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1
Q

In which demographic do sx of Ducheene Muscular dystrophy present?

A

males before age 6

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2
Q

Diagnostic criteria for CRPS (Budapest Criteria)?

A

Budapest Criteria:
At least ONE sx in 3/4 of these categories:

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3
Q

Difference between rigidity and spasticity?

A
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4
Q

What is Capgras’ Syndrome?

A

delusion that can be seen in paranoid schizophrenia in which the patient thinks a familiar individual (usually a spouse or relative) has been replaced with a body double or imposter.

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5
Q

WHich nerve runs through the mandible?

A

inferior alveolar nerve and mental nerve: supplies sensation to the lower lip

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6
Q

MOA of lasmitidan

A

Serotonin 5-HT-1F agonist

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7
Q

MOA of triptan?

A

5-HT1B and D agonist

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8
Q

MOA of -gepants?

A

CGRP antagonists

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9
Q

What chromosome is associated with narcolepsy?

A

6

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10
Q

Which second gen antipsychotic is associated with the greatest inc risk of seizures?

A

Clozapine

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11
Q

Antibody that is significantly higher in patients with MG + thymoma vs MG alone

A

Anti-striated muscle Ab

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12
Q

WHat medications can DEC valproic acid levels?

A
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13
Q

Enzyme deficient in PKU?

A

phenylalanine hydroxylase

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14
Q

Patient’s w/ DiGeorge(Velocardiofacial) Syndrome have 20x inc risk of?

Characteristics of this syndrome?

A

Schizophrenia

CATCH-22

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15
Q

Most common mets to brain?

A

LUNG (#1)
Melanoma
Breast

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16
Q

Multifocal Motor Neuropathy: Antibody and Treatment?

A

anti-GM1, IVIG (does not respond to steroids or PLEX)

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17
Q

What is hippus?

A
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18
Q

BPPV is due to canalolithiasis how?

A

otoliths separate from their attachments to the utricle and saccule.

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19
Q

Mcardles Disease: deficiency in ? due to?

A

myophosphorylase

mutations in PYGM gene

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20
Q

In McArdle’s disease, there is a phenomenon on EMG in which?

A

muscles actively having a contracture while undergoing EMG are electrically silent

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21
Q

Histology for schwannoma?

A

verocay bodies

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22
Q

WHat is the Monro Kellie Doctrine?

A

Sum of the volumes of brain, CSF, and intracranial blood remain constant. Any inc in one causes a decrease in 1 or 2 of the other.

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23
Q

Any role for antivirals in Bells Palsy?

A

Apparently there is a scale for Bells (House-Brackman scale) and it is only sometimes used for severe Bell’s (H-B 4 or greater)

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24
Q

Most common intraocular neoplasm in children? Assoc with what PE finding?

