Neuro Bytes Board Prep Flashcards

Question

Impact of valproic acid on P450 enzymes and subsequent impact on other drugs such as lamotrigine How would you adjust lamotrigine dose?

Answer 1

valproic acid INHIBITS P450 enzymes leading to decreased clearance of lamotrigine and significant increase in the halflife Mneumonic: valproic acid = valley = suppress P450 Maintenance doses of lamotrigine can be reduced by at least 50% when given in combination with valproic acid. Unlike valproic acid, which exhibits P450 inhibition, other antiepileptic drugs, including carbamazepine, phenobarbital, and phenytoin, are P450 inducers.

Answer 2

carbamazepine, phenobarbital, and phenytoin

Answer 3

Folic acid supplementation All women of childbearing age on antiepileptic drugs who may potentially become pregnant should be offered folic acid supplementation, regardless of their antiepileptic drug regimen. Thiamine supplementation is not required. It may not be safe to discontinue lamotrigine, because juvenile myoclonic epilepsy often persists throughout the lifespan, and, in general, the obstetric and teratogenic risks of AEDs are less than the risks to mother and fetus of uncontrolled seizures. Close monitoring of lamotrigine levels is required during and immediately after pregnancy, as levels decrease due to increased glucuronidation. Switching antiepileptic drugs during pregnancy is also not advisable, even to other agents considered relatively safe, such as levetiracetam, and careful consideration should be given to the risks of uncontrolled seizures since breakthrough seizures may occur in this setting.

Answer 4

Recurrent optic neuropathy and the described funduscopic findings, in the context of a family history of similar issues, is consistent with Leber hereditary optic neuropathy. Leber hereditary optic neuropathy is due to one of three known point mutations in mitochondrial DNA and is therefore associated with a non-Mendelian maternal inheritance pattern.

Answer 5

Leber hereditary optic neuropathy is due to one of three known point mutations in mitochondrial DNA and is therefore associated with a non-Mendelian maternal inheritance pattern.

Answer 6

An autosomal dominant inheritance pattern is seen in Kjer-type autosomal dominant optic atrophy, which presents with progressive bilateral vision loss in the first decade of life.

Answer 7

The clinical syndrome experienced by this patient includes difficulty initiating saccades (ie, ocular apraxia), trouble with fine movements under visual guidance (ie, optic ataxia), and difficulty interpreting complex visual stimuli (ie, simultagnosia). bilateral damage to the parietal-occipital association areas. These areas are often affected by watershed (border zone) infarctions occurring in the setting of hypotension, such as septic shock.

Answer 8

Often presenting with foot drop. Examination typically reveals weakness on toe extension, ankle dorsiflexion and eversion, and sensory loss on the dorsum of the foot and lateral aspect of the distal leg. Deep tendon reflexes at the ankle are preserved. A disorder of the L5 nerve root can present with similar symptoms; however, examination will also show weakness on ankle inversion and mild weakness of the proximal leg.

Answer 9

Carbamazepine In contrast, 6 ASDs prescribed for focal epilepsy (phenytoin, carbamazepine, oxcarbazepine, eslicarbazepine, gabapentin, and pregabalin) may worsen myoclonic and absence seizures in idiopathic generalized epilepsy syndromes

Answer 10

Blocks voltage sensitive T-type calcium channels

Answer 11

Although the various mechanisms of action of valproic acid include some sodium channel blockade, its effects on γ-aminobutyric acid–mediated transmission and calcium channel blockade predominate, making it an appropriate option

Answer 12

Levetiracetam reduces neurotransmitter release through binding synaptic vesicle protein SV2A

Answer 13

Like valproate, topiramate’s mechanism of action includes some sodium channel blockade, and although it is ineffective in CAE, it does not clearly worsen the condition.

Answer 14

~150cc at any given moment

Answer 15

CSF forms at a rate of about 0.3–0.4 mL/min translating to 18-25 mL/hour (~20cc/hr) and 430–530 mL/day (you make ~4-5x your volume of CSF during any given day)

Answer 16

~17% of total fluid volume the rest of which lies in the cisterns and subarachnoid space.

Answer 17

CADASIL Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant microvasculopathy characterized by recurrent lacunar and subcortical white matter ischemic strokes and vascular dementia in young and middle age patients without known vascular risk factors. There is disproportionate cortical hypometabolism.

Answer 18

NOTCH3 on chromosome 19p13.12

Answer 19

Often demonstrating widespread confluent white matter hyperintensities on T2-weighted images. More circumscribed T2 hyperintense lesions are also seen in the basal ganglia, thalamus and pons. Although the subcortical white matter can be diffusely involved, in the initial course of the disease involvement of the anterior temporal lobe (86%) and external capsule (93%) are classical. There is relative sparing of the occipital and orbitofrontal subcortical white matter 2, subcortical U-fibers and cortex 3.

Answer 20

Granular deposits in the basal lamina of a small blood vessel

Answer 21

Dermal arteriole with focal aggregates of eosinophilic material which was PAS+ Also focally replaced smooth muscle cells of media

Answer 22

Adamantinomatous craniopharyngioma Tumor with palisading epithelium, Nodules of anucleate squamous cells (squamous whorls), wet keratin, and stellate reticulum associated with surrounding gliosis and Rosenthal fibers Dark brown fluid refers to "motor oil" that may fill cystic spaces Papillary craniopharyngiomas are encountered in adults and may lack the cystic spaces filled with "motor oil" as well as the palisading peripheral rows of epithelial cells, keratinization, or calcification typical of pediatric adamantinomatous craniopharyngioma.

Answer 23

Tabes dorsalis aka syphilitic myelopathy weakness, hyporeflexia, ataxia, personality changes, dementia, deafness vision loss w/ impaired light reflex Argyll Robertson – small, irregular pupils, constrict to accommodation only

Answer 24

Elbow flexion (biceps) also shoulder flexion, extension, abduction (Deltoids), adduction, internal/external rotation

Answer 25

Wrist flexion also elbow pronation

Answer 26

Elbow extension (Triceps) Finger flexion at proximal joint, extension Thumb flexion, extension and abduction in plane of thumb

Answer 27

Finger flexion at MCP joint Thumb opposition, adduction, and abduction perpendicular to palm

Answer 28

Abduction of the 2nd and 5th fingers

Answer 29

hip flexion

Answer 30

Knee extension hip external rotation

Answer 31

foot dorsiflexion hip extension, abduction, internal rotation knee flexion toe metacarpal and interpharyngeal extension

Answer 32

great toe dorsiflexion

Answer 33

foot plantar flexion

Answer 34

DESCENDING Because nerves to the cervical cord are located closest to the middle of the cord, whereas nerves to the sacrum are located farthest towards the edges of the spinal cord. Thus, a central spinal cord lesion may cause neurologic deficits in a descending order.

Answer 35

A - normal anatomy - anterior horn (AH) efferent motor output, dorsal column (DC) afferent fine touch and proprioception, spinothalamic tract (yellow) afferent pain and temperature, lateral corticospinal tract (orange) descending efferent motor output B - anterior cord syndrome/infarct C - posterior cord syndrome aka tabes dorsalis of tertiary syphilis D - subacute combined degeneration from B12 deficiency E - Cord hemisection ie Brown-Sequard syndrome F - central cord syndrome of the syrinx G - poliomyelitis H - ALS

Answer 36

Subacute hemorrhage Bright ring on T1, but does NOT enhance w/ gadolinium Suggestive of intracellular methemoglobin Methemoglobin = dark on T2 Core of hemorrhage contains deoxyhemoglobin Deoxyhemoglobin = gray on T1 and T2

Answer 37

Hyperacute appears... Isointense on T1 Bright on T2/FLAIR Mix of isointense/dark on GRE* Acute appears... Isointense on T1 Dark on T2/FLAIR Dark on GRE Early subacute appears... Bright on T1 (mehemoglobin intracellular) Dark on T2/FLAIR Dark on GRE Late subacute appears... Bright on T1 (methemoglobin extracellular) Bright on T2/FLAIR Dark on GRE Chronic appears... Dark on T1 (hemisiderin) Dark on T2/FLAIR lighter core on GRE

Answer 38

Summation of postsynaptic potentials - inhibitory and excitatory. postsynaptic potentials IPSPs and EPSPs

Answer 39

1 - how old is the patient? 2 - what is the montage? 3 - what is the patient's state? (awake/drowsy/asleep)

Answer 40

Moving downward

Answer 41

Awake = Eye blink/PDR Drowsy = RMTTD - rhythmic mid temporal theta of drowsiness Stage N1 = vertex wave or POSTS Stage N2 = K complex or spindles Stage N3 = slow waves of sleep REM = rapid eye movement and atonia? Coma = no definitive sleep structure

Answer 42

Stage N2 sleep K complex (z appearance with spindle

Answer 43

Vertex waves Stage I and II of sleep

Answer 44

POSTS Sharply appearing discharges in the occipital region bilaterally Why are they not called NOSTS? Even though going up its in reference to the occipital area and because we’re in a “wrong montage” we’re missing that this actually a positive change. If we were to look at a POST in a headband montage it would actually be a positive deflection Stage I and II Classically, they have a "sail-like" appearance, and can come in singles or runs. They arise in stage I sleep, but can persist throughout the later stages of sleep. POSTS have a cousin in the awake state called lambda waves, which are discussed in the artifact section.

