Neuro Bytes Board Prep

Question 1

What are the criteria for manic episode?

Answer 1

Mania is characterized by an (1) abnormally and persistently elevated, expansive, or irritable mood and (2) increased goal-directed activity or energy, lasting at least 1 week and causing gross functional impairments. The additional requirement of increased activity or energy is new in the DSM-5; previously, only elevated mood was required.

To diagnose a manic episode, mood changes should be present for at least 1 week, for most of the day, but can be of any duration if psychiatric hospitalization is required. During the period of mood disturbance and increased energy, three or more of the following symptoms must be present and represent a change from usual behavior:
– inflated self-esteem or grandiosity
– decreased need for sleep
– more talkative than usual or pressure to keep talking
– flight of ideas or racing thoughts
– distractibility and inattention
– increased goal-directed activity or psychomotor agitation
– excessive involvement in high-risk activities.

Question 2

In a patient with bipolar I with history of attempted suicide, what would the ideal medication be?

Answer 2

Lithium

All the medications listed (quetiapine, aripiprazole, lamotrigine, lithium, valproate) are reasonable options for treatment of bipolar I disorder, but lithium is the best option for this patient, as she presented with a history of attempted suicide.
A meta-analysis of 31 bipolar disorder studies involving a total of 85,229 person-years of risk-exposure reported the overall risk of suicide and attempted suicide was FIVE TIMES LESS among subjects treated with lithium than among those not treated with lithium (RR = 4.91, 95% CI 3.82-6.31, P < 0.0001). Valproate is not the best option for this patient as she is of childbearing age, especially when another appropriate alternative is available.

Question 3

What are some associations with meralgia paresthetica?

Answer 3

Pregnancy
External compression (ie belts)
Obesity

Question 4

Where does the lateral femoral cutaneous nerve come from?

Answer 4

It is a pure sensory nerve which branches from the LUMBAR PLEXUS

Formed from fibers of posterior division of anterior rami of spinal nerves L2 and L3

Question 5

What is the most common presentation of neurosarcoidosis?

Answer 5

Chronic meningitis

although cranial nerve palsy, parenchymal involvement, myelopathy, peripheral neuropathy, and myopathy also are possible.

Question 6

What is the most common type of cranial neuropathy in neurosarcoidosis?

Answer 6

Facial nerve palsy

Question 7

What is the most sensitive CSF abnormality in neurosarcoidosis?

Answer 7

he most sensitive but least specific CSF abnormality for neurosarcoidosis is elevated protein. Elevated cell count in the CSF is a useful marker for activity of disease.

Obtaining a CSF angiotensin-converting enzyme level, however, generally is not useful in the diagnosis of neurosarcoidosis.

Question 8

What type of HIV medication is most likely to cause peripheral neuropathy?

Answer 8

The dideoxynucleoside agents like (“-ine”):
didanosine (ddl)
stavudine (d4T)
zalcitabine (ddC)

are among the most toxic

Protease inhibitors (atazanavir, darunavir, lopinavir, ritonavir) are not associated with increased risk of neuropathy after adjusting for other risk factors

Question 9

What term has consolidated chronic major depressive disorder and dysthmic disorder?

Answer 9

Persistent depressive disorder

Question 10

What is the criteria of persistent depressive disorder?

Answer 10

Depressive symptoms must be present for at least 2 years to meet the diagnostic criteria (1 year for children and adolescents), with no longer than 2 months of symptom freedom at a time.

Symptoms include poor appetite or overeating, insomnia or hypersomnia, low energy or fatigue, low self-esteem, poor concentration or difficulty making decisions, and feelings of hopelessness.

A total of 75% of patients with persistent depressive disorder also experience at least one major depressive episode; episodes in these patients tend to be longer and more resistant to treatment than in patients without persistent depressive disorder. The existence of a depressive personality disorder has not been definitively settled; it has not been included since DSM-II.

Question 11

A 1-month-old infant is admitted to the neonatal intensive care unit for hypotonia and failure to thrive. The pregnancy and delivery were normal. On examination, she has paradoxical breathing, tongue fasciculations, diffuse hypotonia, and minimal spontaneous movements. Muscle stretch reflexes are absent.
What is the next best step in diagnosis?

Answer 11

SMN1 gene testing
spinal muscular atrophy (SMA) type 1 given the normal neonatal course followed by the development of fasciculations, hypotonia, respiratory failure, and areflexia. The most appropriate first step would be to test for SMA by obtaining SMN1 gene testing. Should the SMN1 gene test be negative, an EMG/nerve conduction study may confirm the presence of atypical motor neuron disease.

The presence of areflexia and fasciculations suggests a peripheral cause, excluding the need for a brain MRI. Myotonic dystrophy, which would be confirmed with DMPK gene testing, may present in infancy with respiratory failure and hypotonia, although these children do NOT have fasciculations and are not normal at birth. C9OR72 tests for familial amyotrophic lateral sclerosis.

Question 12

What condition is DMPK genetic testing for?

Answer 12

myotonic dystrophy

Question 13

What condition is C9OR72 genetic testing for?

Answer 13

Familial amyotrophic lateral sclerosis

Question 14

What are the fundamental features of a formal thought disorder?

Answer 14

Disturbed and disorganized thinking due to a loss of contact with reality.

Loosening of associations, or derailment, is a feature of formal thought disorder and refers to a changing of reference, even midsentence or thought, often to completely unrelated topics. Patients with a formal thought disorder are commonly evasive, vague, and indirect in their interactions with others rather than direct and straightforward.

