Brain Diseases

Question 1

A decrease in cognitive ability, memory, or function with intact consciousness

Answer 1

Dementia

Question 2

Population with an increased risk of developing Alzheimer

Answer 2

Downs

Question 3

APP (chrom 21), presenilin-1

Chromosome 14, presenilin-2 (chr 1)

Answer 3

Early Onset Alzheimer

Question 4

ApoE4 (chromosome 19)

Answer 4

Late Onset Alzheimer

Question 5

ApoE2 (Chromosome 19)

Answer 5

Protective against Alzheimer

Question 6

Neurofibrillary Tangles

Answer 6

intracellular, hyperphosphorylated tau protein, insoluble

Assoc with Alzheimer

Question 7

Senile Plaques

Answer 7

extracellular beta-amyloid core; may cause intracranial hemorrhage

Question 8

Pick Bodies

Answer 8

spherical tau protein aggregates, Frontotemporal dementia

Question 9

Change in personality, spares parietal and posterior 2/3 of superior temporal gyrus

Answer 9

Frontotemporal Dementia

Question 10

Dementia and visual hallucinations

Answer 10

Lewy Body dementia

Question 11

alpha-synuclein defect

Answer 11

Lewy body demential

Question 12

startle mmyoclonus

Answer 12

Creutzfeldt-Jakob Dx

Question 13

Beta-pleated sheet resistent to proteases

Answer 13

Creutzfeldt-Jakob Dx

Question 14

Spongiform Cortex

Answer 14

Creutzfeldt-Jakob Dx

Question 15

2nd most common cause of dementia in elderly

Answer 15

Multi-infarct

Question 16

Gold Standard for MS diagnosis

Answer 16

MRI (FLAIR)

Question 17

Oligoclonal bands, increase in IgG in CSF

Answer 17

MS

Question 18

Scanning Spech, intention tremor, INO, Nystagmus

Answer 18

MS

Question 19

Tx to slow progression of MS

Answer 19

Beta-IFN, immunosuppression, natalizumab

Question 20

Symptomatic tx for neurogenic bladder associated with MS

Answer 20

catheterization, muscarinic antagonist

Question 21

Symptomatic tx for spasticity associated with MS

Answer 21

baclofen, GABA receptor agonist

Question 22

Most common variant of Guillen-Barre syndrome

Answer 22

Acute Inflammatory Demyelinating Polyradiculopathy

Question 23

Symmetric Ascending weakness/paralysis beginning in lower extremities

Answer 23

Acute Inflammatory Demyelinating Polyradiculopathy (Guillen-Barre)

Question 24

Findings in Guillen-Barre

Answer 24

increased CSF protein with normal cell count, albuminocytologic dissociation
Papilledema

Question 25

Infections associated with Guillen-Barre

Answer 25

Campylobacter jejuni, CMV - autimmune attack of peripheral myelin due to molecular mimicry, innoculatino and stress

Question 26

Tx for Guillen-Barre

Answer 26

Respiratory support, Plasmaphoresis, IVIGs

Question 27

JC virus

Answer 27

Progressive multifocal leukoencephalopathy

Question 28

demyelination of CNS due to destruction of oligodendrocytes; rapidly progressive, usually fatal

Answer 28

PML

Question 29

Natalizumab

Answer 29

Increases risk of developing PML

Question 30

Multifocal perivenular inflammation and demyelination after measles or VZV

Answer 30

Acute disseminated (postinfectious) encephalomyelitis

Question 31

Arylsulfatase A deficiency

Answer 31

Metachromatic Leukodystrophy (Auto Recessive)

Question 32

Build up of sulfatides impairing myelin production, central and peripheral demyelination with ataxia and dementia

Answer 32

Metachromatic Leukodystrophy

Question 33

Hereditary Motor and sensory Neuropathy, defective structural proteins in peripheral nerves or myelin sheath

Answer 33

Charcot-Marie-Tooth Disease

Question 34

High or Flat Arches

Answer 34

Charcot-Marie-Tooth Disease

Question 35

galactocerebroside and psychosine destroys myeline sheath

Answer 35

Krabbe Disease (Auto Recessive)

Question 36

Peripheral neuropathy, developmental delay, optic atrophy, globoid cells

Answer 36

Krabbe Disease

Question 37

Impaired addition of CoA to long chain FA causing excessive build up in nervous system, adrenal gland and testes

Answer 37

Adrenoleukodystrophy

Question 38

Mama

Answer 38

just killed a man