Abstract |
A 39-year-old man (a lifetime non-smoker) presented with a locked left jaw and leg myoclonus. Clinical and electromyographic findings were in keeping with progressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome. A thoracic CT scan demonstrated a 19 mm right hilar nodule, which was proven to be small cell lung cancer on bronchoscopic biopsy. Serological evaluation of the patient's plasma revealed antibodies against glycine receptors (serology negative for anti-GAD, anti-Yo, anti-Hu, anti-Ri, antiamphiphysin, anti-Ma2/Ta, anti-CRMP5 and anti- NMDA receptor). After his cancer was treated with chemotherapy and intravenous immunoglobulins ( IVIg), neurological symptoms resolved but returned several months later without any evidence of cancer recurrence. Symptoms were refractory to corticosteroids and IVIg therapy. Rituximab was then initiated, which led to a dramatic and sustained resolution of symptoms. To our knowledge, this is the first case of PERM related to antiglycine receptor antibodies from paraneoplastic syndrome, which resolved with rituximab.
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Authors | Robert Kyskan, Kristine Chapman, André Mattman, Don Sin |
Journal | BMJ case reports
(BMJ Case Rep)
Vol. 2013
(Jun 21 2013)
ISSN: 1757-790X [Electronic] England |
PMID | 23813517
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Antineoplastic Agents
- Autoantibodies
- Receptors, Glycine
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Topics |
- Adult
- Antineoplastic Agents
(therapeutic use)
- Autoantibodies
(blood)
- Carcinoma, Small Cell
(complications, immunology, therapy)
- Combined Modality Therapy
- Encephalomyelitis
(complications, immunology)
- Humans
- Lung Neoplasms
(complications, immunology, therapy)
- Magnetic Resonance Imaging
- Male
- Muscle Rigidity
(complications, immunology)
- Myoclonus
(complications, immunology)
- Radiotherapy
- Receptors, Glycine
(immunology)
- Treatment Outcome
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