Question 15

What is coprolalia?
What condition is it associated with?

Answer 15

involuntary expression of socially unacceptable words, such as curse words, and is most commonly associated with Tourette syndrome

Question 16

What is dysprosody?

Answer 16

Dysprosody is abnormal expression or comprehension of the emotional and tonal aspects of speech; this can manifest as foreign-accent syndrome and is not a common feature of formal thought disorder.

Question 17

Term for psychosis < 1month…

Answer 17

Brief psychotic disorder

Brief psychotic disorder (<1 mo)
Schizophreniform disorder (1-6 mo)
Schizophrenia >6 months

Question 18

Term for psychosis lasting 1-6 months…

Answer 18

Schizophreniform disorder

Brief psychotic disorder (<1 mo)
Schizophreniform disorder (1-6 mo)
Schizophrenia >6 months

Question 19

Criteria for schizophreniform disorder

Answer 19

Two or more of the following five symptoms must be present for diagnosis:
- delusions
- hallucinations
- disorganized speech representing formal thought disorder
- abnormal psychomotor behavior
- negative symptoms (eg, anhedonia, asociality, apathy, alogia, flat affect)

At least one of these symptoms must be delusions, hallucinations, or thought disorder, and there must be impaired functioning in one or more major life area (eg, work, school, self-care, interpersonal relationships).

Question 20

What aspects of narcolepsy due wake-promoting agents or stimulants treat? What does it NOT treat?

Answer 20

Treats:
- excessive sleepiness
- sleep attacks
- sleep eating
- hypnogogic hallucinations

Does NOT treat:
- cataplexy

Question 21

What are some treatment options of cataplexy?

Answer 21

tricyclic antidepressants
selective serotonin reuptake inhibitors
serotonin-norepinephrine reuptake inhibitors
sodium oxybate.

Question 22

What is the definition of cataplexy?

Answer 22

: a sudden loss of muscle control with retention of clear consciousness that follows a strong emotional stimulus (as elation, surprise, or anger) and is a characteristic symptom of narcolepsy.

Question 23

Patient presents with ophthalmoplegia, ataxia, and areflexia with c/f Miller-Fischer syndrome. What is the best initial treatment?

Answer 23

IVIG
Methylprednisolone has been shown to be ineffective in treating Guillain-Barré syndrome and may result in troubling adverse effects.

Question 24

45-year-old man presented with a six-month history of behavioral changes. He also developed mild to moderate subacute headaches. A head CT demonstrated two areas of cortical subarachnoid hemorrhage in the right parietal and left frontal lobes, with surrounding edema. A spinal puncture demonstrated an elevated protein level and pleocytosis. Vascular imaging showed mid and small size arteries with a “beaded” appearance. Which of the following is the gold standard diagnostic test for this condition?

Answer 24

Brain biopsy.
The clinical presentation, CSF and radiological findings are highly suggestive of primary central nervous system (CNS) angiitis or CNS vasculitis. Brain biopsy is considered the gold standard in establishing the diagnosis. The surgical sampling is usually guided by the locations of abnormalities detected by DSA and MR imaging. Biopsy is performed at the edge of the lesion in order to avoid biopsying only the necrotic core. The biopsy should include have and a cortical vessel. A diagnostic substraction angiogram shows characteristic findings of “beading” or “sausage on string” caused by regions of narrowing interposed with regions of vascular ectasia. However, these changes can also be seen in intracranial atherosclerotic disease, reversible vasoconstriction syndrome and other non-specific vasculopathies.

Question 25

Impact of valproic acid on P450 enzymes and subsequent impact on other drugs such as lamotrigine

How would you adjust lamotrigine dose?

Answer 25

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.

Question 26

Name some of the anti-seizure meds that are P450 inducers

Answer 26

carbamazepine, phenobarbital, and phenytoin

Question 27

What should you recommend for any woman with epilepsy who desires to become pregnant?

Answer 27

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.

Question 28

A 19-year-old man presents to clinic with progressive central visual loss initially affecting the left eye, but now involving both eyes. He is accompanied by his mother, who is concerned because her own mother and younger brother were affected by similar vision loss. Examination reveals bilateral optic disc hyperemia and circumpapillary telangiectasia but is otherwise unremarkable. What is the likely diagnosis?

Answer 28

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.

Question 29

What is the inheritance pattern of Leber hereditary optic neuropathy?

Answer 29

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.

Question 30

Describe Kjer-type autosomal dominant optic atrophy

Answer 30

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.

Question 31

A 68-year-old man admitted for septic shock 2 weeks ago is seen in consultation because of difficulty focusing his vision. He has symmetric pupils and full visual fields but has trouble interpreting complex figures. Instead of describing the entire image, he names individual features. He has difficulty initiating saccadic eye movements on command and has trouble with fine motor activities done under visual guidance. Which of the following is the most likely diagnosis?

Define Balint syndrome
What location is involved?

Answer 31

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.

Question 32

What is the most common neuropathy of the lower extremity?

Answer 32

Peroneal

Question 33

What does peroneal neuropathy present as on exam? How do you differentiate from L5 radiculopathy?

Answer 33

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.

Question 34

What medications might worsen childhood absence epilepsy?

Answer 34

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

Question 35

What is the mechanism of action of ethosuximide?

Answer 35

Blocks voltage sensitive T-type calcium channels

Question 36

What is valproic acid’s mechanism of action?

