Parkinsonian Syndromes

About 10-20% of patients with the clinical features of Parkinson's disease actually have a "Parkinsonian syndrome" (also known as "atypical Parkinson's" or Parkinson's plus)." The importance of recognizing these syndromes is that they respond less reliably to dopaminergic agents and are associated with additional clinical problems.

Atypical Parkinson's should be suspected particularly in patients with poor dopamine responsiveness, early loss of balance, axial greater than appendicular rigidity, prominent early speech difficulty and/or dysphagia, rapid progression, prominent autonomic dysfunction, and little or no tremor.

Progressive Supranuclear Palsy (PSP)

Early onset of balance problems, frequent falls, and (eventually) eye movement problems characterize PSP. Progression is typically more rapid than with "typical" Parkinson's disease. Bulbar symptoms become prominent. The most characteristic eye movement abnormality is loss of downward gaze. Dementia is frequent later in the disease. PSP is more common in men than in women. Symptoms usually begin between ages 50 and 60, with a range from the early forties to the late eighties. For more in depth information go to the WeMove Web site.

Corticobasal Degeneration (CBD)

Symptoms of CBD usually begin after age 60. It is the least common of the Parkinson's variants and often affects patients quite asymmetrically and progresses fairly quickly. Symptoms of CBD include:

• parkinsonism (rigidity, slow movements, postural instability)
• tremor
• myoclonus (sudden, brief jerky movements)
• dystonia, including blepharospasm
• speech difficulty
• mild-to-moderate cognitive impairment (memory loss, difficulty planning or executing unrehearsed movements, dementia)
• sensory loss
• "alien hand/limb" phenomenon (difficulty controlling the movements of a limb, which seems to undertake movements on its own, sometimes combined with a feeling that the limb is not one's own) For more in depth information go to the WeMove Web site.

Multiple System Atrophy

Multiple system atrophy (MSA) is a neurodegenerative disease marked by a combination of symptoms affecting movement, blood pressure, and other body functions; hence the label "multiple system" atrophy. The cause of MSA is unknown.

Symptoms of MSA vary in distribution, onset and severity from person to person. Because of this, three different diseases were initially described to encompass this range of symptoms: Shy-Drager syndrome, striatonigral degeneration, and olivopontocerebellar atrophy.

In Shy-Drager syndrome, the most prominent symptoms are those involving the autonomic system (affecting especially blood pressure control and urinary function). Striatonigral degeneration causes parkinsonian symptoms such as slowed movements and rigidity, but is largely unresponsive to dopamine, and olivopontocerebellar atrophy principally causes ataxia and Parkinsonsm: (affecting balance, coordination, and speech).

Overlaps between these syndromes are common and these diseases are now all considered forms of MSA.

MSA can cause a wide range of symptoms, including:

• stiffness or rigidity
• freezing or slowed movements
• postural instability; loss of balance; incoordination
• Orthostatic hypotension, or a significant fall in blood pressure when standing, causing dizziness, lightheadedness, fainting, or blurred vision
• male impotence
• urinary difficulties
• constipation
• speech and swallowing difficulties
• blurred vision

For more in depth information go to the WeMove Web site.

Lewy Body Disease

Parkinson's disease is characterized by Lewy bodies in the substantia nigra. In Lewy body disease Lewy bodies are found throughout the brain. The clinical features are of fairly rapidly progressive, poorly dopamine responsive Parkinsonism in conjunction with progressive dementia. The dementia of Lewy Body disease is far more prominent and sever than the dementia of other Parkinsonian syndromes.

Vascular Parkinsonism

Multiple small strokes, particularly if located adjacent to a deep part of the brain called the the internal capsule, can cause parkinsonism. Patients with this disorder are more likely to present with gait difficulty than tremor and are more likely to have symptoms that are worse in the lower extremities than upper extremities. Some will also report an abrupt onset of symptoms. Signs on neurological exam may include bilateral slowing, impaired fine movements, increased tone, and a gait disturbance.

Treatment for this condition is the same as for PD.