Case Report

Arnold-Chiari Malformation Type I Associated with Syringomyelia and Atlantoaxial Instability: A Case Report

Eun Jong Kim, Myung Shin Kim, Ju Young Lee, Jin Won Whang, Tae Sik Yoon
Author Information & Copyright
Department of Rehabilitation Medicine, College of Medicine, Ewha Womans University, Korea.

Copyright ⓒ 1998. Ewha Womans University School of Medicine. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Published Online: Dec 31, 1998

Abstract

The Arnold-Chiari Malformation is a cognenital disorder with symptoms such as ataxia, dissociated sensory loss, extremity pain, and frequently associated with other malformations of the same kind, including syringomyelia, basilar impression, atlantoaxial instability. The options for managements of the Chiari malformation have been under considerable debate, especially for case with syringomyelia. Presence of syrinx with evidence of motor weakness, sensory loss and ataxia has been reported as poor prognostic factor and may results in functional disability and dependency in activities of daily living and transfer. We report a case of Arnold-Chiari Malformation type I associated with syringomyelia and atlantoaxial instavility.

A 46 year-old man admitted to neurosurgery department with complaints of ataxic gait and decreased sensation in left extremities which were developed when he was 34 years old and aggravated slowly. 25 years ago he developed progressive weakness of intrinsic muscles of right hand. He was diagnosed as Arnold-Chiari Malformation type I. Under the diagnosis of atlantoaxial instability, the patient was treated with operation of craniocervical fusion and laminectomy at C2-C3 level. Despite these treatment, ataxic gait, motor weakness and sensory dissociation was not improved. After the patient was transferred to rehabilitation department, he received comprehensive rehabilitation program. The patient's functional independence measurement(FIM) score at admission was 72 and improved to 104 discharge. The patient was discharged with the state of the monocane ambulation. Like this case, Arnold-Chiari malformation associated with syringomyelia with the evidence of muscle atrophy, ataxia have poor prognosis, therefore early diagnosis and proper managements along with comprehensive rehabilitation is critical in obtaining the best outcome.

Keywords: Arnold-Chiari malformation; Syringomyelia; Atlantoaxial instability