ABSTRACT
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is an autoimmune disorder involving the brainstem and spinal cord and is sometimes associated with thymoma. We encountered a 75-year-old woman with typical PERM features, glycine receptor antibody, and thymoma. Her neurologic symptoms improved after thymectomy, but she unexpectedly developed anasarca with massive pleural effusions and hypoalbuminemia and finally succumbed to death. The autopsy showed edema and mononuclear infiltration in the pleura but no neuropathological findings typical of PERM. Effective treatment of PERM can reverse the neuropathological signs of encephalomyelitis. The autoimmune nature of anasarca is possible but not proven.
Subject(s)
Autoimmune Diseases/complications , Edema/etiology , Encephalomyelitis/complications , Muscle Rigidity/complications , Myoclonus/complications , Thymectomy/adverse effects , Thymoma/complications , Thymoma/surgery , Thymus Neoplasms/complications , Thymus Neoplasms/surgery , Aged , Autoantibodies/blood , Autoimmune Diseases/surgery , Autopsy , Edema/immunology , Encephalomyelitis/surgery , Fatal Outcome , Female , Humans , Muscle Rigidity/surgery , Myoclonus/surgery , Pleural Effusion/etiology , Pleural Effusion/immunology , Postoperative Complications , Receptors, Glycine/immunology , Serum Albumin/analysisABSTRACT
CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an inflammatory central nervous system disorder that gives rise to brainstem symptoms such as diplopia and articulatory disorders. It shows characteristic "salt-and-pepper" enhancing brainstem lesions on magnetic resonance imaging (MRI) scans. We present an unusual case of CLIPPERS, in which a 29-year-old man initially presented with a seizure. Brain MRI revealed faint open-ring enhancements in the right frontal lobe, and a brain biopsy was performed to make a definitive diagnosis. Immunohistological tests showed perivascular infiltration by primarily CD3- and CD20-positive cells, albeit including a few CD68-positive cells, which confirmed a diagnosis of CLIPPERS. Both the symptoms and the MRI findings were markedly improved by 3 courses of steroid (methylprednisolone) pulse therapy, followed by oral steroid (prednisolone) administration. This case shows that CLIPPERS can present as a phenotype of cortical lesions and seizures as the first signs. A brain biopsy should therefore be actively carried out for differential diagnosis, especially when other, cortically based inflammatory disorders are suspected.
