Status epilepticus and autoimmune pancytopenia: the numerous targets of autoimmunity
Clinical Case, 36 - 39Tag this article
Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of abnormal lymphocyte homeostasis with elevated level of CD4- and CD8-negative T lymphocytes (termed double-negative T cell- DNT cells). Clinical manifestations include noninfectious and nonmalignant splenomegaly and autoimmune pathology. We report the case of a 13 years old girl, who in addition to the expected symptoms of immune dysregulation, manifested alternate side status epilepticus and refractory epilepsy with polymorphic focal seizures and cognitive impairment. An overall clinical improvement was obtained by chronic immunomodulant therapy.
KEY WORDS: status epilepticus, autoimmune lymphoproliferative syndrome (ALPS), CNS autoimmunity.