is used to find Lewy bodies?

Back 74

alpha synuclein

Front 75

Do Lewy bodies accumulate intraneuron, or extraneuron?

Back 75

Intra

Front 76

Can frontotemporal patients copy a figure? Can they draw that figure from recall memory?

Back 76

Yes, no.

Front 77

What's the mean age of onset for frontotemporal dementia?

Back 77

53 (younger than Alzheimer's)

Front 78

Where is the tau gene located (associated with FTD)

Back 78

Chromosome 17

Front 79

What percentage of frontotemporal cases are familial?

Back 79

40%

Front 80

What stain do you do for Pick's disease (a specific kind of FTD)

Back 80

silver stain, looking for intraneuronal inclusions

Front 81

What disease does a subtype of Frontotemporal mimic (up to 10%)?

Back 81

ALS

Front 82

What type of headache is almost never the right answer on the boards?

Back 82

Tension headache!

Front 83

What percentage of people get migraines? What's the male/female ratio?

Back 83

12%, 3:1

Front 84

Migraine can be diagnosed with the following sx's (two of one set, one of another set)

Back 84

two of:
-unilateral
-throbbing
-worsened by movement
-moderate or severe pain

one of:
-nausea or vomiting
-photophobia and phonophobia

Front 85

What percentage of migrainers get auras?

Back 85

20%

Front 86

What are some prodromal migraine symptoms?

Back 86

hyper- or hypo- activity, food cravings, irritability, repetative yawning (!), depression, heightened sensory perception, fluid retention

Front 87

What's an aura?

Back 87

-scotomata, photopsia, visual distortions/hallucinations(!), fortification spectra, paresthesia, unpleasant smells, aphasia (!)

Front 88

What's the least common form of aura?

Back 88

motor weakness and aphasia

Front 89

What two subtypes of hemiplegic migraine (rare but testable hemipolegic aphasia in young adult) are there?

Back 89

familial
sporatic

Front 90

What are the three subtypes of familial hemiplegic migrainers? Genes? *yes you must memorize this drivel*

Back 90

FMH 1 - Chromosome 19p13 mutation is CACNA1A gene

FMH 2 - 1q12 mutation in ATP1A2 gene

FMH 3 - 2q24 mutation in SCN1A gene

Front 91

What is the pathophysiology of an aura? Headache?

Back 91

aura: spreading neuronal depression

headache: "neurovascular" (new theories say more neuronal than vascular)

Front 92

What's the first line migraine prophy drug class? 2nd line?

Back 92

anticonvulsants, then low-dose TCA's.

Front 93

What's the definition of chronic migraine? Only FDA approved treatment?

Back 93

migraine occurs more than 15 days per month x3 months. Botox.

Front 94

Do cluster headache patients want to lie down?

Back 94

No, makes it worse. They pace.

Front 95

What physical manifestations can you see with cluster headaches?

Back 95

ipsilateral Horner's syndrome (autonomic features)

Front 96

What triggers cluster headaches?

Back 96

Alcohol, nitroglycerine

Front 97

Do cluster headaches disturb sleep?

Back 97

Yup, classically are awakened from dreams.

Front 98

How long do cluster headache cycles last and when do they occur?

Back 98

4-12 weeks, fall and spring.

Front 99

TREATMENTS for cluster headache ?

Back 99

sumatriptan INJECTION only
oxygen
ergotamine

Front 100

PREVENTION for cluster headaches?

Back 100

verapamil (treatment of choice)
lithium
divalproex
prednisone (often used as a bridge before verapamil takes affect)

Front 101

How are the paroxysmal hemicranias different from cluster headaches (chronic and episodic)?

Back 101

- shorter (10 min max)
- more frequent attacks
- F>M
-responds to ONLY indomethacin

"As the attacks get shorter, the name gets longer."

Front 102

What percentage of brain tumor patients will complain of headache?

Back 102

60%

Front 103

What are some warning signs that a headache could be a mass?

