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Module 9.3 > Demyelinating Diseases > Flashcards

Demyelinating Diseases Flashcards

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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

Study These Flashcards
A

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

24
Q

Alz - gross findings

Study These Flashcards
A

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

25
Alz - 3 microscopic findings
Neurofibrillary tangles Senile (neuritic) plaques Amyloid angiopathy
26
Alz Pathogenesis
Amyloid (AB) - derived from APP AB peptides aggregate and generate AB found in brain parenchyma & around vessels Gene for APP is on chromo21
27
Development of Alz in trisomy 21
Related to gene dosage effect
28
Alz - Clinical
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
Alz - Dx
``` Medical history Mental status tests Phys exam & diagnostic tests Neuro exam Brain imagins ```
30
Alz - Treatment
Cholinesterase inhibitors: prevents the breakdown of acetylcholine, support communication among nerve cells by keeping acetylcholine levels high Memantine: regulates activity of glutamate
31
Degenerative basal ganglia & brain stem
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
Six cardinal clinical features of Parkinsonism
``` Clinical syndrome: Diminished facial expression Stooped posture Festinating gait Rigidity "Pill rolling" tremor Slowness of voluntary movements ```
33
What pathway in the brain is malfunctioning in the parkinsonism group of diseases
Nigrostriatal dopaminergic system
34
Parks - gross
Pallor of substantia nigra
35
Parks - micro morphology
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
IPD
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
IPD - Clinical
10-15% develop dementia, increasing incidence w/increasing age Treat w/ L-DOPA therapy Neural transplantation
38
H disease: how is it genetically transmitted?
AD
39
H disease movement:
CHOREIFORM: involuntary jerky movements of all parts of the body, writhing movements of extremities are typical Uncontrolled movements, chorea
40
H disease: Brain - gross
Small brain, marked atrophy of caudate nucleus | Severe loss of striatal neurons
41
H disease - affected neurons
Caudate: most severe; putamen: less Lare & small neurons affected, small first Medium sized - use GABA as NTs are affected
42
H disease - trinucleotide repeat? Age of onset?
HD of chromosome 4 CAG repeats 4th and 5th decades
43
H disease - natural course of disease
Early symptoms of higher cortical dysfunction include forgetfulness & thought & affected disorders
44
Degenerative diseases of motor neurons affect what 3 groups of neurons
LMNs - anterior horn of SC, cranial nerve nuclei: 5, 7, 8, 12 UMNs - Betz cells in motor cortex
45
Denervation of muscles leads to what manifestations
Muscle atrophy, hyperreflexia, eventual symmetric weakness, death from respiratory paralysis
46
ALS
Both UMN, LMN degenerate Most common NM disease worldwide Between 40-60 yrs Men
47
ALS - Morphology
UMN & LMN loss
48
ALS - Symptoms
Muscle weakness, atrophy spread | Moving problems, swallowing (dysphagia) and speaking/forming words (dysarthria)
49
ALS - Dx
Signs & symptoms, tests to rule out other diseases EMG - electromyographty NCV - nerve conduction velocity MRI
50
Thiamine deficieny
``` Vit B1 Beriberi Wenicke Korsakoff Common in chronic alcoholism Mamillary bodies - hemorrhage, necrosis ```
51
Neurologic manifestations of VitB12 deficiency?
``` Slight ataxia (coordinate muscle movement), numbness, tingling of LEs Progresses to spastic weakness, complete paraplegia may occur ```
52
Toxic & Acquired metabolic: Hypoglycemia
Hippocampus - Sommer's sector Cerebellum - Purkinje cells Cortex - large pyramidal neurons Global insult
53
Toxic & Acquired metabolic: Hyperglycemia
Ketoacidosis/hyperosmolar coma, dehydration
54
CO
Bilateral necrosis of globus pallidus | Hypoxic changes
55
Methanol
DEgeneration of retinal ganglion cells
56
Ethanol
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
Radiation
Delayed effects: months to years Headaches, nausea, vomiting, papilledema Coagulative necrosis w/ adjacent edema in white matter Tumor induction possible

Module 9.3 (3 decks)

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