Progressive encephalomyelitis with rigidity and myoclonus: Resolution after thymectomy

Progressive encephalomyelitis with rigidity and myoclonus

“…GlyR antibodies have since been identified in patients with hyperekplexia, stiff-person syndrome, and PERM, usually without GAD antibodies. 7,8 The additional features in our patient may relate to the coexistence of NMDAR antibodies. The core spectrum of NMDAR antibody-associated neuro- logic symptoms consists of an encephalitic picture, often in females, and includes seizures, movement disorders, autonomic instability, and reduction in consciousness.…”

Thymoma-Associated Progressive Encephalomyelitis with Rigidity and Myoclonus Successfully Treated with Thymectomy and Intravenous Immunoglobulin

“…PERM is sometimes associated with neoplasms. There has been a report of lung cancer-associated paraneoplastic PERM [4] , and 1 case with thymoma [5] .…”

“…With regard to PERM, plasmapheresis and corticosteroids were reportedly effective in 1 case [12] . Thymectomy was reported to be effective only in a case of thymomaassociated PERM [5] . In our case, the effects of thymectomy alone were not sufficient enough and the patient required 3 courses of IVIG.…”

Autoantibody testing in encephalopathies

“…The patient responded to aggressive immunotherapy with intravenous immunoglobulin, prednisolone, plasma exchange and intravenous cyclophosphamide and returned to work part-time with only some residual spinal rigidity and bilateral ptosis 32. Several other SPS or PERM patients with antibodies to glycine receptors have now been identified 33–35. The clinical spectrum and treatment responses are variable; only one patient had a tumour and he made a complete recovery after thymectomy 34.…”