Rite Exam Neurology

Front 1

sx in serotonin syndrome but not in NMS

Back 1

shivering and myoclonus

Front 2

deletion PMP22

Back 2

hereditary neuropathy with liability to pressure palsies

Front 3

duplication PMP22

Back 3

CMT 1a

Front 4

orofacial dyskinesias lingual and oral dystonia in a 30-year-old patient chorea and peripheral polyneuropathy.

Back 4

neuroacanthocytosis, "thorn" shaped blood cells from membrane dysfunction

Front 5

dominant (left) posterior cerebral artery occlusion

Back 5

alexia w/o agraphia, complete right homonymous hemianopsia, poor color naming

Front 6

leg crossing

Back 6

peroneal palsy from weight loss

Front 7

nystagmus and mild gait btw ataxic attacks. tx acetazolamide. CACNA1A gene on chromosome 19.

Back 7

episodic ataxia 2

Front 8

HA frequent (7 to 22 per day)
episodes of unilateral, severe, but short-lasting (5 to 45 minutes) Indomethacin is the treatment of choice.

Back 8

Paroxysmal hemicrania

Front 9

Unilateral anosmia and optic neuropathy in conjunction with signsof raised ICP (papilledema in the opposite eye) are the clinical features of this disorder, which is most
commonly due

Back 9

to a planum sphenoidale meningioma that could involve both the olfactory groove and optic
canal. The other disorders listed would not typically have this constellation of signs and symptoms.
Foster-Kennedy syndrome

Front 10

Grade II concussion

Back 10

(no loss of consciousness but concussion symptoms greater than 15
minutes) and a more recent Grade III concussion(any loss of consciousness). Guidelines state he should be out
of play for 1 week in this setting of recurrent concussion.

Front 11

stiff person syndrome tx

Back 11

diazepam

Front 12

Anton's syndrome (denial of blindness).

Back 12

It is a result of severe acute bilateral injury to the
medial occipital lobes and adjacent association cortex, ischemia PCA. The syndrome has also occured after traumatic injury to the optic nerve with
associated bifrontal lobe contusions.

Front 13

Mitoxantrone

Back 13

secondary progressive ms

Front 14

Duchenne's muscular dystrophy gene?

Back 14

which is characterized by X-linked recessive
inheritance and a mutation of the dystrophin gene located on Xp21.2

Front 15

toxoplasmosis tx

Back 15

Pyrimethamine

Front 16

tuberous sclerosis

Back 16

mutations tuberin and merlin

Front 17

congenital inability to decarboxylate leucine, isoleucine, and
valine. There is an associated severe metabolic acidosis and hypoglycemia. Death 1st/2nd wk. opisthotonus.

Back 17

Maple syrup urine disease (MSUD) is due to a

Front 18

Brain death o2

Back 18

Presidents Commission recommeded 100% oxygen ventilation for 10 minutes, followed by passive 100%
oxygen for a period long enough to achive a PCO2 of 60.

Front 19

the most common cause of noncommunicating hydrocephalus.

Back 19

normal term infant is 35 cm. A head circumference of 45 cm is greater than the 98th percentile. In the absence of other cerebral malformations, congenital aqueductal stenosis most common

Front 20

coprolalia

Back 20

tourettes swearing, occurs in late adolescence

Front 21

descending paralysis involving
cranial nerves, neck, and shoulder girdle.Sluggish or fatiguable pupils

Back 21

botulism. tx: Human botulism immunoglobulin

Front 22

centrotemporal sharp waves presents from 2 to 13 years of age

Back 22

Benign focal epilepsy childhood. 67% infrequent sz. Carbamez prn

Front 23

striking peripheral neuropathy.

Back 23

Metachromatic leukodystrophy

Front 24

Rett's syndrome has micro or macro-ceph?

Back 24

micro

Front 25

diseases have megaloencephaly and hyperreflexia.

Back 25

Alexander, Canavan, and Tay-Sachs

Front 26

status epilept

Back 26

phenobarbital load 20mg/kg then additional 5 mg/kg x1 prn.
midazolam 0.2 mg/kg slow IV bolus then 0.75 mCg/kg/min to 10 mCg/kg/min IV drip;
pentobarbital 5 mg/kg to 15 mg/kg IV bolus over 1 hour followed by 0.5 mg/kg/h to 3.0 mg/kg/h IV drip.

