NEURODEGENERATIVE DISEASES

by / Monday, 20 December 2010 / Published in Pathology

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DEGENERATIVE DISEASES

ALZHEIMER’S DISEASE

–       Is a very common cause of death

–       Prevalence increases with age

–       Family history is a big contributing factor to the possible development.

–       Diffuse cerebral atrophy occurs secondary to neuronal loss

–       Neurofibrillary tangles are classically found

Signs/Symptoms:

–       Develops slowly over time

–       Death usually occurs within 5-10 years from onset

–       The advanced stages will require the patient to have dependence on others

Stages:

Early – mild forgetfulness, patient will have difficulties learning new information

Intermediate – progressive impairment in memory

Late – patient will require assistance in their activities of daily living

Advanced – patient will experience complete debilitation and depends completely on others

PICKS DISEASE

–       Frontal and temporal lobe dysfunction caused by degeneration

–       Accumulation of tau proteins

–       Pick bodies are characteristic

–       Memory impairment + impulsive behavior + behavioral changes

MOTOR NEURON DEGENERATIVE DISORDERS

AMYOTROPHIC LATERAL SCLEROSIS

ALS is also known as Lou Gehrig’s disease, which is a fatal neurodegenerative disease of both the upper and lower motor neurons.

POLIOMYELITIS

Polio is a virus that affects the anterior horn cells and motor neurons of the spinal cord and brainstem

–       Causes LMN symptoms

Features of Polio:

–       Asymmetric muscle weakness (most commonly the legs)

–       Muscle atrophy

–       Absent of DTR’s

–       Flaccidity

*Sensation is intact *

WERDNIG-HOFFMAN DISEASE

A genetic condition that presents in infancy and/or early childhood.

–       Progressive skeletal muscle atrophy due to anterior horn cell degeneration

–       Often presents with floppy baby at birth (congenital hypotonia)

–       Lack of sucking ability

–       Tongue fasciculation’s

–       Death occurs at a young age due to respiratory muscle failure

BASAL GANGLIA DEGENERATION

PARKINSON’S DISEASE

–       Loss of dopaminergic neurons from the substantia nigra

–       Usually presents in mid-late life (ie >50yr)

–       Diagnosis is completely clinical and based on symptoms

Signs/Symptoms:

–       Resting tremor (pill rolling)

–       Cogwheel rigidity

–       Bradykinesia

–       Difficulty in initiating movement

–       Mask-like facies

–       Brain biopsy will show Lewy bodies

  • certain medications can cause Parkinsonism: Reserpine, Metoclopramide, Haloperidol, Perphenazine, MPTP

HUNTINGTON’S CHOREA

–       Is a genetic condition (AD) where there is atrophy of the caudate nucleus

–       Disease onset is between 30-50yr with a steady worsening of symptoms and death within 15 years of onset.

Signs/Symptoms:

–       Progressive dementia

–       Chorea of the limbs, face, head/neck, and trunk

–       Behavior disturbances such as: Depression, aggression, psychosis, changes in personality.

–       Depression occurs and suicide is somewhat common because patients are aware of their deterioration

  • There is no treatment, only symptomatic management.

SPINOCEREBELLAR DISEASES

FRIEDREICH’S ATAXIA

–       Is an autosomal recessive condition that begins by young adulthood.

Impaired:  Proprioception, vibratory sense, ataxia, and nystagmus

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