Back 103

-increased pain with valsalva
-dull, non-throbbing
-nausea, vomiting
-neuro deficits
-sleep disturbances

Front 104

What's a sentinel bleed?

Back 104

Transient neuro symptoms that resolve during a subarachnoid hemorrhage before the serious symptoms occur.

Front 105

Can you do an LP on a patient you suspect with a subarachnoid without getting a CT first?

Back 105

NO, could have high ICP

Front 106

what's a subhyloid hemorrhage?

Back 106

blood pooling at the base of the retinal on eye exam. Means subarachnoid hemorrhage

Front 107

What menstrual symptom tends to go along with pseudotumor cerebri? What MRI finding?

Back 107

-irregular menses
-slit ventricles, empty sella

Front 108

Which CN is usually affected first by pseudotumor cerebri? What opening pressure on LP is diagnostic?

Back 108

VI, >250

Front 109

What sized vessels does Giant Cell Arteritis attack? Who should we suspect it in?

Back 109

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.

Front 110

Cause of sudden, irreversible vision loss with retinal ischemia?

Back 110

Giant Cell arteritis: ischemic optic neuropathy, central retinal artery occlusion and bilateral occipital lobe infarcts.

Front 111

Trigeminal neuralgia is common in what age range? Worrisome in what age range?

Back 111

Common in elderly, worrisome in the young because it may indicate a demyelinating disease

Front 112

Treatments for trigeminal neuralgia:

Back 112

-carbamazepine
-gabapentin
-amitriptyline
-surgery

Front 113

When does Moro go away?

Back 113

4 months

Front 114

When does hand grasp reflex go away?

Back 114

3 months

Front 115

When does parachute response appear in children (hands out when fear falling)?

Back 115

8-9 months

Front 116

When do babies start to smile?

Back 116

2 months (first two months are therefore brutal)

Front 117

When can babies follow an object 180 degrees?

Back 117

4 months

Front 118

When do babies start reaching for objects?

Back 118

4-5 months

Front 119

When do babies sit? Stand?

Back 119

6-11 months, 9-12 months

Front 120

When do children display the "Cheerio sign" (pincer grasp ability)?

Back 120

12 months

Front 121

Do babies show a hand preference in the first year?

Back 121

Nope.

Front 122

By what age can you expect baby's first steps?

Back 122

9-17 months

Front 123

When can you expect a kid to be able to hop?

Back 123

4 years

Front 124

When can you expect a kid to catch something?

Back 124

5 years

Front 125

At what age can a kid copy a vertical line?

Back 125

2 years

Front 126

At what age can a kid copy a circle?

Back 126

2.5 years

Front 127

At what age can a kid draw a plus sign?

Back 127

3.5 years

Front 128

At what age can a kid draw a square?

Back 128

4.5 years

Front 129

At what age can a kid draw a triangle?

Back 129

5-6 years

Front 130

Head circumference rule of thumb?

Back 130

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

Front 131

Where do the calcifications appear in the brain of someone with a CSV infection?

Back 131

around the ventricles

Front 132

Where do the calcifications appear in the brain of someone with toxo?

Back 132

everywhere "*T*otally all over the place."

Front 133

What are MELAS and MERRF

Back 133

Mitochondrial disorders

Front 134

PKU and homocystenuria are what kind of disorders?

Back 134

metabolic

Front 135

What kind of disorders are Rhett's and Lesch-Nyhan?

Back 135

gray matter disorders

Front 136

What kind of disorders are adrenoleukodystrophy (x-linked) and metachromatic dystrophy?

Back 136

white matter diseases

Front 137

PKU is caused by the absence of what enzyme?

Back 137

phenylalanine hydroxylase

Front 138

What does a homocystinuria patient look like?

Back 138

Marfan's, lens dislocation, mental retardation and psychiatric illness.

Front 139

What is Tay-Sach's disease?

Back 139

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.

Front 140

What is Niemann-Pick disease?