Answer 45

spindles (~14hz) = diagnostic of stage II sleep

Answer 46

stage II sleep - marked by sleep spindles and K complexes

Answer 47

REM sleep has diffuse attenuation of activity with lateral eye movements in the frontal leads Rapid eye movement, or REM sleep, increases in frequency across the span of the night. It is marked by a diffuse attenuation of amplitudes, with a range of frequencies amongst the background that can look similar to an awake state. It derives its name from the rapid eye movements that arise and, on EEG, are seen as very sharply contoured, opposing waveforms (with the upslope usually faster than the downslope) in the left and right frontal regions. These waves arise because the cornea is positively charged, so when a patient looks to their right, the right eye cornea moves closer to the F8 electrode, leading to a positive charge seen at F8; at the same time on the left side, the left eye's cornea moves away from the F7 electrode, leading to a negative charge seen at F7. Thus, opposing frontal waveforms are seen: a positive wave on the side to which you're looking, and a negative wave on the opposite side. In REM sleep one can also see some mild cardiac irregularity and breathing fluctuations, and muscle activity should be absent throughout this stage.undefined

Answer 48

This is eye flutter This is not a seizure because: 1) there’s no evolution 2) it’s only affecting anterior leads 3) it’s very high amplitude and perfectly symmetric 4) the PDR is normal (most important!!)

Answer 49

Positive charge of the cornea is looking to the left Could point to the circle and be like what is going on? Positive charge of cornea causes deflection Creates space that looks like an eyeball how you know which direction going

Answer 50

Chewing artifact PDR not interrupted, maintained Does not evolve, pretty consistent

Answer 51

EKG artifact Small blue bites here Wondering if they’re spikes… Well they are very very regular and real cortical things are usually not that regular. They also have the same morphology every time. And they do not phase reverse or affect other leads. Not bitemporal sharps. These always line up with EKG so this is EKG artifact

Answer 52

Pulse artifact Only in two leads, very regular, seems almost artificial, and lines up with EKG as well This is actually pulse artifact

Answer 53

Sweat artifact Very slow high amplitude kind of swishing “slimy” That’s sweat artifact Very slow but still have alpha activity maintained so there’s something abnormal behind it Look at EKG

Answer 54

Ectropop artifact Look like little sidewise trapezoids it’s the electropop artifact

Answer 55

Mu (normal variant) Not a PDR because it’s central predominant, and it has some spike contours.

Answer 56

Bioccipital epileptiform discharges The marked waveforms, while relatively symmetric as you'd expect with lambda waves, are otherwise wholly different. First off, they have a negative polarity while lambdas are positive. Second, the marked waves have the classic epileptiform morphology of a spike and slow wave, although some show this more clearly than others. Don't let them fool you just because there are a series of them; epileptiform discharges can come in nonevolving runs as you see here.

Answer 57

Benign epileptiform transients of sleep (BETS) are also called small sharp spikes (SSS). While BETS have a slightly oxymoronic name, they are in fact a normal, benign finding that you can see in the drowsy and asleep states. In morphology they are very similar to epileptiform spikes, including having a field, but by definition BETS are low amplitude (technically less than 50µV) and short duration. They can be seen most often in the temporal chains. Differentiating BETS from real epileptiform discharges can be hard when you're starting to learn EEG; a good rule of thumb is that if the discharge is very low amplitude and seen only once or a few times, and only during sleep, its probably a BET but you should make note of it and keep a look out for anything more suspicious.

Answer 58

Wicket's Note temporal location Similar shape Patient is awake but in a drowsiness probably going to go to sleep soon

Answer 59

Small sharp waves Not well localized Phase reversing in some places but not all Not well defined morphology Benign epileptiform transients of sleep (BETS) are also called small sharp spikes (SSS). While BETS have a slightly oxymoronic name, they are in fact a normal, benign finding that you can see in the drowsy and asleep states. In morphology they are very similar to epileptiform spikes, including having a field, but by definition BETS are low amplitude (technically less than 50µV) and short duration. They can be seen most often in the temporal chains. Differentiating BETS from real epileptiform discharges can be hard when you're starting to learn EEG; a good rule of thumb is that if the discharge is very low amplitude and seen only once or a few times, and only during sleep, its probably a BET but you should make note of it and keep a look out for anything more suspicious.

Answer 60

Breach artifact over the left PDR is more sharply contoured and higher amplitude

Answer 61

Encephalopathy Patient has eye blinks but generalized diffuse slowing without clear PDR

Answer 62

Sleepy patient with sleep spindles on the right (stage 2) Upper left is phase reversal sharp wave = left temporal sharp waves

Answer 63

First seizure Bipolar montage Background slow Sharp activity with phase reversal in left frontal activity evolves from a few seconds into with increased speed and then slowing (delta vs theta range in other leads) No muscle artifact - probably subclinical Very short about 11 seconds

Answer 64

Lateralized periodic discharges (~1Hz) left parietal Sharp with slow component

Answer 65

Hypsarrhythmia Associated with West syndrome Tuberous sclerosis Infantile spasm (spikey component then flattening of EEG corresponding with abdominal tonic-clonic flexion)?

Answer 66

Used to be benign rolandic epilepsy

Answer 67

~4Hz polyspike component

Answer 68

Notice there is NOT a polsypike component

Answer 69

EEG showing delta activity with fast activity almost like a paintbrush This is the extreme delta brush seen in anti-NMDA receptor encephalitis (when it is abnormal) It is actually normal in neonates between 28-38 weeks gestational age

Answer 70

If no GTCs - okay to do ethosuximide but if Absence + GTCs then need something more aggressive like VPA

Answer 71

HSV 1 - causes oral herpes and can lead to genital herpes, is most common cause of sporadic encephalitis HSV 2 - main cause of genital herpes, restricted to aseptic meningitis

Answer 72

Dopamine agonist therapy (ie pramipexole)

Answer 73

Inability to read ("word blindness") but retained ability to write and understand spoken word Typically also associated with right hemianopsia, color anomia, and various apraxias (ie inability to tell time) Localization: injury to the dominant occipital lobe + splenium so visual information is not able to get to language center Potential patterns of localization: 1) a single lesion to the splenium of the corpus callosum that stretches posterolaterally so as to interrupt white matter tracts running from the visual cortices of both hemispheres to the left angular gyrus 2) lesions to both the splenium and the left primary visual cortex, or 3) lesions involving both the splenium and the lateral geniculate nucleus. In each case, the language zone is ROBBED OF ITS VISUAL INPUTS but remains intact. Etiologies of these lesions include posterior cerebral artery territory infarct, tumor, or demyelinating disorders such as multiple sclerosis or acute disseminated encephalomyelitis. Case Reference: https://n.neurology.org/content/82/1/e5

Answer 74

Frovatriptan because it is a longer-acting triptan and has been approved for menstrual migraine HOOK: treat Frumpy menses with Frovatriptan Given for 6 days around the time of menses

Answer 75

This is ADEM so the best next step is IVIG!!

Answer 76

C7 causing sensory impairment of middle finger

Answer 77

Somatic symptom disorder - bodily symptoms that are either very distressing or result in significant disruption of functioning as well as excessive and disproportionate thoughts, feelings, and behaviors regarding those symptoms Somatization disorder and hypochondriasis no longer in DSM

Answer 78

Trochlear IV

Answer 79

Bilateral parieto-occipital border zone strokes 1) Oculomotor ataxia: difficulty in grasping or touching an object under visual guidance 2) Oculomotor apraxia: inability to project gaze voluntarily into the peripheral field and to scan it despite the fact that eye movements are full 3) Simultanagnosia: inability to look to the peripheral field or perceive the visual field as a whole

Answer 80

20% of all ischemic strokes

Answer 81

<1.5cm in diameter

Answer 82

**Decremental response when stimulating 2Hz** Decremental response when stimulating 30Hz Decremental response stimulating 50Hz Incremental response stimulating 2Hz No response when stimulating 30HZ [explanation]

Answer 83

Meningioma Intradural, extramedullary dural based enhancing mass Spinal meningioma often have radicular pain Arises from meninges of brain or spinal cord Meningothelial arachnoid cap cells 80% Grade 1 tumor 🡪 20% local recurrence rate Histo 🡪 psammoma bodies Primarily sporadic Also assoc. w/ NFT2 intracranially

