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마미신경총 증후군과 경막확장증이 동반된 강직성 척추염 - 증례 보고 -
강민재,고문주,고길준,허정필,성덕현 대한근전도전기진단의학회 2011 대한근전도 전기진단의학회지 Vol.13 No.2
Ankylosing spondylitis is characterized by inflammation of multiple articular and para-articular structures, frequently resulting in bony ankylosis. The most common presenting symptom is chronic pain and stiffness in the lower part of the spine. Neurological complications such as cauda equina syndrome are very uncommon but can be caused by fibrosis following arachnoiditis, direct compression by arachnoid cyst, or root damage by dural ectasia. We describe a case of a 41-year-old male patient diagnosed with ankylosing spondylitis 11 years ago complaining of recent voiding and defecation problems. Lumbosacral spine MRI and CT imaging revealed dural ectasia with bony erosion of the posterior wall of L3 to S1 vertebral bodies. The electrodiagnostic study was compatible with bilateral lumbosacral polyradiculopathy and the urodynamic study showed underactive detrusor activity during the voiding phase. At 15 month follow-up the patient’s symptoms showed little improvement and he still complained of voiding problems and decreased defecation sense. We report a patient of ankylosing spondylitis with cauda equine syndrome in whom MRI disclosed dural ectasia, and discuss the role of MRI in revealing pathogenesis.
영구적인 근력 약화를 보이는 Andersen-Tawil syndrome
김준석,성덕현,강민재,고길준,기창석,김병준 대한근전도전기진단의학회 2011 대한근전도 전기진단의학회지 Vol.13 No.1
The familial periodic paralysis (FPP) is a rare inherited channelopathy characterized by reversible attacks of muscle weakness. It has been commonly considered to be a benign disease since frequency and severity of the paralytic attacks improve in adulthood. However, with increasing age, a portion of the patients develops permanent weakness and this may be explained by a progressive destruction of muscle tissue. We report a 19 year-old man who had been showing permanent weakness after recurrent paralytic attacks, which began in late childhood. Along with periodic paralysis, he also exhibited cardiac arrhythmia and dysmorphic features. Clinical diagnosis of Andersen-Tawil syndrome was done by these triad. Up to now there are many reports of FPP in Korea, but few or no studies of permanent weakness were reported, especially in young age. We report atypical case of FPP with early onset of permanent weakness.