Answer 36

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

Question 37

What is levetiracetam’s mechanism of action?

Answer 37

Levetiracetam reduces neurotransmitter release through binding synaptic vesicle protein SV2A

Question 38

What is topiramate’s mechanism of action?

Answer 38

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.

Question 39

what is the total volume of CSF in adults?

Answer 39

~150cc at any given moment

Question 40

What rate is CSF produced at?

Answer 40

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)

Question 41

What proportion of CSF is contained in ventricles?

Answer 41

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

Question 42

45yo woman presents with recurrent migraines, TIA, and early-onset dementia

MRI is shown below

What is the diagnosis?

Answer 42

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.

Question 43

What is the mutation of CADASIL?

Answer 43

NOTCH3 on chromosome 19p13.12

Question 44

Describe the classical MRI findings of CADASIL?

Answer 44

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.

Question 45

CADASIL electron microscopy skin findings?

Answer 45

Granular deposits in the basal lamina of a small blood vessel

Question 46

CADASIL light microscope skin biopsy findings?

Answer 46

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

Question 47

A 16 year old boy comes to the clinic because he has been experiencing headaches and vision disturbances. The headaches have worsened over this past year and also are associated with nausea. His head is imaged and a suprasellar mass with calcifications is identified. The mass is removed surgically and gross histological examination reveals the presence of cystic spaces that are filled with thick dark brown fluid. Which of the following is the most likely diagnosis?

Adamantinomatous craniopharyngioma
Epidermoid cyst
Papillary craniopharyngioma
Rathke cleft cyst

Answer 47

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.

Question 48

70yo previously promiscuous gentleman who presented with dementia, vision loss w/ impaired light reflex, unsteady gait, hyporeflexia – condition assoc. w/ findings?

Answer 48

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

Question 49

C5 motor action

Answer 49

Elbow flexion (biceps)

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

Question 50

C6 motor action

Answer 50

Wrist flexion

also elbow pronation

Question 51

C7 motor action

Answer 51

Elbow extension (Triceps)

Finger flexion at proximal joint, extension
Thumb flexion, extension and abduction in plane of thumb

Question 52

C8 motor action

Answer 52

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

Question 53

T1 motor action

Answer 53

Abduction of the 2nd and 5th fingers

Question 54

L2 motor action

Answer 54

hip flexion

Question 55

L3 motor action

Answer 55

Knee extension

hip external rotation

Question 56

L4 motor action

Answer 56

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

Question 57

L5 motor action

Answer 57

great toe dorsiflexion

Question 58

S1 motor action

Answer 58

foot plantar flexion

Question 59

A central cord lesion causes neurologic deficits in a _______ (ascending/descending) order

Answer 59

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.

Question 60

What is the pattern of sensory loss in each of the following?

Complete transection of thoracic cord

Answer 60

Question 61

What is the pattern of sensory loss in each of the following?

Advanced central thoracic lesion

Answer 61

Question 62

What is the pattern of sensory loss in each of the following?

Brown Sequard syndrome (t3)

Answer 62

Question 63

What is the pattern of sensory loss in each of the following?

Cauda equina

Answer 63

Question 64

What is the pattern of sensory loss in each of the following?

Anterior spinal artery syndrome (T4)

Answer 64

Question 65

What is the pattern of sensory loss in each of the following?

Anterior spinal artery syndrome (T4)

Answer 65

Question 66

Describe the following spinal cord syndromes

Answer 66

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

Question 67

Patient is a 80 year old gentleman with history of hypertension who presented with left leg weakness – imaging suggestive of?

Answer 67

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

Question 68

Describe phases of blood on MRI

Answer 68

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

Question 69

What does EEG measure?

Action potentials
Presynaptic potentials
Postsynaptic potentials
Summation of postsynaptic potentials

Answer 69

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

Question 70

What three questions should you ask as approach each EEG?

Answer 70

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

Question 71

What is a normal PDR frequency for the following ages

3-4 months
1 year
2 years
3 years
>8 years

Answer 71

Question 72

What is the frequency of the following rhythms?

delta
theta
alpha
beta
gamma

Answer 72

Question 73

What direction is positive deflection in EEG?

Answer 73

Moving downward

Question 74

Differentiate awake vs each stage of sleep by EEG?

Awake
Drowsy
Stage N1
Stage N2
Stage N3
REM
Coma

Answer 74

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

Question 75

What stage is this patient in?
What is being shown on EEG?

Answer 75

Stage N2 sleep
K complex (z appearance with spindle

Question 76

What is being shown in the following EEG?
What stage of sleep is the patient in?

Answer 76

Vertex waves
Stage I and II of sleep

Question 77

What is being shown in the following EEG? What stage of sleep is this?

Answer 77

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.

Question 78

What is the rule of 2s related to sleep spindles?

Answer 78

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

Question 79

What stage of sleep is shown below?

Answer 79

stage II sleep - marked by sleep spindles and K complexes

Question 80

What stage of sleep is shown below?

Answer 80

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

Question 81

Answer 81

Question 82

What stage of sleep is this?

Answer 82

Question 83

Answer 83

Question 84

What is happening in the following?

Answer 84

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!!)

Question 85

How do you tell what direction gaze is directed at on EEG?

Answer 85

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

Question 86

What is going on in this EEG?

Answer 86

Chewing artifact

PDR not interrupted, maintained
Does not evolve, pretty consistent

Question 87

What is going on in this EEG?

