Symptomatic paroxysmal dysarthria-ataxia in demyelinating diseases

Paroxysmal dysarthria-ataxia syndrome (PDA) is a rare neurological disorder that can be either primary or symptomatic of acute neurological dysfunction. Episodes of symptomatic PDA are poorly documented and there are no video reports. We describe the cases of two patients with symptomatic PDA relate...

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Bibliographic Details
Published in:Journal of neurology 2010-08, Vol.257 (8), p.1369-1372
Main Authors: Marcel, Christophe, Anheim, Mathieu, Flamand-Rouvière, Constance, Heran, Françoise, Masnou, Pascal, Boulay, Clotilde, Mari, Ivan, Tranchant, Christine, Roze, Emmanuel
Format: Article
Language:English
Subjects:
Adult
Anti-Inflammatory Agents, Non-Steroidal - therapeutic use
Anticonvulsants - therapeutic use
Ataxia - etiology
Ataxia - pathology
Ataxia - physiopathology
Biological and medical sciences
Carbamazepine - therapeutic use
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Demyelinating Diseases - complications
Demyelinating Diseases - pathology
Demyelinating Diseases - physiopathology
Diagnosis, Differential
Dysarthria - etiology
Dysarthria - pathology
Dysarthria - physiopathology
Female
Gait Disorders, Neurologic - etiology
Gait Disorders, Neurologic - pathology
Gait Disorders, Neurologic - physiopathology
Humans
Life Sciences
Male
Medical sciences
Medicine
Medicine & Public Health
Multiple Sclerosis - complications
Multiple Sclerosis - pathology
Multiple Sclerosis - physiopathology
Neurology
Neurons and Cognition
Neuroradiology
Neurosciences
Original Communication
Speech Disorders - etiology
Speech Disorders - pathology
Speech Disorders - physiopathology
Treatment Outcome
Young Adult
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Summary:Paroxysmal dysarthria-ataxia syndrome (PDA) is a rare neurological disorder that can be either primary or symptomatic of acute neurological dysfunction. Episodes of symptomatic PDA are poorly documented and there are no video reports. We describe the cases of two patients with symptomatic PDA related to demyelinating diseases. Detailed studies of the patients’ speech disorders showed that the dysarthria and gait disorders were of the ataxic type in both cases. Both patients had midbrain lesions at or below the level of the red nucleus, confirming that this area is critically involved in PDA. The best clinical signs for distinguishing between symptomatic and primary PDA are adult onset and short (
ISSN:0340-5354
1432-1459
1432-1459
DOI:10.1007/s00415-010-5534-3