Parkinson plus syndrome

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Parkinson plus syndrome
Classification and external resources
eMedicine article/1154074

Parkinson-plus syndromes, also known as disorders of multiple system degeneration, are a group of neurodegenerative[1] diseases featuring the classical features of Parkinson's disease (tremor, rigidity, akinesia/bradykinesia, postural instability) with additional features that distinguish them from simple idiopathic Parkinson's disease. Some consider Alzheimer's disease to be in this group.[2]

The atypical parkinsonian or Parkinson Plus syndromes are often difficult to differentiate from Parkinson's disease and each other. They include multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBGD). Dementia with Lewy bodies (DLB), may or may not be part of the PD spectrum, but it is increasingly recognized as the second most common type of neurodegenerative dementia after Alzheimer's disease. These disorders are currently lumped into two groups, the synucleinopathies and the tauopathies.[3]

Additional Parkinson-plus syndromes include Pick's disease and olivopontocerebellar atrophy (OPCA).[4] The latter is characterized by ataxia and dysarthria, and may occur either as an inherited disorder or as a variant of multiple system atrophy. MSA is also characterized by autonomic failure, formerly known as Shy-Drager syndrome.[5]

Nuclear medicine SPECT procedure using 123I-IBZM, is an effective tool in the establishment of the differential diagnosis between patients with Parkinson's disease and Parkinson-Plus syndromes.[6]

[edit] Treatments

The Parkinson-plus syndromes are usually more rapidly progressive and less likely to respond to anti-parkinsonian medication than Parkinson's disease.[7] However, the additional features of the diseases may respond to medications not used in Parkinson's disease.

[edit] See also

[edit] References

  1. ^ Bensimon G, Ludolph A, Agid Y, Vidailhet M, Payan C, Leigh PN (January 2009). "Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS study". Brain 132 (Pt 1): 156–71. doi:10.1093/brain/awn291. PMC 2638696. PMID 19029129. http://brain.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=19029129. 
  2. ^ Cecil Textbook of Medicine, 22nd edition, ISBN 0-7216-9652-X
  3. ^ Mark, M. H. (2001). "Lumping and splitting the Parkinson Plus syndromes: dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, and cortical-basal ganglionic degeneration.". Neurologic Clinics 19 (3): 607–27. PMID 11532646. 
  4. ^ Constance Ward (2006). Journal of Neuroscience Nursing 38 (4): 242–247. PMID 16925000. Characteristics and Symptom Management of Progressive Supranuclear Palsy: A Multidisciplinary Approach. 
  5. ^ Multiple System Atrophy with Orthostatic Hypotension Information Page
  6. ^ Loss of Dopamine-D2 Receptor Binding Sites in Parkinsonian Plus Syndromes
  7. ^ Litvan I, Campbell G, Mangone CA, Verny M, McKee A, Chaudhuri KR, Jellinger K, Pearce RK, D'Olhaberriague L. (Jan 1997). "Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olzewski syndrome) from related disorders". Brain: 65–74. PMID 9055798. 
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