RESUMO
Limbic encephalitis (LE) associated with positive potassium channel complex antibodies often manifests with faciobrachial dystonic seizures (FBDS). We retrospectively analyze two cases, admitted to our video-EEG unit between 2006 and 2014. Both patients were males, aged 66 and 76 years respectively, presenting with brief, but very frequent uni/bilateral dystonic brachial movements, hand posturing and ipsilateral facial grimacing. Severe hyponatremia was found in both patients who went on to develop cognitive impairment. Immunosuppressive therapy improved both seizures and cognitive dysfunction. Serology testing confirmed anti VGKC antibody presence. FBDS are often the first manifestation of LE associated to positive anti VGKC antibodies, and are refractory to treatment with antiepileptic drugs. Early diagnosis and treatment of FBDS with immunosuppressive therapy is important, not only because of seizure suppression, but also because it may help limit the extent of the cognitive damage.
Assuntos
Neuropatias do Plexo Braquial/etiologia , Distúrbios Distônicos/etiologia , Encefalite Límbica/complicações , Convulsões/etiologia , Idoso , Autoanticorpos/sangue , Neuropatias do Plexo Braquial/sangue , Distúrbios Distônicos/sangue , Eletroencefalografia , Humanos , Hiponatremia/sangue , Terapia de Imunossupressão , Masculino , Estudos RetrospectivosRESUMO
Status Dystonicus (SD) is characterized by generalized muscle contractions in dystonic patients. We report 5 cases of SD, two of which in patients with dystonic cerebral palsy, one in a patient with primary segmental dystonia, one in a patient with Hallervorden-Spatz syndrome and one in a patient with Wilson's disease (WD). Three patients were admitted to an intensive care unit and treated with propofol and midazolam, and two were submitted to neurosurgical procedures (bilateral pallidotomy and bilateral pallidal deep brain stimulation). Triggering factors were identified in three patients as follows: infection, stress-induced and zinc therapy for WD. On follow-up, two patients presented with significant improvement of dystonia, whereas the other three cases the clinical picture ultimately returned to baseline pre-SD condition.
Assuntos
Distúrbios Distônicos/tratamento farmacológico , Distúrbios Distônicos/cirurgia , Adulto , Criança , Creatina Quinase/sangue , Distúrbios Distônicos/sangue , Terapia por Estimulação Elétrica , Humanos , Masculino , Pessoa de Meia-Idade , Técnicas Estereotáxicas , Resultado do TratamentoRESUMO
O "Status Dystonicus" (SD) é uma situação clínica caracterizada por contratura muscular generalizada em pacientes com distonias. Relatamos cinco casos de SD, que ocorreram em dois pacientes com distonia associada à paralisia cerebral, um paciente com distonia segmentar primária, um com síndrome de Hallervorden-Spatz e um com doença de Wilson (DW). Três pacientes foram submetidos a tratamento em terapia intensiva com uso de propofol e midazolam e dois pacientes foram submetidos ao tratamento neurocirúrgico (um paciente com palidotomia bilateral e outro com estimulador cerebral profundo palidal bilateral). Em três casos foram identificados fatores desencadeantes: infecção, estresse acentuado e o uso de zinco no tratamento da DW. Em dois pacientes ocorreu melhora importante do quadro distônico, e outros três retornaram à situação clínica pré-SD.