Syndrome of fixed dystonia in adolescents--short term outcome in 4 cases.
We describe the clinical features, investigations and outcome of 4 adolescents aged 13, 16, 17 and 19 years, with fixed dystonia. The diagnosis was made within 6 months of the onset of symptoms. One patient had an identifiable traumatic precipitant. All the affected extremities had pain, sudomotor and vascular changes which were consistent with complex regional pain syndrome. The extremities affected by dystonia were the foot and the hand. The dystonia spread to affect other extremities in one patient. One patient had hemifacial spasm. Examination of the central and peripheral nervous system and allied investigations failed to reveal an organic cause. Common genetic causes for dystonia were excluded. The response to physical treatments for the affected extremities, such as Botulinum Toxin and surgery was poor. In all our cases there were significant psychological and psychiatric factors. Three patients fully met the criteria for psychogenic dystonia and responded well to psychological intervention. Fixed dystonia in adolescents is an uncommon disorder of unknown aetiology, usually presenting in girls, which can be very disabling and difficult to treat. The affected parts of the body are usually painful and show vascular changes. The condition is allied to CRPS. Treatment with multidisciplinary approach including psychological measures and physiotherapy is more likely to be successful and may prevent unnecessary physical measures.
Majumdar
Anirban
A
Department of Paediatric Neurology, Bristol Royal Hospital for Children, Level 6 UBHT Education Centre, Upper Maudlin Street, Bristol BS2 8AE, United Kingdom. anirban98@hotmail.com
López-Casas
Jesús
J
Poo
Pilar
P
Colomer
Jaume
J
Galvan
Marta
M
Lingappa
Lokesh
L
Short
Clare
C
Jardine
Philip E
PE
Fernández-Alvarez
Emilio
E
D000293:Adolescent; D001007:Anxiety / Q000523:psychology; D001340:Autonomic Nerve Block; D001733:Bites and Stings / Q000150:complications; D019274:Botulinum Toxins, Type A / Q000627:therapeutic use; D020918:Complex Regional Pain Syndromes / Q000150:complications; D003975:Diazepam / Q000627:therapeutic use; D004421:Dystonia / Q000188:drug therapy / Q000473:pathology* / Q000628:therapy*; D005260:Female; D005528:Foot / Q000473:pathology; D019569:Hemifacial Spasm / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D001523:Mental Disorders / Q000150:complications; D009125:Muscle Relaxants, Central / Q000627:therapeutic use; D009460:Neurologic Examination; D009771:Obsessive-Compulsive Disorder / Q000150:complications; D013746:Tetany / Q000150:complications; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult
D009125:Muscle Relaxants, Central; D019274:Botulinum Toxins, Type A; D003975:Diazepam