Answer 84

Hemifacial spasm MRI displays small vessel abutting proximal left facial nerve w/o demyelination or pathologic enhancement (e.g. Ramsay hunt syndrome) Build up is typically gradual, typically involving the eye and then lower facial muscles over months to years Assoc. w/ chronic irritation of CN VII from various means (vessels, masses) TX: Botox; carbamazepine, gabapentin, clonazepam or surgery

Answer 85

Hemifacial spasm MRI displays small vessel abutting proximal left facial nerve w/o demyelination or pathologic enhancement (e.g. Ramsay hunt syndrome) Build up is typically gradual, typically involving the eye and then lower facial muscles over months to years Assoc. w/ chronic irritation of CN VII from various means (vessels, masses) TX: Botox; carbamazepine, gabapentin, clonazepam or surgery

Answer 86

Arachnoid cyst, anterior temporal Likely unrelated to patient’s headaches w/o evidence of papilledema Embryonic origin assoc. w/ abnormal arachnoid splitting May be assoc. w/ Aicardi syndrome, Mucopolysarccharidosis, Marfan syndrome Primarily arise in middle or anterior cranial fossa Fluid filled lesions, typically asymptomatic Tx if compression of structures or neuro deficits

Answer 87

Substantia nigra with loss of dopaminergic neurons in B demonstrating Parkinson's disease Right corner Lewy body (alpha synuclein)

Answer 88

Pompe Disease Glycogen storage disease type II Finding **severe vacuolar myopathy** Acid maltase (acid alpha glucosidase, GAA) deficiency Autosomal dominant Cardiomegaly, hepatomegaly, progressive muscle weakness, macroglossia, hypotonia DX: clinical evaluation, measure GAA activity TX: Symptom support Enzyme replacement 🡪 IV recombinant human acid alpha-glucosidase

Answer 89

Mutations in VG K ch (KCNQ2 and KCNQ3) AD inheritance HOOK: Familial = AD Convulsions – C sounds like K – potassium channel

Answer 90

Carbamazepine and phenytoin are enzyme inducers which thus accelerate vpa metabolism and reduce blood concentrations Aspirin and other NSAIDs inhibit UGT thereby reducing vpa metabolism by glucoronidation and increasing blood concentrations

Answer 91

LTG must be reduced by 50% because VPA inhibits lamotrigine clearance thereby increasing levels

Answer 92

They decrease LTG levels thereby potentially reducing its efficacy

Answer 93

Usual titration schedule 25 mg daily 50 mg daily after 3 weeks Then addition of 50 mg every week or every other week after 5 weeks of treatment initiation The typical maintenance then is 225 mg to 375 mg given in 2 divided doses When it is prescribed along with carbamazepine, phenytoin, phenobarbital, primidone, rifampin, lopinavir/ritonavir, and atazanavir, or ritonavir, Starting dose of 50 mg daily for 3 weeks. Then 50 mg twice daily for 2 weeks Then increase by 100 mg every week or every other week maintenance usually ranges from 300 mg to 500 mg given in 2 divided doses daily When it is prescribed along with valproate, the level of lamotrigine will be increased so lamotrigine should be dose reduced by 50%

Answer 94

Never faster then 50mg/min (rec 20-50mg/min)

Answer 95

Phenytoin is usually prescribed in the 300-400 mg range. Due to the non-linear pharmacokinetics of the drug, even a small change in dosing can result in markedly increased levels and toxicity. This is because the metabolism of the drug starts becoming saturated over time, leading to reduced elimination. This saturation can occur even when the drug is given within the therapeutic range. Therefore, even a small adjustment can lead to a large increase in plasma levels of the drug. Adjustments: Level <7mg/L = increase by 100mg/d Level 7-12mg/L = increase 50mg/d Level 12-16mg/L = increase 30mg/d >16mg/L = little to no increase

Answer 96

Valproic acid (8.9%) Phenobarbital(5.9%) Topiramate (4.4%) Carbamazepine (3.0 %) Phenytoin (2.8 %) Lamotrigine (2.1%) Levetiracetam (2.0 %).

Answer 97

The concentration of an antiepileptic drug in breast milk is determined by the extent it binds to proteins. Primidone Carbamazepine Lamotrigine Phenobarbital Valproate being highly protein-bound does NOT appear in substantial concentrations in breast milk and is safer than most other drugs during lactation.

Answer 98

ViGABAtrin inhibits gaba transaminase Key side effect is that it can cause visual field loss

Answer 99

PerAMPAnel is an AMPA receptor blocker Key side effect is FDA black box warning for serious psychiatric events like homicidal ideation

Answer 100

Unknown, binds to SV2A protein

Answer 101

Na+ channel (slow) blocker

Answer 102

Na+ and CA++ channel blocker

Answer 103

1. Increased frequency of tonic-clonic seizures, increased seizure refractoriness. 2. Poor compliance with treatment, non-therapeutic levels of antiepileptic medications (AEDs). 3. Young age (20-40) and early age onset of seizures. 4. Male gender. 5. Multiple AED use. 6. Nocturnal seizures

Answer 104

Accidental death Status epilepticus Suicide SUDEP

Answer 105

EMP2A mutation

Answer 106

Landau-Kleffner

Answer 107

Lennox Gastaut

Answer 108

Benign Rolandic epilepsy Benign Childhood Epilepsy with centrotemporal spikes (BECTS Autosomal dominant inheritance with variable penetrance

Answer 109

JME type 1 associated with EFHC1 mutation GABRA1 (CACNB4)

Answer 110

CHRNA4, nicotinic Ach gene receptor

Answer 111

dantrolene is a ryanodine receptor antagonist that is indicated in the management of malignant hyperthermia. Patients with malignant hyperthermia present with hyperthermia, rigidity, tachypnea, and tachycardia.

Answer 112

atropine is a competitive antagonist that acts on the muscarinic M1 receptors. It is used for the management of acetylcholinesterase inhibitor poisoning

Answer 113

GCS 15 = grade 1 no matter FND or not Sequential 2 points off on GCS THEN motor deficit Then GCS points off x2

Answer 114

HSV-1 although only accounts for 10% of all encephalitis cases

Answer 115

Irreversible cerebellar toxicity Characterized by selective loss of Purkinje cells

Answer 116

Amphotericin B and flucytosine is the correct option because using these agents together has shown a lower incidence of relapse, failure, and nephrotoxicity, as compared to amphotericin B alone. Detailed trials have shown positive results. Additionally, it is safe to use with an abnormal renal profile, as in the instant case.

Answer 117

Primidone is introduced at a low dose and increased incrementally as patients can often be sensitive to this drug. The side effect profile of this drug includes sleepiness, low mood, dizziness, paresthesia, and ataxia. This drug can also cause hypocalcemia and macrocytic anemia secondary to folate deficiency.

Answer 118

Tolcapone is a COMT inhibitor which has CENTRAL action and can be used for wearing off. It has an important potentially fatal side effect of hepatotoxicity

Answer 119

RLS is a sensory-motor disorder with 4 cardinal features, including (1) an uncomfortable sensation or urge to move the legs (2) discomfort worse in the evening or night (3) discomfort worse at rest (4) discomfort better with movement of the affected limbs. There are 2 forms of RLS, which include primary (familial) and secondary (acquired). Most sufferers of RLS (more than 85%) have periodic leg movements during sleep, which also may disrupt sleep.

Answer 120

The Westphal variant of Huntington’s disease develops before the age of 21 years with bradykinesia, dystonia, and parkinsonian features presenting more dominantly than chorea. It is associated with neuropsychiatric manifestations, myoclonus, and epilepsy.

Answer 121

Ergot-derived dopamine agonists, such as cabergoline, can often cause retroperitoneal, pulmonary, and cardiac fibrosis. For this reason, a baseline serum creatinine, chest x-ray, and echocardiogram should be obtained before treatment is initiated.

Answer 122

Multisystem atrophy MSA Inspiratory laryngeal stridor is a more common clinical manifestation than expiratory stridor in a patient with MSA. One pathological mechanism identified in this population is laryngeal narrowing which results from degeneration of the nucleus ambiguous; subsequent paralysis of the posterior cricoarytenoid muscle occurs. Continuous positive airway pressure and tracheostomy may be offered to these individuals.

Answer 123

Literature reports that chorea is a significant adverse effect of oral contraceptive pills in young women with underlying asymptomatic or mildly symptomatic SLE. Therefore, all young, healthy women with movement disorders should be evaluated for SLE despite the absence of classical features. This patient has symptoms and unilateral chorea, which may be attributed to OCPs or an underlying autoimmune disorder triggered by the OCPs.

Answer 124

Chorea gravidarum tends to develop in primigravida women, particularly in the first trimester. Usually occurs in women who had syndenham chorea in the past

Answer 125

Amantadine Amantadine is an NMDA antagonist that increases dopamine availability in the synapse and the only drug that has been approved for treating levodopa-induced dyskinesia.