Front 27

progressive extrapyramidal dysfunction, dementia, gait impairment, dystonic posturing, dysarthria, and loss of memory. Onset is most commonly in late childhood. Fe deposition, eye of the tiger sign.

Back 27

pantothenate kinase
deficiency (PKAN).

Front 28

A child from a hilly region in the Upper Midwest presenting
during the late summer with fever, headache, stiff neck, and focal seizures has....

Back 28

La Crosse encephalitis.

Front 29

newborn with microcephaly, organ
involvement, and subependymal calcifications

Back 29

CMV

Front 30

an X-linked dominant syndrome, presumed lethal in males, with agenesis of corpus
callosum, retinal lacunae, development disabilities, and frequently associated with infantile spasms.

Back 30

Aicardi syndrome is

Front 31

Clusters of head drops with flexor spasms of the trunk occuring around meal time in an otherwise healthy
7-month-old with a normal EEG suggest

Back 31

benign myoclonus of infancy.

Front 32

weakness, ataxia, absent tendon jerks, Babinski sign, abnormal position sense. Romberg sign Hammer toes, pes cavus deformities, and scoliosis

Back 32

Friedreich's ataxia

Front 33

chronic transfusion to keep hemoglobin S below 30%

Back 33

sickle cell dz

Front 34

Inc intracran pressure from a toxin (peds)

Back 34

lead

Front 35

vitamin cause of pseudotumor

Back 35

Vitamins A or D can cause
pseudotumor cerebri,

Front 36

BPPV in peds predicts what dz l8r?

Back 36

migraines

Front 37

generalized, fast (4 Hz to 6 Hz) spike-and-wave complexes.

Back 37

juvenile myoclonic epilepsy generalized, fast (4 Hz to 6 Hz) spike-and-wave complexes. Valproic acid is the treatment of
choice and recurrence of seizures is likely to occur if treatment is stopped.

Front 38

congenital myasthenic syndromes

Back 38

are not related to an immune process but are caused by genetic defects
affecting the neuromuscular junction. These include defects in acetylcholine synthesis and packaging (familial
infantile myasthenia gravis), end-plate deficiency of acetylcholinesterase, acetylcholine receptor deficiency, and
the slow channel syndrome.

Front 39

opsoclonus, myoclonus, ataxia syndrome, alternatively called "OMS", "dancing eyes
syndrome" or Kinsbourne's Syndrome)

Back 39

OMS: a paraneoplastic manifestation of occult neuroblastoma CT chest, abdomen, and
pelvis using oral contrast

Front 40

driving w/ dementia, clinical dementia rating...

Back 40

0.5 eval, 1 strongly consider not driving

Front 41

right parietal lobe mass showed a tumor composed of cells with monotonous round nuclei
surrounded by prominent perinuclear halos ("fried egg" apppearance). These features are characteristic of
oligodendroglioma.

Back 41

These features are characteristic of
oligodendroglioma.

Front 42

a cephalic disorder that refers to the disproportionate enlargement of the occipital horns of the lateral ventricles and is usually diagnosed early after birth due to seizures. I

Back 42

Colpocephaly is

Front 43

schizencephaly

Back 43

brain loss with fluid-filled component entirely lined by heterotopic grey matter (porencephalic cyst is lined mostly by white matter. ) SG, PW = Sharon Gan POW!

Front 44

Leptomeningeal enhancement, parenchymal enhancement, and, especially, pituitary stalk/hypothalamic
involvement in a patient with cranial nerve symptoms is characteristic of

Back 44

neurosarcoidosis.

Front 45

pachygyria

Back 45

neuronal migration

Front 46

T2-weighted sequence (T2-CISS) shows a cluster of vessels on the dorsal aspect of the cord compatible with
venous dilation due to arteriovenous shunting by a dural fistula.

Back 46

Foix-Alajouanine

Front 47

supplies the anteroinferior portion of the caudate nucleus, the
putamen, and the anterior limb of the internal capsule. off the ACA

Back 47

recurrent artery of Heubner arises from the A1
segment of the anterior cerebral artery and

Front 48

It is almost always found just posterior to the foramen of Monro in the anterior aspect of the third ventricle, originating from the roof of the ventricle. Because of its location, it can cause obstructive hydrocephalus

Back 48

colloid cyst is a cyst containing gelatinous material in the brain.

