RESUMEN
BACKGROUND: The correlation between detection of autoantibodies and the pattern and severity of symptoms in patients with encephalitis was the crucial factor for the initiation of immune therapy. The elimination of autoantibodies using therapeutic apheresis by plasma exchange (PE) and immunoadsorption (IA) is a pathophysiologically guided therapeutic approach. The aim was to evaluate the current use of PE and for the first time also of IA for patients with autoimmune encephalitis. METHODS: A nationwide data collection was performed and the modified Rankin score (mRS) was used to evaluate the severity of neurological symptoms. RESULTS: Data of 31 treatment courses (30 patients and 1 relapse) were documented and 22 patients were positive for autoantibodies (NMDA-R, GABA, VGKC, Hu). In 23 cases PA was performed, tryptophan IA in 7 cases and in 1 patient both methods were applied. In 67 % of the treatment courses the mRS improved and the mean mRS of all patients was 3.2 before apheresis and 2.2 after apheresis (p < 0.05). All patients who were treated with IA improved clinically from a mean mRS of 3.9 before IA to 1.9 after IA (p < 0.01). CONCLUSIONS: For immune-mediated forms of encephalitis rapid elimination of autoantibodies with PA and IA seems to be an effective therapeutic option as part of a multimodal immune therapy and is already established in many clinics in Germany.
Asunto(s)
Autoanticuerpos/aislamiento & purificación , Eliminación de Componentes Sanguíneos/métodos , Encefalopatías/epidemiología , Encefalopatías/terapia , Enfermedad de Hashimoto/epidemiología , Enfermedad de Hashimoto/terapia , Sistema de Registros , Adolescente , Adulto , Distribución por Edad , Anciano , Autoanticuerpos/inmunología , Encefalopatías/inmunología , Encefalitis , Femenino , Alemania/epidemiología , Enfermedad de Hashimoto/inmunología , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Distribución por Sexo , Resultado del Tratamiento , Adulto JovenRESUMEN
A 53-year-old woman had suffered from watery diarrhoea and substitution-requiring hypokalaemia for 18 months. Ultrasonography and abdominal x-ray revealed multiple calcifications in the liver. Retrograde cholangiopancreaticography, performed because an endocrine-secreting pancreatic tumour was suspected, demonstrated occlusion of the major pancreatic duct and calcification in the body of the pancreas. Gastrointestinal hormone levels were elevated (vasoactive intestinal polypeptide 950 pg/ml, pancreatic polypeptide 1000 pmol/l and neurotensin 86 pmol/l) in the blood, and immunohistochemical results on cells of an adenocarcinoma obtained by needle biopsy confirmed a metastasizing VIPoma. Administration of the somatostatin analogue SMS 201-995 achieved marked improvement.