Exam 3 -- Idiopathic Intracranial Hypertension and Prion Diseases Flashcards Preview

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Flashcards in Exam 3 -- Idiopathic Intracranial Hypertension and Prion Diseases Deck (30):

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

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


What are the major risk factors for idiopathic intracranial hypertension?

Female of childbearing age, obesity


Some medications can induce IIH. Name some of them.

Oral contraceptives, tetracyclines, and retinoids.


What are some systemic conditions that are associated with IIH?

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


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

Venous stenosis and/or venous sinus thrombosis


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

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


What are the symptoms of IIH?

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


What are the ocular clinical findings associated with IIH?

Papilledema is the major one; also visual field loss.


What might imaging show that could be due to IIH?

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.


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.

True; can elevate to more than 250 mmH20


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

Modified Dandy criteria


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

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


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

Optic nerve sheath fenestration, CSF shunting.


What disease makes up 90% of prion diseases?

Creutzfeldt-Jakob disease


What are the signs of Creutzfeldt-Jakob disease?

Rapidly deteriorating mental status and myoclonus.


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

Spongiform degeneration (pockets left by misfolded proteins)


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

Hyperintensity lesions at caudate, putamen, cortex


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

Periodic sharp wave complexes


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

14-3-3 protein


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

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


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

CJD: 65 years
vCJD: 29 years


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

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


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

CJD: 6 months; vCJD: 14 months


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

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


Where would an MRI show hyperintensity in vCJD?



Variant CJD involves amyloid plaques with PrPsc.

Free card.


What is the name for the "shivering disease" or "laughing sickness"?



What uncommon practice is associated with kuru?



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

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


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

9 to 24 months