Multiple sclerosis with early childhood onset: a case report

Vergani, Maria Isabel Campos; Reimão, Rubens; Silva, Ana Maria Camara; Muskat, Mauro; Espósito, Sandro; Diament, Aron

doi:10.1590/S0004-282X1988000200012

Abstracts

A 2 year old boy was admitted owing to a subacute episode of ataxic gait and hearing deficit. Computerized tomography (CT) was normal and cerebrospinal fluid (CSF) analysis revealed gamma globul.ns level of 15.4% (normal 7 to 14%). There was spontaneous remission after 7 months. At 5 years of age the boy incurred a second episode with predominantly right apendicular ataxia and tonic gaze deviation to the right side. CT showed a low-density lesion in the white matter adjacent to the right frontal horn. Visual and auditory evoked potentials were abnormal. CSF revealed a mild increase in gamma globulins level of 14.5% with an abnormal T lymphocyte subsets study. The combination of visual, cerebellar, brain stem and paraventricular lesions with clear remissions and exacerbations, supported by CT, CSF and evoked potentials findings suggests the diagnosis of multiple sclerosis even at this early age.

Os autores relatam o caso de um menino com provável esclerose múltipla, de início aos 2 anos de idade, com um segundo surto aos 5 anos. O acometimento foi predominantemente de tronco cerebral, cerebelo, nervo óptico e paraventricular. Tomografia computadorizada, exame do líquido cefalorraqueano com eletroforese de proteínas e estudo de subpopulações linfocitárias T, bem como a avaliação dos potenciais evocados visuais, auditivos e sômato-sensitivos sugeriram o diagnóstico.

Maria Isabel Campos Vergani^I; Rubens Reimão^II; Ana Maria Camara Silva^I; Mauro Muskat^I; Sandro Espósito^I; Aron Diament^III

^IResident - Division of Child Neurology, Department of Neurology, University of São Paulo School of Medicine

^IIAssistant Professor - Division of Child Neurology, Department of Neurology, University of São Paulo School of Medicine

^IIIHead, Division of Child Neurology - Division of Child Neurology, Department of Neurology, University of São Paulo School of Medicine

SUMMARY

A 2 year old boy was admitted owing to a subacute episode of ataxic gait and hearing deficit. Computerized tomography (CT) was normal and cerebrospinal fluid (CSF) analysis revealed gamma globul.ns level of 15.4% (normal 7 to 14%). There was spontaneous remission after 7 months. At 5 years of age the boy incurred a second episode with predominantly right apendicular ataxia and tonic gaze deviation to the right side. CT showed a low-density lesion in the white matter adjacent to the right frontal horn. Visual and auditory evoked potentials were abnormal. CSF revealed a mild increase in gamma globulins level of 14.5% with an abnormal T lymphocyte subsets study. The combination of visual, cerebellar, brain stem and paraventricular lesions with clear remissions and exacerbations, supported by CT, CSF and evoked potentials findings suggests the diagnosis of multiple sclerosis even at this early age.

RESUMO

Os autores relatam o caso de um menino com provável esclerose múltipla, de início aos 2 anos de idade, com um segundo surto aos 5 anos. O acometimento foi predominantemente de tronco cerebral, cerebelo, nervo óptico e paraventricular. Tomografia computadorizada, exame do líquido cefalorraqueano com eletroforese de proteínas e estudo de subpopulações linfocitárias T, bem como a avaliação dos potenciais evocados visuais, auditivos e sômato-sensitivos sugeriram o diagnóstico.

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Acknowledgement - The authors wish to thank A. Spina-França, M.D., J.A. Livramento, M.D. and L.R. Machado, M.D., for kindly providing the CSF studies.

Departamento de Neurologia, Faculdade de Medicina da Universidade de São Paulo - Caixa Postal 3461 - 01051 São Paulo SP - Brasil.

