Neurol India. 2003 Jun;51(2):183-8.
Parkinsonism plus syndrome--a review.
Mitra K, Gangopadhaya PK, Das SK.
Movement Disorders Clinic, Bangur Institute of Neurology, Kolkata 700-025,
India.
Parkinsonism plus syndrome is a group of heterogeneous degenerative
neurological
disorders, which differ from the classical idiopathic Parkinson's disease in
certain associated clinical features, poor response to levodopa, distinctive
pathological characteristics and poor prognosis. Associated clinical
features
include symmetrical onset, infrequent or atypical tremor, prominent rigidity
in
axial musculature, bradykinesia, early postural instability, supranuclear
gaze
palsy, early autonomic failure, pyramidal affection, cerebellar involvement,
alien limb phenomenon, apraxia and significant early cognitive dysfunction
in
some cases. Progressive supranuclear palsy (PSP), multiple system atrophy
(MSA)
and dementia with Lewy body disease (DLB) are commoner disorders. Less
frequent
disorders are cortico-basal ganglionic degeneration (CBGD), frontotemporal
dementia with chromosome 17 (FTDP-17), Pick's disease, parkinsonian-dementia
complex of Guam, Pallidonigral degeneration, Wilson's disease and a rigid
variant of Huntington's disease. During the last 3 decades, major progress
has
been made in understanding PSP, CBGD and FTDP-17, which are tau disorders.
MSA
and DLB together with idiopathic Parkinson's disease are called
alpha-synucleinopathies. Recent studies show that the diagnosis of these
Parkinsonism plus syndromes improves when strict diagnostic criteria are
used.
However, unusual presentations may pose a diagnostic challenge. The
shortcomings
of the current studies demand the need for further research to identify
biologic
markers that may allow earlier diagnosis, and understanding of the factors
leading to alpha-synuclein or tau aggregation. Identification of therapeutic
strategies that may prevent the aggregation of these proteins and rescue
dysfunctional cells has been stressed. This review focuses on the advances
in
the clinical, neuroimaging, pathologic, genetic and management aspects of
these
disorders.
PMID: 14570999 [PubMed - in process]
_____________________________________________
No pessimist ever discovered the secrets of the stars,
or sailed to uncharted land,
or opened a new heaven to the human spirit.
- Helen Keller
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