improve dystonia, 13 myoclonus, 14 paroxysmal choreoatheto- sis, 15 and levodopa-induced dyskinesia. 16 Our case report sug- gests that LEV could play a role in the treatment of chorea from CP and might be evaluated for chorea from other causes (e.g., Huntington’s disease). An advantage of LEV over agents such as haloperidol is that it is not known to cause tardive dyskine- sia. Further testing of LEV in a case series or a controlled trial of choreiform patients is warranted to further assess its safety and efficacy. Legend to the Video A 35-year-old woman with cerebral palsy developed chorea when she experienced a fever from a nonstreptococcal infec- tion. A: Video before treatment with levetiracetam (Keppra, LEV) shows the patient with severe chorea. B: The patient 48 hours after starting LEV 1,000 mg twice a day. Substantial improvement in chorea is demonstrated. References 1. Paneth N, Stark R. Cerebral palsy and mental retardation in rela- tion to indicators of perinatal asphyxia: an epidemiologic over- view. Am J Obstet Gynecol 1983;147:960 –966. 2. Kerrigan JF, Chugani HT, Phelps ME. Regional cerebral glucose metabolism in clinical subtypes of cerebral palsy. Pediatr Neurol 1991;7:415– 425. 3. Hagberg B, Hagberg G, Olow I. The changing panorama of cere- bral palsy in Sweden 1954 –1970. II. Analysis of the various syndromes. Acta Paediatr Scand 1975;64:193–200. 4. Erickson GR, Chun RW. Acquired paroxysmal movement disor- ders. Pediatr Neurol 1987;3:226 –229. 5. Rosen JA. Paroxysmal choreoathetosis. Associated with perinatal hypoxic encephalopathy. Arch Neurol 1964;11:385–387. 6. Harbord MG, Kobayashi JS. Fever producing ballismus in patients with choreoathetosis. J Child Neurol 1991;6:49 –52. 7. PeBenito R, Talamayan RC. Fever-induced protracted ballismus in choreoathetoic cerebral palsy. Clin Pediatr (Phila) 2001;40:49 –51. 8. Guy W. ECDEU assessment manual for psychopharmacology. Washington, DC: Public Health Service, US Department of Health, Education and Welfare; 1976. 9. Anand KS, Thakur LC. Cryptogenic chorea. J Trop Med Hyg 1991;94:393–394. 10. Cereghino JJ, Biton V, Abou-Khalil B, et al. Levetiracetam for partial seizures: results of a double-blind, randomized clinical trial. Neurology 2000;55:236 –242. 11. Shorvon SD, Lowenthal A, Janz D, et al. Multi-center double- blind, randomized, placebo-controlled trial of levetiracetam as add-on therapy in patients with refractory partial seizures. Euro- pean Levetiracetam Study Group. Epilepsia 2000;41:1179 –1186. 12. Ikeda A, Shibasaki H, Tashiro K, et al. Clinical trial of piracetam in patients with myoclonus: nationwide multi-institution study in Japan. Mov Disord 1996;11:691–700. 13. Deleu D. Levetiracetam in the treatment of idiopathic hemifacial spasm. Neurology 2004;8:2134 –2135. 14. Frucht SJ, Louis ED, Chuang C, Fahn S. A pilot tolerability and efficacy study of levetiracetam in patients with chronic myoclonus. Neurology 2001;57:1112–1114. 15. Piperidou C, Maltezos E, Kafalis G, et al. Piracetam for choreo- athetosis. Lancet 1988;2:906. 16. Zesiewicz TA, Sullivan KL, Maldonado JA, Tatum WO, Hauser RA. Levetiracetam (Keppra) in the treatment of levodopa-induced dyskinesia in Parkinson’s disease (P6.138). Neurology 2004; 62(Suppl. 5):A510. Levodopa-Responsive Infantile Parkinsonism Due to a Novel Mutation in the Tyrosine Hydroxylase Gene and Exacerbation by Viral Infections Katharina Diepold, MD, 1 Barbara Schu¨tz, MD, 2 Kevin Rostasy, MD, 1 * Bernd Wilken, MD, 3 Pia Hougaard, 4 Flemming Gu¨ttler, MD, PhD, 4 Anne Romstad, PhD, 4 and Lisbeth Birk Møller, PhD 4 1 Department of Pediatrics, Division of Neuropediatrics, University of Go¨ttingen, Go¨ttingen, Germany 2 Department of Pediatrics, Division of Neuropediatrics, Division Neurology, Charite´ Virchow-Klinikum, Berlin, Germany 3 Department of Neuropediatrics, Klinikum Kassel, Kassel, Germany 4 The John F. Kennedy Institute, Glostrup, Denmark Abstract: Autosomal recessive forms of infantile dystonia due to mutations in the tyrosine hydroxylase (TH) gene have been described recently. The main clinical manifestations are Sega- wa’s disease, or infantile hypokinetic rigid Parkinsonism. Here, we report on a patient with hyperrigidity, psychomotor devel- opmental delay, and dystonic posturing of the hands, symptoms that appeared after a viral infection at the age of 14 months. Low homovanillic acid/5-hydroxyindolacetic acid (HVA/ 5HIAA) ratio in cerebrospinal fluid suggested a TH deficiency. Molecular analysis revealed a novel (H246Y) and a known (D498G) compound heterozygote mutation in the TH gene. The patient showed a remarkable response to treatment with levodopa. The new mutation and the association of viral infections with the onset and worsening of symptoms are discussed. © 2005 Movement Disorder Society Key words: autosomal recessive dystonia; infantile dysto- nia; tyrosine hydroxylase; levodopa; viral infection In recent years, the genetic basis of several forms of primary dystonias have been elucidated. They may occur as autosomal dominant, autosomal recessive or as X-linked recessive forms, and are inherited as monogenic traits. 1 Mutations in the respon- sible genes, lead to biochemical defects in the neurotransmitter synthesis of the basal ganglia, whereas neuroanatomical struc- tures are usually preserved. 1 Dopa-responsive dystonia with childhood-onset dystonia and a dramatic response to levodopa is due to at least two different genetic defects. The most common form is caused by mutations in the GTP-cyclohydrolase I gene, the first enzyme in the biosynthe- sis of tetrahydrobiopterin (BH4) from GTP. This form is inherited *Correspondence to: Dr. Kevin Rostasy, Department of Pediatrics, Division of Neuropediatrics, University of Go¨ttingen, 37099 Go¨ttin- gen, Germany. E-mail: krostasy@excite.com Received 24 July 2004; Revised 6 November 2004; Accepted 10 November 2004 Published online 3 March 2005 in Wiley InterScience (www. interscience.wiley.com). DOI: 10.1002/mds.20416 764 CLINICAL/SCIENTIFIC NOTES Movement Disorders, Vol. 20, No. 6, 2005