Demyelinating Diseases Flashcards

1
Q

Most common demyelinating disorder

A

MS

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2
Q

MS defined

A

Autoimmune demyelinating disorder = distinct episodes of neuro deficits, separated in time, attributable to white matter lesions that are separated in space

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3
Q

Myelin

A

Promotes transmission of a nerve impulse along an axon, rich in lipids & proteins, form layers around nerve fibers, produced in the CNS by oligodendrocytes

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4
Q

Relapsing-remitting

A

Attacks of worsening neuro function

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5
Q

MS epidemiology

A

2-3x more common in women than men
Genetic factors play a role
ID twin - 1 in 4 chance of developing disease

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6
Q

MS morphology

A

Lesions (plaques) spread throughout white matter
Plaques - sharply circumscribed, depressed, glassy & gray-tan, irregularly shaped lesion; commonly located @ angle of lateral ventricles (optic nerves & chiasm, brain stem, cerebellum, spinal cord)

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7
Q

MS symptoms

A

Most common: fatigue, numbness, walking, balance & coordination problems, bladder dysfunction

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8
Q

MS diagnosis

A

No specific symptom, physical finding or lab test…

Need evidence of damage in at least 2 areas of CNS, & that damage occurred at least 1 month apart

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9
Q

CSF Studies - MS

A

Mildly elevated protein, proportion of gamma globulin increased, oligoclonal bands; B-cell proliferation w/in nervous system

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10
Q

MS Clinical

A

Unilateral vision impairment, optic nerve involved

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11
Q

Central pontine myelinolysis

A

Loss of myelin in symmetrical pattern involving the basis pontis & portions of pontine tegmentum

Believed due to rapid correction of hyponatremia

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12
Q

What 3 groups of pts does CPM occur in

A

Alcoholics, severe electrolyte osmolar imbalance & orthotopic liver transplant

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13
Q

CPM clinical findings

A

Rapidly evolving quadriplegia

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14
Q

What is lost in degenerative diseases of the brain?

A

Progressive loss of neurons of gray matter

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15
Q

Two general characteristics of degenerative diseases of the brain

A

Development of protein aggregates resistant to normal degredation (recognized histologically as inclusions), aggregates usually cytotoxic

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16
Q

Degenerative Diseases

A

Alzheimers

Movement motor disorders: amyotrophic lateral sclerosis, Parkinson’s, Huntington’s

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17
Q

Dementia

A

Progressive loss of cognitive function independent of state of attention - not a part of normal aging, represents a pathologic process

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18
Q

What is the most common cause of dementia in the elderly?

A

Alzheimer’s

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19
Q

Delerium

A

The onset of mental status change, such as confusion, disorientation, agitation; often due to drugs, hypoxia, metabolic condition

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20
Q

Alzheimer’s

A

Impairment of higher intellectual functions w/alterations in mood & behavior

Later progressive disorientation, memory loss, aphasia

5-10 years patient disabled, mute & immobile

5-10% familial

1% of 60-64 y/o’s, 40% of 85-89 y/o’s

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21
Q

Alzheimer’s history

A

Memory probs, difficulty speaking, paranoia.
Died from bedsores & pneumonia

Autopsy: Cortex shrunk, dead/dying brain cells, abnormal deposits in & around cells

Entered med. literature @ 1907, in 1910 named after Dr Alzheimer

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22
Q

Alzheimers - cont’d

A

10mi baby boomers will develop

6th leading cause of death

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23
Q

Alzheimer’s RFs

A

Age, fam history, genetics: APOLIPOPROTEIN E-e4, deterministic gene - familial alzheimers disease

24
Q

Alz - gross findings

A

Variable degree of cortical atrophy: frontal, temporal, parietal; compensatory ventricular enlargement (hydrocephalus)

25
Q

Alz - 3 microscopic findings

A

Neurofibrillary tangles
Senile (neuritic) plaques
Amyloid angiopathy

26
Q

Alz Pathogenesis

A

Amyloid (AB) - derived from APP
AB peptides aggregate and generate AB found in brain parenchyma & around vessels

Gene for APP is on chromo21

27
Q

Development of Alz in trisomy 21

A

Related to gene dosage effect

28
Q

Alz - Clinical

A

Course runs more than 10 yrs
Initially forgetfulness, other memory disturbances
Later language deficits, loss of math skills, loss of learned motor skills
FInal incontinent, mute, unable to walk
Pneumonia often terminal event

29
Q

Alz - Dx

A
Medical history
Mental status tests
Phys exam & diagnostic tests
Neuro exam
Brain imagins
30
Q