Answer 87

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

Question 88

What is going on in this EEG?

Answer 88

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

Question 89

What is going on in this EEG?

Answer 89

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

Question 90

What is going on in this EEG?

Answer 90

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

Question 91

What rhythm seeing here?

Answer 91

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

Question 92

Explain eye blinks on EEG

Answer 92

Question 93

Explain lateral eye movement positivity

Answer 93

Question 94

What is going on in the following EEG?

Answer 94

Question 95

What is going on in the following EEG?

Answer 95

Question 96

What is going on in the following EEG?

Answer 96

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.

Question 97

What is going on in the following EEG?

Answer 97

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.

Question 98

What is going on in this EEG?

Answer 98

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

Question 99

What is going on in this EEG?

Answer 99

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.

Question 100

What is going on in this EEG?

Answer 100

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

Question 101

What is going on in this EEG?

Answer 101

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

Question 102

What is going on in this EEG?

Answer 102

Sleepy patient with sleep spindles on the right (stage 2)

Upper left is phase reversal sharp wave = left temporal sharp waves

Question 103

What is going on in this EEG?

Answer 103

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

Question 104

What is going on in this EEG?

Answer 104

Lateralized periodic discharges (~1Hz) left parietal

Sharp with slow component

Question 105

What is going on in this EEG?
What syndrome is this associated with?

Answer 105

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

Question 106

What is going on in this EEG?
What syndrome is associated?

Answer 106

Used to be benign rolandic epilepsy

Question 107

Patient aged 7-13 with hemifacial twitching, excessive salivation, inability to speak

Answer 107

BECTS?

Question 108

What is going on in this EEG?
What is the associated syndrome?

Answer 108

~4Hz polyspike component

Question 109

What is going on in this EEG?

Answer 109

Notice there is NOT a polsypike component

Question 110

What syndrome is associated with this EEG?

Answer 110

Question 111

Patient with vomiting episodes, migraine, autonomic episodes, and this EEG…

What is the syndrome?

Answer 111

Question 112

What is going on in this EEG?
What is this associated with when it is abnormal?
When is it normal?

Answer 112

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

Question 113

What consideration do you need to take into account when choosing anti-seizure medication for treatment of a child with absence seizures?

Answer 113

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

Question 114

Differentiate HSV 1 and HSV 2 in terms of infections and populations impacted

Answer 114

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

Question 115

What types of medications have side effect of sleep attacks?

Answer 115

Dopamine agonist therapy (ie pramipexole)

Question 116

Localization of alexia without agraphia

Answer 116

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

Question 117

Which triptan should be selected in patients who have migraines mostly around the time of menses? Why?

Answer 117

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

Question 118

57-year-old woman seen in ICU for AMS. She had been ill the week before with an upper respiratory tract infection. On the day of admission she had a generalized tonic-clonic seizure lasting <1min and was brought to the ED. On exam she rouses only to painful stimuli and is unable to follow commands. No focal deficits. EEG demonstrates diffuse slowing. Brain MRI shows multiple enhancing lesions in white matter. HSV PCR in CSF is negative. Which is the most appropriate next step in management?

Answer 118

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

Question 119

What is the most common nerve root affected in cervical radiculopathy?

Answer 119

C7 causing sensory impairment of middle finger

Question 120

What is the DSM5 designation for patients with multiple somatic symptoms?

Answer 120

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

Question 121

A 25 year old man reports vertical strabismus after a TBI sustained during a football game. Which of the following cranial nerves is most likely involved?

Answer 121

Trochlear IV

Question 122

What is the triad of Balint syndrome?

Answer 122

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

Question 123

What proportion of ischemic strokes are lacunar infarctions?

Answer 123

20% of all ischemic strokes

Question 124

Lacunar size definition

Answer 124

<1.5cm in diameter

Question 125

Which of the following is observed during repetitive nerve stimulation studies in patients who have botulism?

Decremental response when stimulating 2Hz
Decremental response when stimulating 30Hz
Decremental response stimulating 50Hz
Incremental response stimulating 2Hz
No response when stimulating 30HZ

Answer 125

Decremental response when stimulating 2Hz
Decremental response when stimulating 30Hz
Decremental response stimulating 50Hz
Incremental response stimulating 2Hz
No response when stimulating 30HZ

[explanation]

Question 126

Patient is a 40yo gentleman presents with complaint of right side arm and leg weakness, ataxia, loss of vibration and proprioception and left side loss of pain/temp from the neck down – likely lesion?

Answer 126

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

Question 127

Patient is a 50yo lady who presents complaining of involuntary tonic/clonic contractions of the left side of face – initially beginning with involuntary closure of the left eye – likely diagnosis?

Answer 127

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

Question 128

Patient is a 50yo lady who presents complaining of involuntary tonic/clonic contractions of the left side of face – initially beginning with involuntary closure of the left eye – likely diagnosis?

Answer 128

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

Question 129

Patient is a 40yo gentleman who presented with a complaint of migraine w/ scintillations beginning when he was 15yo. Imaging consistent with?

Answer 129

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

Question 130

What structure is shown in these images? What disease is being shown in B?
What is being shown in right corner?

Answer 130

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

Question 131

3 month old infant – rapidly progressive muscle weakness, hypotonia, large/protruding tongue, feeding/swallowing difficulties, respiratory difficulty. Echo found hypertrophic cardiomyopathy – condition?

