Life-threatening dystonia-dyskinesias in a child: Successful treatment with bilateral pallidal stimulation

• Published in: Movement disorders Vol. 15; no. 5; pp. 1010 - 1012
• Main Authors: Angelini, Lucia, Nardocci, Nardo, Estienne, Margherita, Conti, Chiara, Dones, Ivano, Broggi, Giovanni
• Format: Journal Article
• Language: English
• Published: New York John Wiley & Sons, Inc 01-09-2000; Wiley
• Subjects: Adolescent; Biological and medical sciences; Cognition Disorders - etiology; Deep brain stimulation; Diagnosis, Differential; Diseases of the nervous system; Dysarthria - etiology; Dyskinesias - etiology; Dystonic Disorders - complications; Dystonic Disorders - physiopathology; Dystonic Disorders - therapy; Electric Stimulation Therapy - methods; Electrodes, Implanted; Globus Pallidus - surgery; Humans; Male; Medical sciences; Pallidal stimulation; Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects); Refractory dyskinesia; Refractory dystonia; Severity of Illness Index; Stereotaxic Techniques; Treatment Outcome; Videotape Recording; Human; Nervous system diseases; Critically ill; Electrical stimulus; Instrumentation therapy; Bilateral; Male; Pallidum; Involuntary movement; Case study; Refractory; Instrumental stimulation; Striated muscle disease; Evolution; Dystonia; Neurological disorder; Child; Extrapyramidal syndrome; Dyskinesia
• ISSN: 0885-3185; 1531-8257
• DOI: 10.1002/1531-8257(200009)15:5<1010::AID-MDS1039>3.0.CO;2-5

We report a 13‐year‐old boy who developed severe, refractory dystonia–dyskinesias as an abrupt worsening of a previously nonprogressive movement disorder. The movements became continuous, requiring artificial respiration and continuous sedation in the intensive‐care unit. Various drugs and drug combinations failed to achieve control. The child was then treated successfully with bilateral pallidal (GPi) stimulation as shown in the videotape. Four months later and without medication, the boy regained autonomous gait and audible speech; his neurologic condition continues to improve.