Front 49

_________(tumor) typically produce a large cyst with superficially positioned mural nodule. Most
lesions arise within the temporal or parietal lobes.

Back 49

Pleomorphic xanthoastrocytoma

Front 50

most common site for hypertensive hemorrhage

Back 50

Putamen, also thalamic haemorrhage
pontine haemorrhage
cerebellar haemorrhage

Front 51

open ring on MRI

Back 51

(Schilder's) variety of multiple sclerosis (large tumor-like in appearance, bilateral.

Front 52

pseudorosettes

Back 52

ependymoma

Front 53

pseudopalisading

Back 53

GBM

Front 54

fried egg

Back 54

oligodendroglioma

Front 55

MRI spine: hyperintense signal is seen on both T1-weighted and T2-weighted images,

Back 55

Benign lipoma

Front 56

The microscopic appearance is that of a capillary-rich neoplasm with abundant foamy cells containing lipid.

Back 56

hemangioblastoma The tumor arises independently or in conjunction with von Hippel-Lindau disease.

Front 57

tumor: are composed of astrocytes with elongate, bipolar, eosinophilic cell
processes; these tumors often show Rosenthal fibers.

Back 57

Pilocytic astrocytomas, also
commonly found in the cerebellum,

Front 58

tumor: commonly found in the cerebellum, composed of astrocytes with elongate, bipolar, eosinophilic cell
processes; these tumors often show Rosenthal fibers.

Back 58

Pilocytic astrocytomas,

Front 59

Toxoplasmosis resides also in basal ganglia, but then there is usually also mass effect.
________enters the intracranial space through the perivascular spaces, and the organisms are surrounded by
gelatinous material that is seen as high signal foci. This correlates with "soap-bubble" abscesses seen grossly.

Back 59

The correct answer is cryptococcus infection.

Front 60

____is caused by migrational arrest of the
affected neuroblasts. The cause of the incomplete migration is unknown. Three kinds of heterotopic gray matter
have been described: (1) nodular (periventricular), (2) laminar (in deep white matter), like in this patient, and (3)
band heterotopia (double cortex).

Back 60

Heterotopia

Front 61

__________________ occurs in Hispanic
individuals and is characterized by intraparenchymal cavernous malformations that can produce seizures,
impairment of function, or hemorrhage. The photograph illustrates a multi-channeled vascular malformation
composed of closely juxtaposed hyalinized, non-arterial vessels.

Back 61

Autosomal dominant cerebral cavernous angioma (malformation) (CCM1) syndrome

Front 62

________________ the most common of the neural tube closure defects. Folate supplementation has been
known to lower the recurrence risk of these defects however a recent report also documents the presence of
anti-folate receptor antibodies in some pregnancies complicated by neural tube closure defects.

Back 62

anencephaly,

Front 63

______________ typically produce a large cyst with superficially positioned mural nodule. Most
lesions arise within the temporal or parietal lobes. This lesion is surrounded with minimal hemosiderin rim but
no edema, and this excludes abscess and hematomas.

Back 63

Pleomorphic xanthoastrocytoma

Front 64

The signal characteristics heterogeneous (popcorn).

Back 64

cavernous hemangioma

Front 65

Megalocephaly (abnormally enlarged head). defective ASPA gene which is responsible for the production of the enzyme aspartoacylase.
early infancy and progress rapidly, may include mental retardation, loss of previously acquired motor skills, feeding difficulties, abnormal muscle tone (i.e., floppiness or stiffness), poor head control, Paralysis, blindness, or seizures may also occur.

Back 65

Canavan disease, also called Canavan-Van Bogaert-Bertrand disease, aspartoacylase deficiency or aminoacylase 2 deficiency,[1] is an autosomal recessive[2] degenerative disorder that causes progressive damage to nerve cells in the brain.

Front 66

dorsal column PLUS dorsal spinocerebellar columns

Back 66

Friedrich's ataxia. autosomal recessive inherited disease that causes progressive damage to the nervous system, resulting in symptoms ranging from gait disturbance to speech problems; it can also lead to heart disease and diabetes.

Front 67

intradural, extramedullary mass in the thoracic spine is causing significant cord compression.

