Related Experiment Video
Updated: May 25, 2026

Comprehensive Autopsy Program for Individuals with Multiple Sclerosis
Published on: July 19, 2019
Probable hereditary multiple system atrophy-autonomic (MSA-A) in a family in the United States
1Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA. hohlera@hotmail.com
Abstract:
Multiple system atrophy-autonomic (MSA-A) is a typically spontaneous neurological disorder. The disease, distinguished by a "hot cross bun" sign on MRI, causes a series of autonomic dysfunctions including orthostatic hypotension and genitourinary and gastrointestinal problems. We present an 84 year-old woman with MSA-A symptoms who was positive for a "hot cross bun" sign. Genetic testing was used to rule out other possible ataxias. Importantly, the patient's two sisters also presented with similar symptoms indicating a possible autosomal dominant linkage. To our knowledge, this is the first report of hereditary MSA-A in the USA.
Related Concept Videos
Multiple Sclerosis l: Introduction
Myasthenia Gravis ll: Pathophysiology
Huntington Disease l: Introduction
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...
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Myasthenia Gravis: Diagnostic Tests
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