Answer 131

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

Question 132

Genetic mutation and mode of inheritance in Benign familial neuonatal convulsions (BFNC)

Answer 132

Mutations in VG K ch (KCNQ2 and KCNQ3)
AD inheritance

HOOK:
Familial = AD
Convulsions – C sounds like K – potassium channel

Question 133

Describe Ohtahara syndrome

Answer 133

Question 134

Describe Dravet syndrome

Answer 134

Question 135

Describe infantile spasms/West syndrome

Answer 135

Question 136

Describe Panayiotopoulos syndrome

Answer 136

Question 137

Describe Landau-Kleffner syndrome

Answer 137

Question 138

What effect do the following drugs have on valproic acid blood levels?

CBZ
PHT
ASA

Answer 138

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

Question 139

What dose adjustment needs to be made when using VPA and LTG together?

Answer 139

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

Question 140

What impact do OCPs have on LTG?

Answer 140

They decrease LTG levels thereby potentially reducing its efficacy

Question 141

What is the usual titration schedule of lamotrigine?

What about when prescribed with an enzyme inducer? (CBZ)

What about when prescribed with an enzyme inhibitor? (VPA)

Answer 141

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%

Question 142

What is the maximum speed of phenytoin IV infusion?

Answer 142

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

Question 143

Explain phenytoin starting dosing and adjustments

Answer 143

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

Question 144

What are the antiseizure medications with the most teratogenic side effects (top 3)?

Answer 144

Valproic acid (8.9%)
Phenobarbital(5.9%)
Topiramate (4.4%)

Carbamazepine (3.0 %)
Phenytoin (2.8 %)
Lamotrigine (2.1%)
Levetiracetam (2.0 %).

Question 145

What antiseizure medications are the most likely to transmit through breast milk and thus most dangerous during breastfeeding?

Answer 145

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.

Question 146

What is the mechanism and key side effect of vigabatrin?

Answer 146

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

Question 147

Perampanel mechanism of action and key side effect

Answer 147

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

Question 148

Levetiracetam mechanism of actino

Answer 148

Unknown, binds to SV2A protein

Question 149

Lacosamide mechanism of action

Answer 149

Na+ channel (slow) blocker

Question 150

Lamotrigine mechanism of action

Answer 150

Na+ and CA++ channel blocker

Question 151

Basic mechanisms of anti-seizure drugs

Answer 151

Question 152

SUDEP risk factors

Answer 152

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

Question 153

Causes of mortality with epilepsy from most to least common:

Answer 153

Accidental death
Status epilepticus
Suicide
SUDEP

Question 154

Describe myoclonic epilepsy with red ragged fibers (MERRF)

Answer 154

Question 155

Describe Lafora disease

Answer 155

EMP2A mutation

Question 156

EEG rapid fire

Spike and sharp waves over temporoparietal and occipital regions during sleep, surprisingly normal when awake

Answer 156

Landau-Kleffner

Question 157

EEG rapid fire

Generalized slow spike-and wave discharges when awake and busts of paroxysmal fast activity when asleep

Answer 157

Lennox Gastaut

Question 158

EEG rapid fire

high-voltage centrotemporal spike-wave activity that may shift from side to side with normal background

Answer 158

BECTS

Question 159

Describe BECTS aka Rolandic

Answer 159

Benign Rolandic epilepsy
Benign Childhood Epilepsy with centrotemporal spikes (BECTS

Autosomal dominant inheritance with variable penetrance

Question 160

Describe Lennox-Gastaut syndrome

Answer 160

Question 161

Describe Juvenile myoclonic epilepsy

Answer 161

JME type 1 associated with EFHC1 mutation
GABRA1 (CACNB4)

Question 162

Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) associated mutation

Answer 162

CHRNA4, nicotinic Ach gene receptor

Question 163

Alternating hemiplegia of childhood mutation

Answer 163

Question 164

Episodic ataxia

Answer 164

Question 165

Dantrolene use and mechanism

Answer 165

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.

Question 166

Atropine mechanism

Answer 166

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

Question 167

WFNS grade

Answer 167

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

Question 168

Most common cause of sporadic fatal encephalitis in the US

Answer 168

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

Question 169

Cytarabine can cause acute _____ toxicity

Answer 169

Irreversible cerebellar toxicity
Characterized by selective loss of Purkinje cells

Question 170

Treatment of cryptococcal meningitis?

Answer 170

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.

Question 171

Side effects of primidone

Answer 171

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.

Question 172

Side effect of tolcapone

Answer 172

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

Question 173

Four cardinal features of RLS

Answer 173

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.

Question 174

Describe Westphal variant of HD

Answer 174

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.

Question 175

What tests should be obtained prior to use of ergot-derived DA agonists ie cabergoline?

Answer 175

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.

Question 176

Describe Friedrich ataxia

Answer 176

Question 177

What parkinson’s plus syndrome is associated with INSPIRATORY stridor?

Answer 177

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.

Question 178

When might chorea be more likely to present in a patient with lupus?

Answer 178

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.

Question 179

When does chorea gravidarum tend to develop?

Answer 179

Chorea gravidarum tends to develop in primigravida women, particularly in the first trimester.

Usually occurs in women who had syndenham chorea in the past

Question 180

What medication can be used to help treat levodopa-associated dyskinesias?

Answer 180

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.

Question 181

What is a major side effect of tetrabenazine?

Answer 181

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

Question 182

What is amantadine helpful for in Huntington’s disease?

