Cervical myelopathy in athetoid and dystonic cerebral palsy: retrospective study and literature review

The early onset of degenerative cervical lesions has been well described in patients suffering from athetoid or dystonic cerebral palsy. Myelopathy can occur and aggravate of their unstable neurological status. Diagnosis and treatment are delayed and disrupted by the abnormal movements. This retrospective study was implemented to evaluate the symptoms, the anatomical findings, and the surgical management of seven patients from 20 to 56 years old suffering from cervical myelopathy and athetoid or dystonic cerebral palsy. The mean delay in diagnosis was 15 months and the mean follow-up was 33 months. The initial symptoms were spasticity, limbs weakness, paresthesias and vesico-sphinteric dysfunction. In addition to abnormal movements, imaging demonstrated disc herniation, spinal stenosis and instability. All patients were managed surgically by performing simultaneous spinal cord decompression and fusion. Two patients benefited from preoperative botulinum toxin injections, which facilitated postoperative care and immobilization. Strict postoperative immobilization was achieved for 3 months by a Philadelphia collar or a cervico-thoracic orthosis. All patients improved functionally with a mean Japanese Orthopaedic Association score gain of 1.5 points, in spite of the permanent disabilities of the myelopathy. Complications occurred with wound infection, metal failure and relapse of cervical myelopathy at an adjacent level in one case each. All the previous authors advised against isolated laminectomy but no consensus emerged from the literature analysis. Spinal fusion is usually recommended but can be complicated by degenerative adjacent deterioration. Surgical management provides good outcomes but requires a long-term follow-up.