Progressive Supranuclear Palsy

(Steele-Richardson-Olszewski Syndrome)

Progressive supranuclear palsy is an uncommon disorder characterized by parkinsonian symptoms, eye movement abnormalities, and dementia. Diagnosis is by history and physical examination. Treatment is supportive.

Geriatric Essentials

• Progressive supranuclear palsy is often misdiagnosed, most frequently as Parkinson's disease.
• End-of life issues should be discussed early.

About 4% of patients with parkinsonian symptoms have progressive supranuclear palsy. Onset usually occurs at age >= 50, but some patients develop signs in their 40s. Occurrence is sporadic. The cause is unknown. Degenerative changes occur in the brain stem, basal ganglia, and elsewhere. Neurofibrillary tangles are present.

Symptoms and Signs

Voluntary vertical gaze is impaired. The first symptom may be difficulty looking up or down without extending or flexing the neck or difficulty going up and down stairs. Other ophthalmic symptoms and signs may include square wave jerks (inability to fix the gaze on a stationary or moving object), blurred vision, diplopia, photophobia, burning, tearing, and retraction of the upper eyelids leading to a staring, astonished appearance. Other findings may include gait unsteadiness with falling (typically backwards), dysarthria, dysphagia, rigidity, bradykinesia, deep nasolabial folds, and dystonic neck extension. Subtle resting tremor is occasionally present. Depression and dementia are common later in the disease; patients may also have sleep disturbances (insomnia or hyposomnia), agitation, irritability, apathy, and pseudobulbar affect (emotional lability with a propensity to laugh or cry easily). Deficits, like those due to multiple strokes, tend to occur in steps.

Usually, marked incapacity occurs within 3 to 5 yr, and death occurs within 10 yr, typically due to infection or other complications of immobility.

Diagnosis

Diagnosis is by history and physical examination. Characteristic findings on MRI include mild to moderate atrophy of the midbrain and, on sagittal views, thinning of the quadrigeminal plate. However, neuroimaging findings are not specific, and neuroimaging is not required for diagnosis.

Treatment

Treatment is far less effective than that for Parkinson's disease. Treatment with levodopa, amitriptyline, or dopamine agonists may partially relieve rigidity. Early in the disease, patients may use weighted walking aids or even wheelchairs to prevent falls. Specially adapted glasses (eg, with prisms or bifocals) may improve vision. Supportive therapy (speech, occupational, and physical) is important.

This topic was last updated June 2006.