Pathological description of a non-motor variant of multiple system atrophy
- 1 Neurology Service, Hospital Clínic and Institut D’Investigació Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain
- 2 Otorhinolaryngology Service, Hospital Clínic and Institut D’Investigació Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain
- 3 Banc de Teixits Neurologics de la Universitat de Barcelona- Hospital Clinic, Hospital Clínic and Institut D’Investigació Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain
- Dr A Iranzo, Neurology Service. Hospital Clinic de Barcelona, C/Villarroel 170, Barcelona 08036, Spain; airanzo{at}clinic.ub.es
- Received 23 January 2008
- Revised 23 April 2008
- Accepted 26 April 2008
Abstract
Multiple system atrophy (MSA) is a neurodegenerative disorder that usually presents clinically as a combination of parkinsonism, cerebellar syndrome and autonomic failure. Patients with MSA can present other clinical features, such as inspiratory stridor and rapid eye movement (REM) sleep behaviour disorder (RBD). We report a patient with pathologically confirmed MSA who presented with a longstanding history of stridor, RBD and autonomic disturbances but did not develop overt parkinsonism or cerebellar signs. This case illustrates that MSA may present clinically without its cardinal motor symptoms, and that stridor and RBD may be clues to recognise the disease in a patient with autonomic failure.
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Competing interests: None.
