Related Experiment Video
Updated: Sep 25, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
Multiple system atrophy
1Department of Neurology, University Hospital Innsbruck, Austria. gregor.wenning@uibk.ac.at
Abstract:
Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder that usually manifests in the early fifties and progresses relentlessly with a mean survival of 9 years. Clinically, MSA is dominated by autonomic/urogenital failure which may be associated with either levodopa unresponsive parkinsonism in 80p.cent of cases (MSA-P subtype) or with cerebellar ataxia in 20p.cent of cases (MSA-C subtype). Pathologically, MSA is characterized by a neuronal multisystem degeneration and abnormal glial cytoplasmic inclusions containing alpha-synuclein aggregates. Pharmacological treatment of motor features is disappointing except for a transient L-Dopa response in a minority of MSA-P patients. In contrast, autonomic and urogenital features of MSA should be identified early on because they can be treated effectively in many instances. To improve the outcome of this devastating disorder which causes early severe and progressive disability more research into etiology, pathogenesis, early diagnosis and therapeutic management is necessary. Several research groups have been formed in Europe (EMSA-SG, NNIPPS) and USA (NAMSA-SG) to address these unmet needs.
Insights
Multiple system atrophy (MSA) is a rare neurodegenerative disease affecting people in their fifties. Early diagnosis of autonomic dysfunction is crucial for effective treatment, despite limited options for motor symptoms.
Area of Science:
- Neuroscience
- Neurology
- Pathology
Background:
- Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder with a mean survival of 9 years.
- Clinical presentation includes autonomic/urogenital failure, parkinsonism (MSA-P), or cerebellar ataxia (MSA-C).
- Pathology involves neuronal degeneration and alpha-synuclein aggregates in glial cytoplasmic inclusions.
Purpose of the Study:
- To highlight the clinical and pathological characteristics of MSA.
- To emphasize the importance of early diagnosis and effective treatment of autonomic dysfunction.
- To underscore the need for further research into MSA etiology, pathogenesis, diagnosis, and therapeutics.
Main Methods:
- Clinical case review and pathological analysis of MSA patients.
- Review of current pharmacological treatments for motor and autonomic features.
- Analysis of research initiatives addressing unmet needs in MSA.
Main Results:
- Motor features in MSA have disappointing treatment outcomes, with only transient L-Dopa response in some MSA-P patients.
- Autonomic and urogenital features of MSA can be effectively treated if identified early.
- Significant disability and progressive nature of MSA necessitate further research.
Conclusions:
- Early identification and management of autonomic/urogenital symptoms are vital for improving MSA patient outcomes.
- Current treatments for motor symptoms are largely ineffective, highlighting a critical unmet need.
- Collaborative research efforts are essential to advance understanding and treatment of this devastating disorder.
Related Concept Videos
Multiple Sclerosis l: Introduction
Myasthenia Gravis ll: Pathophysiology
Myasthenia Gravis: Overview and Treatment
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Alterations in Muscle Tone lll
Parkinson's Disease: Overview
Parkinson Disease ll: Pathophysiology

