Clinical Features

Progressive supranuclear palsy (PSP) affects middle-aged and elderly individuals. The disease is sporadic, but a few familial occurrences have been reported, some associated with mutations in the tau gene. The annual incidence rate is 5 per 100,000 population; this figure increases with advancing age.

The disease presents a broad spectrum of symptoms and signs including (a) supranuclear vertical and horizontal gaze palsy; (b) motor disturbances, such as bradykinesia-akinesia, rigidity of the neck, axial, and

TABLE 5.12.

Diseases with Parkinsonism

Degenerative

Acquired

Hereditary

Progressive supranuclear palsy Striatonigral degeneration Corticobasal dementia Chromosome 17-linked dementia Parkinsonism-dementia complex of Guam Postencephalitic parkinsonism Multiple system atrophy

Infection Trauma

Dementia pugilistica Vascular diseases Hypoxic-ischemic insult Mass lesion

Toxin MPTP

Carbon monoxide Manganese Neuroleptics

Wilson's disease Neuroacanthocytosis Hallervorden-Spatz disease

Progressive supranuclear palsy Striatonigral degeneration Corticobasal dementia Chromosome 17-linked dementia Parkinsonism-dementia complex of Guam Postencephalitic parkinsonism Multiple system atrophy

Infection Trauma

Dementia pugilistica Vascular diseases Hypoxic-ischemic insult Mass lesion

Toxin MPTP

Carbon monoxide Manganese Neuroleptics

Wilson's disease Neuroacanthocytosis Hallervorden-Spatz disease

TABLE 5.13(A).

Neurodegenerative Diseases with *Parkinsonism

**Diseases

Differentiating Clinical Features

PSP

Gaze palsy, Axial dystonia, Pseudobulbar palsy

CBD

Limb apraxia, Alien-limb phenomenon, Cortical sensory deficit, Aphasia

FTDP-17

Autosomal dominant inheritance.

Mutation in tau gene on chromosome 17

MSA

Cerebellar ataxia, Dysautonomia of Shy-Drager syndrome

*Parkinsonism refers principally to motor disturbances of idiopathic Parkinson's disease (iPD).

**PSP, progressive supranuclear palsy; CBD, corticobasal degeneration; FTDP-17, frontotemporal dementia parkinsonism linked to chromosome 17; MSA, multiple system atrophy.

limb muscles, neck dystonia, impaired gait, postural instability with frequent falls, and pseudobulbar palsy; and (c) behavioral changes and cognitive decline. The clinical course averages from 5 to 10 years.

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