Back 140

Symptoms appear sometime within the first year of life. Deficiency in sphingomylinase causes retardation, regression (key symptom), seizures, hypotonia, jaundice and hepatosplenomegaly

Front 141

What is Krabbe disease (also known as globoid cell leukodystrophy)?

Back 141

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

Front 142

Metachromatic leukodystrophy

Back 142

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.

Front 143

What does MELAS stand for?

Back 143

Myopathy, Encephalopathy, Lactic Acidosis, Stroke

Front 144

What does MERRF stand for?

Back 144

Myoclonus, Epilepsy/encephalopathy, Ragged Red Fibers (in the muscle)

Front 145

Gene for Rett syndrome?

Back 145

X linked Xq28, can be seen in XXY boys, lethal en utero for XY boys

Front 146

Age range for Rett syndrome apperance?

Back 146

6-18 months

Front 147

What's the inheritance pattern for Lesch-Nyhan syndrome?

Back 147

X-linked recessive, child nl until 1st year

Front 148

What's the deficient enzyme in Lesch-Nyhan syndrome?

Back 148

Hypoxanthine phosphoribosyl Transferase

Front 149

What's SSPE?

Back 149

Subacute sclerosing panencephalitis. A late complication of a MEASLES infection. Might show elevated anti-measles titer in CSF

Front 150

What is Adrenoleukodystrophy?

Back 150

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

Front 151

What substance abuse can look like a leukodystrophy syndrome?

Back 151

Touluene abuse

Front 152

Kleinfelter syndrome genetic anomaly? Physical features?

Back 152

XXY. Tall long and lean. Sterile but with nl penis. Gynicomastia and sparse 2ndary sexual characteristics, MR in 25-30%.

Front 153

Turner syndrom genetic anomaly? Physical features?

Back 153

XO. Mental retardation in 18%. short stature, webbed neck. High palate, flat nose, wide mouth. no ovaries.

Front 154

Fragile X syndrome genetic anomaly? Physical features?

Back 154

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)

Front 155

XYY syndrome

Back 155

Tall, tend to be prisoners, severe acne in puberty. Behavioral disorders in up to 50%, often with speech delay as a kid

Front 156

XXX syndrome

Back 156

some developmental delays, but otherwise no clinical symptoms. Sometimes have increased anxiety or depression.

Front 157

William's syndrome kids look like what? Retardation? Special gift?

Back 157

Elves, yes mild to moderate, musically gifted with perfect pitch and very sociable

Front 158

Prader-Willi & Angelman syndromes have what gene problem?

Back 158

AUTOSOMAL DOMINANT Micro-deletion or translocation on chromosome 15

Prader-willi is if inherited from dad
Angelman is if inherited from mom

Front 159

VCF (Velocardiofacial syndrome) is what? What psych diagnosis is it strongly associated with?

Back 159

-*V*elopharyngeal dysfunction (cleft palate, very nasal voice)
-*C*ardiac abnormalities
-*F*acial abnormalities

SCHIZOPHRENIA, up to 30%

Front 160

Differences between NF1 and NF2? Chromosomes?

Back 160

NF1 - "mostly everything's on the outside," chromosome 17

NF2 - "mostly everything's on the inside," chromosome 22

Front 161

How can you tell the difference between NF1, NF2 and Tuberous sclerosis complex?

Back 161

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.

Front 162

What is Ataxia-telangiectasia? What's the treatment?

Back 162

big, visible blood vessels in eyes and abdomen. Deficiencies in IgA and IgE. Progressive cerebellar ataxia, leukemia, lymphoma, diabetes, delayed sexual development. Treatment: IVIG

Front 163

What problems can a Sturge-weber patient have? What will the brain xray look like?

Back 163

seizures, intellectual impairment, glaucoma. Brain xray will look like waves.

