Progressive multifocal leukoencephalopathy in a child with acquired immunodeficiency syndrome (AIDS)

Araujo, Alexandra Prufer de Q.C.; Pereira, Heloisa S.; Oliveira, Ricardo H.; Frota, Ana C.; Esperança, José C.; Duarte, Francisco

doi:10.1590/S0004-282X1997000100019

Abstracts

Progressive multifocal leukoencephalopathy is a rare viral-induced demyelinating disease associated to immunodeficiency. A 10-year-old boy with AIDS is reported, who developed subacute cerebellar signs and symptoms with multiple cranial nerve involvement and dementia. A computed tomography scan revealed a focal nonenhancing area of low attenuation in the cerebellum. On magnetic resonance imaging high signal lesions in T2 weighted sequences were shown. The biopsy of one of those lesions showed the typical histological findings of progressive multifocal leukoencephalopathy. It seems important to consider this diagnosis in children with AIDS who present with progressive neurological features.

progressive multifocal leukoencephalopathy; acquired immunodeficiency syndrome; children; diagnosis

A leucoencefalopatia multifocal progressiva é uma doença desmielinizante rara, induzida por vírus em indivíduos imunodeficientes. Relata-se o caso de um menino de 10 anos de idade com síndrome da imunodeficiência adquirida (SIDA), que desenvolveu quadro subagudo de sinais e sintomas cerebelares com acometimento de nervos cranianos e demência. A tomografia computadorizada de crânio mostrou uma área de hipodensidade não captante no cerebelo. A ressonância magnética evidenciou áreas de hipersinal na seqüência em T2. A biópsia de uma destas lesões revelou os achados histológicos típicos da leucoencefalopatia multifocal progressiva. Esta hipótese, portanto, deve ser considerada em crianças com SIDA que apresentem deterioração neurológica progressiva.

leucoencefalopatia multifocal progressiva; síndrome da imunodeficiência adquirida; criança; diagnóstico

Progressive multifocal leukoencephalopathy in a child with acquired immunodeficiency syndrome (AIDS)

Leucoencefalopatia multifocal progressiva em uma criança com síndrome da imunodeficiênda adquirida

Alexandra Prufer de Q.C. Araujo^I; Heloisa S. Pereira^II; Ricardo H. Oliveira^III; Ana C. Frota^III; José C. Esperança^I; Francisco Duarte^IV

^IChild Neurology and Infectious Diseases Units of the Department of Pediatrics, and Department of Pathology, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil: M.D., M.Sc, Assistant Professor

^IIChild Neurology and Infectious Diseases Units of the Department of Pediatrics, and Department of Pathology, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil: M.D., Postgraduate in Neuropediatrics

^IIIChild Neurology and Infectious Diseases Units of the Department of Pediatrics, and Department of Pathology, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil: M.D., Pediatrician

^IVChild Neurology and Infectious Diseases Units of the Department of Pediatrics, and Department of Pathology, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil: M.D., Professor

ABSTRACT

Progressive multifocal leukoencephalopathy is a rare viral-induced demyelinating disease associated to immunodeficiency. A 10-year-old boy with AIDS is reported, who developed subacute cerebellar signs and symptoms with multiple cranial nerve involvement and dementia. A computed tomography scan revealed a focal nonenhancing area of low attenuation in the cerebellum. On magnetic resonance imaging high signal lesions in T2 weighted sequences were shown. The biopsy of one of those lesions showed the typical histological findings of progressive multifocal leukoencephalopathy. It seems important to consider this diagnosis in children with AIDS who present with progressive neurological features.

Key words: progressive multifocal leukoencephalopathy, acquired immunodeficiency syndrome, children, diagnosis.

RESUMO

A leucoencefalopatia multifocal progressiva é uma doença desmielinizante rara, induzida por vírus em indivíduos imunodeficientes. Relata-se o caso de um menino de 10 anos de idade com síndrome da imunodeficiência adquirida (SIDA), que desenvolveu quadro subagudo de sinais e sintomas cerebelares com acometimento de nervos cranianos e demência. A tomografia computadorizada de crânio mostrou uma área de hipodensidade não captante no cerebelo. A ressonância magnética evidenciou áreas de hipersinal na seqüência em T2. A biópsia de uma destas lesões revelou os achados histológicos típicos da leucoencefalopatia multifocal progressiva. Esta hipótese, portanto, deve ser considerada em crianças com SIDA que apresentem deterioração neurológica progressiva.

Palavras-chave: leucoencefalopatia multifocal progressiva, síndrome da imunodeficiência adquirida, criança, diagnóstico.

Full text available only in PDF format.

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Aceite: 24-outubro-1996.

Dra. Alexandra Prufer Araujo - Rua Gildásio Amado 55/211 - 22631-020 Rio de Janeiro RJ - Brasil.