Answer 182

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)

Question 183

Paraneoplastic cerebellar degeneration characterized by what pathologic feature?

Answer 183

Extensive loss of purkinje cells

Question 184

What is the pathologic feature of spinocerebellar degeneration?

Answer 184

Axonal loss in the posterior columns

Question 185

What is the pathologic feature of limbic encephalitis?

Answer 185

Reactive gliosis and neuronal loss affecting gray matter of limbic structures

Question 186

What is the mechanism of memantine?

Answer 186

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

Question 187

What is the main treatment of behavioral variant of FTD?

Answer 187

SSRIs like fluoxetine

Question 188

What neurotransmitter is produced in the locus coeruleus?

Answer 188

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.

Question 189

AD protocol treatment

Answer 189

Question 190

Where is orexin produced?

Answer 190

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

Question 191

Describe Isaac’s syndrome

Answer 191

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.

Question 192

Satoyoshi disease

Answer 192

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

Question 193

Treatment of hepatic encephalopathy

Answer 193

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.

Question 194

A dramatic loss of proprioception and vibratory sense with relatively intact motor function should prompt consideration of…

Answer 194

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

Question 195

Describe Alpers-Huttenlocher syndrome

Answer 195

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.

Question 196

A 75-year-old woman had left upper teeth extraction 3 months ago. She was doing well until 2 weeks ago, when she noted the onset of numbness and constant pain in the left cheek. Neurologic examination reveals decreased pin and soft touch in the left cheek and upper lip that also involves the medial and lateral upper incisors, canine teeth, and adjacent gingiva, but spares more posterior teeth and gums. The examination was otherwise normal. Which one of the following diagnoses is the most likely diagnosis for this patient?

Answer 196

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.

Question 197

Treatment of hypoglycemia caused by sulfonylureas

Answer 197

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.

Question 198

Describe oculopharyngeal muscular dystrophy

Answer 198

Question 199

What is the most common cause of generalized MSK pain?

What is the FDA approved medication to treat it?

Answer 199

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

Question 200

Tolosa Hunt syndrome

Answer 200

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.

Question 201

Treatment Prader Willi syndrome

Answer 201

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.

Question 202

What sensory receptor is activated by COOL temperatures or COOLING agents?

Answer 202

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)

Question 203

Describe domains of GCS

Answer 203

Question 204

Describe trigeminal neuralgia

Answer 204

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.

Question 205

Describe characteristics of pheochromocytoma headaches

Answer 205

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.

Question 206

What is the clinical picture from anterior spinal cord infarction?

Answer 206

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.

Question 207

Testicular seminoma is associated with the presence of _____ antibodies.

Answer 207

anti-Ma2 antibodies

Question 208

Anti-Hu antibodies can be seen in ________ cancer

Answer 208

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

Question 209

Anti-glutamic acid decarboxylase antibodies are associated with _____ cancer

Answer 209

Thymoma and small cell carcinoma

Question 210

Anti-CRMP 5 antibody is the most common antibody found in patients with paraneoplastic _______

Answer 210

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

Question 211

At what point does hypoglycemia cause neuronal death ?

Answer 211

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).

Question 212

A left-handed patient presents with a stroke in the right temporal lobe. Which one of the following language disturbances is the patient most likely to suffer from?

A. Difficulty with comprehension and repetition
B. Difficulty with comprehension only
C. Difficulty with fluency and repetition
D. Difficulty with recognizing and producing affective content
E. No language deficit

Answer 212

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.

Question 213

What deficits would you expect from R posterior spinal artery infarction?

Answer 213

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.

Question 214

Describe clinical features of central cord syndrome

Answer 214

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)

Question 215

What is the primary site of production of serotonin?

Answer 215

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.

Question 216

What is the primary site of acetylcholine production and AD?

Answer 216

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.

Question 217

What neurotransmitter is synthesized in nucleus accumbens?

Answer 217

GABA

Question 218

Describe tobacco-alcohol amblyopia

Answer 218

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.

Question 219

Describe ASIA impairment scale for spinal injuries

Answer 219

Question 220

Describe radiation induced plexopathy

Answer 220

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.

Question 221

What should pregnant women who receive phenytoin be supplemented with?
Newborn?

Answer 221

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.

Question 222

‘Optic glioma is associated with what genetic syndrome…

Answer 222

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.

Question 223

Syndrome associated with increased risk of non-Hodgkin’s lymphoma

Answer 223

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.

Question 224

Giant cell astrocytoma development is associated with what neurocutaneous disorder….

Answer 224

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.

Question 225

Renal cell carcinoma development is associated with what inherited disorder….

Answer 225

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.

Question 226

NF2 is a type of ____ function mutation

Answer 226

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.

Question 227

Most common myopathy of AIDS patients….

Answer 227

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.

Question 228

What type of inflammation is seen on pathology in dermatomyositis?

Answer 228

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.

Question 229

Describe Krabbe’s disease features

Answer 229

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.

Question 230

Describe Tay-Sachs

Answer 230

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.

Question 231

Describe Fabry disease

Answer 231

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.

Question 232

Describe Nieman Pick

Answer 232

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?

Question 233

A 50-year-old sailor presents to the clinic with repeated falls for the past 4 months. He states that his legs suddenly “turn to jelly” and he has problems climbing the stairs. He also has to swallow repeatedly and has developed troublesome regurgitation of food. He has been recently diagnosed with diabetes mellitus. The patient has a 100 pack-year smoking history with a chronic smoker’s cough. On examination, he has weakness of knee extension and hip flexion, which is more marked on the left side. He is unable to keep his fingers flexed against resistance. The remainder of the examination, including sensation and cranial nerves, is normal. Serum creatine kinase is within range. Which of the following is the most likely diagnosis in this patient?

