Severe neurological complications associated with Tourette syndrome

Amy L. Patterson MD,  Asim F. Choudhri MD,  Masanori Igarashi MD,  Kathryn McVicar MD, Namrata Shah MD and Robin Morgan MD Severe neurological complications associated with Tourette syndrome. Pediatric Neurology   Article in press.

…Tourette syndrome has associated behavioral and psychiatric comorbidities, including sleep disorders, attention-deficit hyperactivity disorder, obsessive-compulsive behaviors and disorder, and anxiety and mood disorder.  Cheung et al.  described malignant Tourette syndrome as two or more emergency room visits or one or more hospitalization for Tourette syndrome symptoms or associated behavioral comorbidities. Only 5% of patients referred to specialty clinics meet these criteria.

Tourette syndrome and tics may rarely be associated with serious medical complications including cervical myelopathy, cervical disc herniation, stroke and/or dissection, self-mutilation, retinal detachment, and bone fracture.  These complications arise as the result of violent motor tics. We present here the clinical and radiologic features of four adolescents with Tourette syndrome who were treated for life-threatening complications…

His tics consisted of facial grimacing with flaring of his nostrils, mouth stretching, flexion of his arms, and violent head and neck twisting with lateral flexion to the left…

MRI of the brain showed cervical (C2) cord compression due to odontoid nonunion with anterior subluxation. The subluxation was not present on a comparison study three months before. Computed tomography (CT) of the cervical spine revealed a chronic-appearing nonunited odontoid fracture with sclerotic margins with approximately 3.5-mm anterior subluxation with respect to the base of the odontoid process and body of C2…

His tics consisted of biting his cheeks, face twisting, inward sucking movements of the cheeks, and dancing-like movements of his leg and foot…

MRI showed cervical disk herniation with cord compression and cord edema. Congenital narrowing of the anteroposterior diameter of the central canal predisposed him to injury in the setting of this relatively small disc bulge….

Patient 3 is a 14-year-old boy with a four to five year history of motor and vocal tics consisting of eye rolling, sniffing, coughing, arching of his back, neck popping, and swinging his neck from side to side…

MRI of the brain revealed embolic infarction in the right inferior cerebellar hemisphere and the right inferolateral aspect of the cerebellar vermis. Magnetic resonance angiography demonstrated an absence of flow within the V4 segment of the right vertebral artery. Conventional arteriography confirmed right vertebral artery dissection…

His motor tics had increased during the three to four months prior to admission, with aggressive head and neck thrusting and jerking of his body…

CT of the head was found to have a wedge-shaped hypodensity in the medial aspect of the right occipital lobe and smaller more subtle hypodensities in the right cerebellar hemisphere suggesting infarction in the territory of the right posterior cerebral artery. MRI of the brain revealed multiple areas of infarction involving the posterior inferior cerebellar artery territory of the inferior right cerebellar hemisphere, the lateral aspect of the pons, and the parasagittal aspect of the right occipital lobe. Additional punctate areas of infarction were present in both thalami…

These four patients demonstrate that forceful neck tics in patients with Tourette syndrome can be associated with cervical myelopathy and arterial dissection, leading to potentially serious neurological complications including spinal cord injury and stroke. Treatment for tics can include pharmacological therapy, including α 2 agonists, dopamine depleters, and dopamine antagonists; behavior therapy, which can lead to a significant decrease in tic severity; cervical collar, for stabilization and prevention of initial injury and/or progression of existing injury; and treatment with botulinum injection