| 제목 |
Progressive multifocal leukoencephalopathy in Systemic Lupus Erythematosus |
| 소속 |
Samsung Changwon Hospital, Sungkyunkwan University school of Medicine, Dapartment of Physical Medicine & Rehabilitation1 |
| 저자 |
Chang woo Kim1*, Young Sook Park1†, Hyun Jung Chang1, Eun sol Cho1, Jae Sam Seo1, Kyo Hun Ku1 |
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Progressive multifocal leukoencephalopathy (PML) is rare and serious demyelinating disease characterized by progressive damage in the white matter of a brain. PML occurs to patients with severe immune deficiency and most commonly to those infected by the human immunodeficiency virus (HIV). We present a rare case of PML in systemic lupus erythematosus (SLE). A 21-year-old SLE woman visited our outpatient clinic for left ankle motor weakness. For evaluation of peripheral neuropathy, nerve conduction study was done but result was normal. For evaluation of neuropsychiatric SLE like cerebrovascular disease, evoked potential study was carried out. There was dysfunction of motor evoked potential on her left abductor hallucis muscle recording. A brain MRI was done and it showed a manifestation of non-enhancing and non-territorial localized confluent hyper-intensities in her right corona radiate and fronto-parietal lobe. To treat the neuropsychiatric SLE, steroid pulse therapy put in place, using 1000mg of methylprednisolone for 5 days, but the left ankle motor was gradually deteriorating. We transferred the patient to the rheumatology department of Samsung Seoul Medical Center for additional studies. In cooperation with neurology, a CSF study was undertaken and a brain biopsy was performed. The JC virus was detected by polymerase chain reaction (PCR) in the CSF study, and SV 40 (polyomavirus) was found in viral particles in her brain tissue. PML was confirmed. Currently, she is in a general ward with a stupor mental state and is undergoing a palliative treatment. This suggests that PML may be underdiagnosed in patients with SLE. A possibility of PML must therefore be considered when undertaking a differential diagnosis of SLE patients who demonstrate unexplained neurologic symptoms or signs. |
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