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Thymoma-associated Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM) with Myasthenia Gravis.
- Source :
-
Internal medicine (Tokyo, Japan) [Intern Med] 2017; Vol. 56 (13), pp. 1733-1737. Date of Electronic Publication: 2017 Jul 01. - Publication Year :
- 2017
-
Abstract
- We report a case of a 72-year-old woman who initially presented with symptoms of bulbar myasthenia and was positive for anti-acetylcholine receptor antibodies. She subsequently developed painful muscle spasms, myoclonus, and stiffness. Thymoma was detected, and both anti-glycine receptor and anti-glutamic acid decarboxylase antibodies were found. She was diagnosed with thymoma-associated progressive encephalomyelitis with rigidity and myoclonus (PERM). She experienced marked improvement after thymectomy followed by plasma exchange and intravenous immunoglobulin and prednisolone. This case suggests that thymectomy followed by sufficient immunosuppression may be useful in the treatment of thymoma-associated PERM. Myasthenia gravis may develop in thymoma-associated PERM patients.
- Subjects :
- Aged
Autoantibodies
Diagnosis, Differential
Encephalomyelitis therapy
Female
Glutamate Decarboxylase immunology
Humans
Muscle Rigidity therapy
Myasthenia Gravis complications
Receptors, Glycine
Thymectomy
Thymoma therapy
Thymus Neoplasms therapy
Encephalomyelitis complications
Encephalomyelitis diagnosis
Muscle Rigidity complications
Muscle Rigidity diagnosis
Thymoma complications
Thymoma diagnosis
Thymus Neoplasms complications
Thymus Neoplasms diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 1349-7235
- Volume :
- 56
- Issue :
- 13
- Database :
- MEDLINE
- Journal :
- Internal medicine (Tokyo, Japan)
- Publication Type :
- Academic Journal
- Accession number :
- 28674368
- Full Text :
- https://doi.org/10.2169/internalmedicine.56.7979