NPF overview of six atypical parkinsonism syndromes

Here’s the National Parkinson Foundation ( overview of atypical parkinsonism syndromes.  In addition to the four that are part of Brain Support Network – PSP, CBD, MSA, and DLB – NPF also includes drug-induced parkinsonism and vascular parkinsonism.

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What are the Different Types of Atypical Parkinsonism Syndromes?
National Parkinson Foundation

Progressive Supranuclear Palsy (PSP)

* PSP is one of the more common forms of atypical Parkinsonism.

* Symptoms of PSP usually begin after age 50 and progress more rapidly than PD.

* These symptoms include: imbalance and frequent falls early on in the disease, rigidity of the trunk, voice and swallowing changes and eye-movement problems including the ability to move eyes up and down.

* Dementia develops later in the disease. There is no specific treatment for PSP.

* Dopaminergic medication treatment is often tried and may provide some modest benefit.

* Other therapies such as speech therapy, physical therapy, and antidepressants are important for management of patients with PSP.

* No laboratory/brain scan testing exists for PSP. In rare cases, some patients may have shrinking of a particular part of the brain, called the “Pons”, which can be seen on an MRI of the brain.

Corticobasal Degeneration (CBD)

* CBD is the least common of the atypical causes of Parkinsonism

* CBD develops after age 60 and progresses more rapidly than PD.

* The initial symptoms of CBD include asymmetric bradykinesia, rigidity, limb dystonia and myoclonus (rapid jerking of a limb), postural instability, and disturbances of language.

* There is often marked and disabling apraxia of the affected limb, where it becomes difficult or impossible to perform coordinated movements of the affected limb even though there is no weakness or sensory loss. Sometimes this can be so severe that the movements of the affected limb cannot be controlled and is called ‘alien limb’ phenomenon.

* No laboratory/brain scan tests exist to confirm the diagnosis of CBD. CBD is a clinical diagnosis.

* There is no specific treatment for CBD.

* Supportive treatment such as botulinum toxin (Botox) for dystonia, antidepressants, speech and physical therapy may be helpful.

* Levodopa and dopamine agonists (common PD medications) seldom help.

Multiple System Atrophy (MSA)

* MSA is a larger term for several disorders in which one or more system in the body deteriorates.

* Included in the category of MSA are: Shy-Drager syndrome (DSD), Striatonigral degeneration (SND) and OlivoPontoCerebellar Atrophy (OPCA).

* In 2007, a new classification was proposed with two major subtypes: MSA- P (similar to SND) in which parkinsonian signs predominate and MSA-C a cerebellar dysfunction type which resembles OPCA. The term Shy-Drager Syndrome is now rarely used.

* The mean age of onset is in the mid-50s.

* Symptoms include: bradykinesia, poor balance, abnormal autonomic function, rigidity, difficulty with coordination, or a combination of these features.

* Initially, it may be difficult to distinguish MSA from Parkinson’s disease, although more rapid progression, poor response to common PD medications, and development of other symptoms in addition to Parkinsonism, may be a clue to its diagnosis.

* No laboratory/brain scan testing exists to confirm the diagnosis of MSA.

* Patients respond poorly to PD medications, and may require higher doses than the typical PD patient for mild to modest benefits.

Dementia with Lewy bodies (DLB)

* Did you know that Dementia with Lewy Bodies is second to Alzheimer’s as the most common cause of dementia in the elderly?

* DLB is a neurodegenerative disorder that results in progressive intellectual and functional deterioration.

* Patients with DLB usually have early dementia, prominent visual hallucinations, fluctuations in cognitive status over the day, and Parkinsonism.

* It is not uncommon for patients to present with cognitive problems particularly language problems, known as aphasia.

* Other cognitive changes in patients with DLB include deficits in attention, executive function (problem solving, planning) and visuospacial function (the ability to produce and recognize figures, drawing or matching figures).

* There are no known therapies to stop or slow the progression of DLB.

Drug-induced Parkinsonism

* Side effects of some drugs, especially those that affect dopamine levels in the brain such as anti-psychotic or anti-depressant medication, can actually cause symptoms of Parkinsonism.

* Although tremor and postural instability may be less severe, this condition may be difficult to distinguish from Parkinson’s disease.

* Medications that can cause the development of Parkinsonism include:
– Antipsychotics
– Metaclopramide
– Reserpine
– Tetrabenazine
– Some calcium channel blockers
– Stimulants such as amphetamines and cocaine
– Usually after stopping those medications Parkinsonism gradually disappears over weeks to months

Vascular Parkinsonism

* Multiple small strokes 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 part of the body.

* Some will also report the abrupt onset of symptoms or give a history of step-wise deterioration (symptoms get worse, then plateau for a period).

* Dopamine is tried to improve patients’ mobility although the results are often not as successful.

* Vascular Parkinsonism is static (or very slowly progressive) when compared to other neurodegenerative disorders.

Page reviewed by Dr. Joash Lazarus, NPF Movement Disorders Fellow, Department of Neurology at Emory University School of Medicine.