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Question 1

What are the clinical features of IIH and risk factors?

Answer 1

Clinical presentation: obese woman of childbearing age with headache and papilloedema. Other sx include transient visual obscurations, intracranial noises (pulsatile tinnitus), photopsia, back pain, retrobulbar pain, diplopia, sustained visual loss.Headache – not necessarily typical pattern – lateralizing and throbbing or pulsatile, intermittent or persistent, daily or less frequently. Retrobulbar pain and pain on eye movements are more specific. Transient visual obscurations: bilateral or unilateral lasting for seconds. Can change with posture.

Question 2

DDx for IIH

Answer 2

Malignant hypertension:Also presents with headache and papilloedemaSecondary intracranial hypertension: any cause that increased intracranial hypertension including: intracranial mass lesion, obstruction to venous outflow (venous sinus thrombosis), obstructive hydrocephalus, decrease CSF absorption or increased CSF production, malignant systemic hypertension

Question 3

How is IIH diagnosed?

Answer 3

Normal or characteristic findings on MRI / MRVopening pressure >25 on LP (20-25 = equivocal)

Question 4

Treatment for IIH?

Answer 4

Weight lossAcetazolamide (carbonic anhydrase inhibitor) decreases the rate of CSF productionTopiramate (less effective, alternative if SE of acetazolamide not tolerated)

Question 5

What is a chiari malformation? What are the types? How does it present? What is the treatment?

Answer 5

Chiari malformation describes a group of structural defects of the cerebellum, characterized by brain tissue protruding into the spinal canal.Chiari malformations are often associated with myelomeningocele, hydrocephalus, syringomyelia, and tethered cord syndrome.Chiari type 1 refers to herniation of the cerebellar tonsils alone, and radiologically as simple tonsillar herniation 5 mm or greater below the foramen magnum (Elster and Chen 1992); type 2 refers to herniation of both the cerebellum and lower brainstem; type 3 refers to a rare type of brainstem herniation in association with a cervical or occipital encephalocele; type 4 involves extreme cerebellar hypoplasia and caudal displacement of the posterior fossa contents. Presentation is often dependent on type of malformation and associated structures - Many individuals with Chiari 1 malformation do not have symptoms and go undiagnosed until adolescence. Alternatively, because of brainstem involvement, type 2 malformations are typically diagnosed earlier in childhood. Although studies of etiology are few, an increasing number of specific genetic syndromes are found to be associated with Chiari malformations. Management primarily targets supportive care and neurosurgical intervention when necessary.