Juvenile parkinsonism – Dystoniaparkinsonism, Ataxia-parkinsonism, Spastic paraplegia-parkinsonism and Parkinsonism due to brain iron accumulation – part II

Authors

Keywords:

Juvenile parkinsonism, atypical parkinsonism, Segawa syndrome, Wilson disease

Abstract

Parkinsonism can manifest within a clinical spectrum of syndromes, in combination with other motor symptoms (dystonia, ataxia, spastic paraparesis, etc.) which severity usually is equal or exceeding that of the parkinsonism. The onset of the disease is usually in childhood and adolescence. Dopa-responsive dystonia (Segawa's disease) starts most commonly with fluctuant dystonia responding well to levodopa, with asymmetric leg dystonia leading to gait disturbance and subsequent progression to segmental or generalized dystonia.
Parkinson symptoms such as static tremor and postural instability are also possible. Parkinsonism may be part of the clinical picture of some spinocerebellar ataxias (SCA2 SCA3, SCA17 etc.). The akinetic-rigid phenotype is described more frequently compared to static tremor. It is also possible that the disease starts with Parkinson syndrome, with cerebellar manifestations appearing later in the disease course. Neurodegenerative diseases due to the accumulation of metals in the basal ganglia (iron, copper, calcium, etc.) are a heterogeneous group of diseases manifesting with extrapyramidal symptoms.
Timely diagnosis of Wilson's disease is of particular importance because of the possibility of anti-copper therapy. We present the clinical course of dystonia-parkinsonism, ataxia-parkinsonism, spastic paraplegia-parkinsonism and parkinsonism resulting from metal accumulation in the brain.

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Published

30.12.2023

How to Cite

Aleksandrova, I., Asenova, A., & Bojinova, V. (2023). Juvenile parkinsonism – Dystoniaparkinsonism, Ataxia-parkinsonism, Spastic paraplegia-parkinsonism and Parkinsonism due to brain iron accumulation – part II. Bulgarian Neurology, 24(3), 106–112. Retrieved from https://www.nevrologiabg.com/journal/index.php/neurology/article/view/148

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