Answer 233

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.

Question 234

What part of the thalamus is targeted in DBS for essential tremor?

Answer 234

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.

Question 235

What are features of good candidacy for DBS?

Poor candidacy/contraindictions?

Answer 235

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.

Question 236

Metabolic finding in alcohol intoxication

Answer 236

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

Question 237

What percent of patients with status epilepticus will go on to develop epilepsy?

Answer 237

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

Question 238

What is the response rate of epilepsy surgery in patients with mesial temporal sclerosis?

Answer 238

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.

Question 239

Down syndrome associations

Answer 239

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.

Question 240

Adult opsoclonus-myoclonus is most commonly associated with….

Answer 240

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.

Question 241

Describe symptoms of homocystinuria and treatment

Answer 241

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.

Question 242

Gabapentin enacarbil (brand: Horizant) benefit and use

Answer 242

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

Question 243

Describe peripheral neuropathy due to isoniazid

Answer 243

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.

Question 244

When do fontanelles close?

Answer 244

By 18 months, US before then

Question 245

Treatment of vasculitic neuropathy

Answer 245

Combination steroids and rituximab

Question 246

Treatment of sudden sensorineural hearing loss

Answer 246

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

Question 247

Differentiate focal clonic from focal myoclonic seizure

Answer 247

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.

Question 248

Location of injury in capgras syndrome

Answer 248

Right frontal cortex lesions

Question 249

A 23-year-old gentleman presents with severe dystonia of the trunk and a reduced range of motion in all his limbs. He has a history of juvenile Huntington’s disease. He has mild chorea. His neurological examination reveals brisk tendon reflexes and an intermittent tremor in his left hand. How should this patient be managed?

Answer 249

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.

Question 250

Describe MRI aging of hemorrhage

Answer 250

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

Question 251

Indications for multiple sleep latency test (MLST)

Answer 251

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.

Question 252

POTS diagnostic criteria

Answer 252

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.

Question 253

Most common presenting symptoms in myasthenia gravis

Answer 253

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

Question 254

Describe Lafora disease and associated mutation

Answer 254

Question 255

What is the main inhibitory NT in the spinal cord?

In the brain?

Answer 255

Spinal cord - glycine

Brain - GABA

Question 256

What is the most common neurotoxic effect of L-asparginase?

Answer 256

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.

Question 257

Cyclophosphamide neurologic adverse effects

Answer 257

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

Question 258

Describe Fabry disease

Answer 258

Question 259

Treatment of pituitary apoplexy

Answer 259

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

Question 260

Most common cervical fracture resulting in central cord syndrome

Answer 260

Question 261

Symptoms of Caisson’s disease

Answer 261

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.

Question 262

What is Foix-Alajouanine syndrome?

Answer 262

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.

Question 263

What nerve root is predominantly involved in dorsiflexion of the foot?

Answer 263

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.

Question 264

What muscles in the foot does S1 supply?

Answer 264

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).

Question 265

Mneumonic for brachial blexus

Answer 265

3 muscateers
2 were assasinated
4 men
5 rats
2 unicorns

Question 266

Paraneoplastic optic neuropathy is associated with…antibodies

Answer 266

Paraneoplastic optic neuropathy presents with blindness and a swollen disc. Anti-CRMP5 antibodies are also positive.

Primary usually small cell lung cancer

Question 267

Diagnosis of ADHD in adults requirements

Answer 267

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.

Question 268

C botulinum is what type of bacteria?

Answer 268

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

Question 269

What are some clinical symptoms of BIFRONTAL lesions?

Answer 269

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.

Question 270

Describe Kluver Bucy syndrome

Answer 270

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

Question 271

What is the distinct movement disorder associated with Whipple’s disease?

Answer 271

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

Question 272

Negri body association

Answer 272

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

Question 273

Hirano body

Answer 273

AD

Question 274

Pick bodies in dentate gyrus

Answer 274

FTD

Question 275

What conditions are associated with pathology shown here?

Answer 275

DLB, PD

Question 276

treatment hyponatremia

Answer 276

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.

Question 277

What is the main structure implicated in positive symptoms of schizophrenia?

Answer 277

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

Question 278

Describe all four dopaminergic pathways of the brain

Answer 278

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

Question 279

Describe antipsychotic impact on tuberoinfundibular pathway and impacts

Answer 279

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

Question 280

What action on receptors differentiates first from second gen antipsychotics? Why is this so important?

Answer 280

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

Question 281

Treatment of EPS dystonia

Answer 281

Tx with anticholinergics like benztropine or benadryl

Question 282

Tardive dyskinesia treatment

Answer 282

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

Question 283

Treatment of akathesia

Answer 283

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

Question 284

What are the features of NMS? (hook)

Answer 284

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

Question 285

What is the MoA of dantroline?

Answer 285

Binds ryanodine receptor to decrease Ca2+ intracellularly and reduce rigidity

Question 286

What is the treatment of serotonin syndrome

Answer 286

Cyptoheptadine

Question 287

Describe BG direct and indirect pathway

Answer 287

Question 288

Wilson’s disease genetics

Answer 288

Autosomal recessive defect in ATP7B (Cu2+ transporter)

Hook: Wilson A+

Question 289

Wilson syndrome clinical features & Dx

Answer 289

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

Question 290

Wilson’s disease treatment

Answer 290

Penicillamine - FIRST LINE
Triantine second line

Question 291

What is the underlying cause of dopamine-responsive dystonia?

