Review Questions 2

Question 1

A neurologic disorder characterized pathologically by loss of neurons in Substantia Nigra

Answer 1

Parkinson Disease

Question 2

• Tremors AT REST
• Muscular rigidity
• Expressionless/Mask-like Face
• Emotional lability (depression/dementia)
• Drooling
• Very little blinking
• Cog-wheel rigidity

Answer 2

Clinical features of Parkinson Disease

Question 3

Which neurotransmitter is deficient in Parkinson Disease?

Answer 3

Dopamine (produced by Substantia Nigra, are responsible for relaying messages that plan/control body movement)

Question 4

When does Parkinson Disease usually appear?

Answer 4

• 6th decade (50-59 yrs)
• 8th decade (70-79 yrs)

Question 5

When does Parkinson Disease incidence decline?

Answer 5

After 80 yrs old

Question 6

What % of N. America will develop Parkinson Disease?

Answer 6

More than 2%

Question 7

Is not genetic, sex, or racial

Answer 7

Parkinson Disease

Question 8

Vast majority of cases of Parkinson Disease are due to which etiology?

Answer 8

Idiopathic

Question 9

Genetic factor of Parkinson Disease

Answer 9

• Rare, autosomal dominant
• Early onset
• Point mutation of chromosome 4

Question 10

What part of the brain is affected by Parkinson Disease?

Answer 10

• Substantia Nigra (relays info to the Basal Glanglia) through dopaminergic synapses

Question 11

What group of brain tumors account for 60% of primary intracranial neoplasms?

Answer 11

Gliomas (tumors of neuroectodermal origin)

Question 12

Corticol disease (outside of brain affected)

Answer 12

Alzheimer Disease

Question 13

• Most common cause of dementia in elderly (1/2 of all cases)
• Before what age is the prevalence 1 - 2%?
• After what age is prevalence over 10%

Answer 13

Alzheimer Disease

• under 65 yrs
• over 85 yrs

Question 14

Which chromosome has the Alzheimer gene?

Answer 14

21

Question 15

Which sex is affected twice as often by Alzheimer Disease?

Answer 15

Women

Question 16

What % of Alzheimer Disease has a familial variant (genetic)

Answer 16

5 - 10%

Question 17

Etiology of Alzheimer Disease

Answer 17

Idiopathic, but there is suspicion for viral cause which is not proven

Question 18

Significant advances of what pathogenesis have been made for Alzheimer Disease?

Answer 18

Beta-protein amyloid deposition in senile plaques

Question 19

located in areas of the cerebral cortex and are linked to intellectual function and are a constant feature of Alzheimer Disease

Answer 19

Senile Plaques

Question 20

What 3 microscopic features are needed to dx Alzheimer Disease?

Answer 20

1. Senile Plaques
2. Neurofibrillary tangles
3. Beta-protein Amyloid Angiopathy

Question 21

Origin of deposits in the brain

Answer 21

Beta-protein amyloid (found in walls of cerebral blood vessels)

Question 22

• Paired helical filaments
• abnormal form
• microtubule protein
• proper axonal transport

Answer 22

Neurofibrillary tangles

Question 23

What happen to the Gyri and Sulci w/ AD?

Answer 23

• Gyri narrow
• Sulci widen

Question 24

• Which parts of the brain are affected by AD?
• Which part is spared?

Answer 24

• Bilateral cortical atrophy of frontal, temporal, parietal, and hippocampal cortices
• Spares occiput

Question 25

Brain loses how many grams over how many years?

Answer 25

* 200 grams * 3 - 8 years

Question 26

Which part of the brain is the best place to examine for the 3 microscopic findings to dx AD?

Answer 26

Hippocampus

Question 27

discrete spherical masses of silver-staining neuritic processes surrounding a central amyloid core. Found predominantly in the hippocampus and amygdala, as well as the cortex.

Answer 27

Senile (neuritic) Plaques

Question 28

* bundles of paired, helical filaments in the cytoplasm of cortical neurons or hippocampal pyramidal cells that displace or encircle the nucleus (flame cells). * Are dramatically demonstrated on silver stains and are commonly found in the cortex, hippocampus, and the amygdala.