Back 67

This
represents either neurofibroma or meningioma

Front 68

temp lesion: T1-weighted images and high signal on T2-weighted images. Enhances.

Back 68

HSV

Front 69

spongiform change of the cerebral cotex . Neuronal loss and
gliosis

Back 69

consistent with a prion disease

Front 70

coronal images note that the sulci at the high parietal convexity are compressed, as
compared to the markedly dilated posterior extent of the Sylvian fissure.

Back 70

communicating hydrocephalus

Front 71

is associated with periodic long-interval diffuse discharges in the EEG that
recur every 4 to 15 seconds.

Back 71

Subacute sclerosing panencephalitis from measles/rubeola)

Front 72

CT and MRI scan findings are highly consistent with Cerebral lesions in _____________ are most frequently found in the parieto-occipital area. On CT scans, parenchymal calcification is found
adjacent to the leptomeningeal lesions in a pericapillary distribution in the fourth cortical layer, possibly
secondary to chronic tissue hypoxia. Noncontrast MR images in SWS typically show unilateral cerebral atrophy
with enlargement of the ipsilateral subarachnoid spaces and ventricular cavities. Enlarged tubular regions of
flow void and gyriform hypointensities (decreased signal on T1-weighted and T2-weighted images) reflect
venous collateralization and cortical "serpentine" calcification, respectively

Back 72

Sturge-Weber syndrome (SWS).

Front 73

Familial frontotemporal dementia chromosome?

Back 73

that arises from a mutation on chromosome 17 results in a mutation of the
gene encoding for tau protein.

Front 74

inorganic arsenic, lead, mercury, and thallium intox can cause......

Back 74

Peripheral neuropathies can be caused by

Front 75

Aluminum intoxication
can produce

Back 75

encephalopathy but does not produce a neuropathy.

Front 76

open
ring,

Back 76

a finding characteristic of the large lesion (Schilder's) variety of multiple sclerosis.

Front 77

subacute process, with quick worsening

Back 77

listeriosis preferencefor the brainstem.

Front 78

popcorn

Back 78

cavernous anioma

Front 79

meningioma, neurofibroma, or schwannoma...do they enhance?

Back 79

yes!

Front 80

refers to increased perfusion in the periphery of an ischemic area. It is best documented with
PET or SPECT. Some authors have used the term to refer to gray matter enhancement, which is best seen on
MRI 3 days to several weeks after a cerebral infarct and results from contrast leaking into the perivascular space
in an area of ischemic insult.

Back 80

Luxury perfusion

Front 81

Toluene abuse or "glue sniffing" Huffing glue causes.....

Back 81

as become widespread, especially among children or ... imaging (MRI) findings have shown diffuse white matter demyelination and gliosis (diffuse leukoencephalopathy_

Front 82

Dolichoectasia (fusiform aneurysm) most commonly affects the supraclinoid segment of the internal carotid
artery and the basilar artery, and is seen in patients with>>>>

Back 82

advanced cerebral atherosclerosis.

Front 83

is chronic inflammation of the brain, with infiltration of T lymphocytes into the brain tissue. This affects only one cerebral hemisphere,

Back 83

Rasmussen's encephalitis

Front 84

Critical care myopathy is an underrecognized disorder characterized by selective loss of ________ from myofibers.

Back 84

myosin.
Also known as myosin-losing myopathy, this disorder is most commonly seen in critically ill patients treated
with corticosteroids and neuromuscular blockers.

Front 85

methanol poisoning causes damages to _____ part of the brain

Back 85

Hemorrhagic necrosis of the putamen is characteristic of

Front 86

_________ classically presents with progressive asymmetric apraxias, rigidity, and/or aphasia due
to neuronal loss and atrophy of the peri-Sylvian region. Ballooned neurons and astrocytic plaques with
tau-immunoreactivity are a diagnostic feature of _________

Back 86

Corticobasal degeneration

Front 87

anti jo

Back 87

dermatomyositis

Front 88

perifasicular atrophy

Back 88

dermatomyositis

Front 89

Neurogenic atrophy (group atrophy) of skeletal muscle is characteristic of

Back 89

spinal
muscular atrophy type I (Werdnig-Hoffman disease). Crappy, most kids die >2 years

Front 90

The lesions (cysts) most pronounced in the distribution of the anterior and middle cerebral arteries. They are the
result of circulatory disturbances during the latter half of pregnancy or the neonatal period.