Answer 291

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.

Question 292

Describe Machado-Joseph disease

Answer 292

AKa spinocerebellar ataxia 3

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

Question 293

What is the most common genetic mutation for:
- autosomal dominant inheritance of parkinsonS?

Answer 293

LRRK2 (park - lark)

Associated with N. African Arabs and Ashkenazi jews
Typically late-onset

Question 294

What is the most common genetic mutation for:
- autosomal recessive inheritance of parkinsonS?

Answer 294

Parkin - PARK2
Young onset, NO Lewy bodies in brain

Question 295

What is a key ddx for young onset (<40) Parkinson’s?

Answer 295

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

Question 296

What medication is approved for Parkinson’s disease dementia?

Answer 296

Rivastigmine is FDA approved for PD dementia
MoA: cholinesterase inhibitor

Question 296

What is a key exclusionary symptom/exam feature for Parkinson’s disease?

Answer 296

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

Question 296

PD preferred antipsychotics

Answer 296

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

Question 297

What dose is an adequate trial of levodopa?

Answer 297

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

Question 298

Parkinson’s plus syndrome associated with INSPIRATORY stridor…

Answer 298

MSA

Question 299

MSA symptoms

Answer 299

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

Question 300

Full penetrance HD

Answer 300

> 40 repeats of CAG

Question 301

Huntington’s before age 21…

Answer 301

Westphal variant - presents as rigid hypokinetic syndrome

Question 302

Treatment of chorea

Answer 302

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

Question 303

Essential myoclonus is inherited in…

Answer 303

AD fashion
Usually begins in childhood

Question 304

Name progressive myoclonic epilepsies

Answer 304

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

Question 305

Non-tic steotypies to know
Hand-wringing…
Self-injurious behavior…

Answer 305

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

Question 306

Treatment of Tourette syndrome

Answer 306

DA antagonists: pimozide, haloperidol
Clonidine or guanfacine

(off label: topiramate, tetrabenazine)

Question 307

Generalized dystonia mutation - presents in childhood

Answer 307

Autosomal dominant DYT1 mutation
Associated with Ashkenazi jews

Question 308

Geste antagoniste

Answer 308

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

Question 309

Describe neuroacanthocytosis

Answer 309

Question 310

Dystonia treatments

Answer 310

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

Question 311

RLS is more prominent in…

Answer 311

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

Question 312

What is a critical test to get in patient with GBS?

Answer 312

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.

Question 313

How does topiramate interact with phenytoin?

Answer 313

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.

Question 314

What is an important psychiatric side effect of phenytoin toxicity?

Answer 314

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

Question 315

Describe hyperkalemic periodic paralysis. It is due to a mutation in what kind of channel?

Answer 315

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.

Question 316

Describe hypOkalemic periodic paralysis?

Answer 316

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.

Question 317

Dix-Hallpike maneuver is considered positive when ____ seen when reclining and ____ seen sitting up

Answer 317

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

Question 318

What nerve supplies serratus anterior?

Answer 318

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.

Question 319

What is the cause of lateral scapular winging?

Answer 319

Dysfunction of trapezius muscle and spinal accessory nerve

Question 320

What nerve roots are involved in long thoracic nerve?

Answer 320

C5, C6, C7

Question 321

Describe L5-S1 disc herniation symptoms

Answer 321

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

Question 322

Describe sensory dermatomes of the leg and foot

Answer 322

Question 323

Describe sensory dermatomes of the torso

Answer 323

Question 324

Describe sensory dermatomes of the arm and hand

Answer 324

Question 325

Describe hypersensitivity reactions

Answer 325

Question 326

Differentiate from Marfan syndrome and homocystinuria

Answer 326

don’t put homos down

Question 327

Describe phenylketouria (PKU)

Answer 327

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.

Question 328

What clinical syndrome is temporal dispersion associated with?

Answer 328

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.

Question 329

Describe Refsum disease

Answer 329

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.

Question 330

α-galactosidase mutation….

Answer 330

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.

Question 331

β-galactosidase mutation….

Answer 331

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.

Question 332

Arylsulfatase A and B mutation…

Answer 332

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.

Question 333

ABC1 protein mutation…

Answer 333

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

Question 334

Most common urodynamic finding in MS patients

Answer 334

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

Question 335

Describe porphyria (hook)

Answer 335

“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.

Question 336

Epilepsy w/ red-ragged fibers

Dx and inheritance

Answer 336

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.

Question 337

Most common (3) presenting features of MS

Answer 337

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

Question 338

GBS NCS results

Answer 338

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.

Question 339

Autism screening tools by age

Answer 339

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.

Question 340

Describe Eagle syndrome

Answer 340

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?

Question 341

MoA of oxybutynin

Answer 341

Question 342

Describe insulin neuroitis

Answer 342

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.

Question 343

Presence of this signifies?

Answer 343

Splenic impairment as seen in sickle cell disease with auto asplenia

Question 344

Hirschsprung disease sx and mutation

Answer 344

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.

Question 345

Diagnostic triad of paraneoplastic optic retinopathy

Answer 345

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

Question 346

Downbeat nystagmus localization

Answer 346

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.