CLINICAL/SCIENTIFIC NOTES
Levodopa responsive dystonia secondary to virus encephalitis
Distonia sensível a levedopa secundária a encefalite viral
Rita Lucena; Eduardo Cardoso; Ailton Melo
DINEP - Divisão de Neurologia e Epidemiologia/Universidade Federal da Bahia, Salvador BA, Brazil
Movement disorders secondary to virus encephalitis is an uncommom feature and is mainly observed after the acute or subacute stage of Japanese encephalitis. This condition, described in Asian countries, is characterized by pleomorphic neurologic signs and poor outcome as 30% of patients die and around half of the survivors have severe neurological sequelae1. In Brazil, although some extra-piramidal syndromes such as chorea continues to be an endemic post-infectious disease, dystonia has seldom been related to central nervous system infection2.
In previous paper, our group showed that the neurological sequelae of post infectious meningoencephalitis are extremely pleomorphic, however no case of dystonia was described3.
CASE
The patient, a 13-year-old girl, was born to a healthy Brazilian family. At the age of 7, she started to have persistent headache, vomiting and high fever followed by epileptic crisis and decreasing consciousness level. CSF examination showed lymphomononuclear pleocytosis (85 cels/mm3), mild increase protein (60 mg%) and normal glucosis. She had the diagnosis of viral encephalitis and received acyclovir during ten days. After the beginning of treatment she continued in coma and left conjugated eye deviation for more five days with progressive improvement. In the day of hospital discharge, she had right spastic hemiparesis and motor aphasia. After neurological rehabilitation, she persisted with the motor deficit without any language disturbance. At the age of 11, she changed schools and had to walk for long distances. After approximately one month, she began to present pain in the right muscle triceps and involuntary flexion of foot toes at the same side, which disappeared after rest. At the age of 12, the episodes became more frequently, occurring after 200 meters of walking.
In April 2004, she was seen by the movement disorders group of the Division of Neurology and Epidemiology - Federal University of Bahia and her neurological examination revealed mild spastic hemiparesis and right gastrocnemius atrophy. After walking 100 meters in accelerated steps, she had intense pain associated with dystonia and flexion of right toes that persisted for five minutes after interruption of walking. The T2-weighted MRI showed marked, bilateral gliosis of substance nigra with no other encephalic involvement and motor symptoms disappeared after oral administration of 200 mg plain levodopa with carbidopa. So far, the effects have been sustained without any side effects.
DISCUSSION
We report the first case of levodopa responsive dystonia occurring several years after viral encephalitis. Although a number of movement disorders has been associated with virological diseases such as Coxsackie B, measles, polio, herpesvirus, encephalitis lethargica, western equine encephalitis and Japanese encephalitis4-8, dystonia is an uncommon sequelae of post-viral encephalitis usually, it has been associated with other parkinsonian phenomena9. In cases of Japanese encephalitis, the most common of the above diseases, extrapiramidal symptoms were described in 70% of patients10, but the majority of them had parkinsonian features as the main symptom and even patients with dystonia rigidity, hypokinesia, masked face and tremor were frequent. However, contrary to the cases of Misra and Kalita, which described movement disorders in the acute and subacute stage of JE, in our patient dystonia occurred several years after encephalitis and only under exercise stress resembling that it only appeared after wasting the reserve of nigro-striatal levodopa. However, as it has been sufficiently described, degeneration of dopamine neurons in the substancia nigra is responsible for idiopathic Parkinson's disease, juvenile parkinsonism, hereditary progressive dystonia, and levodopa responsive dystonia, yet the clinical phenotypes in the three disease states are different, which means that they also have different physiopathological process11. Regarding our specific case, some particular features, such as the beginning of symptoms after several years of encephalitis, points to a progressive decline of dopamine, which is pronounced after exercise. As it has been stated elsewhere, processes of nigrostriatal dysfunction vary, and it is very possible that the clinical manifestations based on the striatal dysfunction maintain continuity and that variable clinical phenotype may develop after several years12.
In conclusion, our case illustrates the occurrence of post-encephalitic dystonia in an adolescent with bilateral gliosis of substancia nigra, and draws attention to the excellent improvement after levodopa therapy.
Received 1 February 2008, received in final form 25 November 2008. Accepted 12 February 2009.
Dr. Ailton Melo - Av. Magalhães Neto 735/802 - 41820-020 Salvador BA - Brasil. E-mail: asm@ufba.br
- 1. Solomon T, Dung NM, Kneen R et al. Japanese encephalitis. J Neurol Neurosurg Psychiatry 2000; 68:405-415.
- 2. Ferraz HB, Andrade LA. 3. Gomes I. Symptomatic dystonia: clinical profile of 46 Brazilian patients. Can J Neurol Sci 1992; 19:504-507.
- 3. Gomes I, Melo A, Lucena R, et al. Prognosis of bacterial meningitis in children. Arq Neuropsiquiatr 1996; 54:407-411.
- 4. Peatfield RC. Basal ganglia damage and subcortical dementia after possible Coxsachie virus encephalitis. Acta Neurol Scand 1987; 76:340-345.
- 5. Ward CD. Neuropsychiatric interpretations of postencephalitic movement disorders. Mov Disord 2003; 18:623-630.
- 6. Borghi E, Pagani E, Mancuso R, et al. Detection of herpesvirus-6A in a case of subacute cerebellitis and myoclonic dystonia. J Med Virol 2005; 75:427-429.
- 7. Schultz DR, Barthal JS, Garrett G. Western equine encephalitis with rapid onset of parkinsonism. Neurology 1977; 27:1095-1096.
- 8. Murgod UA, Muthane UB, Ravi V, Radhesh S, Desai A. Persistent movement disorders following Japanese encephalitis. Neurology 2001; 57:2313-2315.
- 9. Pradhan S, Pandey N, Shashank S, Gupta RK, Mathur A. Parkinsonism due to predominant involvement of substantia nigra in Japanese encephalitis. Neurology 1999; 10:1781-1786.
- 10. Misra UK, Kalita J. Prognosis of Japanese encephalitis patients with dystonia compared to those with parkinsonian features only. Postgrad Med J 2002; 78:238-241.
- 11. Segawa M. Hereditary progressive dystonia with marked diurnal fluctuation. Brain Dev 2000; 22(Suppl):S65-S80.
- 12. Yokochi M. Development of the nosological analysis of juvenile parkinsonism. Brain Dev 2000; 22(Suppl):S81-S86.
Publication Dates
-
Publication in this collection
05 June 2009 -
Date of issue
June 2009