Alz - Treatment

A

Cholinesterase inhibitors: prevents the breakdown of acetylcholine, support communication among nerve cells by keeping acetylcholine levels high

Memantine: regulates activity of glutamate

31
Q

Degenerative basal ganglia & brain stem

A

Movement disorders:
Rigidity, abnormal posture, chorea (involuntary jerky movements)
Reduction of voluntary or abundance of involuntary movement
Involves nigrostriatal system (dopamine pathway involved in production of movement)

32
Q

Six cardinal clinical features of Parkinsonism

A
Clinical syndrome:
Diminished facial expression
Stooped posture
Festinating gait
Rigidity
"Pill rolling" tremor
Slowness of voluntary movements
33
Q

What pathway in the brain is malfunctioning in the parkinsonism group of diseases

A

Nigrostriatal dopaminergic system

34
Q

Parks - gross

A

Pallor of substantia nigra

35
Q

Parks - micro morphology

A

Loss of pigmented catecholaminergic neurons w/gliosis

Lewy bodies (protein aggregates): eosinophilic, round inclusions w/dense core surrounded by paler rim

Neurofilament antigens present

36
Q

IPD

A

Dopaminergic neurons of substantia nigra project to striatum - loss in IPD reduced striatal dopamine content

Can partially correct motor probs w/L-dopa (can cross brain barrier)

37
Q

IPD - Clinical

A

10-15% develop dementia, increasing incidence w/increasing age

Treat w/ L-DOPA therapy

Neural transplantation

38
Q

H disease: how is it genetically transmitted?

A

AD

39
Q

H disease movement:

A

CHOREIFORM: involuntary jerky movements of all parts of the body, writhing movements of extremities are typical

Uncontrolled movements, chorea

40
Q

H disease: Brain - gross

A

Small brain, marked atrophy of caudate nucleus

Severe loss of striatal neurons

41
Q

H disease - affected neurons

A

Caudate: most severe; putamen: less
Lare & small neurons affected, small first
Medium sized - use GABA as NTs are affected

42
Q

H disease - trinucleotide repeat? Age of onset?

A

HD of chromosome 4
CAG repeats

4th and 5th decades

43
Q

H disease - natural course of disease

A

Early symptoms of higher cortical dysfunction include forgetfulness & thought & affected disorders

44
Q

Degenerative diseases of motor neurons affect what 3 groups of neurons

A

LMNs - anterior horn of SC, cranial nerve nuclei: 5, 7, 8, 12
UMNs - Betz cells in motor cortex

45
Q

Denervation of muscles leads to what manifestations

A

Muscle atrophy, hyperreflexia, eventual symmetric weakness, death from respiratory paralysis

46
Q

ALS

A

Both UMN, LMN degenerate
Most common NM disease worldwide
Between 40-60 yrs
Men

47
Q

ALS - Morphology

A

UMN & LMN loss

48
Q

ALS - Symptoms

A

Muscle weakness, atrophy spread

Moving problems, swallowing (dysphagia) and speaking/forming words (dysarthria)

49
Q

ALS - Dx

A

Signs & symptoms, tests to rule out other diseases
EMG - electromyographty
NCV - nerve conduction velocity
MRI

50
Q

Thiamine deficieny

A
Vit B1 
Beriberi
Wenicke
Korsakoff
Common in chronic alcoholism
Mamillary bodies - hemorrhage, necrosis
51
Q

Neurologic manifestations of VitB12 deficiency?

A
Slight ataxia (coordinate muscle movement), numbness, tingling of LEs
Progresses to spastic weakness, complete paraplegia may occur
52
Q

Toxic & Acquired metabolic: Hypoglycemia

A

Hippocampus - Sommer’s sector
Cerebellum - Purkinje cells
Cortex - large pyramidal neurons
Global insult

53
Q

Toxic & Acquired metabolic: Hyperglycemia

A

Ketoacidosis/hyperosmolar coma, dehydration

54
Q

CO

A

Bilateral necrosis of globus pallidus

Hypoxic changes

55
Q

Methanol

A

DEgeneration of retinal ganglion cells

56
Q

Ethanol

A

Symptoms of cerebellar dysfunction from choric alcoholism: truncal ataxia, unsteady gait, nystagmus

Cerebellar degneration : anterior vermis (Bergman gliosis)

FAS: growth retardation, facial abnormalities, cardiac septal defects, joint probs, delayed development

57
Q

Radiation

A

Delayed effects: months to years

Headaches, nausea, vomiting, papilledema

Coagulative necrosis w/ adjacent edema in white matter

Tumor induction possible