Front 164

Neurologic causes of Austic symptoms ("Really Frustrating Autistic Toddler"):

Back 164

Rett
Fragile X
Angeleman
Tuberous Sclerosis

Front 165

Genetic Causes of Tall stature (XXY men kill homies):

Back 165

XXY syndrome
Marfan's
Klinefelter's
Homocystenuria

Front 166

Genetic causes of short stature ("Too Tiny Triple X"):

Back 166

Trisomy 21
Turner's XO
XXX syndrome

Front 167

Differences between NF1 and NF2?

Back 167

NF1 - "mostly everything's on the outside," chromosome 17

NF2 - "mostly everything's on the inside," chromosome 22

Front 168

How can you tell the difference between the NF syndromes and Tuberous sclerosis complex?

Back 168

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.

Front 169

What is Ataxia-telangiectasia? What's the treatment?

Back 169

big, visible blood vessels in eyes and abdomen. Deficiencies in IgA and IgE. Progressive cerebellar ataxia, leukemia, lymphoma, diabetes, delayed sexual development. Treatment: IVIG

Front 170

What problems can a Sturge-weber patient have? What will the brain xray look like?

Back 170

seizures, intellectual impairment, glaucoma. Brain xray will look like waves.

Front 171

Neurologic causes of Austic symptoms ("Really Frustrating Autistic Toddler"):

Back 171

Rett
Fragile X
Angeleman
Tuberous Sclerosis

Front 172

Genetic Causes of Tall stature ("XXY men kill homies"):

Back 172

XXY syndrome
Marfan's
Klinefelter's
Homocystenuria

Front 173

Genetic causes of short stature ("Too Tiny Triple X"):

Back 173

Trisomy 21
Turner's XO
XXX syndrome

Front 174

How you can tell weakness is a myopathy vs. a neuropathy.

Back 174

mypoathies are proximal weakness, neuropathies are distal.

Front 175

Genetic inheritance pattern of Duchenne's?

Back 175

sex linked recessive

Front 176

muscle membrane protein lost in
Duchenne's?

Back 176

Dystrophin

Front 177

Is there MR in Duchenne's?

Back 177

Can be, in 1/3 of cases. Also occasional cardiac involvement

Front 178

What's the genetic inheritance pattern in Myotonic dystrophy? The specific gene problem?

Back 178

autosomal dominant, trinucleotide repeats on #19

Front 179

pathophysiology of Myotonic dystrophy?

Back 179

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.

Front 180

consider mtDNA disorder when:

Back 180

episodic confusion with acidosis
strokes in kids and young adults
progressive ocular muscle paresis

Front 181

What else has a "dive bomber" sound on EMG besides Myotonic dystrophy?

Back 181

Myasthenia Gravis

Front 182

What symptoms are in botulism poisoning not found in Myasthenia Gravis?

Back 182

DILATED pupils. Also constipation in kids.

Front 183

What's the Eaton-Lambert syndrome associated with?

Back 183

small cell lung cancer

Front 184

What neurotransmitters does clostridium tetani interfere with?

Back 184

GABA and glycine

Front 185

Can you tell tetanus and strychnine poisoning apart, based on symptoms?

Back 185

no

Front 186

What is Werdig-Hoffman disease?

Back 186

ALS in kids.

: (

Front 187

What usually preceeds Guillain-Barre syndrome?

Back 187

GI or respiratory tract infection, but could be any infection

Front 188

What eye stuff does CN III enervate?

Back 188

up, down, in, pupil and lids

Front 189

What does CN VI enervate?

Back 189

Out gaze!

Front 190

If eyes deviate towards body hemiparesis are on the same side, where's the stroke?

Back 190

contralateral pons

Front 191

if eyes deviate opposite of hemiparesis, where's the stroke?

Back 191

contralateral frontal lobe

Front 192

What's the cause of a CN III palsy that is PAINFUL and spares the pupil?

Back 192

Diabetes, pain is caused by an infarction.

Front 193

What happens if the MLF is disrupted?

Back 193

CNIII nucleus affected, ipsilateral eye can't adduct. Rest of CN II functions remain intact.

