CH 14--Nervous System 5/Degenerative Disorders Flashcards

1
Q

Cerebrocortical degenerations

Manifest with dementia as a common symptom

Includes:

  1. Alzheimer’s disease
  2. Pick’s disease
  3. Senile dementia
A

KNOW

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2
Q

Cerebrocortical degenerations

Dementia

A–symptom complex characterized by the following
chronological sequence of events:
1. Anxiety and depression WITH difficulty in ______ and
comprehension
2. Decreased recent memory and judgment
-Loss of ability to solve problems
-Disorientation
-Aphasia
-________ (loss of ability to carry out familiar purposeful
movement)
3. The patient is totally withdrawn, motionless and mute

A

memory

Apraxia

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3
Q

Cerebrocortical degenerations

8 Causes of Dementia:

  • Alzheimer’s disease (50% of cases)
  • Chronic alcoholism
  • Pick’s disease
  • Senile dementia
  • Subdural hematomas
  • Meningioma
  • Chronic meningitis
  • Hydrocephalus
A

KNOW THE 8

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4
Q

Cerebrocortical degenerations——8 Causes of Dementia:

  1. Alzheimer’s disease
Properties:
- Progressive loss of \_\_\_\_\_\_\_\_\_in the entire cerebral
cortex especially frontal lobes dementia
Etiology:
1. Unknown
2. \_\_\_\_\_\_\_\_\_ abnormality (21 chromosome)
3. Familial incidence (about 25%)
4. Deficiency of enzymes required for synthesis
of acetyl choline

Manifestations:

  • Age: over 50 years
  • Loss of high ______ functions:
    i. loss of ___________memory
    ii. loss of ability to solve problems
    iii. disorientation
    iv. emotional lability
  • Progressive dementia, patient can not carry out daily activity
  • Motionless & mute
  • A terminal bronchopneumonia is the usual outcome
A

neurons

Genetic

cortical

recent

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5
Q

Cerebrocortical degenerations——8 Causes of Dementia:

  1. Pick’s disease

Properties:
- Age: pre-senile, age group from 40-65 years
- Cause: unknown
- Clinically: similar to _________ disease
- No neurofibrillary tangles, no senile plaques
- Affected neurons show round eosinophilic cytoplasmic inclusions (Pick bodies)
*****The marked atrophy of Pick’s disease, a senile
dementia, produces “_____–_____” thinning of the gyri in frontal lobes and temporal lobes.

A

Alzheimer’s

knife-like

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6
Q

Cerebrocortical degenerations——8 Causes of Dementia:

  1. Senile dementia OUT OF 8 PRESENTED!

Properties:

  • Age: usually over 60 years
  • Evidence of _________
  • Neurofibrillary tangles & senile plaques are less abundant than Alzheimer’s disease
A

atherosclerosis

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7
Q

3 TYPES OF Basal Ganglia Degenerations

Degenerative diseases of the basal ganglia are 
characterized by:
-Involuntary movements
-Interference with voluntary movements
-Disturbed muscle tone

Includes:

  1. Parkinson’s disease
  2. Huntington disease
  3. Wilson’s disease
A

KNOW THESE 3

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8
Q

3 TYPES OF Basal Ganglia Degenerations

  1. _____________disease (Paralysis Agitans)
    Properties:
    - Affects 5% of persons over 70 years of age
    Etiology:
    - unknown
    - degeneration of pigmented neurons of brain stem
    (substantia nigra) that normally project in basal
    ganglia where they secrete dopamine
    - failure of dopamine synthesis
    dysfunction of extrapyramidal system
Manifestations:
- mask-like face
- SLOW  difficult speech
- SLOW  difficult movements
- increased muscle rigidity (lead pipe rigidity)
- resting tremors
- gait: walking stooped forward with short rapid 
shuffling steps
- dementia (20%)
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_\_
A

Parkinson’s

**A spherical eosinophilic inclusion within the
cytoplasm of a pigmented dopaminergic neuron,
termed a “Lewy body.”

Levodopa

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9
Q

3 TYPES OF Basal Ganglia Degenerations

2.____________ Disease

Properties:

  • Inherited, autosomal dominant
  • Onset: 30-50 years
  • Atrophy of caudate nucleus & putamen + may be some atrophy in cerebral cortex especially frontal lobes
  • Loss of neurons AKA_______

Manifestations:

