RESUMO
BACKGROUND: Globus Pallidus Interna (GPi) deep brain stimulation (DBS) is an effective treatment for DYT1-associated dystonia, but long-term results are lacking. OBJECTIVE: To evaluate the long-term effects of GPi DBS in patients with DYT1 dystonia. METHODS: A retrospective chart review (cohort study) of 47 consecutive DYT1+ patients treated by a single surgical team over a 10-year period and followed for up to 96 months (mean, 46 months) was performed. Symptom severity was quantified with the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor (M) and disability (D) sub-scores. RESULTS: As measured with the BFMDRS (M), symptom severity was reduced to less than 20% of baseline after 2 years of DBS therapy (P = .001). The disability scores were reduced to <30% of baseline (P = .001). Symptomatic improvement was durable throughout available follow-up. Sixty-one percent of patients had discontinued all dystonia-related medications at their last follow-up. Ninety-one percent had discontinued at least 1 class of medication. Infections requiring removal and later reimplantation of hardware occurred in 4 of 47 patients (8.5%). Hardware malfunction including lead fractures occurred in 4 of 47 cases (8.5%). Lead revision to address poor clinical response was performed in 2 of 92 implanted leads (2.2%). CONCLUSION: GPi DBS is an effective therapy for DYT1-associated torsion dystonia. Statistically significant efficacy is maintained for up to 7 years. Neurologic complications are rare, but long-term hardware-related complications can be significant.
Assuntos
Estimulação Encefálica Profunda/efeitos adversos , Estimulação Encefálica Profunda/métodos , Distonia Muscular Deformante/diagnóstico , Distonia Muscular Deformante/prevenção & controle , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/etiologia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
Introducción: Los antipsicóticos atípicos o de segunda generación producen efectos extrapiramidales con poca frecuencia. Risperidona a dosis bajas produce pocos efectos extrapiramidales. El síndrome de Pisa, una forma de distonía tadía, es un cuadro extremadamente raro. Con risperidona se ha comunicado recientemente algún caso. Caso Clínico: Paciente de 60 años de edad, diagnosticada de esquizofrenia desde los 16 años. Ha seguido tratamiento con diversos neurolépticos a los largo de 44 años. Con risperidona siguió tratamiento durante 1 año. Desarrolló un cuadro de distonía tadía grave, que remitió a lo largo de varios meses con clozapina, después de intentar otras estrategias. Discusión y conclusiones: la fisiopatología, la clínica y el tratamiento de este raro cuadro se discute a la luz de este caso. La resolución después de varios meses con clozapina es una de las posibles actuaciones aconsejadas
Introduction: The atypical antipsychotics of second generation produce not frequently extrapyramidal effects. Risperidone in low doses produces a few extrapyramidal effects. The Pisa syndrome, a form of tardive distony, is an extremely rare medical-board. With risperidone some cases have been reported recently. Clinical case: A sixty years old patient was diagnosticated of schizophrenia since she was sixtee. She has continued treatment with different neuroleptics during forty-four years. Se went on treatment with risperidone for a year. Se developed a grave tardive distony medical-board that remitted all along several months with clozapine, after have attempted another strategics. Discussion and conclusions: The physiopathology, the clinical and the treatment in this rare medica´l-board is discussed at the light of this case. The resolution after several months with clozapine is one of the possible adviced actuations
