RESUMO
Hashimoto's Encephalopathy (HE) is a very rare condition characterized by psychosis, seizures, cognitive fluctuations, and myoclonus. In a few published cases, plasma exchange has been used due to the theoretical removal of antithyroid peroxidase antibodies (anti-TPO), one of the postulated causes of the condition. We report a case of HE treated by plasma exchange where no clinical or neurophysiologic improvement was observed despite documented reduction of the anti-TPO antibody to levels below the limits of laboratory detection. We discuss these findings in the context of the known literature for this disease process.
Assuntos
Remoção de Componentes Sanguíneos/métodos , Encefalite/terapia , Doença de Hashimoto/terapia , Troca Plasmática/métodos , Autoanticorpos/sangue , Transtornos Cognitivos/complicações , Encefalite/complicações , Feminino , Doença de Hashimoto/complicações , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Iodeto Peroxidase/imunologia , Pessoa de Meia-Idade , Transtornos Paranoides/complicações , Plasmaferese/métodos , Estado Epiléptico/complicações , Esteroides/uso terapêuticoRESUMO
ABSTRACT: Patients with Ehlers-Danlos syndrome (EDS) have many associated symptoms of unclear cause, most recently suggested to be due to small fiber neuropathy (SFN). Small fiber neuropathies are sorely underestimated and with minimal treatment options. We report 2 cases of patients with EDS with presumed immune-mediated SFN, successfully treated with IV immunoglobulins. There is a stark need for further investigational studies into immunosuppressant treatments for immune-mediated SFN as well as the link between EDS and immune-mediated SFN.