  1. Involuntary movements (chorea)
  2. Dementia, Depression
  3. Slow progressive course
  4. Death in 15-20 years (suicide & infections)
A

Huntington

gliosis

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10
Q

3 TYPES OF Basal Ganglia Degenerations

  1. ______disease

Properties:
- Hepatolenticular degeneration
- Inherited, autosomal recessive
- Disorder of copper metabolism (decreased biliary
excretion) deposited in many organs:
a. Liver: hepatitis, cirrhosis = FAILURE!
b. Eye:_________-_________rings, greenish-brown
deposits of copper at corneal angles
c. Basal Ganglia (especially putamen) atrophy,
brownish discoloration, cavitation
extrapyramidal dysfunction

A

Wilson’s

Kayser-Fleicher

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11
Q

Spinocerebellar Degenerations–Friedreich’s ataxia

Properties:

  • Inherited _______ recessive
  • Degeneration of:
    a. Posterior columns&raquo_space;»»»loss of sensation
    b. Spinocerebral tracts»»> ataxia
    c. Lateral corticospinal tracts&raquo_space;»»>paralysis
    d. Peripheral nerves

Manifestations:

  • loss of coordination
  • muscle weakness
A

autosomal

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12
Q

Motor Neuron Diseases X 4
A group of disorders characterized by degeneration of
the motor neurons anywhere in the CNS.
-Associated with a progressive ATROPHY and
DEGENERATION of the _________

Four clinical types:

  1. Amyotrophic lateral sclerosis
  2. Progressive muscular atrophy
  3. Progressive bulbar palsy
  4. Pseudobulbar palsy
Properties:
- Degeneration of both UPPER & LOWER 
MOTOR neurons (purely motor)
- Etiology: unknown
- Age: Over \_\_\_\_\_ years
A

muscles

KNOW THE FOUR COMING UP

50

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13
Q

Motor Neuron Diseases—Four clinical types:

  1. Amyotrophic lateral sclerosis “Lou Gehrig’s disease”

Properties:
A— Degeneration of ___________ __________ UPPER motor neuron paralysis in extremities:
B–ALS is a type of motor neuron disease that causes nerve cells to gradually break down and die.
C—ALS often begins with muscle twitching and weakness in an arm or leg, or sometimes with slurring of speech. Eventually, ALS can affect your ability to control the muscles needed to move, speak, eat and breathe. ALS can’t be cured and eventually leads to death.

A

corticospinal tracts

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14
Q

Motor Neuron Diseases—Four clinical types:

  1. Progressive ______________ atrophy

Properties:

  • Degeneration of ANTERIOR horn cells LOWER motor neuron paralysis in extremities
  • Neuronal degeneration is associated with irregular discharge muscle fasciculation (small involuntary contractions) followed by atrophy
A

muscular

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15
Q

Motor Neuron Diseases—Four clinical types:

  1. Progressive __________ palsy

Properties:
- Degeneration of motor cranial nuclei = LOWER motor neuron paralysis of jaw, tongue and pharyngeal muscles

A

bulbar

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16
Q

Motor Neuron Diseases—Four clinical types:

  1. Pseudobulbar palsy

Properties:
- ___________ UPPER motor neuron paralysis of
jaw, tongue and pharyngeal muscles

A

Bilateral

17
Q

Demyelinating Diseases—–TWO TYPES:

  1. Multiple Sclerosis

A—Properties:
- Incidence: 0.5 million Americans
- Age: 20-40 years
- Sex: more frequent in __________ (2:1)
B—Etiology:
- unknown
- increased familial incidence
- increased frequency of HLA-A3, B7, DR2
C—Pathological lesions:
- Well-demarcated areas of ____________(plaques)
- Microscopically:
i. plaques show demyelination and preserved axons
ii. lymphocyte and macrophage infiltrate
D—Manifestations:
- Visual disturbances
- Sensory: parasthesias, loss of sensation
- Muscular: weakness in limbs, paralysis, incontinence
- Cerebellar: incoordination, disturbed gait
* CSF: lymphocytesprotein (IgG)
* Chronic course, remission & relapses over several years

A

females

demyelination

18
Q

Demyelinating Diseases—–TWO TYPES:

  1. Schilder’s Disease
Properties:
Similar to multiple sclerosis but:
- Age: primarily in\_\_\_\_\_\_\_\_\_\_\_
- Lesions: LARGER & more DESTRUCTIVE
- Course: rapid, progressive, NO \_\_\_\_\_\_\_\_
A

children

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