Back 90

multicystic encephalopathy or multicystic encephalomalacia.

Front 91

capillary-rich neoplasm with abundant foamy cells containing lipid. This
is a hemangioblastoma. The cell origin of the hemangioblastoma is still unknown, despite many investigative
efforts. The tumor arises independently or in conjunction with __________ disease. The most common
location is in the cerebellum, but the spinal cord can also be a site of origin.

Back 91

von Hippel-Lindau disease.

Front 92

__________ syndrome occurs in Hispanic
individuals and is characterized by intraparenchymal cavernous malformations that can produce seizures,
impairment of function, or hemorrhage. The photograph illustrates a multi-channeled vascular malformation
composed of closely juxtaposed hyalinized, non-arterial vessels.

Back 92

Autosomal dominant cerebral cavernous angioma (malformation) (CCM1)

Front 93

________ infarcts often result in profound apathy, lack of motivational intent, akinetic mutism,
weakness of the lower extremities and urinary incontinence.

Back 93

Bilateral anterior
cerebral artery territory

Front 94

periventricular leukomalacia (PVL) occurs at what age

Back 94

prematurity

Front 95

nucleus of________ of the sacral cord is spared in motor neuron disease

Back 95

Onufrowicz

Front 96

Paraneoplastic cerebellar degneration is most commonly associated with what cancers.....

Back 96

ovarian carcinoma and some breast
tumors. It can also be seen with Hodgkin's lymphoma and small cell carcinoma of the lung.

Front 97

_______ nucleus is site of the major cholinergic projections to the cerebral
cortex, and with depopulation of this projection nucleus there is a corresponding reduction in cortical choline
acetyltransferase.

Back 97

basal nucleus

Front 98

_________ toxicity has birefringent calcium oxalate deposits in and around blood vessels. Oxalic acid
crystals may be detected in the urine.

Back 98

Ethylene glycol

Front 99

__________ results from missense mutations of the Notch 3 gene on chromosome 19. The small deep white matter vessels
show replacement of the media by eosinophilic, periodic acid-Schiff positive, Congo red negative, granular
material. Ultrastructurally, there is compact electron-dense material known as granular osmophilic material
surrounding myocytes in the arterial media. Although the predominant effects of ________ are on the central
nervous system, it is a systemic vasculopathy that can be appreciated on skin or nerve biopsy. Vasculitis is not
seen in this dz.

Back 99

dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

Front 100

______ has the following: astrocytic hamartomas
called tubers and candle gutterings are present. Subependymal giant cell astrocytomas are true neoplasms with malignant degeneration.
in the vicinity of the foramen of Monro. These tumors may produce CSF obstruction, and they may rarely

Back 100

TSclerosis

Front 101

_____ is a variant of meningioma that often has aggressive clinical behavior with numerous
recurrences and the potential for metastasis. Grade III.

Back 101

Rhabdoid meningioma

Front 102

The type of edema seen with acute, obstructive, high-pressure hydrocephalus

Back 102

is interstitial edema.

Front 103

________ edema is seen when high arterial pressures overcome cerebrovascular resistance and drive water across the
capillary walls into the extracellular space.

Back 103

Hydrostatic

Front 104

_________ is the most frequent type of edema and is seen
around tumors or traumatic lesions.

Back 104

Vasogenic edema

Front 105

cytotoxic edema is most commonly caused by

Back 105

hypoxia.

Front 106

Neurosarcoidosis anatomic site most common......

Back 106

hypothalamus

Front 107

cadasil which chromosome

Back 107

19, notch 3

Front 108

triptans act where?

Back 108

5HT1 receptors,
specifically 1b and 1d, resulting in decreased activity in the trigeminovascular system. Drugs acting at
adrenergic, specifically beta and alpha, receptors provide prophylactic effects.

Front 109

____________________ are molecular gateways in sensory systems, an interface between
the environment and the nervous system. Several (channels) transduce thermal, chemical, and mechanical stimuli
into inward currents, an essential first step for eliciting thermal and pain sensations.

Back 109

Transient receptor potential (TRP) ion channels

Front 110

Cluster headache It may be triggered by ______ . TX w/ ______ or (other options) acutely. Tx ppx w/ ______.

