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Updated: Aug 8, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
[Multiple system atrophy]
Ida Wilkens1, Sabrina Katzdobler1,2, Alexander Bernhardt1,2
1LMU Klinikum, Neurologische Klinik und Poliklinik, Bayern, Germany, München.
Abstract:
Multiple system atrophy is a rare, rapidly progressive neurodegenerative disorder within the spectrum of atypical parkinsonian syndromes. It is clinically defined by the combination of parkinsonism, cerebellar ataxia, and autonomic failure. Neuropathological findings reveal misfolded α-synuclein accumulation in oligodendrocytes, leading to multisystem neurodegeneration. Diagnosis is based on the Movement Disorder Society criteria and characteristic MRI findings but can only be definitively confirmed post mortem. Currently, no disease-modifying therapies are available; management is symptomatic and requires a multidisciplinary approach. Ongoing research aims to establish sensitive biomarkers, identify prodromal disease stages, and develop targeted interventions addressing key pathophysiological mechanisms such as α-synuclein aggregation and neuroinflammation.
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