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 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 (<1 min) episodes in the former. If these signs are present, brain MRI should be used to identify a cause of symptomatic PDA.
Marcel
Christophe
C
Département de Neurologie, Hôpital Civil de Strasbourg, 1 Place de l'Hôpital, BP426, 67091 Strasbourg, France.
Anheim
Mathieu
M
Flamand-Rouvière
Constance
C
Heran
Françoise
F
Masnou
Pascal
P
Boulay
Clotilde
C
Mari
Ivan
I
Tranchant
Christine
C
Roze
Emmanuel
E
D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D001259:Ataxia / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D002220:Carbamazepine / Q000627:therapeutic use; D003711:Demyelinating Diseases / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D003937:Diagnosis, Differential; D004401:Dysarthria / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D020233:Gait Disorders, Neurologic / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008297:Male; D009103:Multiple Sclerosis / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D013064:Speech Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D016896:Treatment Outcome; D055815:Young Adult
D000894:Anti-Inflammatory Agents, Non-Steroidal; D000927:Anticonvulsants; D002220:Carbamazepine