Answer 126

It is a dopamine depleter so it can worsen EPS like akathisia and voluntary muscle control

Answer 127

Amantadine is a good option as it treats chorea, dystonia, bradykinesia, and tremors and does not worsen the extrapyramidal symptoms (unlike tetrabenazine which is a VMAT inhibitor that depletes dopamine thereby treating chorea)

Answer 128

Extensive loss of purkinje cells

Answer 129

Axonal loss in the posterior columns

Answer 130

Reactive gliosis and neuronal loss affecting gray matter of limbic structures

Answer 131

It is an NMDA receptor antagonist of glutamate used in AD to slow neuronal death and progression

Answer 132

SSRIs like fluoxetine

Answer 133

Norepinephrine Locus coeruleus is the sole source of NE to the neocortex, hippocampus, cerebellum, and most of the thalamus. It plays a vital role in arousal and sleep-wake states. Disruption of this region affects the reticular activating system.

Answer 134

orexin is produced in the posterior hypothalamus and plays a role in wakefulness and arousal.

Answer 135

Isaac's syndrome is also known as acquired neuromyotonia in which patients develop SPONTANEOUS muscular activity resulting from repetitive motor unit action potentials of peripheral origin. These patients also have painful spasms in limbs resulting in walking difficulty but they do not respond to benzodiazepines and have doublet, triplet, or multiplet single-unit discharges that have a high, irregular intraburst frequency, fasciculations, and fibrillations on EMG studies. Some patients with Isaac syndrome also have voltage-gated antibodies to potassium channels.

Answer 136

Satoyoshi disease is described as painful muscle cramps with joint and bone deformities and endocrine disturbances manifesting as diarrhea, baldness, and reproductive dysfunction. Muscle cramp may be due to inhibition of the spinal interneuron and excitability of the anterior horn cell. In patients with Satoyoshi disease, there is cross-reaction with an 85 kDa protein of the human brain lysate. No response to benzos

Answer 137

Oral lactulose is currently recommended as first-line therapy in treating patients with an initial episode of HE. It is also recommended for the prevention of recurrent episodes of HE. As per current clinical evidence, rifaximin is recommended as an add-on therapy to lactulose for the prevention of recurrent episodes of hepatic encephalopathy (HE). Rifaximin monotherapy is currently not indicated in the treatment of HE.

Answer 138

the clinician should immediately consider a SENSORY NEURONOPATHY or dorsal root ganglionopathy. In addition to the severe proprioceptive and vibration deficits, sensory neuronopathies usually have a pan-modality sensory loss in the affected extremities.Light touch and pain sensation are also affected due to injury to all sensory cell bodies. The most common causes of a sensory neuronopathy or dorsal root ganglionopathy include paraneoplastic disease, Sjogren's syndrome, and idiopathic sensory neuronopathy. Other causes include toxicity of cis-platinum and other analogs, vitamin B6 toxicity, and HIV-related sensory neuronopathy

Answer 139

Alpers-Huttenlocher syndrome is a rare type of hepatocerebral mitochondrial-DNA depletion syndrome (MDS). It classically presents with episodic psychomotor retardation, intractable seizures, cortical blindness, and hepatic dysfunction. The MOST specific indicator of this diagnosis is a valproate-induced fulminant hepatic failure. Valproate is strictly contraindicated in hepatocerebral MDS.

Answer 140

Numb cheek syndrome - Malignant tumor infiltrating the trigeminal nerve The maxillary division of the trigeminal nerve may be damaged at the lower lateral wall of the cavernous sinus, at the foramen rotundum, in the pterygopalatine fossa, in the floor of the orbit, at the infraorbital foramen, or in the face. Numbness or discomfort in a maxillary distribution may be the initial presentation of a nasopharyngeal tumor, as these tumors often arise in the lateral nasopharyngeal wall (fossa of Rosenmüller) and extend via the foramen lacerum to involve the region of the middle cranial fossa and cavernous sinus. Lesions affecting this nerve in the cavernous sinus usually affect other cranial nerves as well. More distal lesions (e.g., infraorbital nerve damage secondary to maxillary fracture) result in sensory disturbances that are confined to the cutaneous supply of the maxillary nerve. Lesions in the infraorbital foramen may cause the numb cheek syndrome, in which numbness involves 1 cheek and the upper lip in an infraorbital nerve distribution. The hypesthesia in this syndrome may also involve the medial and lateral upper incisors and canine teeth and adjacent gingiva, but spare more posterior teeth and gums (e.g., the molar and premolar teeth and gums that are innervated by the posterior and middle superior alveolar nerves). In two-thirds of patients, the numb cheek syndrome heralded recurrent squamous cell carcinoma of the skin. Image - Anatomy of the mental nerve and associated area of numbness From the gasserian ganglion (A), the mandibular nerve (B) exits the skull via the foramen ovale. The posterior trunk divides, giving rise to the auriculotemporal (C), inferior alveolar (D), and lingual (E) nerves. The inferior alveolar nerve travels in the mandible, entering through the mandibular canal. It then gives rise to the mental nerve (F), which exits through the mental foramen. The shadowed area (G) corresponds to the sensory region of the mental nerve and is the classically affected area in numb chin syndrome. Figure courtesy of Lucas Roberts.

Answer 141

Glucose repletion but ALSO octreotide In this condition, administration of dextrose may stimulate the pancreatic B-cells to secrete insulin, resulting in persistent hypoglycemia. Administration of octreotide can block the secretion of insulin by inhibiting the B-cells.

Answer 142

Fibromyalgia is the most common cause of generalized musculoskeletal pain with a prevalence of 1-6% of the general population. The exact mechanism is unknown; however, it is closely associated with depressive, anxiety, and trauma symptoms. First line treatments involve lifestyle modifications (sleep behavior modifications, exercise, and diet.) If lifestyle modifications are insufficient, then augmenting with a medication is warranted. Other interventions include CBT and physical therapy. The first line medications used for fibromyalgia are SNRIs (milnacipran and duloxetine) and pregabalin. Milnacipran is FDA approved to treat fibromyalgia NOT SSRIs (citalopram, escitalopram, fluoxetine) NOT gabapengin

Answer 143

Tolosa-Hunt syndrome is characterized by the chronic granulomatous inflammation of the cavernous sinus, which presents as severe pain behind or around the eyes, which is not present in this patient.

Answer 144

Growth hormone and testosterone is the correct option because this patient's presentation is consistent with Prader-Willi syndrome. Moreover, growth hormone deficiency (short stature), premature adrenarche, hypogonadism (a risk factor for osteoporosis), and hyperphagia with obesity are observed as the children grow. Therefore, the administration of growth hormone and sex hormones is the treatment of choice to manage obesity, growth hormone insufficiency, and hypogonadism.

Answer 145

transient receptor potential cation channel subfamily M member 8 (TRPM8) is found in sensory neurons and activated by cold temperatures or cooling agents (menthol and icilin)

Answer 146

trigeminal neuralgia. This disorder is characterized by recurring paroxysmal attacks of sharp, stabbing pains in the distribution of the 5th cranial nerve and its branches. These episodes are sudden in onset and termination and are typically triggered by talking, chewing, applying makeup, and brushing teeth. The second and third divisions of the trigeminal nerve are more commonly involved than the first. Patients usually have a normal neurological examination.

Answer 147

A 40-year-old man reports recurrent explosive bilateral throbbing headaches occurring several times a day, sometimes provoked by Valsalva maneuver or micturition. The headaches are diffuse and typically last less than one hour. Which one of the following diagnoses is the most likely? In patients with pheochromocytoma: 80% of patients have headache; 10% present with headache alone; The headache has a rapid buildup and is often 'explosive'; The headache is bilateral, often throbbing; Nausea/vomiting are common; Nocturnal attacks are common; Duration of headache: 50% less than 15 minutes; 70% less than 60 minutes. The headache may be provoked by sleep, Valsalva, exertion, micturition.

Answer 148

The clinical picture resulting from anterior spinal artery syndrome is a motor and sensory weakness, spared proprioception and vibration, and bowel or bladder dysfunction. Treatment aims at measures to re-perfuse the spinal cord by decreasing the pressure on the spinal cord by reducing cerebrospinal fluid through a lumbar drain, reducing complications resulting from the occlusion, and addressing the cause of occlusion. In the case of an abdominal surgical pathology such as aortic dissection or major surgery, dual antiplatelet therapy is contraindicated. In such cases, single antiplatelet therapy is given to minimize the risk of thromboembolic events.

Answer 149

anti-Ma2 antibodies

Answer 150

Small cell lung cancer Associated with limbic and cerebellar encephalitis/degeneration

Answer 151

Thymoma and small cell carcinoma

Answer 152

paraneoplastic optic neuropathy associated with small-cell lung cancer (SCLC).

