Medicine:Parkinson plus syndrome
| Parkinson-plus syndromes | |
|---|---|
| Other names | Disorders of multiple system degeneration |
| Specialty | Neurology |
Parkinson-plus syndromes is a group of neurodegenerative[1] diseases featuring the classical features of Parkinson's disease (tremor, rigidity, akinesia/bradykinesia, and postural instability) with additional features that distinguish them from simple idiopathic Parkinson's disease (PD). Some consider Alzheimer's disease to be in this group.[2] Parkinson-plus syndromes are either inherited genetically or occur sporadically.[3]
Atypical parkinsonism, and other Parkinson-plus syndromes are often difficult to differentiate from PD and each other. They include multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). 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.[4][5] They may coexist with other pathologies.[6]
Additional Parkinson-plus syndromes include Pick's disease and olivopontocerebellar atrophy.[7] 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.[8]
Presentation
Clinical features that distinguish Parkinson-plus syndromes from idiopathic PD include symmetrical onset, a lack of or irregular resting tremor, and a reduced response to dopaminergic drugs (including levodopa).[3] Additional features include bradykinesia, early-onset postural instability, increased rigidity in axial muscles, dysautonomia, alien limb syndrome, supranuclear gaze palsy, apraxia, involvement of the cerebellum including the pyramidal cells, and in some instances significant cognitive impairment.[3]
Diagnosis
Accurate diagnosis of these Parkinson-plus syndromes is improved when precise diagnostic criteria are used.[3] Since diagnosis of individual Parkinson-plus syndromes is difficult, the prognosis is often poor. Proper diagnosis of these neurodegenerative disorders is important as individual treatments vary depending on the condition. The nuclear medicine SPECT procedure using 123I‑iodobenzamide (IBZM), is an effective tool in the establishment of the differential diagnosis between patients with PD and Parkinson-plus syndromes.[9]
Treatments
Parkinson-plus syndromes are usually more rapidly progressive and less likely to respond to antiparkinsonian medication than PD.[10][11] However, the additional features of the diseases may respond to medications not used in PD.
Current therapy for Parkinson-plus syndromes is centered around a multidisciplinary treatment of symptoms.[12][13]
These disorders have been linked to pesticide exposure.[14]
See also
- Frontotemporal dementia and parkinsonism linked to chromosome 17
References
- ↑ "Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS study". Brain 132 (Pt 1): 156–71. January 2009. doi:10.1093/brain/awn291. PMID 19029129.
- ↑ Cecil Textbook of Medicine, 22nd edition, ISBN:0-7216-9652-X
- ↑ 3.0 3.1 3.2 3.3 Mitra K.; Gangopadhaya P. K.; Das S. K. (2003). "Parkinsonism plus syndrome—a review". Neurol India 51 (2): 183–188. PMID 14570999.
- ↑ 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. doi:10.1016/S0733-8619(05)70037-2. PMID 11532646.
- ↑ "The Differential Diagnosis and Treatment of Atypical Parkinsonism.". Dtsch Arztebl Int. 113 (5): 61–9. February 5, 2016. doi:10.3238/arztebl.2016.0061. PMID 26900156.
- ↑ Brittany N. Dugger; Charles H. Adler; Holly A. Shill; John Caviness; Sandra Jacobson; Erika Driver-Dunckley; Thomas G. Beach; the Arizona Parkinson’s Disease Consortium (May 2014). "Concomitant pathologies among a spectrum of parkinsonian disorders.". Parkinsonism Relat Disord. 20 (5): 525–9. doi:10.1016/j.parkreldis.2014.02.012. PMID 24637124.
- ↑ Constance Ward (2006). "Characteristics and symptom management of progressive supranuclear palsy: a multidisciplinary approach". Journal of Neuroscience Nursing 38 (4): 242–247. doi:10.1097/01376517-200608000-00007. PMID 16925000. Archived from the original on 2008-07-23. https://web.archive.org/web/20080723181442/http://www.aann.org/ce/pdf/0608b.pdf.
- ↑ "Multiple System Atrophy with Orthostatic Hypotension Information Page". http://www.ninds.nih.gov/disorders/msa_orthostatic_hypotension/msa_orthostatic_hypotension.htm.
- ↑ Hierholzer, Johannes; Cordes, Michael; Venz, Stephan; Schelosky, Ludwig; Harisch, Cordula; Richter, Wolf; Keske, Uwe; Hosten, Norbert et al. (1998-06-01). "Loss of Dopamine-D2 Receptor Binding Sites in Parkinsonian Plus Syndromes" (in en). Journal of Nuclear Medicine 39 (6): 954–960. ISSN 0161-5505. PMID 9627325. http://jnm.snmjournals.org/content/39/6/954.
- ↑ "Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olzewski syndrome) from related disorders". Brain 120 (1): 65–74. Jan 1997. doi:10.1093/brain/120.1.65. PMID 9055798.
- ↑ David R. Williams; Irene Litvan (October 2013). "Parkinsonian syndromes". Continuum (Minneap Minn) 19 (5 Movement Disorders): 1189–212. doi:10.1212/01.CON.0000436152.24038.e0. PMID 24092286.
- ↑ Molloy, F. M., & Healy, D. G. (2011). Parkinsonism Plus Syndromes. In O. Hardiman & C. P. Doherty (Eds.), Neurodegenerative Disorders (181-196). London: Springer London. doi:10.1007/978-1-84996-011-3_9
- ↑ Ling H (2016). "Clinical Approach to Progressive Supranuclear Palsy". J Mov Disord 9 (1): 3–13. doi:10.14802/jmd.15060. PMID 26828211.
- ↑ Milestones in Atypical and Secondary Parkinsonisms
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