Pathology VI

Question 1

What is a possible compilcation of cerebellar tonsillar herniation into the foramen magnum?

Answer 1

Coma and death when herniation compresses the brainstem (p.448)

Question 2

What causes central vertigo?

Answer 2

Brainstem or cerebellar lesion (e.g stroke affecting vestibular nucleu or posterior fossa timor) (p.446)

Question 3

What clinical findings are present in a patient with central vertigo?

Answer 3

Directional change of nystagmus, skew deviation, diplopia, dysmetria (p.446)

Question 4

What positional testing results indicate central vertigo?

Answer 4

Immediate nystagmus in any direction which may change directions (p.446)

Question 5

What is the difference between positional testing results of peripheral vs central vertigo?

Answer 5

Peripheral- delayed horizontal nystagmus; central- immediate nystagmus in any direction that may change directions (p.446)

Question 6

Name four neurocutaneous disorders.

Answer 6

Sturge-Weber syndrome, Tuberous sclerosis, neurofibromatosis type I (von Recklinghausen’s disease), von Hippel Lindau Disease (p.446)

Question 7

What is Sturge-Weber syndrome?

Answer 7

A congenital disorder with port-wine stains typically in the V1 opthalmic distribution. Sporadic occurance (p.446)

Question 8

What symptoms and findings are characteristic of Sturge-Weber syndrome?

Answer 8

Ipsilateral leptomeningeal angiomas, pheochromocytomas, glaucoma, seizures, hemiparesis, mental retardation (p.446)

Question 9

What symptoms are characteristic of Tuberous Sclerosis?

Answer 9

Hamartomas in CNS and skin; adenoma sebaceum (cutaneous angiofibromas); Mitral regurgitation; Ash-Leaf spots; cardic rhabdomyoma; Tuberous sclerosis; mental retardation; renal angiomyolipoma; seizures (p.446)

Question 10

How is tuberous sclerosis inherited?

Answer 10

Autosomal dominant (p.446)

Question 11

What symptoms are characteristic of neurofibromatosis type I (von Recklinghausen’s diseae)?

Answer 11

Cafe-au-lait spots, Lisch nodules (pigminted iris hamartomas), neurofibromas in skin, optic gliomas, pheochromocytomas (p.446)

Question 12

Describe the mechanism of inheritance of Neurofibromatosis type I.

Answer 12

Autosomal dominant; 100% penetrant with variable expression (p.446)

Question 13

What gene is mutated in Neurofibromatosis type I?

Answer 13

NF1 gene on chromosome 17 (p.446)

Question 14

What symptoms are characteristic of von Hippel-Lindau disease?

Answer 14

Cavernous hemangiomas in skin, mucosa, organs; bilateral renal cell carcinoma, hemangioblastomas in retina, brainstem, cerebellum; pheochromocytomas (p.446)

Question 15

Describe the mechanism of inheritance of von Hippel Lindau disease.

Answer 15

Autosomal dominant; mutated tumor suppressor VHL gene on chromosome 3 (p.446)

Question 16

Name the five types of primary adult brain tumors.

Answer 16

Glioblastoma multiforme (grade IV astrocytoma); Meningioma, Schwannoma, Oligodendroglioma; Pituitary adenoma (p.447)

Question 17

What is the most common type of primary adult brain tumor?

Answer 17

Glioblastoma multiforme (grade IV astrocytoma) (p.447)

Question 18

Where is a glioblastoma multiforme typically found?

Answer 18

In the cerebral hemispheres and can cross the corpus callosum (p.447)

Question 19

What histological staining can pick up a glioblastoma multiforme?

Answer 19

GFAP (stains for astrocytes) (p.447)

Question 20

What characterizes histology of glioblastoma multiforme tumors?

Answer 20

Pseudopalisading, pleomorphic tumor cells that border central areas of necrosis and hemorrhage (p.447)

Question 21

What is the second most common type of primary adult brain tumor?

Answer 21

Meningioma (p.447)