Answer 153

Hypoglycemia causes neuronal death when glucose levels have fallen below 1 mM (18 mg/dL) is the correct response. Neuronal death from hypoglycemia occurs when the EEG becomes flat, which typically happens after glucose levels have fallen below the level previously mentioned. Symptoms of hypoglycemia are those of sympathoadrenal activity (including tremor, palpitations, sweating, anxiety, and hunger) and of neuroglycopenia (such as blurring of vision, confusion, incoordination, impaired intellectual function, and inability to concentrate).

Answer 154

The correct answer is:D. Difficulty with recognizing and producing affective content Explanation: The nondominant hemisphere is responsible for affective aspects of speech, and patients with strokes in that area frequently have difficulty discerning the emotional content of language. Although the patient is left-handed, 60%-70% of left-handed people still have a left-dominant hemisphere. In a patient who is left-dominant for language, a stroke in the right temporal lobe would not be expected to cause problems with comprehension, repetition or fluency.

Answer 155

unilateral loss of vibratory sense. motor and pain sensations are intact. Injury of the right posterior spinal artery would result in infarction of the right dorsal column, which is responsible for ipsilateral vibratory and proprioceptive senses.

Answer 156

Bilateral motor paresis (upper > lower extremities and distally > proximally) Variable sensory impairment (burning pain in the arms, loss of pain and temp in the arms) Sacral sparing (Because the corticospinal fibers that innervate the anterior horn cells of the sacral segments are located in the most lateral part of the lateral corticospinal tract)

Answer 157

Raphe nucleus is correct because paroxetine is a selective serotonin reuptake inhibitor (SSRI) that is indicated in the treatment of major depressive disorder (MDD). The raphe nucleus is a primary site of the production of serotonin. It is located in the midbrain. Current studies show that low levels of serotonin in the central nervous system are associated with depression. Therefore, drugs such as paroxetine that increase levels of serotonin in the synaptic cleft, help improve mood.

Answer 158

The basal nucleus of Meynert is a site of the production of acetylcholine in the brain. Decreased levels of acetylcholine have been associated with Alzheimer’s disease.

Answer 159

Vision loss due to synergistic action of alcohol and tobacco These patients should be supplemented with vitamin B 12 along with avoidance of both alcohol and tobacco. Both alcohol and tobacco act synergistically to cause optic nerve dysfunction, which is further exacerbated by his vitamin B12 deficiency. Therefore, the patient should also be counseled to avoid alcohol and tobacco.

Answer 160

This form of brachial plexopathy is slowly progressive and often painless. It typically affects the upper trunk of the brachial plexus more commonly than the lower trunk. Myokymic discharges or fasciculations seen on electromyography (EMG) are strongly suggestive of radiation-induced damage. Although chronic radiation fibrosis often demonstrates low signal intensities on T2-weighted MRI images, high signal intensities have also been reported.

Answer 161

When pregnant women are given phenytoin, they are also given folate and Vitamin K supplementation due to phenytoin's antifolate activity and interference with vitamin K metabolism. Even the newborn infant must be supplemented with vitamin K to prevent bleeding.

Answer 162

NF type 1 Other features: seizure disorder and physical exam findings of axillary and inguinal freckling, café au lait spots, neurofibromas, Lisch nodules, and scoliosis, the most likely diagnosis is neurofibromatosis type 1 (NF1). Individuals with NF1 are at increased risk for developing optic gliomas. Such patients should undergo annual ophthalmologic examination and have a brain and orbit MRI. Of note, these patients are also at increased risk for pheochromocytomas.

Answer 163

Non-Hodgkin's lymphoma is incorrect. Patients with ataxia telangiectasia (AT) are at increased risk for lymphoid malignancies, including non-Hodgkin's lymphoma, Hodgkin's lymphoma, and several forms of leukemia. AT is an autosomal recessive disorder characterized by the appearance of ataxia at an early age. A significant number of patients are wheelchair-bound by their teens. Noted in the conjuctiva, eyelid, cheek, and limb skin folds, telangiectasias can be identified by 3-6 years of age. Respiratory tract infections are frequent in this population.

Answer 164

Tuberous sclerosis Giant cell astrocytoma is incorrect, because this is a finding associated with tuberous sclerosis (TS). Individuals with TS often have hypopigmented ash-leaf spots (also known as Fitzpatrick patches), renal angiomyolipoas, cardiac rhabdomyoma, cortical tubers, subependymal nodules, and facial angiofibromas.

Answer 165

Giant cell astrocytoma is incorrect, because this is a finding associated with tuberous sclerosis (TS). Individuals with TS often have hypopigmented ash-leaf spots (also known as Fitzpatrick patches), renal angiomyolipoas, cardiac rhabdomyoma, cortical tubers, subependymal nodules, and facial angiofibromas.

Answer 166

Loss of function This gene, also called neurofibromin 2, is located on chromosome 22q. About 90 percent of merlin mutations result in an abnormally shortened version of the merlin protein. This short protein cannot perform its normal tumor suppressor function in cells. Research suggests that the loss of merlin allows cells, especially Schwann cells, to multiply too frequently and form noncancerous tumors. The most common tumors in NF2 are vestibular schwannomas, which develop along the vestibular nerve. NF2 is an autosomal dominant disorder.

Answer 167

Polymyositis is the most common myopathy in AIDS patients. Clinical features of polymyositis are myalgia, symmetric proximal muscle weakness, and wasting. CK levels are very elevated in these patients, and muscle biopsy shows intrafascicular inflammatory infiltrates composed of CD8+ T cells. Patients with polymyositis show an excellent response to steroids.

Answer 168

Dermatomyositis is an inflammatory myopathy with prominent skin changes. A heliotrope rash, Gottron papules, and shawl sign are often observed in these patients. PERIFASICULAR inflammatory infiltrates are seen on muscle biopsy.

Answer 169

Krabbe's disease characteristics: deficient enzyme is galactocerebrosidase; associated with galactocerebroside substrate; is autosomal recessive; and presents with peripheral neuropathy, optic atrophy, developmental delay, and/or globoid cells.

Answer 170

deficient enzyme is hexosaminidase A; associated with GM2 ganglioside substrate; is autosomal recessive; and presents with progressive neurodegeneration, developmental delay, cherry-red spots on macula, and lysosomes with onion skin.

Answer 171

Fabry – X-linked recessive deficient enzyme is a-galactosidase A; associated with ceramide trihexoside substrate; is X-linked recessive; and presents with peripheral neuropathy, keratomas, and/or CV/renal disease.

Answer 172

deficient enzyme is sphingomyelinase; associated with sphingomyelin substrate; is autosomal recessive; and presents with progressive neurodegeneration, hepatosplenomegaly, cherry-red spot on macula, and/or foam cells. *** check menumonic: pick a sphinger?

Answer 173

Inclusion body myositis is the correct option. It is an inflammatory myosotis that is predominantly seen in older Caucasian men. Patients may present with variable degrees of limb weakness, loss of finger dexterity, and grip strength. The signs are often asymmetrical, and unlike polymyositis, both proximal and distal muscles are involved. Knee extension and ankle dorsiflexion are impaired earlier in the disease, and dysphagia occurs in 50% of patients. Muscle biopsy is diagnostic.

Answer 174

ventral intermediate (VIM) nucleus of the thalamus shows great improvement of essential tremors in patients who do not respond to medical treatment, and this improvement lasts for 5-7 years.

Answer 175

Good candidates: - Respond well to levodopa - Main sx of concern: rigidity, bradykinesia, tremors, dystonia, and medication-induced dyskinesia Poor candidates: - predominent sx: dementia, gait freezing, apathy, POSTURAL INSTABILITY & FALLS when on (may get worse with DBS) - Depression even in the on periods that is not well controlled (black box warning suicidality) - atypical parkinsonism (parkinson+ syndromes) DBS can be done in eligible patients irrespective of age.

Answer 176

The presence of alcohol in the body promotes ketoacidosis and thereby leads to high anion gap metabolic acidosis.

Answer 177

Approximately 40% of patients who have had one episode of status epilepticus subsequently develop epilepsy.

Answer 178

Proceed with epilepsy surgery is the correct answer because the patient here has the most likely diagnosis of mesial temporal lobe epilepsy (MTLE) with hippocampal sclerosis that is drug-resistant. Epilepsy surgery has an excellent response rate in MTLE after temporal lobectomy or selective amygdalohippocampectomy. 60 to 80% of the patients are seizure-free after the surgery.

Answer 179

Early-onset Alzheimer's disease is a major complication of Down's syndrome and occurs in almost every patient who is more than 50 years old. Other important disease associations of Down's syndrome are ostium primum cardiac defects, hypothyroidism, hematological malignancies, moyamoya disease, and atlantoaxial subluxation.

Answer 180

In adults, opsoclonus-myoclonus-ataxia is the most common manifestation of breast cancer, mainly when associated with positive anti-Ri antibodies. Opsoclonus-myoclonus-ataxia syndrome is most commonly a manifestation of neuroblastoma in children. In adults, neuroblastoma is less likely.