Back 110

It may be triggered or exacerbated by vasodilating substances such as nitroglycerin, histamine, and
ethanol. Drugs such as triptans, steroids, and dihydroergotamine may also be helpful in acute headache
management. Verapamil, lithium, and methysergide can be effective prophylactic agents for cluster headaches.

Front 111

Cessation of electrical activity due to hyperpolarization that occurs acutely after an ischemic insult is the result
of activation of _____ channels.

Back 111

potassium (K+) membrane

Front 112

Direct dopa agonists: name and SE

Back 112

Pramipexole, bromocriptine, , ropinirole, rotigotine, and injectable apomorphine
SE neuropsychiatric hallucinations

Front 113

dopa SE

Back 113

dyskinesias

Front 114

__________________ is a prodrug that is transformed in the liver to a potent agonist of alpha receptors in arteries and veins.
Given its predictable absorption and peak effect, it is the sympathomimetic of choice for treatment of
neurogenic orthostatic hypotension. One common side effect is scalp pruritus, a manifestation of alpha
adrenergic mediated piloerection. Its most potentially serious side effect is supine hypertension.

Back 114

Midodrine

Front 115

RLS worse with >>>>, tx w/ >>>>>

Back 115

iron deficiency, uremia, pregnancy,
neuropathy, and possibly other conditions. Among the current treatment options offered for the treatment of
RLS, dopaminergic agents cabergoline, lisuride, pergolide, (pramipexole, ropinirole are non-ergot fewer SE), rotigotine, have provided the best evidence for efficacy in symptom relief.

Front 116

is characterized by progressive loss of pain and temperature sensation,
lancinating pain, and severe generalized autonomic failure. Mutations transthyretin, the protein that transports thyroxine and retinol binding protein.

Back 116

Type 1 (Andrade) familial amyloidosis, inherited as an autosomal dominant, is associated with amyloid
deposition in peripheral nerves. It

Front 117

_________________in classic cases, manifests in early childhood with walking problems due to dystonia of the lower limbs. The dystonia is frequently accompanied by parkinsonian features such as reduced
facial expression or slowing of fine finger movements. Biochemically, the disorder is typically characterized by
low levels of the neurotransmitter metabolite homovanillic acid and reduced levels of neopterin and
tetrahydrobiopterin (BH4) in the cerebrospinal fluid. This is due to heterozygote mutations of the GTP
cyclohydrolase I gene, which is the rate-limiting enzyme in the synthesis of BH4. BH4 is an essential cofactor
for tyrosine hydroxylase (TH), the rate-limiting enzyme in the synthesis of dopamine. Reduced levels of BH4
lead to __________________ because of reduced TH activity. Other genes implicated in the
pathogenesis of this disorder are the TH gene itself and the parkin gene.

Back 117

Dopa-responsive dystonia (DRD),

Front 118

tx postherpetic neuralgia

Back 118

tx antiviral w/in 72 hrs of onset of rash....post herp neur occurs 1-6 months later, However, tricyclic antidepressants (amitriptyline, nortriptyline, desipramine, and maprotiline), gabapentin,
pregabalin, opioids, and topical lidocaine patches are effective in reducing pain and improving quality of life and
should be used in the treatment of postherpetic neuralgia.

Front 119

___________________ impairs mitochondrial fatty acid
oxidation and oxidative phosphorylation. ____________ raises levels of malonylcarnitine that inhibits CPT1, blocking
long chain fatty acid transport into mitochondria. Complex II function is secondarily inhibited. Children have
limited carbohydrate stores, and require larger doses of ________________for sedation, thus placing them at higher risk of
this syndrome than adults. Inadequate caloric intake before or during the infusion further increases the risk, by
leading to inability to meet metabolic demands and suppress fat metabolism. Hemofiltration has been shown to
correct the metabolic abnormalities in propofol infusion syndrome. Carbohydrate intakes of 6 mg/kg/min to 8
mg/kg/min during propofol infusion might prevent it.

Back 119

The findings are most consistent with propofol infusion syndrome. Propofol. Follow CK, triglyc, lactate.

Front 120

best way to tx opiod w/drawal:

Back 120

combination of naltrexone, clonidine, and buprenorphine. mydriasis, lacrimation, rhinorrhea,
piloerection, yawning, sneezing, anorexia nausea, vomiting, and diarrhea.