Front 194

Most common cause of MLF being damaged?

Back 194

Multiple Sclerosis

Front 195

What spot damaged in the optic tracks will give you a bilat quadrantanopsia?

Back 195

optic chiasm (usually pituitary adenoma)

Front 196

Is it possible to get HIV related dementia and have good viral load counts/CD4 ct?

Back 196

YES

Front 197

Distinctive characteristics of HIV dementia?

Back 197

subcortical dementia, diffuse neurologic signs, extrapyramidal features, frontal release signs, gait impairment

Front 198

Mechanism of AIDS related dementia?

Back 198

probably cytokines, NOT primary neuronal infection

Front 199

What does AIDS related dementia look like on imaging?

Back 199

random, mostly symmetric subcortical white spots, not predominantly periventricular

Front 200

What infection is most common infectious mass lesion in HIV pt's? Treatment?

Back 200

Toxoplasmosis, treated with Pyrimethamine or sulfadiazine. Serum titers may be false negative.

Front 201

What's the most common HIV associated FUNGAL CNS infection? Treatment?

Back 201

Cryptococcus, amphotericin

Front 202

Most common cause of blindness in HIV?

Back 202

CMV retinitis

Front 203

What ages are most likely to get meningitis? What populations are most at risk?

Back 203

<5 and <60. Crowded populations: military barracks, college dorms, etc.

Front 204

What viral meningitis can also have RBC's in it?

Back 204

Herpes Simplex

Front 205

Where would a primary CNS lymphoma (B cell tumors) be most likely to show up on an MRI?

Back 205

Periventricular area

Front 206

On SPECT what's the difference between lymphoma and Toxoplasmosis?

Back 206

Lymphoma is "hot" and toxo is "cold."

Front 207

If you can't tell by imaging if lesions are cancer or toxo, what should you do?

Back 207

treat empirically for toxo before biopsying, it's safer

Front 208

If a VDRL is given as negative in a test question, should you assume it's not syphilis?

Back 208

NO! Negative in up to 25% of cases!

Front 209

What's with Argyll-Robertson pupils?

Back 209

They accomodate (to a close object) but don't react (to light).

Front 210

Where do first order sensory neurons synapse?

Back 210

dorsal root ganglion, talks to spinothalamic tract

Front 211

Nocioceptor fibers are which kind (2 types)?

Back 211

unmylinated C fibers and mylinated A-delta fibers.Memory aid: "A dull constant pain"

Front 212

Where does descending pain modulation come from?

Back 212

Periaqueductal gray and rafe nucleus (enkephalins and seratonin)

Front 213

What is Dejerine-Roussy syndrome?

Back 213

centrally caused pain 2ndary to thalamic infarction, presumably due to interruption to communication with the spinothalamic tract.

Front 214

Is there a widely accepted cure for intention tremor?

Back 214

Nope.

Front 215

what genetic disorder has a similar tremor to essential tremor?

Back 215

Fragile X

Front 216

Treatments for essential tremor:

Back 216

propranolol, metoprolol, primidone, DB stimulator in thalamus(Ventralis intermedius nucleus)

Front 217

Hallmarks of a psychogenic tremor.

Back 217

-oscilates in 2-3 planes
-variable frequency, amplitude, location
-fatigues
-when bilat it's symmetrical ("coherent")

Front 218

Corticospinal (pyramidal) tract does what? Is where? What's the major disease when it's lost?

Back 218

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")

Front 219

What do fasiculus gracilis and F. cuneatus (posterior columns) do?

Back 219

position, vibration and light touch.

Front 220

What does the lateral spinothalamic tract carry? From which side of the body?

Back 220

pain and temp from CONTRALATERAL body

Front 221

What does the spinocerebellar tract carry? What does spinocerebellar ataxia look like?

Back 221

feedback to cerebellum. High foot arch (pes cavus), decreased reflexes, loss of position and vibration sense.

Front 222

What's Friedrich's ataxia?

