Abstract

Introduction: Arnold–Chiari malformation  is the brain stem and cerebellum tonsils descent into the foramen Magnum and often associated with Syrinx. Most patients are symptomatic when descending tonsils are over 12mm. Diagnosis is based on history and physical examination and MRI findings. The present study reports the case with claw hand deformity and syrinx.

Case Report: A 36-year-old construction worker who had no history of previous illness presented with mild weakness and deformity of the upper limbs and had experienced 8 years ago this situation without pain in the left hand with the gradual progress. In neurological examination, atrophy in both the upper limbs with bilateral claw hands and in the lower extremity spasticity and increased reflexes and positive bilateral Babinski sign were seen. MRI shows Arnold–Chiari malformation with syrinx of cervical cord from C2 to T4. He was referred to neurosurgery Service for further treatment.

Conclusion: In any patients with symptoms associated with progressive weakness and atrophy of the upper limb deformity which is painless and progressive onset, Syrinx is suspected and we need to confirm the diagnosis of cervical lesions with magnetic resonance imaging (MRI) .