부산대학교 도서관

This article discusses a case of progressive encephalomyelitis with rigidity and myoclonus (PERM) associated with anti-DPPX antibodies. PERM is a severe form of stiff-person-syndrome spectrum disorders characterized by brainstem and autonomic involvement, as well as axial and limb rigidity. The patient in this case presented with a neuropsychiatric prodrome, including behavioral changes, weight loss, and movement disorders. The diagnosis was confirmed through positive serum and cerebrospinal fluid DPPX antibodies. Treatment with immunotherapies resulted in significant improvement in symptoms. The document provides a table summarizing the symptoms, CSF findings, and therapy response of patients with PERM associated with different antibodies. The authors emphasize the importance of early recognition and treatment of this condition. [Extracted from the article]