Front 121

___________________ typically presents in the first 3 decades with episodes of
weakness occurring following rest after exercise, particularly if the subject is exposed to high-carbohydrate
meals, emotional stress, or cold. The respiratory and cardiac muscles are almost always spared, and attacks last
for hours. Symptoms often resolve in later life, although patients may be left with residual weakness. Most
patients have mutations in the skeletal muscle calcium channel gene, although mutations may be found in
sodium and potassium channels in the remainder. Secondary hypokalemic paralysis occurs in older patients with
chronic medical problems predisposing them to hypokalemia. Preventive treatment with acetazolamide is usually
effective in abolishing or attenuating episodes, and oral potassium supplements may be helpful during acute
attacks. Some patients require the use of potassium-sparing diuretics such as spironolactone or triamterene.

Back 121

Autosomal dominant hypokalemic periodic paralysis

Front 122

Autosomal dominant hypokalemic periodic paralysis where is the mutation?

Back 122

mutations in the skeletal muscle calcium channel gene, although mutations may be found in
sodium and potassium channels in the remainder. Nephrolithiasis is a complication of long-term therapy with acetazolamide and should be screened for with
annual ultrasound examinations.

Front 123

HA throughout the day.....and tx.....

Back 123

Chronic paroxysmal hemicrania is considered a syndrome with two pivotal characteristics: (1) unilateral
headache of moderate intensity, with episodes lasting under 45 minutes on average, occurring multiple times
throughout the day, and at a 3:1 ratio female:male; and (2) absolute response to indomethacin. Patients may also
complain of a continuous low-grade headache in between the more severe attacks (hemicrania continua).

Front 124

tx resting tremor in PD.

Back 124

trihexyphenidyl (Anticholinergic drugs) for younger PD patients (<60 years) with severe resting
tremor but cognitive function is preserved. Levodopa is probably more effective for the control of tremor and other parkinsonian features than any other drug. COMT inhibitors andMAO-B inhibitors such as tolcapone and selegiline, and amantadine are indicated for motor fluctuations associated with PD and to provide neuroprotection. No propanolol.

Front 125

prodrug that is converted in the liver to an alpha agonist. Its predictable absorption and
pharmacokinetics makes it the drug of choice for treatment of orthostatic hypotension unresponsive to
fludrocortisone.

Back 125

Midodrine esp with MSA

Front 126

hypocretin-1 and hypocretin-2. The cause of. Over 90%
of patients with ________________ carry HLA-DQB1*0602.

Back 126

narcolepsy with cataplexy is still under investigation

Front 127

A-delta nociceptive fibers use _______________ as their fast transmitter.

Back 127

glutamate

Front 128

C-fiber slower transmitted nociception involves
a number of neurotransmitters and neuromodulators, the most important of which is:

Back 128

substance P.

Front 129

TX Juvenile myoclonic epilepsy

Back 129

valproate.
is a common type of epilepsy with onset occurring during adolescence. This is a
fairly common type of epilepsy, which can develop between 8 and 26 years of age but usually starts between
ages 12 and 16. Historically, the large majority of patients become seizure-free when treated with valproate.

Front 130

_____________ is a rare condition of spontaneous and continuous muscle fiber activity of peripheral nerve
origin. It represents the more severe phenotype of peripheral nerve hyperexcitability and, when acquired, is often
associated with antibodies to voltage-gated potassium channels. There are no specific published
electromyographic or clinical diagnostic criteria for this disorder. Carbamazapine, topiramate, and gabapentin
have all shown efficicacy.

Back 130

Neuromyotonia

Front 131

Neuromyotonia has AB to ______________ channels.

Back 131

voltage-gated potassium

Front 132

HyperKalemic periodic paralysis. tx

Back 132

Glucose or other
carbohydrates (sugars) given during an attack may reduce the severity. Intravenous calcium or diuretics such as
furosemide may need to be given to stop sudden attacks. Intravenous glucose and insulin cause potassium to
move into the cell and may reduce weakness without a loss of total body potassium. A high-carbohydrate diet
may be recommended. Acetazolamide, a medication that prevents attacks of familial periodic paralysis, is also
effective in preventing attacks of hyperkalemic periodic paralysis. Thiazide diuretics such as chlorothiazide are
also effective and have fewer side effects than acetazolamide.

