Neurol India Home 

Year : 2003  |  Volume : 51  |  Issue : 2  |  Page : 183--188

Parkinsonism plus syndrome – A review

K Mitra, PK Gangopadhaya, SK Das 
 From Movement Disorders Clinic, Bangur Institute of Neurology, Kolkata-700025., India

Correspondence Address:
S K Das
74, Minto park Govt. Housing, 247/1, A. J. C. Bose Road, Kolkata-700027

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 a-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 a-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.

How to cite this article:
Mitra K, Gangopadhaya P K, Das S K. Parkinsonism plus syndrome – A review .Neurol India 2003;51:183-188

How to cite this URL:
Mitra K, Gangopadhaya P K, Das S K. Parkinsonism plus syndrome – A review . Neurol India [serial online] 2003 [cited 2023 Dec 11 ];51:183-188
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