Cns Degeneration, Demyelination And Tumors

Front 1

Whic diseases reflect inherited disturbances in formation and preservation of myelin

Back 1

Leukodystrophies

Front 2

What is the most common leukodystrophy?

Back 2

Metachromatic leukodstrophy

Front 3

Krabbe disease is caused by defficiency of _

Back 3

Galactocerebroside - beta galactosidase

Front 4

X linked inherited disorder of adrenal cortex and demyelination of nervous system is called _

Back 4

ALD - adrenoleukodystrophy

Front 5

Alexander disease is rare neurological disorder that causes loss of _ and increased accumulation of irregular extracellular fibers called _

Back 5

Myelin in brain

Rosenthal fibers

Front 6

Most common chronic CNS disease of young adults in USA

Back 6

Multiple sclerosis

Front 7

Mean age of MS patients

Back 7

30 years

Front 8

People living where have increased risk of MS

Back 8

Colder climates

Front 9

What is a hallmark of multiple sclerosis - where is it located

Back 9

Demyelinating plaque in white matter

Front 10

Demyelinating plaque of multiple sclerosis has preference for _

Back 10

Optic nerves, chiasm

Front 11

Which part of neuron is affected in MS

Back 11

AXONS

Front 12

What is usually the presenting complaint of patient with MS

Back 12

Loss of vision in one eye

Front 13

What is the name of MS variant occuring in young individuals and has fulminant course over several months

Back 13

Acute MS - MARBURG DISEASE

Front 14

Central pontine myelolysis is loss of myelin with sparing of neurons and axons - what is the most likely cause of this disease

Back 14

RAPID CORRECTION OF HYPONATREMIA

Front 15

What is the most common type of Parkinsons

Back 15

Idiopathic

Front 16

What is an early treatment of Parkinsons

Back 16

L dopa

Front 17

What is the name of rare disorder that mimicks Parkinsons but is less pathological

Back 17

Multiple system atrophy

Front 18

ALS affects _

Back 18

Motor neurons of brain and spinal cord

Front 19

What is the inheritance pattern of ALS

Back 19

Autosomal dominant

Front 20

In ALS there is missense mutation that codes for _

Back 20

SOD1

Front 21

How would patient with ALS originally present ?

Back 21

Complain that he cant brush his teeth or shave due to weakness and wasting of muscles of hand (often with painful cramps)

Front 22

What muscle changes occur in ALS

Back 22

Fasciculations

Front 23

What is the inheritance pattern of Huntingtons

Back 23

Autosomal dominant

Front 24

In Huntingtons there is degeneration of -

Back 24

Striatal neurons

Front 25

Which trunucleotide repeat involved in Huntingtons

Back 25

CAG

Front 26

What is the most common inherited ataxia

Back 26

Friedreich ataxia

Front 27

What is the inheritance pattern of Friedreich ataxia

Back 27

Autosomal recessive

Front 28

What are the hallmarks of Friedrich ataxia

Back 28

Combined ataxia of both upper and lower limbs + systemic abnormalities of skeletal system (scoliosis, pes cavus)
- Hypertrophic cardiomyopathy which commonly causes death
- Diabetes mellitus

Front 29

In Friedrich ataxia there is abnormality of chromosome _

Back 29

9

Front 30

In Friedreich ataxia what is the trinucleotide repeat involved

Back 30

GAA

Front 31

What is the stain specific for amyloid deposits in Alzheimers

Back 31

CONGO RED

Front 32

Are neurofibrillary tangles specific for Alzheimers

Back 32

NO - can occur in other diseases as well

Front 33

What is a constant feature of Alzheimers

Back 33

Amyloid beta deposits

Front 34

What is the name of protein which is abnormal in neurofibrillary tangles in Alzheimers

Back 34

TAU (phosphorylated)

Front 35

How does brain look in patient with Alzheimers

Back 35

Atrophied - gyri smaller, sulci wider

Front 36

Most of the cases of Alzheimers are associated with what cytoplasmic inclusions

Back 36

LEWY bodies

Front 37

In Alzheimers cortical atrophy is _

Back 37

Bilateral and symmetrical

Front 38

How do you distinguish Pick disease from Alzheimers

Back 38

It is initially unilateral and becomes bilateral with progression, in Alzheimers cortical atrophy is bilateral and symmetrical

Front 39

Pick disease is localized to _

Back 39

Frontotemporal region

Front 40

Progression to death in Alzheimers occurs in _

Pick disease

Back 40

15-20 years

3-10

Front 41

What is the most common origin of brain metastases?

Back 41

Lung (followed by breast)

Front 42

Do primary brain tumors metastasize

Back 42

NO

Front 43

What is the most common brain tumor in adults

Back 43

Glioblastoma multiform

Front 44

Which astrocytoma is relatively benign

Back 44

Pliocytic astrocytoma

Front 45

Oligodenroglioma mainly occurs where

Back 45

White matter

Front 46

In children ependymoma most often occurs in _ , in adults in _

Back 46

Children - ventricles

Adults - spinal cord

Front 47

Describe subependymoma

Back 47

Solid, sometimes calcified - difficult to remove

Front 48

Most common brain tumor in children

Back 48

Medulloblastoma

Front 49

Medulloblastoma occurs exclusively in _ what part of brain?

Back 49

Cerebellum

Front 50

Which tumor of cerebellum occurs in adults

Back 50

Hemangioblastoma

Front 51

What is the best treatment for medulloblastoma

Back 51

Surgery followed by radiation - very radiosensitive

Front 52

What patient population usually gets primary CNS lymphomas

Back 52

IMMUNOSUPPRESSED

Front 53

What type of lymphomas are in CNS

Back 53

EXTRANODAL

Front 54

In lymphomas of CNS majority of the cells are _

Back 54

B cells

Front 55

What is the most common benign pituitary tumor

Back 55

Prolactinoma

Front 56

What is the most common suprasellar tumor (above sella turcica) and how does it present

Back 56

CRANIOPHARYNGIOMA - loss of peripheral vision and headache, more often in children

Front 57

What is the most common benign brain tumor in adults

Back 57

Meningioma - slow growing, in adults, usually attached to dura

Front 58

How do you distinguish primary brain tumor from the metastases coming from somewhere else

Back 58

MULTIPLE TUMORS - metastases

SINGLE TUMOR - primary

Front 59

Tumor in lung or brain causes what kind of paraneoplastic syndrome?

Back 59

Hyperthyroid or other endocrine problems