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Progressive Encephalomyelitis With Rigidity and Myoclonus With Thymoma: A Case Report and Literature Review
Yana Su1, Li Cui1, Mingqin Zhu1
1Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China.
Abstract:
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is part of the variant type of the Stiff Person Syndrome (SPS) and is a rare neurological disease. We report here a patient with PERM who had thymoma and was positive for anti-glutamic acid decarboxylase (anti-GAD) antibodies. Her symptoms improved after treatment with hormones and gamma globulin. We also summarized the literature review of patients with PERM accompanied by tumors reported.
Insights
Progressive encephalomyelitis with rigidity and myoclonus (PERM), a rare neurological disorder and variant of Stiff Person Syndrome (SPS), can be associated with thymoma. This case study shows symptom improvement with hormonal and gamma globulin therapy.
Area of Science:
- Neurology
- Immunology
Background:
- Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disorder, considered a variant of Stiff Person Syndrome (SPS).
- PERM is characterized by encephalomyelitis, rigidity, and myoclonus, often with autonomic dysfunction.
Observation:
- A patient with PERM presented with thymoma and positivity for anti-glutamic acid decarboxylase (anti-GAD) antibodies.
- The patient's neurological symptoms showed improvement following treatment with hormones and intravenous gamma globulin.
Findings:
- This case highlights a potential association between PERM, thymoma, and anti-GAD antibodies.
- Literature review indicates that PERM can occur in patients with various tumors.
Implications:
- Early diagnosis and treatment of PERM, especially when associated with tumors, may lead to improved patient outcomes.
- Further research is warranted to understand the immunological links between PERM, thymoma, and anti-GAD antibodies.
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