Case Review Questions Flashcards
What are the diagnostic criteria for NF-2?
- Bilateral vestibular schwannoma.
- Family history of NF-2 (first degree relative) plus unilateral VS.
- Family history of NF-2 plus two of the following: meningioma, schwannoma (nonvestibular), glioma, neurofibroma, juvenile posterior subcapsular lenticular cataract, or opacity.
What is the 50/50 rule for serviceable hearing?
- A pure tone (PTA) showing values of < 50 dB and speech discrimination with recognition of > 50% indicate serviceable hearing.
Differential diagnosis for intraventricular lesions?
CENTRAL MS
- Choroid plexus papilloma or carcinoma, colloid cyst, central neurocytoma, cavernoma.
- Ependymoma, epidermoid/dermoid.
- Neurocytoma.
- Teratoma, tuber.
- “Rule out” infection.
- Astrocytoma, AVM, aneurysm, abscess.
- Lipoma, lymphoma.
- Mets, meningioma.
- Subependymoma, SEGA.
Which neurocutaneous syndrome is associated with SEGAs?
- Tuberous sclerosis.
What are some complications that can be seen with transcallosal approaches to the lateral ventricles?
- Weakness.
- Akinetic mutism.
- Memory deficits.
Which vein does the thalamostriate vein empty into?
- Internal cerebral vein.
What are some findings associated with Sturge-Weber?
- Port wine stain (usually affecting V1).
- Leptomeningeal angiomas.
- Seizures.
- Hemiparesis.
- MR or learning disability.
- Glaucoma.
What are some indications for hemispherectomy?
- Intractable epilepsy with unilateral hemisphere damage.
- Congenital hemiplegia.
- Chronic encephalitis.
- Hemimegalencephaly.
- Sturge-Weber syndrome.
What lab abnormality can be seen with hemangioblastomas?
- Polycythemia.
Genetic mutation seen in NF-1?
- NF1 gene mutation on chrom. 17.
Genetic mutation seen in NF-2?
- NF2 gene mutation on chrom. 22.
Genetic mutation seen in tuberous sclerosis?
- TSC1 and TSC2 gene mutations on chrom. 9 and 16.
Genetic mutation seen in VHL?
- VHL gene mutation on chrom. 3P.
What are the 3 main types of spinal AVMs?
- Dural AV fistula.
- Perimedullary fistula.
- Intramedullary AVMs.
Pathophysiology of dural AV fistulas.
- Lesion consists of a small AV fistula within or just beneath the dura at the point where a feeding radicular artery enters the dura at the nerve root sleeve.