Front 133

tx tics in tourettes

Back 133

Alfa-adrenergic agents such as guanifencine and clonidine are first-choice treatments for TS.
Neuroleptics are the most consistently useful medications for tic suppression; a number are available but some are more effective than others (for example, haloperidol and pimozide).

Front 134

Cabergoline, pergolide, bromocriptine, and dihydroergocriptine are all ergot-derived dopamine agonists that have been associated with what side effect.

Back 134

serosal fibrosis syndromes. Ropinirole is a nonergoline and does not have this adverse effect.

Front 135

Vigabatrin enhances GABAergic transmission by inhibiting _________________ thus increasing GABA
concentration at the synapse.

Back 135

GABA transaminase,

Front 136

In compression neuropathies, sensory fibers are usually affected first with conduction changes. The median
palmar sensory latency prolongation is the earliest change seen on EMG/nerve conduction studies in patients

Back 136

with mild carpal tunnel syndrome.

Front 137

In a _______________ rapid repetitive stimulation causes no change in the
compound muscle action potential.

Back 137

postsynaptic disorder (ie, myasthenia gravis), There would typically be a >10% decrement seen on slow repetitive
stimulation.

Front 138

A significant increment on rapid repetitive stimulation is typically seen in

Back 138

presynaptic disorders
(Lambert-Eaton myasthenic syndrome, botulism).

Front 139

periodic lateralized sharp wave discharges in a patient with a recent febrile illness and onset of
seizures would strongly suggest

Back 139

herpes simplex encephalitis.

Front 140

presence of generalized periodic 1 Hz sharp
waves with progressive dementia is strongly suggestive

Back 140

of Creutzfeldt-Jakob disease.

Front 141

Injury to _______________ weakens the toe
and foot dorsiflexors, with sensory changes over the web of the skin between the first and second toe.

Back 141

the deep peroneal

Front 142

Lesion of
the ____________ affects the everters, with sensory deficit over most of the dorsum of the foot.

Back 142

superficial peroneal

Front 143

Injury to the _________
weakens the quadriceps muscle.

Back 143

femoral nerve

Front 144

Injury to the __________ weakens the adductors, internal and
external rotators of the thigh.

Back 144

obturator nerve

Front 145

Injury to the ___________ weakens the foot inverters, dorsiflexors and intrinsic foot
muscles.

Back 145

tibial nerve

Front 146

Mutations of the FIL-1 gene in chromosome Xq28, encoding for filamin-1, produce _____________
This reflects a complete failure of neuronal migration that may cause seizures. It is dominant in females and
lethal in males. Filamin-1 interacts with actin and is critically involved in the early phases of migration.

Back 146

periventricular heterotopy.

Front 147

Familial periodic paralyses

Back 147

most common channel episodic-weakness phenotypes is a long-lasting depolarization that inactivates sodium ion (Na+)
channels, initiating the action potential.

Front 148

Drugs that inh metabolism of carbamez:

Back 148

These include phenytoin,
cimetidine, diltiazem, erythromycin, verapamil, fluoxetine, and isoniazid.

Front 149

carbamazapine can accelerate hepatic breakdown of a number of drugs, including its own metabolism.

Back 149

The most common interaction is with oral contraceptives, sodium valproate, ethosuximide, corticosteroids, anticoagulants,
antipsychotics, cyclosporine, and methylphenidate.

Front 150

Meperidine and methylphenyldate can _________ the seizure
threshold in epilepsy patients resulting in worsening seizures independent of antiepileptic therapy.

Back 150

lower

Front 151

worst loss of acetylchol in what type dementia

Back 151

DLB

Front 152

AED that has also been associated with changes in serum lithium
levels and lithium toxicity and therefore would not be indicated for primary treatment of epilepsy in bipolar
patients well controlled on lithium.

Back 152

Topiramate

Front 153

____________ interacts with actin and may contribute to
structural and functional stability of the plasma membrane.

Back 153

dystrophin

Front 154

AED......can produce hair loss that rarely requires discontinuation of the drug.

Back 154

Valproate

Front 155

In compression neuropathies, _______________ fibers are usually affected first with conduction changes.

Back 155

sensory