Session Information
Date: Saturday, November 16, 2019
Session Title: Pediatrics Case Report
Session Time: 11:15am-12:45pm
Location: Research Hub - Kiosk 3
Disclosures: Kendl M. Sankary, MD: Nothing to disclose
Case Description: The patient developed hypotonia and severe upper extremity and cervical dystonic posturing in infancy following a term, uncomplicated birth. He underwent extensive workup as to the cause of his movement disorder including genetic evaluations, metabolic screens, and muscle biopsy all unrevealing of a diagnosis. Artane and baclofen were initiated in an attempt to control his movement disorder, however severe dystonia persisted. At age 7, he developed neck pain, urinary incontinence, and bilateral lower extremity weakness. Imaging revealed os odontoideum and atlantoaxial instability with cord compression and myelomalacia at the C2 level. He underwent spinal decompression and occiput to C2 fusion with occipital plate and laminar screws at C2, and C1 laminectomy.
Setting: Tertiary care children’s hospital.
Patient: A 7-year-old boy with severe choreoathetoid and dystonic movement disorder of unknown etiology who developed cervical myelopathy.
Assessment/Results: Following the spinal decompression and fusion, his lower extremity symptoms improved, and urinary function was intact. His postoperative course was complicated by hardware instability due to dystonia, which was managed with botulinum toxin injection to neck extensors and placement of a halo brace. After 6 months, bony fusion was seen on imaging and he was transitioned to a hard cervical collar.
Discussion: Although cervical myelopathy has been reported in young adults with cervical dystonia, this case was the first example of cervical myelopathy related to movement disorder in a child.
Conclusion: This case demonstrates the challenges in diagnosis of neurological changes in a patient with existing movement disorder, the risk of cervical myelopathy in children, the challenge of maintaining hardware stability in someone with cervical dystonia, and the importance of a multidisciplinary approach to treatment.
Level of Evidence: Level V
To cite this abstract in AMA style:
Sankary KM, Pham KL, Carpenter B. Cervical Dystonia of Unknown Etiology Leading to Cervical Myelopathy in a Child: A Case Report [abstract]. PM R. 2019; 11(S2)(suppl 2). https://pmrjabstracts.org/abstract/cervical-dystonia-of-unknown-etiology-leading-to-cervical-myelopathy-in-a-child-a-case-report/. Accessed September 7, 2024.« Back to AAPM&R Annual Assembly 2019
PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/cervical-dystonia-of-unknown-etiology-leading-to-cervical-myelopathy-in-a-child-a-case-report/