Cervical myelopathy in an adolescent with hallervorden-spatz disease☆

Abstract 

Hallervorden-Spatz disease is a rare neurodegenerative disorder characterized by progressive dystonia, rigidity, and dementia. In these patients, chronic repeated dystonic movements, especially of the head and neck, can lead to excessive stress on the cervical spine, resulting in early degenerative changes and myelopathy. This report focuses on a young patient with Hallervorden-Spatz disease who presented with C₄ to C₅ cervical disk extrusion and cord compression because of premature spondylotic changes of the cervical spine. Other authors have documented cervical spondylosis caused by movement disorders, but there had been no reported cases of cervical myelopathy as a complication of Hallervorden-Spatz disease. Because these patients already manifest a longstanding and progressive neurologic disorder, clinicians may encounter difficulties recognizing the symptoms and signs of new cervical pathology, especially if the spondylosis and myelopathy has an insidious onset. For follow-up of patients with Hallervorden-Spatz disease who have multiple disabilities, a multidisciplinary approach with active involvement of physiotherapists, occupational and speech therapists, experienced nurses, caregivers, and clinicians is recommended. The clinician should also monitor the neurologic and functional status of the patient and screen for cervical pathology if suspicion arises. Good control of dystonia may be helpful in prevention of cervical spondylosis but may be difficult to achieve. Treatment modalities for dystonia are also discussed. Fung GPG, Chan KY. Cervical myelopathy in an adolescent with Hallervorden-Spatz disease.