Answer 181

His physical exam shows Marfanoid habitus, inferonasal lens subluxation, and intellectual disability. This suggests homocystinuria, most likely caused by a deficiency of cystathionine synthase. Vitamin B6 is a cofactor of cystathionine synthase. Homocystinuria caused by cystathionine synthase deficiency can be treated with B6 supplementation.Vitamin B6 is a cofactor of cystathionine synthase.

Answer 182

FDA-approved drug for RLA. It is an extended-release version of Gabapentin with twice as much bioavailability.

Answer 183

Isoniazid-induced peripheral neuropathy is usually dose-dependent and starts as paresthesia that begins in the feet extends to the arms and hands. The offending medicine should be discontinued; timely therapeutic intervention with high-dose vitamin B6 can reduce the long-term morbidity associated with this easily REVERSIBLE condition.

Answer 184

By 18 months, US before then

Answer 185

Combination steroids and rituximab

Answer 186

Current guidelines recommend salvage hyperbaric oxygen therapy (HBOT) with steroid therapy, within one month of onset of SSNHL.

Answer 187

Regular vs irregular Focal clonic seizures is correct. Because only one hemisphere is involved, the seizure is classified as focal. Focal motor seizures can include automatisms (such as lip-smacking) and clonic activity (which would entail regularly spaced, repeated stereotypical jerking movements). During a focal onset seizure, an individual can either remain aware of the surroundings or have impaired awareness. In this particular example, the patient maintained awareness. Focal myoclonic seizures is incorrect, because the patient had rhythmic jerking of his limbs (which is characteristic of clonic movements), as opposed to irregular jerking of the limbs.

Answer 188

Right frontal cortex lesions

Answer 189

Amantadine is a good choice for this patient as it counteracts the parkinsonism and should help this patient with his dystonia, tremor, and bradykinesia. It can even help with the mild chorea.Tetrabenazine should be avoided in this patient. Tetrabenazine is useful for treating chorea, but that is not the main complaint of this patient. Tetrabenazine can reduce the dopamine effect required to treat this patient and should, therefore, be avoided in this patient.

Answer 190

Hyperacute (w/i 24 hours) - isointense T1 and hyperintense T2 Some references say isointense on both but to me more that T2 hyperintense at this stage Acute (1-3d) - isointense on T1 and hypointense on T2 Early subacute (3-7d) - hyperintense on T1 and hypointense on T2 Late subacute (7-14d) - hyperintense on T1 and T2 Chronic (>14d) - hypointense on T1 and T2

Answer 191

The Multiple Sleep Latency Test (MSLT) is done in a laboratory setting in which the patient is assessed for the length of time it takes to fall asleep and to enter REM sleep during scheduled naptimes. The test is repeated several times throughout the day at 2 hour intervals. Indications for the MSLT are:(1) evaluation of suspected narcolepsy to confirm the diagnosis;(2) evaluation of suspected idiopathic hypersomnia to differentiate it from narcolepsy. The MSLT is not routinely indicated for the initial evaluation and diagnosis of obstructive sleep apnea syndrome or in the assessment of change following treatment with nasal continuous positive airway pressure (CPAP). It is not used for circadian rhythm disorders or primary insomnia.

Answer 192

Diagnostic criteria include an increase in heart rate of 30 bpm within 10 minutes without a postural drop in blood pressure. It may be idiopathic or caused by medications like calcium channel blockers, diuretics, tricyclic antidepressants, opioids, and selective serotonin reuptake inhibitors. The first step in the management is discontinuing the offending agents, patient education, and increasing salt and water intake. Exercise conditioning improves symptoms and should be advised to all patients.

Answer 193

Diplopia - 41% Ptosis - 25% Dysarthria - 16% Generalized weakness - 11% Dysphagia - 10%

Answer 194

Spinal cord - glycine Brain - GABA

Answer 195

The most common neurotoxic effects of L-asparaginase are cerebrovascular accidents, including cerebrovascular THROMBOSIS AND HEMORRHAGE L-asparaginase would cause further depletion of protein C and S levels, along with a reduction in anti-thrombin-III, platelet activation, and decline in plasminogen levels to promote thrombosis. The neurological adverse effects related to the use of cyclophosphamide are secondary to metabolic encephalopathy and syndrome of inappropriate anti-diuretic hormone (SIADH). These are not the most dangerous neurotoxic effects expected in this patient’s case.Daunorubicin is incorrect because daunorubicin is an anthracycline antineoplastic medication that does not cross the blood-brain barrier, so there is little evidence of any direct neurotoxic effects from its use.Vincristine is incorrect because vincristine causes peripheral neuropathy, which is not a dangerous, adverse effect and not specifically probable in this patient. Other neurotoxicities associated with vincristine use include encephalopathy, seizures, and, very rarely, Guillain-Barre syndrome. These adverse effects are possible in this patient but less dangerous and probable than L-asparaginase neurotoxicity.Methylprednisolone is incorrect because methylprednisone is not a chemotherapeutic agent; it is a corticosteroid that is used in chemotherapy regimens for leukemia to reduce hypersensitivity reactions to medications and to hamper inflammatory response from the tumor cells. Methylprednisolone does not have direct neurotoxic effects. However, it may cause indirect secondary effects by causing metabolic derangements.

Answer 196

The neurological adverse effects related to the use of cyclophosphamide are secondary to metabolic encephalopathy and syndrome of inappropriate anti-diuretic hormone (SIADH).

Answer 197

Glucocorticoids are the correct response because they are first-line management to treat pituitary apoplexy. Corticosteroids should be administered immediately because most patients have hypopituitarism which causes corticotroph deficiency. Corticotroph deficiency is considered a life-threatening condition. Thus, it should be corrected first

Answer 198

Caisson's = acute decompression sickness resulting from the impinging of blood vessels of the spinal cord by nitrogen bubbles from a sudden ascent in divers from high atmospheric pressure to low atmospheric pressures. The most common symptoms are headaches and visual disturbances. Patients may also experience seizures and blindness if complete vessels are occluded. Acute decompression syndrome may involve any of the organ systems, but the neurological system is commonly affected.

Answer 199

Foix-Alajouanine syndrome is caused by arteriovenous malformation of the spinal cord resulting in gradual progressive limb weakness and neurological deficit depending on the compression level of the spinal cord. A diagnosis of congestive myelopathy, secondary to a spinal vascular malformation, needs a high index of suspicion. Any patient with slowly progressive paraplegia with T2 hyperintensity in the cord and flow voids in the spinal subarachnoid space should be investigated cautiously with a spinal digital subtraction angiography.

Answer 200

L4 is the correct answer because it is the predominant nerve supply of the muscle anterior tibialis, which is involved in the dorsiflexion of the foot. L5 also supplies the anterior tibialis BUT L4 is the predominant nerve root that supplies this muscle.

Answer 201

it supplies the extensor digitorum longus and brevis, extensor hallucis longus (which causes dorsiflexion of toes), and peroneus longus and brevis (which causes foot eversion).

Answer 202

3 muscateers 2 were assasinated 4 men 5 rats 2 unicorns

Answer 203

Paraneoplastic optic neuropathy presents with blindness and a swollen disc. Anti-CRMP5 antibodies are also positive. Primary usually small cell lung cancer

Answer 204

diagnosis of ADHD in adults requires the presence of 5 or more symptoms of inattention and/or hyperactivity and impulsivity in greater than 2 settings for 6 or more months. This patient exhibited more than 5 symptoms of inattention, hyperactivity, and impulsivity. He, therefore, fulfills the criteria of ADHD in adults.

Answer 205

Botulinum toxin is produced by Clostridium botulinum, which is a gram-positive, spore-forming rod.

Answer 206

Effects of bifrontal disease are bilateral hemiparesis, spastic bulbar (pseudobulbar) palsy, abulia or akinetic mutism, inability to stay attentive and solve complex problems, thinking problems, social incompetence, behavioral disinhibition, inability to anticipate, labile mood, and varying combinations of grasping, sucking, obligate imitative movements, and utilization behavior. Sphincter incontinence and a gait disorder can also be present. Effects of left frontal lobe disease are right-sided hemiplegia, Broca's aphasia with agraphia, sympathetic apraxia of the left hand, contralateral gaze paresis, apathy, and loss of grasp and suck reflexes. Effects of right frontal lobe disease are left-sided hemiplegia, contralateral gaze paresis, apathy, and loss of grasp and suck reflexes. Effects of unilateral temporal lobe disease are homonymous contralateral upper quadrantanopia, amnesic aphasia, visual agnosia, amusia, Wernicke's aphasia, and, occasionally, amnesic (Korsakoff) syndrome. Effects of bitemporal disease are Korsakoff's amnesic defect, apathy and, Klüver-Bucy syndrome.

