Exam 3 -- Idiopathic Intracranial Hypertension and Prion Diseases

Question 1

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

Answer 1

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

Question 2

What are the major risk factors for idiopathic intracranial hypertension?

Answer 2

Female of childbearing age, obesity

Question 3

Some medications can induce IIH. Name some of them.

Answer 3

Oral contraceptives, tetracyclines, and retinoids.

Question 4

What are some systemic conditions that are associated with IIH?

Answer 4

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

Question 5

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

Answer 5

Venous stenosis and/or venous sinus thrombosis

Question 6

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

Answer 6

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

Question 7

What are the symptoms of IIH?

Answer 7

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

Question 8

What are the ocular clinical findings associated with IIH?

Answer 8

Papilledema is the major one; also visual field loss.

Question 9

What might imaging show that could be due to IIH?

Answer 9

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.

Question 10

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.

Answer 10

True; can elevate to more than 250 mmH20

Question 11

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

Answer 11

Modified Dandy criteria

Question 12

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

Answer 12

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

Question 13

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

Answer 13

Optic nerve sheath fenestration, CSF shunting.

Question 14

What disease makes up 90% of prion diseases?

Answer 14

Creutzfeldt-Jakob disease

Question 15

What are the signs of Creutzfeldt-Jakob disease?

Answer 15

Rapidly deteriorating mental status and myoclonus.

Question 16

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

Answer 16

Spongiform degeneration (pockets left by misfolded proteins)

Question 17

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

Answer 17

Hyperintensity lesions at caudate, putamen, cortex

Question 18

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

Answer 18

Periodic sharp wave complexes

Question 19

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

Answer 19

14-3-3 protein

Question 20

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

Answer 20

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

Question 21

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

Answer 21

CJD: 65 years
vCJD: 29 years

Question 22

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

Answer 22

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

Question 23

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

Answer 23

CJD: 6 months; vCJD: 14 months

Question 24

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

Answer 24

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

Question 25

Where would an MRI show hyperintensity in vCJD?

Answer 25

Pulvinar

Question 26

Variant CJD involves amyloid plaques with PrPsc.

Answer 26

Free card.

Question 27

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

Answer 27

Kuru

Question 28

What uncommon practice is associated with kuru?

Answer 28

Cannibalism

Question 29

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

Answer 29

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

Question 30

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

Answer 30

9 to 24 months