Exam 3 -- Idiopathic Intracranial Hypertension and Prion Diseases Flashcards

1
Q

Idiopathic intracranial hypertension is due to an increase in CSF and is caused by what?

A

Trick question; there is no underlying cause (diagnosis is of exclusion)

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2
Q

What are the major risk factors for idiopathic intracranial hypertension?

A

Female of childbearing age, obesity

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3
Q

Some medications can induce IIH. Name some of them.

A

Oral contraceptives, tetracyclines, and retinoids.

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4
Q

What are some systemic conditions that are associated with IIH?

A

Sleep apnea, polycystic ovary syndrome, coagulation disorders, anemia, lupus

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5
Q

The proposed mechanism of IIH involves decreased outflow of CSF via?

A

Venous stenosis and/or venous sinus thrombosis

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6
Q

True or false: the normal volume of CSF is between 60-150ml, and the body produces about 250ml a day.

A

False; there is indeed 60-150ml of CSF, but the body produces 500ml per day

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7
Q

What are the symptoms of IIH?

A

Headache is the major one; can also get transient visual obscurations, pulsatile tinnitus, and diplopia (particularly CN VI)

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8
Q

What are the ocular clinical findings associated with IIH?

A

Papilledema is the major one; also visual field loss.

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9
Q

What might imaging show that could be due to IIH?

A

Empty sella turcica, flattening of posterior sclera, enlargement of subarachnoid space around the optic nerve, turtuosity of the optic nerve, slit-like ventricles, narrowing of venous sinus.

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10
Q

True or false: a lumbar puncture of a patient with IIH would show CSF that is normal in composition, but highly elevated in opening pressure.

A

True; can elevate to more than 250 mmH20

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11
Q

What is the name for the set of criteria used to determine if a case qualifies as IIH?

A

Modified Dandy criteria

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12
Q

Weight loss is one of the main treatment options for IIH. What pharmacologic options are available?

A

Carbonic anhydrase inhibitor (acetazolamide), topiramate (also used for epilepsy and associated with a myopic shift and angle closure), furosemide or methazolamide (diuretics)

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13
Q

What surgical options might be considered for a patient with IIH?

A

Optic nerve sheath fenestration, CSF shunting.

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14
Q

What disease makes up 90% of prion diseases?

A

Creutzfeldt-Jakob disease

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15
Q

What are the signs of Creutzfeldt-Jakob disease?

A

Rapidly deteriorating mental status and myoclonus.

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16
Q

What would a brain biopsy show in a patient with Creutzfeldt-Jakob disease?

A

Spongiform degeneration (pockets left by misfolded proteins)

17
Q

What would an MRI show in a patient with Creutzfeldt-Jakob disease?

A

Hyperintensity lesions at caudate, putamen, cortex

18
Q

What would an EEG show in a patient with Creutzfeldt-Jakob disease?

A

Periodic sharp wave complexes

19
Q

What would show in the CSF of a patient with Creutzfeldt-Jakob disease?

A

14-3-3 protein

20
Q

True or false: though there is no treatment for Creutzfeldt-Jakob disease, patients usually live for 5 years after diagnosis.

A

False; patients live for about 6 months (and there is no treatment)

21
Q

Compare and contrast the age of the typical patient with Creutzfeldt-Jakob disease and variant CJD.

A

CJD: 65 years
vCJD: 29 years

22
Q

How do the symptoms of variant CJD compare with those of CJD?

A

They include the rapid deterioration of mental status and myoclonus, as well as psychiatric symptoms and paresthesia.

23
Q

How long do the symptoms last in Creutzfeldt-Jakob disease compared to variant CJD?

A

CJD: 6 months; vCJD: 14 months

24
Q

True or false: both CJD and vCJD commonly have a periodic sharp wave complex (PSWC) on an EEG

A

False; CJD does indeed have this finding, but in vCJD, it is rare and occurs later in the disease.

25
Q

Where would an MRI show hyperintensity in vCJD?

A

Pulvinar

26
Q

Variant CJD involves amyloid plaques with PrPsc.

A

Free card.

27
Q

What is the name for the “shivering disease” or “laughing sickness”?

A

Kuru

28
Q

What uncommon practice is associated with kuru?

A

Cannibalism

29
Q

Where would PrPsc plaques show up in a patient with kuru? What symptoms might occur in a patient with this disease?

A

Cerebellum; symptoms would include postural instability, myoclonus, and late onset dementia

30
Q

Death in a patient with kuru occurs within how long after diagnosis?

A

9 to 24 months