Treatment of Involuntary Movements with Carbamazepine in Dystonic-Choreoathetoid Cerebral Palsy

Abstract

Choreoathetoid movements are quite common in cerebral palsy (CP). This is the first report of a patient with choreoathetoid CP who was successfully treated with carbamazepine. Therefore, clinicians should try carbamazepine for involuntary movements in CP patients before pursuing other procudures.Keywords: cerebral palsy, dyskinetic cerebral palsy, dystonic-choreoathetoid cerebral palsy, carbamazepine, levetiracetam.Cerebral palsy (CP) is a disorder of posture and movement resulting from a static injury to the developing central nervous system occurring in utero, during delivery, or within the first 2 years of life. 1 The Surveillance of Cerebral Palsy in Europe (SCPE) has classified CP into three main groups, one of which is dyskinetic CP (which includes the dystonic and choreoathetotic subtypes). 2 CP remains a leading cause of disability in children affecting 2 to 3 per 1,000 live births, with~10% to 15% of these children having dystonic-choreoathetoid CP typically with little or no cognitive impairment. 1 ,2 Medical treatment of dystonic-choreoathetoid CP remains less than satisfactory, but there are reports of mild improvement with trihexyphenidyl 3 and a report of 2 children who improved with levetiracetam. 4 Many patients are currently undergoing bilateral globus pallidus interna (GPi-DBS) stimulation for symptomatic improvement. 5 We present a patient who responded well to carbamazepine (CBZ), the withdrawal of which resulted in significant worsening of his choreoathetoid movements, and reinstitution of therapy resulted in amelioration of his involuntary movements. Case ReportA 45-year-old man was diagnosed with generalized tonic clonic seizures at age two secondary to CP. His seizures were well controlled on CBZ 150 mg bid and valproate 600 mg bid. Mild choreoathetoid movements were noticed while on CBZ therapy but were not bothersome to the patient or the mother. He was diagnosed with HIV and hepatitis C at age 18 secondary to multiple blood transfusions for hemophilia. CBZ was changed to levetiracetam to avoid hepatic enzyme induction during HAART therapy for HIV. He then developed generalized, but asymmetric, involuntary, almost constant choreoathetoid movements involving upper more than lower extremities (Video, Part 1). With the intention of controlling the movements, levetiracetam was titrated to a total dose of 1250 mg/ day and tried for four and a half months without any improvement. The movements would improve with diazepam or sleep. EEG confirmed the movements were non-epileptic. MRI of the brain showed moderate cortical atrophy but no new lesions. CBZ was restarted and after 2 weeks at a dose of 250 mg bid the patient had complete cessation of the abnormal movements (Video, Part 2). The patient's function improved and he was able to better engage in his own ADLs. This is a first demonstration of effective treatment of dystonicchoreoathetoid CP by CBZ. While Vles and colleagues 4 have reported significant improvement of choreoathetoid movements with levetiracetam in two children with ...