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논문 기본정보

Left ventricular hypertrabeculation/noncompaction in juvenile neuronal ceroid lipofuscinosis.

논문 개요

기관명, 저널명, ISSN, ISBN 으로 구성된 논문 개요 표입니다.
기관명 NDSL
저널명 臨床神經學 = Clinical neurology
ISSN 0009-918x,1882-0654
ISBN

논문저자 및 소속기관 정보

저자, 소속기관, 출판인, 간행물 번호, 발행연도, 초록, 원문UR, 첨부파일 순으로 구성된 논문저자 및 소속기관 정보표입니다
저자(한글) Finsterer, Josef,St?llberger, Claudia,Yoshida, Taemi
저자(영문)
소속기관
소속기관(영문)
출판인
간행물 번호
발행연도 2015-01-01
초록 A 44-year-old man with a bilateral hand tremor suffered from a decline in concentration and abnormal vision for several months. He also complained of easily falling down because of muscle stiffness and cramps in his lower limbs. On admission, he demonstrated lower limb stiffness, muscle cramps, diplopia, hyperhidrosis, left upper limb ataxia and dysesthesia in all limbs. Laboratory examination showed a marked elevation in his serum creatine kinase level (26,890?U/l), and needle electromyography demonstrated myokymic discharges in the muscles of his lower extremities. Isaacs #039; syndrome was diagnosed based on a positive voltage-gated potassium channel antibody titer of 1,007?pM. Administration of an anticonvulsant (phenytoin, 200?mg/day) did not resolve his symptoms; however, high-dose intravenous methylprednisolone therapy (1?g/day for 3 days) resulted in marked clinical improvement. This case suggests that high-dose intravenous methylprednisolone therapy for Isaacs #039; syndrome might be as effective as other immunosuppressive therapies such as plasma exchange or intravenous immunoglobulin.
원문URL http://click.ndsl.kr/servlet/OpenAPIDetailView?keyValue=03553784&target=NART&cn=NART73928255
첨부파일

추가정보

과학기술표준분류, ICT 기술분류,DDC 분류,주제어 (키워드) 순으로 구성된 추가정보표입니다
과학기술표준분류
ICT 기술분류
DDC 분류
주제어 (키워드)