Back 222

Most common cause of inherited ataxia, it's a spinocerebellar ataxia. Caused by trinucleotide repeats, can be autosomal dominant or recessive.

Front 223

Symptoms of tabes dorsalis?

Back 223

lightening-like pains, position sense loss, high steppage gait, Argyl-Robertson pupil

Front 224

What's "combined systems disease" and what are the symptoms?

Back 224

b12 deficiency/pernicious anemia. Loss of lateral and posterior columns. Sometimes they give a vignette about eating raw fish?!

Front 225

What does copper deficiency look like? What are common vignettes used on the boards for this?

Back 225

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.

Front 226

What's a syrinx and what causes it?

Back 226

loss of pain and temp sensation from damage of the spinothalamic tract fibers *where they cross.* Position sensation intact. Usually in fingers

Front 227

What happens with an injury at C4?

Back 227

paralyzed below the neck, APNEA, bladder, bowel, sexual impairment.

Front 228

What happens with an injury at T4?

Back 228

paralyzed below the nipples, bladder, bowel, sexual impairment.

Front 229

What happens with an injury at T10?

Back 229

paralyzed below the belly button, bladder, bowel, sexual impairment.

Front 230

What happens with an injury at S1-3?

Back 230

leg extensor weakness, bladder, bowel, sexual impairment.

Front 231

What medication was mom probably taking if the kid has a meningocele or a menigomyelocele?

Back 231

valproate or carbamazapine

Front 232

What genetic syndrome is associated with meningocele or menigomyelocele?

Back 232

Arnold Chiari malformation

Front 233

What's a Dandy Walker malformation?

Back 233

cyst where cerebellum should be. No vermis! Also often with hydrocephalus and enlarged 4th ventricle.

Front 234

What's Brown-Sequard syndrome? What are the symptoms?

Back 234

lateral hemi-transection of the spinal cord. Ipsilateral paresis with hyeractive DTR's, and contralateral position, vibration, pain and temp sense loss

Front 235

What's cauda equina syndrome? Symptoms?

Back 235

Injury to L2-S5. pain in legs and low back, flaccid areflexic paraperesis, incontinence.

Front 236

What's the mechanism of action for Baclofen?

Back 236

GABA B agonist

Front 237

What's an MRI for an MS patient look like?

Back 237

Periventricular demylination/plaques (bright spots)

Front 238

Depression is most common in what type of MS?

Back 238

Relapsing/remitting

Front 239

What's fingolimod?

Back 239

An immunomodulator used for MS treatment.

Front 240

What's mitroxantrone?

Back 240

Immunomodulator for MS

Front 241

What's the problem with using Natalizumab (Tysabri) for MS patients?

Back 241

may allow development of PML!

Front 242

Which brain region is most sensitive to a lithium level > than 3.5?

Back 242

cerebellum (we don't know why)

Front 243

What's pseudobulbar palsy?

Back 243

emotional incontinence (after head injury)

Front 244

Rapid dementia with myoclonus, sporatic not inherited

Back 244

CJD

Front 245

What's Variant CJD?

Back 245

Genetically inherited (Brits) personality changes, sensory problems then cognitive changes in young adults (average age 27)

Front 246

What is SSPE?

Back 246

Subacute Sclerosing Panencephaliis, a reaction to measles, dementia and myoclonus with onset in childhood. DIAGNOSTIC: CSF contains anti-measles antibodies. 90% mortality.

Front 247

Where does Wernicke-Korsakoff cause hemorrhaging?

Back 247

lymbic system (mamillary bodies and periaqueductal gray)

Front 248

Can anorexia cause Wernicke-Korsakoff syndrome?

Back 248

Nope, almost never.

Front 249

What vitamin deficiency does bariatric surgery commonly cause?

Back 249

B12

Front 250

What's TGA (Transient Global Amnesia)? How can you tell it's not a fugue?

Back 250

anteriograde amnesia that lasts only hours, PERSONAL INFORMATION IS RETAINED unlike in a fugue.