Answer 207

Hypersexuality and hyperactivity Hyperorality Bulimia Visual agnosia Decreased emotional reaction Hypermetamorphosis, characterized as "an irresistible impulse to notice and react to everything within sight"

Answer 208

Oculomasticatory myorhythmia refers to acquired pendular vergence oscillations of the eyes associated with concurrent contraction of the masticatory muscles. When the myorhythmia also involves nonfacial skeletal muscles, it is called oculofacial-skeletal myorhythmia. There is a smooth, rhythmic eye convergence which cycles at a frequency of approximately 1 Hz, followed by divergence back to the primary position. Rhythmic elevation and depression of the mandible is synchronous with the ocular oscillations that persist in sleep and are unaltered by stimuli. Patients with oculomasticatory myorhythmia may also have paralysis of vertical gaze, progressive somnolence, and intellectual deterioration. This distinct movement disorder has been recognized only in Whipple's disease. Whipple's disease may also cause convergence nystagmus at approximately 1 Hz (pendular vergence oscillations). Whipple's disease is caused by Tropheryma whipplei, a bacteria. It is thought that infection with this bacteria alters T cell function leading to some of the symptomatology Pathophys - foamy macrophages

Answer 209

Negri bodies are cytoplasmic eosinophilic inclusions within neurons composed of Rabies virus. Most frequently found in pyramidal cells of hippocampus and Purkinje cells of cerebellum

Answer 210

In symptomatic patients with acute hyponatremia (in which the known duration is less than 24-48 hours), urgent correction by 4-6 mmol/L is advised to prevent neurological damage from cerebral ischemia as well as brain herniation. Sodium levels should be monitored at 2-hour intervals to ensure that the sodium correction is not taking place too quickly. A bolus of 100 ml 3% NaCl IV should be administered over 10 min and repeated up to 3 doses until acute symptoms subside. The rate of sodium correction should not exceed 4-6 mmol/L/day.

Answer 211

Mesolimbic pathway - Dopaminergic neurons from ventral tegmental area (VTA) (hook: vhere the trouble arises) to the nucleus accumbens

Answer 212

1) Mesolimbic pathway (+ schizo sx) - VTA to nucleus accumbens 2) Mesocortical pathway (- schizo sx) - VTA to cortex 3) Nigrostriatal (EPS) - substantia nigra to striatum 4) Tuberoinfundibular - hypothalamus to pituitary gland, inverse relationship - increased dopamine leads to decreased prolactin

Answer 213

Antipsychotics inhibit dopaminergic pathways, by inhibiting tuberoinfundibular pathway decreased DA from hypothalamus decreases inhibition of pituaitary leading to increased prolactin and subsequent amenorrhea and galactorrrhea

Answer 214

First gen antipsychotics inhibit D2 receptors and have HIGH rates of EPS Second gen antipsychotics inhibit D2 but ALSO inhibit 5HT2a which returns some of the DA back and decreases total dopaminergic inhibition enough that positive symptoms are decreased but lower rates of EPS

Answer 215

Tx with anticholinergics like benztropine or benadryl

Answer 216

Discontinue the antipsychotic Clozapine might help Valbenazine (VMAT2) might help

Answer 217

Beta-blockers Second line: anti-cholinergics, mirtazepine, clonidine

Answer 218

NMS - Kidney Disease K - K+, cK, Killer cells (leukocytosis) D - Discontinue antisychotic, Dantroline, Dopamine

Answer 219

Binds ryanodine receptor to decrease Ca2+ intracellularly and reduce rigidity

Answer 220

Cyptoheptadine

Answer 221

Autosomal recessive defect in ATP7B (Cu2+ transporter) Hook: Wilson A+

Answer 222

Suspect in patient <40 with dysarthria, gait changes, tremor, dystonia, droling, pyschiatric disease, hyperreflexia DX with elevated 24hr Cu urine collection reduced ceruloplasmin Kayser Fleischer ring MRI - high T2 signal in BG, abnormal thalamus, midbrain, or other TREATABLE and potentially FATAL so boards favorite

Answer 223

Penicillamine - FIRST LINE Triantine second line

Answer 224

Deficiency in dopamine production Mutation in the DYT5 gene coding for GTP cyclohydrolase 1 is Autosomal DOMINANT This enzyme constitutes part of the tetrahydrobiopterin synthetic pathway, the cofactor for tyrosine hydroxylase.

Answer 225

AKa spinocerebellar ataxia 3 Autosomal DOMINANT (all spinocerebellar ataxias) Slowly progressive limb and gait ataxia, dystonia, +symptoms of parkinsonism

Answer 226

LRRK2 (park - lark) Associated with N. African Arabs and Ashkenazi jews Typically late-onset

Answer 227

Parkin - PARK2 Young onset, NO Lewy bodies in brain

Answer 228

Make sure thinking about treatable conditions: - Wilson's disease - DA- responsive dystonia Genetic young onset: - AR PD w/ Parkin mutation Juvenile HD may initial present parkinsonian rather than w/ chorea

Answer 229

Rivastigmine is FDA approved for PD dementia MoA: cholinesterase inhibitor

Answer 230

The ABSENCE of ataxia If ataxia is present, it's not PD. Think about SCA3 (Machado-Joseph disease) or parkinson's plus syndrome. Idiopathic parkinson's disease should always have narrow base and shuffling gait

Answer 231

Quetiapine + Clozapine - least EPS SE Pimavanserin - 5HT inverse agonist (FDA approved)

Answer 232

900-1000mg/day (build up to this)

Answer 233

Remember MSA-P and MSA-C and can have mix between the two So Parkinson's + autonomic sx + cerebellar sx Autonomic fx: orthostatic hypotension, ED, inspiratory nocturnal stridor, sleep apnea, higher perception of pain, temp intolerance Nystagmus, ataxia

Answer 234

>40 repeats of CAG

Answer 235

Westphal variant - presents as rigid hypokinetic syndrome

Answer 236

DA depleters like tetracenazine and detrabenazine (FDA approved for HD) Note that they can also WORSEN depression and suicidality may need to tx with SSRI

Answer 237

AD fashion Usually begins in childhood

Answer 238

EPM = progressive myoclonic epilepsies Dentatorubral-pallidoluysian atrophy - DRPLA - autosomal dominant - progressive ataxia, myoclonus, epilepsy, intellectual disability Lafora's disease - autosomal recessive - EPM2A Myoclonic epilepsy and red ragged fibers MERRF - aka Fukuhara syndrome - avoid VPA given increased liver risks Gaucher's - impaired glucocerebrosidase Galactosialidosis Neroinal ceroid lipofusciosis Baltic myoclonus aka Unverricht-Lundborg aka progressive myoclonus 1 - EPM1 - impaired lysosomal function

Answer 239

Hand-wringing - Rett syndrome Self-injurious behavior - Lesch-Nyan syndrome

Answer 240

DA antagonists: pimozide, haloperidol Clonidine or guanfacine (off label: topiramate, tetrabenazine)

Answer 241

Autosomal dominant DYT1 mutation Associated with Ashkenazi jews

Answer 242

Sensory tick touching chin improves dystonia through feedback Do NOT confuse with psychogenic

Answer 243

Botox for focal dystonias Levo-dopa trial for possible dopamine-responsive dystonia Oral meds less effective - trihexyphenidyl, benzos, baclofen DBS for refractory cases

Answer 244

Iron deficiency (ferritin <50) Patients w/ family history Pts on SSRIS Pts with renal disease Pregnancy

Answer 245

EKG You should be monitoring for autonomic symptoms!!! Patients necessitate cardiac monitoring and measurement of forced vital capacity as they may develop fatal cardiac arrhythmias.

Answer 246

Topiramate is metabolized by and is an inhibitor of cytochromes CYP2C19 and CYP3A4, which are needed for the metabolism of phenytoin, leading to a toxic build-up of this drug.

Answer 247

Increased suicidality and depression (w Phenytoin levels above 30 μg/mL.)

Answer 248

Autosomal dominant disorder is due to a mutation in the SODIUM channel. Attacks typically begin in infancy or early childhood. These sudden episodes of muscle weakness tend to last for a few minutes or up to an hour; rarely, they can last for 1 or 2 days. Potassium levels are elevated during an event but return to a normal level in between episodes. Attacks may be triggered by exposure to cold, rest after vigorous exercise, eating foods high in potassium, stress or fatigue. Between attacks, patients may complain of muscle stiffness. Myotonia is evident both proximally and distally, particularly during an attack.

Answer 249

This autosomal dominant disorder is a skeletal muscle channelopathy in which voltage-gated potassium, calcium, or sodium ion channels are mutated, leading to attacks of muscle paralysis. The disease usually presents in childhood or adolescence. The episodes of muscle weakness have a sudden onset and may last for hours or even days; they are associated with concomitant hypokalemia. Attacks may be brought on after a period of rest, particularlyAFTER EATING a meal high in carbohydrates. They can occur late at night, or the patient may awaken with symptoms. Presentation can vary from mild weakness of the legs to total paralysis of all the limb and trunk muscles. During the attack, muscle fibers do not respond to electrical stimulation, and deep tendon reflexes are decreased or absent. Once the attack subsides, the neurological exam is usually normal. The interval between attacks may vary from annually to multiple attacks occurring daily. NO MYOTONIA is evident.

