– 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
– 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
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
– 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.
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
*Sensation is intact *
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
– Loss of dopaminergic neurons from the substantia nigra
– Usually presents in mid-late life (ie >50yr)
– Diagnosis is completely clinical and based on symptoms
– Resting tremor (pill rolling)
– Cogwheel rigidity
– Difficulty in initiating movement
– Mask-like facies
– Brain biopsy will show Lewy bodies
- certain medications can cause Parkinsonism: Reserpine, Metoclopramide, Haloperidol, Perphenazine, MPTP
– 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.
– 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.
– Is an autosomal recessive condition that begins by young adulthood.
Impaired: Proprioception, vibratory sense, ataxia, and nystagmus