Front 251

What do white striae on fingernails mean?

Back 251

Arsenic poisoning.

Front 252

What toxicity causes hair to fall out?

Back 252

Thallium

Front 253

When interpreting an EEG, what seizure type has regular spikes and waves?

Back 253

Absence

Front 254

What's a normal EEG background frequency?

Back 254

alpha 8-13 Hz

Front 255

What medications will speed up EEG?

Back 255

benzos, barbiturates

Front 256

What medications will slow down an EEG?

Back 256

Phenothiazines, lithium

Front 257

What medications will cause spikes in an EEG?

Back 257

Lithium, clozipine, TCAs

Front 258

What meds lower the seizure threshold?

Back 258

neuroleptics, TCAs, wellbutrin, benzo or barbiturate withdrawal

Front 259

What's non-convulsant status epilepticus look like?

Back 259

semi-purposeful behavior, intermittently responsive. R/o drug intoxication and fugue state.

Front 260

Treatment of choice for complex seizures (either simple or complex?

Back 260

oxcarbamaepine, carbamazapine, phenotoin, lamotrigine, topiramate

Front 261

What seizures happen in sleep?

Back 261

frontal lobe seizures. Sometimes ONLY happen during sleep. Weird looking, often easily mistaken for psychogenic.

Front 262

if a prolactin level is neg, is that proof the pt has a pseudoseizure?

Back 262

Nope.

Front 263

What two antiseizure meds are renally cleared?

Back 263

gabapentin and levetiracetam

Front 264

medications of choice for tonic-clonic seizures?

Back 264

valproate, carbamazepine, phenytoin

Front 265

How does one treat erythema multiforme after you've caused it with one of our anticonvulsants?

Back 265

steroids!

Front 266

What can phenobarbital do to kids?

Back 266

Cause hyperactivity!

Front 267

Major carbamazepine side effects

Back 267

diplopia, osteopenia, leukopenia, liver damage, neural tube defects, hyponatremia (though not as bad as oxcarbazepine)

Front 268

LAbs you need to monitor for carbamazepine? Is it an inducer or an inhibitor of P450

Back 268

lft's, CBC, platelets, sodium and levels monthly for 3 months, then at least quarterly. It's a p450 inducer.

Front 269

Who should you not give carbamazepine to?

Back 269

People of asian descent, since they're at higher risk for Stevens-Johnson syndrome.

Front 270

What's a side effect of valproate for unborn infants?

Back 270

lower IQ.

Front 271

If a patient on valproate gets a tremor, what should you do? What if they get abdominal pain?

Back 271

Check ammonia level. Check amylase level.

Front 272

what two antiepileptic drugs can cause psychosis and aggression?

Back 272

Keppra and zonisamide

Front 273

What causes burst-suppression in an EEG?

Back 273

post anoxic injury

Front 274

what causes 3hz spike and wave in an EEG?

Back 274

Absence seizure

Front 275

What causes slow disorganized background in an EEG?

Back 275

delirium

Front 276

What causes periodic complexes in an EEG?

Back 276

CJD

Front 277

What kind of sleep causes sleep spindles and K complexes in an EEG?

Back 277

Non-rem sleep

Front 278

what causes 8-12hz occipital rhythm?

Back 278

Nothing, that's normal

Front 279

What anti epileptic drug can cause PCOS?

Back 279

valproic acid

Front 280

Are suicide rates the same amongst those with seizures and the nl population?

Back 280

No! 4-5 times higher!

Front 281

What may aggressive, violent behavior be a manifestation of?

Back 281

frontal lobe seizures.

Front 282

What's postictal psychosis?

Back 282

Psychosis that shows up days after a psychosis, usually after a lucid period

Front 283

What's interictal psychosis?

Back 283

hallucinations and thought disorder with clear sensorium. Not temporally related to seizures. DURATION OF 6 MONTHS if not treated. NOT genetic.