1. Bejar JM, Ziegler DK - Onset of multiple sclerosis in a 24-month-old child. Arch Neurol 41: 881, 1984.
2. Brandt S, Gyldensted C, Offner H, Melchior JC - Multiple sclerosis with onset in a two-year-old boy. Neuropediatrics 12: 75, 1981.
3. Buggenhout E, Ketelaer P, Carton H - Success and failure of evoked potentials in detecting clinical and subclinical lesions in multiple sclerosis patients. Clin Neurol Neurosurg 84: 3, 1982.
4. Bye AME, Kendall B, Wilson J - Multiple sclerosis in childhood: a new look. Develop Med Child Neurol 27: 215, 1985.
5. Duquette P, Murray TJ, Pleines J, Ebers GC, Sandwick D, Weldon P, Warren S, Paty DW, Upton A, Hader W, Nelson R, Auty A, Neufeld B, Meltzer C - Multiple sclerosis in childhood: clinical profile in 125 patients. J Pediatr 111: 359, 1987.
6. Gall JC, Hayes AB, Siekert RG, Keith HM - Multiple sclerosis in children: a clinical study of 40 cases with onset in childhood. Pediatrics 21: 703, 1958.
7. Ghezzi A, Manara F - Multiple sclerosis in childhood. Acta Neurol (Napoli) 33: 157, 1978.
8. Hauser SL, Bresnan MJ - Childhood multiple sclerosis: clinical features and demonstration of changes in T cells subsets with disease activity. Ann Neurol 11: 463, 1982.
9. Kelly R - Clinical aspects of multiple sclerosis. In Koestier JC (ed): Handbook of Clinical Neurology. Elsevier, Amsterdam, 1985, vol 47.
10. Livramento JA, Machado LR, Spina-França A - Sinalização do líqüido cefalorraqueano em doenças inflamatórias crônicas do sistema nervoso central. Arq Neuro-Psiquiat (São Paulo) 44: 351, 1986.
11. Low NL, Carter S - Multiple sclerosis in children. Pediatrics 18: 24, 1956.
12. Machado LR - Subpopulações linfocitárias do liqüido cefalorraqueano: II. Técnica. Arq Neuro-Psiquiat (São Paulo) 41: 132, 1983.
13. Machado LR - Subpopulações linfocitárias no líqüido cefalorraqueano: III. Valores normais. Arq Neuro-Psiquiat (São Paulo) 41: 137, 1983.
14. Poser CM - Diseases of the myelin sheat. In Baker AB, Baker LH (eds): Clinical Neurology. Harper & Row, Philadelphia, 1982, vol 2, pg 21.
15. Rose A, Ellison G, Miyers L, Tourtellotte W - Criteria for the clinical diagnosis of multiple sclerosis. Neurology 26: 20, 1976.
16. Schumacher G, Beebe G, Kibler R, Kurland L, Kurtzke J, McDowell F, Nagler B, Sibley W, Tourtellotte W, Willmon R - Problems of experimental trials of therapy in multiple sclerosis. Ann NY Acad Sci 122: 552, 1965.
17. Sheramata W, Brown SB, Curless RR - Childhood multiple sclerosis: a report of 12 cases. Arm Neurol 10: 304, 1981.

Multiple sclerosis with early childhood onset: a case report

Esclerose múltipla na infância: relato de um caso

Publication Dates

Publication in this collection
21 June 2011
Date of issue
June 1988

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Bejar JM, Ziegler DK - Onset of multiple sclerosis in a 24-month-old child. Arch Neurol 41: 881, 1984.

[2] 2. Brandt S, Gyldensted C, Offner H, Melchior JC - Multiple sclerosis with onset in a two-year-old boy. Neuropediatrics 12: 75, 1981.

[3] 3. Buggenhout E, Ketelaer P, Carton H - Success and failure of evoked potentials in detecting clinical and subclinical lesions in multiple sclerosis patients. Clin Neurol Neurosurg 84: 3, 1982.

[4] 4. Bye AME, Kendall B, Wilson J - Multiple sclerosis in childhood: a new look. Develop Med Child Neurol 27: 215, 1985.

[5] 5. Duquette P, Murray TJ, Pleines J, Ebers GC, Sandwick D, Weldon P, Warren S, Paty DW, Upton A, Hader W, Nelson R, Auty A, Neufeld B, Meltzer C - Multiple sclerosis in childhood: clinical profile in 125 patients. J Pediatr 111: 359, 1987.

[6] 6. Gall JC, Hayes AB, Siekert RG, Keith HM - Multiple sclerosis in children: a clinical study of 40 cases with onset in childhood. Pediatrics 21: 703, 1958.

[7] 7. Ghezzi A, Manara F - Multiple sclerosis in childhood. Acta Neurol (Napoli) 33: 157, 1978.

[8] 8. Hauser SL, Bresnan MJ - Childhood multiple sclerosis: clinical features and demonstration of changes in T cells subsets with disease activity. Ann Neurol 11: 463, 1982.

[9] 9. Kelly R - Clinical aspects of multiple sclerosis. In Koestier JC (ed): Handbook of Clinical Neurology. Elsevier, Amsterdam, 1985, vol 47.

[10] 10. Livramento JA, Machado LR, Spina-França A - Sinalização do líqüido cefalorraqueano em doenças inflamatórias crônicas do sistema nervoso central. Arq Neuro-Psiquiat (São Paulo) 44: 351, 1986.

[11] 11. Low NL, Carter S - Multiple sclerosis in children. Pediatrics 18: 24, 1956.

[12] 12. Machado LR - Subpopulações linfocitárias do liqüido cefalorraqueano: II. Técnica. Arq Neuro-Psiquiat (São Paulo) 41: 132, 1983.

[13] 13. Machado LR - Subpopulações linfocitárias no líqüido cefalorraqueano: III. Valores normais. Arq Neuro-Psiquiat (São Paulo) 41: 137, 1983.

[14] 14. Poser CM - Diseases of the myelin sheat. In Baker AB, Baker LH (eds): Clinical Neurology. Harper & Row, Philadelphia, 1982, vol 2, pg 21.

[15] 15. Rose A, Ellison G, Miyers L, Tourtellotte W - Criteria for the clinical diagnosis of multiple sclerosis. Neurology 26: 20, 1976.

[16] 16. Schumacher G, Beebe G, Kibler R, Kurland L, Kurtzke J, McDowell F, Nagler B, Sibley W, Tourtellotte W, Willmon R - Problems of experimental trials of therapy in multiple sclerosis. Ann NY Acad Sci 122: 552, 1965.

[17] 17. Sheramata W, Brown SB, Curless RR - Childhood multiple sclerosis: a report of 12 cases. Arm Neurol 10: 304, 1981.