Two Cases of Holosyringomyelia with Atypical Clinical Features

J Korean Neurol Assoc > Volume 16(1); 1998 > Article

Journal of the Korean Neurological Association 1998;16(1): 73-76.

비전형적 임상증상을 보인 전(全)척수공동증 2례

황신이, 조은경, 노숙영, 박기덕, 최경규

이화여자대학교 의과대학 신경과

Two Cases of Holosyringomyelia with Atypical Clinical Features

SY Hwang, M.D., EK Cho, M.D., SY Roh, M.D., KD Park, M.D., KG Choi, M.D.

Department of Neurology, College of Medicine, Ewha Womans University

Abstract

Syringomyelia is a well known clinical syndrome that has typical neurologic manifestations such as sensory dissociation. The authors experienced 2 cases of holosyringomyelia with unusual clinical manifestations. The first case was 26-year-old man complaining of vague sensory symptoms scattered over the trunk but no other neurologic abnormality. At age 14, he suffered from thoracic scoliosis which was corrected with total spine brace. Neurologic examination on admission revealed no sensory dissociation or motor weakness except focal areas of decreased pin-prick on the trunk. Spine MRI scan, surprisingly revealed holosyrinx involving almost entire cord associated with typeⅠArnold-Chiari malformation. The second case was 38-year-old male who presented with cervical sensory level and absence of position and vibration senses in the lower extremities. The MRI scan revealed syrinx involving entire spinal cord. These cases suggest huge or extensive syringomyelia can be manifested without typical features of syrinx and definite motor weakness and the spine scoliosis associated with vague sensory symptoms may be a sign of syringomyelia.