Answer 250

Dix-Hallpike maneuver: upward nystagmus when patient is reclining (head extended back 20 degrees and tilted at 45 degrees), downward nystagmus when sitting up.

Answer 251

Long thoracic nerve supplies the serratus anterior, which stabilizes the scapula and enables its upward rotation. Weakness in this muscle results in an inability to raise the arm over the head and scapular winging of its MEDIAL border, which can be seen clearly when the arm is outstretched and pushed forward against resistance.

Answer 252

Dysfunction of trapezius muscle and spinal accessory nerve

Answer 253

C5, C6, C7

Answer 254

A disc herniation usually affects the spinal nerve BELOW the level of the lesion so this typically involves S1 radiculopathy leading to a weak Achilles tendon reflex

Answer 255

don't put homos down

Answer 256

Defect in the phenylalanine hydroxylase (PAH) gene. If untreated, subsequent accumulation of phenylalanine results in permanent intellectual disability, developmental delay, seizures, psychiatric issues, and behavioral problems. Individuals with this disease have eczema, a musty odor, and lighter hair and skin color compared to their family members who do not have PKU. Management of PKU involves the dietary restriction of phenylalanine.

Answer 257

The tibial CMAP response shows evidence of temporal dispersion. Temporal dispersion is associated with ACQUIRED demyelinating conditions, such as CIDP. Hereditary Sensori-Motor Neuropathy Type I (CMT) is a demyelinating neuropathy; but not acquired so it is NOT associated with temporal dispersion.

Answer 258

Phytanoyl-αCoA-hydroxylase is the correct answer because the aforementioned case describes a patient with a likely diagnosis of Refsum disease. Refsum disease is a rare inherited autosomal recessive disorder characterized by the presence of retinitis pigmentosa, ataxia, and chronic polyneuropathy with increased blood phytanic acid. Refsum disease occurs due to deficiency of the peroxisomal enzyme, phytanoyl-coenzyme A (CoA) hydroxylase, which leads to the accumulation of phytanic acid. The patient commonly presents with night blindness and visual field defects preceding the onset of neuropathy; the neuropathy is sensorimotor, distal, and symmetrical in distribution and affects the lower limbs more than the upper limbs. There is a global sensory impairment with absent deep tendon reflexes.

Answer 259

FABRY DISEASE, which is a sex-linked disorder caused by the deficiency of alpha-galactosidase. It leads to the accumulation of glycolipid (ceramide trihexoside) in peripheral nerves, which is characterized by pain, primarily in the fingers and toes, and paresthesias of the palms and soles.

Answer 260

Krabbe disease, which is characterized by the accumulation of galactocerebroside and leads to the destruction of oligodendrocytes and depletion of lipids in the cerebral white matter. Most patients die by the end of the first year of life; survival beyond 2 years is unusual.

Answer 261

Metachromatic Leukodystrophy, which is an autosomal recessive metabolic disorder characterized by the congenital absence of arylsulfatase and leads to accumulation of sulfatide throughout the nervous system. There is progressive cerebral deterioration, hyporeflexia muscular atrophy, and decreased nerve conduction velocity.

Answer 262

Tangier disease, which is an inherited autosomal recessive small-fiber neuropathy characterized by the presence of sensory impairment, especially of pain and temperature sensation, over the entire body with preserved tactile and proprioception

Answer 263

Detrusor overactivity DO occurs due to demyelination in the frontal cerebral cortex impairing the detrusor reflex and thus resulting in uninhibited bladder contractions. Patients complain of urinary frequency, urgency, and urge incontinence. Can also develop detrusor-sphincter dyssynergia is described as the contraction of the detrusor muscle in the absence of relaxation of the urethral sphincter

Answer 264

“five P's” of acute intermittent porphyria (AIP) Painful abdomen, Polyneuropathy, Psychologic disturbances, Precipitated by drugs/alcohol, and Purple pee Hemin is recommended for the management of symptoms in patients of acute intermittent porphyria (AIP). It inhibits the function of δ-aminolevulinate synthase. This decreases heme synthesis which limits the production of intermediates, resulting in symptom control.

Answer 265

MELAS or MERRF - inherited by mitochondrial DNA heteroplasmy Ragged red fibers are the hallmark of MELAS. A trichrome stain shows a brilliant red appearance of the ragged red fibers with occasional cytoplasmic bodies. Diseases caused by mutations in mitochondrial DNA, such as MELAS, can only be passed onto the children from the mother. Patients with MELAS show heteroplasmy, in which mutant and wild-type mitochondrial DNA molecules exist in the same cell.

Answer 266

1. Sensory disturbance - including paresthesia, hypoesthesia, pins and needle sensation, tingling, and dysesthesias; Lhermitte symptom. 2 - Motor symptoms - gait disturbance, limb weakness, and reduced muscle power 3 - Visual loss - 13% of the patients with multiple sclerosis present with vision loss

Answer 267

Reduced conduction velocity Conduction block Small compound motor and sensory potentials Mechanism: SEGMENTAL (aka focal)demyelination EMG/NCS is the correct answer because abnormalities of nerve conduction are detected early in patients with Guillain–Barre syndrome (GBS), including a decrease in the amplitude of muscle action potentials, slowed conduction velocity, and motor nerve conduction block; these are dependable indicators of the disease, as compared to CSF analysis.

Answer 268

Toddlers (<14mo) - ESAT Young children (<8yo) - PEDS Children 7-16yo - ASSQ or DAWBA Adults - Social Responsiveness Scale for adults (SRS-A) is a 65-item questionnaire completed by the caregiver.

Answer 269

Eagle syndrome is the correct option. This patient has glossopharyngeal neuralgia, as evidenced by severe pain in the territory of the glossopharyngeal nerve distribution. The possible causes include autoimmune disorders, infections, demyelination, carcinoma of the tongue, and benign tumors like an acoustic neuroma. In this patient, Eagles syndrome or stylalgia is the most likely etiology. Treatment includes carbamazepine, duloxetine, and valproic acid. A 26-year-old woman presents to the emergency department with complaints of severe stabbing and electric shock-like pain in her right ear, the base of the tongue, and posterior jaw. It occurs without warning and is precipitated by chewing, swallowing, and yawning. Although the pain lasts for a few seconds, it is excruciating and leaves her drained. Physical examination, including examination of the ears and tongue, is unremarkable. Apart from a styloid process of 26 mmHg, imaging is unremarkable. Which of the following is the most likely underlying cause of this patient's presentation?

Answer 270

Insulin neuritis is also known as treatment-induced neuropathy. It presents with a RAPID-onset of PAINFUL but reversible hyperalgesia in the fingers and toes, which occurs after rapid normalization of previously impaired blood sugar levels. It occurs due to endoneurial ischemia, microvascular injury to neurons, and regenerating nerve firing. Treatment is supportive.

Answer 271

Splenic impairment as seen in sickle cell disease with auto asplenia

Answer 272

Delayed passage of meconium and distended gut loops on abdominal radiographs. About 10% of the patients may present with diarrhea or perforation due to enterocolitis. It is associated with Down's syndrome and RET proto-oncogene/ A full-thickness rectal biopsy is confirmed as it reveals the absence of ganglion cells in the Meissner and Auerbach plexus of the terminal rectum.

Answer 273

The diagnostic triad of paraneoplastic optic retinopathy constitutes: 1. photosensitivity 2. ring scotoma 3. attenuation of retinal arterioles; SCC of the lung is the most common malignancy associated with paraneoplastic optic retinopathy but is NOT a part of the diagnostic triad of paraneoplastic optic retinopathy. Decreased vision is also not a part of the diagnostic triad. A patient with paraneoplastic optic neuropathy can present with a decrease in vision, but such a symptom is not diagnostic. SCC of the lung is the most common malignancy associated with paraneoplastic optic retinopathy but is not a part of the diagnostic triad of paraneoplastic optic retinopathy. SCC of the lung is the most common malignancy associated with paraneoplastic optic retinopathy but is not a part of the diagnostic triad of paraneoplastic optic retinopathy

Answer 274

Downbeat nystagmus may occur with cervicomedullary junction disease, midline medullary lesions, posterior midline cerebellar lesions, or diffuse cerebellar disease. Most responsible lesions affect the vestibulocerebellum (flocculus, paraflocculus, nodulus, and uvula) and the underlying medulla. Deficient drive by the posterior semicircular canals, whose central projections cross in the floor of the fourth ventricle, has been postulated as an explanation for downbeat nystagmus. Downbeat nystagmus is common with cervical-medullary junction processes, e.g., Chiari malformation. Nystagmus or vertigo induced by coughing or the Valsalva maneuver may occur with Chiari malformation or perilymph fistulas.