A

Retinoblastoma

Leukocoria

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25
MOA of buspirone?
Partial agonist at 5HT1A serotonin receptors and D2 dopamine receptors
26
benzos with least affect on the liver?
oxazepam and lorazepam
27
Common causes of painless drug induced myopathy?
28
Which antipsychotics are generally the safest during pregnancy?
First gens, like haldol CBZ, VPA, Lithium are all contraindicated.
29
What age range is most common for non-REM sleep arousal disorders?
20-30 yo
30
Most common tumors in the CP angle?
1. acoustic schwannoma - T2 bright, no restricted diff 2. Meningioma - same 3. Epidermoid cyst - iso, restricted diff.
31
MOA of topiramate?
BLOCKS: NA, carbonic anhydrase, glutamate STIMS: GABA
32
Absence EEG?
3Hz spike and slow wave
33
Manganese Toxicity findings?
T1 changes in globus pallidus/subthalamic nucleus on MRI Sx: "manganese madness": cognitive changes/AMS + Parkinsonism. Tx: Levodopa/EDTA (partial/no benefit)
34
What genes are associated with familial hemiplegic migraine?
FHM1: CACNA1A FHM2: ATP1A2 FHM3: SCN1A
35
Inheritance of Niemann Pick?
AR
36
Deficiency in Niemann Pick?
deficiency of Sphingomyelinase
37
Sx of Niemann Pick?
38
Peak incidence of vasospasm after SAH?
6-8 days, but can occur anywhere from 3-15d
39
intercostal nerve travels?
along the inferior border of the corresponding rib
40
Most common side effects of clozapine?
Sedation, drooling/hypersalivation
41
elevations in CHIN EMG on PSG is indicative of?
Bruxism
42
Main differences between fosphenytoin and phenytoin?
Fosphenytoin is more water soluble, allowing IV administration without as many side effects such as purple glove syndrome.
43
Findings in Mercury toxicity?
found in thermometers, dental fillings. Causes gingivitis, tremor, behavioral changes.
44
Findings in n-hexane toxicity?
found in many household glues. Causes symmetric ascending motor neuropathy similar to GBS, as well as autonomic dysfunction such as bluish discolorations on the feet and numbness in the toes. Can also cause facial numbness, maculopathy and optic neuropathy.
45
Findings in acrylamide toxicity?
Found in wastewater management, ore processing, gels. palmar erythema/dermatitis, ataxia and encephalopathy
46
Findings in carbon disulfide toxicity?
found in perfumes/insecticides Encephalopathy, seizures, coma respiratory failure, mucosal irritation and burning in the nose (it is typically inhaled)
47
Findings in ethylene oxide toxicity?
found in pesticides. Causes encephalopathy and pulmonary edema and peripheral neuropathy down the line.
48
Lesion of which nerve leads to hoarseness in the voice?
Recurrent laryngeal nerve.
49
Lesion at the nucleus ambiguus would cause?
50
Lesion at the nucleus tractus solitarius would cause?
51
Lesion of the vagus nerve proximal to the pharyngeal branch would cause?
sensory defects of the pharynx, as well as hoarseness.
52
CNS lyphoma in patients with AIDS almost always have?
EBV in the CSF
53
What nerve is most commonly affected in Lyme disease assoc neuropathies?
Facial nerve
54
What is the criteria to diagnose major cognitive disorder?
55
Sx of Klein Levin Syndrome?
excessive daytime sleepiness + hypersexuality, hyperphagia, aggressiveness
56
Most common cause of spinal epidural abscess?
staphylococcus
57
What therapy is used in conduct disorder?
Multi-systemic therapy?
58
What therapy is used in anxiety/depression?
CBT
59
What therapy is used in borderline?
DBT
60
Geschwind Syndrome?
Epilepsy that originates in temporal lobe with impaired awareness: focal seizures + hyper-religious, hyposexual, hypergraphia, intense emotional responses.
61
Findings in LEMS on EMG?
incremental increase in CMAP amplitude after repetitive stimulation at high frequency.
62
Telencephalon becomes?
Cerebral hemispheres, limbic system, basal ganglia
63
Diencephalon becomes?
thalamus, hypothalamus, optic vesicle
64
Mesencephalon becomes?
midbrain, red nucleus, substantia nigra, periaqueductal grey matter
65
Metencephalon becomes?
pons/cerebellum
66
myelencephalon becomes?
medulla
67
which drug can show a false positive for PCP?
venlafaxine
68
Triad of spasmus nutans?
Head positioning abnormalities (torticollis), head bobbing, nystagmus
69
Most common causes of RCVS?
triptans, SSRI, marijuana
70
What medication has been shown to be an effective adjunct for OSA?
modafinil
71
What is the first line treatment for REM sleep behavior disorder?
Melatonin
72
Why do 2nd gen antipsychotics have less EPS sx?
because they are also partial antagonists of 5HT2A serotonin receptors.
73
Skin lesion associated with Fabry disease?
Angiokeratomas
74
triad in medial medullary syndrome?
75
Gene and inheritance associated with Dravet Syndrome?
SCN1A, AD
76
What medications can increase the metabolism of OCPs?
Topiramate, carbamazepine, OXC, phenytoin, phenobarbital,
77
Unique aspect of phenytoin metabolism?
Zero-order kinetics: constant amount of drug eliminated per unit of time, regardless of concentration.
78
Unique aspect of phenytoin metabolism?
Zero-order kinetics: constant amount of drug eliminated per unit of time, regardless of concentration.
79
Which medication can worsen absence epilepsy?
oxcarb
80
deep brain stimulation for drug resistant epilepsy targets what?
anterior nucleus of the thalamus: believed to cut off Papez circuit
81
MOA of ethosuxamide
T type calcium channel blockade
82
Side effects of dalfampradine?
decreases seizure threshold, renal injury
83
Tx for Rasummen encephalitis
hemispherectomy
84
postictal nose wipe localization?
usually ipsilateral temporal lobe
85
Cerebral palsy most common cause?
intrauterine injury, most commonly infarcts and hypoxemic injury
86
Treatment for bruxism in children?
it is common and usually self limiting, no tx is needed
87
Ulrich's congenital muscular dystrophy?
dystrophy (absent reflexes/hypotonia) + shoulder contractures and distal limb hyper-laxity
88
Walker-Warburg muscular dystrophy?
dystrophy (absent reflexes/hypotonia) + ocular abnormalities like cataracts
89
Margaret Mahler's stages of development
1. Autistic phase 2. symbiotic phase 3. separation-individuation phase
90
What class of drugs can cause drug induced PD?
classically high potency first gen antipsychotics
91
HSAN types 1-4
1: AD, BL LE sensory loss/pain + hypohidrosis 2: AR, loss of sensation + insensitivity to pain => mutilation of hands/feet/lips, no autonomic sx 3: AR, autonomic dysfunction without any numbness 4: congenital insensitivity to pain and anhidrosis
92
Features of congenital syphilis?
deafness, interstitial keratitis and Hutchinson teeth (pegged shaped incisors)
93
Neuroacanthocytosis - inheritance, mut, pres
AR VPS13A gene mutation chorea, seizures, areflexia, tics, orolingual-facial dystonias that lead to mouth and lip biting, violent trunk spasms that can lead to head banging, frontal lobe disinhibition, psychosis
94
MOA of memantine?
low affinity antagonist of NMDA rec
95
there is a relationship between NMS and which element?
low iron stores it is believed that this reduced dopamine receptor function
96
What is a positive finding on FDG PET in patients with epilepsy?
reduced glucose uptake in the pathologic site
97
which hypnotic has the shortest half life?
zaleplon
98
Which therapy has the most evidence for SUBSTANCE abuse?
Family therapy
99
When a patient with tremor has ASTHMA, whats the best medication?
propranolol is CONTRAINDICATED, so primidone is first line in this case.
100
What cofactor is needed to normalize a patient's thiamine (ex: an alcoholic with Wernicke's)
Magnesium

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