Progressive supranuclear palsy

Alternative names:

dementia-nuchal dystonia; palsy - progressive supranuclear; Richardson-Steele-Olszewski syndrome

Definition:

A disorder associated with damaged nerve cells of the brain and characterized by progressive lack of coordination, stiffness of the neck and trunk, difficulties with eye movement, and mild dementia.

Causes, incidence, and risk factors:

Progressive supranuclear palsy was first described as a distinct and separate disorder in 1963 by Richardson, Steele, and Olszewski. It is a disorder characterized by symptoms similar to Parkinson's disease, including unsteady gait, stiff movements, and mild dementia. Other similar disorders include dysfunction of the cerebellum, Creutzfeldt-Jacob disease, and senile dementia/Alzheimer's type.

The cause is unknown. It is suspected to be a degenerative condition triggered by a viral infection. The disorder involves damage to multiple cells of the brain, with loss of the myelin sheath (the covering of the nerve cell that speeds nerve impulse conduction) in some nerves and destruction of the entire nerve in other areas. There are deposits in the tissues that resemble the deposits found in Alzheimer's disease. There is atrophy (loss of tissue) in most areas of the brain.

The disorder is most often seen in people over 60 years old, and is somewhat more common in men. It occurs in approximately 1 out of 100,000 people.