Answer 28

Neurofibrillary Tangles

Question 29

found within cerebral blood vessels seen with **congo red stains.**

Answer 29

Amyloid Angiopathy

Question 30

Clinical assessment + modern radiologic methods allow acurate dx in what % of cases of AD?

Answer 30

80 - 90%

Question 31

What is the usual cause of death of a bedridden patient with late stage AD?

Answer 31

Terminal bronchopneumonia

Question 32

What is the most common clinical presentation of someone w/ an ependymoma of the 4th ventricle?

Answer 32

**Hydrocephalus**, secondary to progressive obstruction of the 4th ventricle rather than invasion of the pons or medulla.

Question 33

Arises in white matter, usually in cerebral hemispheres in adults

Answer 33

Oligodenroglioma

Question 34

5 - 15% of gliomas are which one?

Answer 34

Oligodendroglioma

Question 35

* Well circumscribed * Gelatinous * Grey masses * often w/ cysts * focal hemorrhage * calcifications seen in 90% of tumors

Answer 35

oligodendroglioma

Question 36

* Slow growing tumor due to absence of mitotic figures and necrosis * Have a better prognosis than patients w/ astrocytomas * Several yrs of neuro complaints (seizures)

Answer 36

Oligodendroglioma

Question 37

* Accounts for 20% of all brain tumors in children * Arises exclusively in the cerebellum

Answer 37

Medulloblastoma

Question 38

Small, round, blue cell tumors of childhood (neuroblastoma, retinoblastoma, Ewing's Sarcoma)

Answer 38

Medulloblastoma

Question 39

Where do medullosblastomas infiltrate the brain?

Answer 39

Vermis of the cerebellum (aggressively and frequently disseminates through CSF)

Question 40

Well circumscribed, gray, friable

Answer 40

Medulloblastoma

Question 41

A child with a medulloblastoma will present with which of 2 things?

Answer 41

* Cerebellar dysfunction * Hydrocephalus

Question 42

What is the most common genetic alteration of a medulloblastoma?

Answer 42

Loss of material from short arm of **chromosome 17**

Question 43

Highly malignant, but is extremely radiosensitve tumor if dx early enough. If untreated is rapidly fatal.

Answer 43

Medulloblastoma

Question 44

With total excision and radiation of a medulloblastoma, the 10 year survival rate is \_\_\_%.

Answer 44

only 50%

Question 45

What is the chromosomal abberation seen in meningiomas?

Answer 45

Either deletion or mutation of chromosome 22

Question 46

* Intracranial tumor * Arises from arachnoid villi * Produces sxs by compressing brain tissue

Answer 46

Meningioma

Question 47

Peak incidence of meningiomas is which 2 decades of life?

Answer 47

* 4th (30 - 39 yrs) * 5th (40 - 49 yrs)

Question 48

Intracranial tumor most common in: * parasagital areas * convexities of cerebral hemispheres * olfactory groove * lateral wing of sphenoid

Answer 48

Meningioma

Question 49

* Has propensity to erode contiguous bone * Slightly more common in females (60:40) * Benign tumor

Answer 49

Meningioma

Question 50

* Majority arise sporadically * Can be induced by cranial radiation w/ latent period of 10 - 15 yrs * Well circumscribed, firm, bosselated masses of variable size * Gray fibrous patter (when cut) * Cause HA

Answer 50

Meningioma

Question 51

* Meningiomas in which location cause anosmia? * Meningiomas in which location lead to visual defects?

Answer 51

* Olfactory groove * Suprasellar region

Question 52

* Depending on location of tumor, pt usually has seizures rather than neuro deficits * If not completely excised, tend to recur * Doubles in size every 2 years

Answer 52

Meningioma

Question 53

Pain down back/leg/knee when hip is flexed

Answer 53

Kernig's Sign (sign of bacterial meningitis)

Question 54

Spontaneous flexion of knees and hips when neck is flexed

Answer 54

Brudzinski sign (sign of bacterial meningitis)