Discinesias paroxísticas / Paroxysmal dystonia

RESUMEN Existen enfermedades y condiciones que se presentan con movimientos involuntarios hipercinéticos de aparición súbita y remisión espontanea (episódicas). Dichas condiciones generan dificultades diagnósticas ya que por su carácter intermitente, en ocasiones no pueden ser evaluadas por el clínico.

SUMMARY During the last years, several drugs has been tried to try to diminish the impact of this condition and improve quality of life the people who suffer from dystonia. Oral therapy alone or in combination generates only partial symptom relief and most of the cases end up requiring other more invasive therapies.

[Non-epileptic paroxysmal sleep disorders]. / Trastornos paroxísticos no epilépticos durante el sueño.

Non-epileptic paroxysmal disorders during sleep are a great challenge for the clinician. It is important to know the various clinical manifestations for appropriate differential diagnosis, since alterations in sleep, mostly motor, are part of these disorders. Our paper describes the normal sleep stages and electroencephalographic characteristics and polysomnography basic data. The confusions especially with nocturnal frontal lobe epilepsy are frequent and cause unnecessary drugs administered, the emotional burden of the parents or caretakers, which is the diagnosis of epilepsy. We discuss the possible causes of diagnostic errors.

TITLE: Trastornos paroxisticos no epilepticos durante el sueño.Los trastornos paroxisticos no epilepticos durante el sueño son un gran reto para el clinico. Por ello, es importante conocer las diferentes manifestaciones clinicas que permitan llevar a cabo un diagnostico diferencial adecuado, ya que las alteraciones, sobre todo motoras en el sueño, son parte de estos trastornos. En el presente trabajo se describen las fases del sueño normal y sus caracteristicas electroencefalograficas, asi como datos basicos de la polisomnografia. Las confusiones, sobre todo con la epilepsia nocturna del lobulo frontal, son frecuentes y provocan que se administren farmacos innecesarios, asi como una carga emocional en los padres o cuidadores del paciente, que resulta del diagnostico de epilepsia. Se enuncian las posibles causas de los errores de diagnostico.

Botulinum-A toxin in the treatment of painful post-stroke nocturnal paroxysmal dystonia triggered by periodic limb movements of sleep: case report / Toxina botulínica tipo A no tratamento da distonia paroxística noturna dolorosa pós-isquemia cerebral desencadeada por movimentos periódicos do sono: relato de caso

INTRODUCTION: Sleep disorders presenting involuntary movements may be very annoying to patients, apart from their negative influence on sleep. OBJECTIVE: To report the use of botulinum type-A toxin (BoNT-A) to manage the case of a patient whose sleep was severely disrupted by episodes of dystonic posturing of the right lower limb triggered by periodic limb movements of sleep (PLMS). METHOD: A 79-year-old woman with mild post-stroke right hemiparesis presented with recurrent painful episodes of dystonia of the right lower limb, which disrupted her sleep. The dystonic episodes could also be voluntarily triggered by extension of the right hallux. Polysomnography confirmed that the dystonic episodes were triggered by PLMS. Twenty units of BoNT-A (20U/500U vial) were injected into her right extensor hallucis longus. RESULTS: Shortly after BoNT-A was injected, the dystonic symptoms abated, and the patient achieved better sleep efficiency. CONCLUSION: The PLMS-related involuntary extension of the hallux was probably triggering the nocturnal post-stroke lower limb dystonic paroxysms. BoNT-A injection into the right extensor hallucis longus was effective in managing this condition and thus resolved the associated disruption of sleep.

INTRODUÇÃO: Desordens do sono apresentando movimentos involuntários podem ser bastante perturbadoras aos pacientes, além de sua influência negativa no sono. OBJETIVO: Descrever o uso da toxina botulínica tipo-A (BoNT-A) no manejo do caso de um paciente cujo sono estava gravemente fragmentado por episódios de distonia do membro inferior direito, desencadeados por movimentos periódicos do sono (MPS). MÉTODO: Uma paciente com 79 anos portadora de hemiparesia direita leve seqüelar a isquemia cerebral (AVCI) procurou-nos por episódios dolorosos recorrentes de distonia noturna de seu membro inferior direito, os quais fragmentavam seu sono. Os episódios de distonia também podiam ser desencadeados voluntariamente, por extensão do hálux direito. Uma polisonografia confirmou que os episódios distônicos eram desencadeados pelos MPS. Vinte unidades de BoNT-A (20 U/frasco de 500 U) foram injetadas no seu extensor longo do hálux. RESULTADOS: Alguns dias após a injeção de BoNT-A os sintomas distônicos regrediram, e o sono da paciente tornou-se eficiente. CONCLUSÃO: As extensões involuntárias do hálux relacionadas aos movimentos periódicos do sono estavam provavelmente desencadeando os paroxismos distônicos noturnos pós-AVCI. A injeção de BoNT-A no extensor longo do hálux foi eficaz no manejo desta condição, resolvedo assim a fragmentação do sono.

Botulinum-A toxin in the treatment of painful post-stroke nocturnal paroxysmal dystonia triggered by periodic limb movements of sleep: case report.

INTRODUCTION: Sleep disorders presenting involuntary movements may be very annoying to patients, apart from their negative influence on sleep. OBJECTIVE: To report the use of botulinum type-A toxin (BoNT-A) to manage the case of a patient whose sleep was severely disrupted by episodes of dystonic posturing of the right lower limb triggered by periodic limb movements of sleep (PLMS). METHOD: A 79-year-old woman with mild post-stroke right hemiparesis presented with recurrent painful episodes of dystonia of the right lower limb, which disrupted her sleep. The dystonic episodes could also be voluntarily triggered by extension of the right hallux. Polysomnography confirmed that the dystonic episodes were triggered by PLMS. Twenty units of BoNT-A (20U/500U vial) were injected into her right extensor hallucis longus. RESULTS: Shortly after BoNT-A was injected, the dystonic symptoms abated, and the patient achieved better sleep efficiency. CONCLUSION: The PLMS-related involuntary extension of the hallux was probably triggering the nocturnal post-stroke lower limb dystonic paroxysms. BoNT-A injection into the right extensor hallucis longus was effective in managing this condition and thus resolved the associated disruption of sleep.

Treatment of periodic leg movements with a dopaminergic agonist in subjects with total spinal cord lesions.

OBJECTIVE: To investigate the effect of L-dopa on the PLM/h index of spinal cord injured subjects. SETTING: São Paulo, Brazil. METHODS: Thirteen male volunteers with spinal cord section between T7 - T12, and mean age of 31.6+/-8.3 years participated in the study. L-dopa or placebo were administered for 30 days, 1 h before the volunteers went to sleep, in a double blind, crossover design. Polysomnographic recordings were performed on ten occasions: Phase 1: Basal night, following an adaptation night at the sleep laboratory; phase 2: after 1, 7, 21 and 30 days of L-dopa administration; phase 3: first night of L-dopa or placebo withdrawal; phase IV: 1, 7, 21 and 30 days after placebo ingestion. RESULTS: The index of PLM/h on the first night of L-dopa or placebo withdrawal (phase III) was lower than on both the basal night and the first night of L-dopa treatment. At the time of polysomnographic analysis, volunteers were divided into two groups: index of PLM/h below five and those whose index was above five. Comparison between L-dopa and placebo treatments revealed that only those volunteers with an index above five revealed a reduction in PLM in L-dopa. CONCLUSION: These results indicate that despite the spinal cord lesions, L-dopa treatment is capable of minimizing PLM during sleep.