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Syringomyelia presenting as paroxysmal arm posturing resembling seizures
Mícheál Macken1, Elaine Wyllie, William E Bingaman
1Department of Neurology, The Cleveland Clinic Foundation, Ohio 44195, USA.
Pediatric Neurology
|May 7, 2002
Summary
This case study highlights cervical cord disease as a cause of paroxysmal arm posturing, mimicking epilepsy. Surgical intervention for a syrinx resolved the dystonic episodes, underscoring the importance of considering spinal cord issues.
Area of Science:
- Neurology
- Neurosurgery
- Pediatrics
Background:
- Epileptic seizures are common in children, but paroxysmal arm posturing can be misdiagnosed.
- Chiari I malformation is a structural defect in the cerebellum, often associated with syringomyelia.
Observation:
- A 9-year-old girl experienced refractory paroxysmal bilateral arm posturing, initially presumed to be epileptic seizures.
- Normal electroencephalograms (EEG) and brain MRI showing Chiari I malformation were noted.
- Spinal MRI revealed a large cervical syrinx associated with the Chiari I malformation.
Findings:
- Video EEG monitoring showed no epileptiform discharges during the arm posturing episodes.
- Surgical intervention for the cervical syrinx led to complete resolution of the paroxysmal bilateral dystonic arm posturing.
- The patient also had staring spells and generalized tonic-clonic convulsions, which were not the focus of the intervention.
Implications:
- Cervical cord disease, specifically syringomyelia secondary to Chiari I malformation, should be considered in the differential diagnosis of pediatric paroxysmal dystonic arm movements.
- This case emphasizes the importance of comprehensive neuroimaging, including spinal MRI, in refractory movement disorders.
- Prompt diagnosis and surgical management of cervical syrinx can lead to significant improvement in neurological symptoms.