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J Neurocrit Care 2008;1(1): 54-58.
반복성 간질중첩증으로 발현한 신경베체트병 1예
구영호, 오승헌·김원찬·김현숙·김옥준
포천중문의과대학 신경과학교실
A Case of Neuro-Behcet’s Disease Presenting with Recurrent Status Epilepticus
Young-Ho Koo, MD Seung-Hun Oh, MD, Won-Chan Kim, MD, Hyun Sook Kim, MD and Ok Joon Kim, MD, PhD
Department of Neurology, Pochon CHA University College of Medicine, Seongnam, Korea
Background: Central nervous system involvement accounts for about 4-49% of Behcet’s disease (Neuro-Behcet’s disease: NBD). However, status epilepticus (SE) is rare manifestation in NBD.
Case Report: A man who had past history of Behcet’s disease for 6 years was admitted with sudden SE. Electroencephalography showed focal sharp waves and frequent ictal discharges in the left temporo- occipital region. Although brain MRI showed diffuse atrophic change of brain without any active lesion, a magnetic resonance spectroscopy (MRS) showed reduced NAA/Cr ratio in the left temporo-occipital region compared with the right side. Various antiepileptic drugs showed minimal improvement but seizure persisted. Additional steroid pulse therapy ceased seizure.
Conclusions: Although a pathophysiology of SE appeared in NBD is still unclear, an active microstructural lesion of cerebral cortex may induce epileptic discharges in NBD. An additional steroid therapy may be helpful in seizure control in NBD patients refractory to anticonvulsant therapy.
Key Words: Neuro-Behcet’s disease·Status epilepticus·Steroid
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