1. Araujo APQC. Neurologic findings in Brazilian children with human immunodeficiency virus infection. Pediatric Aids 1994;5:33-34.
2. Araujo AQC, Araujo APQC, Novis SAP. A neuropatogenia do vírus da imunodeficiência humana. Arq Neuropsiquiatr 1996;54:335-345.
3. Behnan AL, Diamond G, Dickson D, Houroupian D, Llena J, Lantos G, Rubinstein A. Pediatric acquired immunodeficiency syndrome: neurologic syndromes. Am J Dis Child 1988;142:29-35.
4. Berger JR, Coocha M. Progressive multifocal leukoencephalopathy: the evolution of a disease once considered rare. J Neurovirol 1995;1:5-18.
5. Berger JR, Kaszovitz B, Post MJD, Dickinson G. Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection: a review of the literature with a report of sixteen cases. Ann Intern Med 1987;107:78-87.
6. Berger JR, Mucke L. Prolonged survival and partial recovery in AIDS-associated progressive multifocal leukoencephalopathy. Neurology 1988;38:1060-1066.
7. Berger JR, Scott G, Albrecht J, Behnan AL, Tornetore C, Major EO. Progressive multifocal leukoencephalopathy in HIV-infected children. AIDS 1992;6:837-841.
8. Brooks BR, Walker DL. Progressive multifocal leukoencephalopathy. Neurol Clin 1984;2:299-313.
9. Butel J. Papova viruses. In Kendall RR, Stroop WG. Handbook of neurovirology. New York: Marcel Decker, 1994:339-354.
10. Chimelli L, Rosemberg S, Hahn MD, Lopes MB. Pathology of the central nervous system in patients infected with the human immunodeficiency virus (HIV): a report of 252 autopsy cases from Brazil. Neuropathol Appl Neurobiol 1992;5:478-488.
11. Epstein LG, Sharer LR, Oleske JM, Connor EC, Goudsmit J, Bagdon L, Robert-Guroff M, Koenigsberger R. Neurologic manifestations of human immunodeficiency virus infection in children. Pediatrics1986;78:678-687.
12. Greenle JE. Papovavirus diseases. In Kendall RR, Stroop WG. Handbook of neurovirology. New York: Marcel Decker, 1994:355-367.
13. Henson J, Rosemblum M, Armstrong D, Furneaux H. Amplification of JC virus DNA from brain and cerebrospinal fluid of patients with progressive multifocal leukoencephalopathy. Neurology 1991 ;41:1967-1971.
14. Jakobsen J, Diemer NH, Gaub J, Brun B, Helweg-Larsen S. Progressive multifocal leukoencephalopathy in a patient without other clinical manifestations of AIDS. Acta Neurol Scand 1987;75:209-213.
15. Katz DA, Berger JR, Hamilton B, Major EO, Post MJD. Progressive multifocal leukoencephalopathy complicating Wiskott-Aldrich syndrome. Arch Neurol 1994;51:422-426.
16. Knight RS, Hyman NM, Gardner SD, Gibson PE, Esiri MM, Warlow CP. Progressive multifocal leukoencephalopathy and viral antibody litres. J Neurol 1988;235:458-461.
17. Krasinski K, Borkowsky W, Holzman RS. Prognosis of human immunodeficiency virus infection in children and adolescents. Pediatr Infect Dis J 1989;8:216-220.
18. Levy JD, Cottingham KL, Campbell RJ, Moore GK, Gyorkey F, Ashizawa T, Goldman AM. Progressive multifocal leukoencephalopathy and magnetic resonance imaging. Ann Neurol 1986;19:399-401.
19. Major EO, Ault GS. Progressive multifocal leukoencephalopathy: clinical and laboratory observations on a viral induced demyelinating disease in the immunodeficient patient. Curr Op Neurol 1995;8:184-190.
20. Mark AS, Atlas SW. Progressive multifocal leukoencephalopathy in patients with AIDS: appearance on MR images. Radiology 1989;173:517-520.
21. Suhrland MJ, Koslow M, Perchick A, Weiner E, Greco MA, Colquhoun F, Muller WD, Burstein DE. Cytologic findings in progressive multifocal leukoencephalopathy: report of two cases. Acta Cytologica 1987;31:505-511.
22. Stoner GL, Walker DL, Webster HE Age distribution of progressive multifocal leukoencephalopathy. Acta Neurol Scand 1988;78:307-311
23. Taguchi F, Kajioka J, MiyamuraT. Prevelance rate and age of acquisition of antibodies against JC virus and BK virus in human sera. Microbiol Immunol 1982;26:1057-1064.
24. Vandersteenhoven JJ, Ddaibo G, Boyko OB, Hulette CM, Anthony DC, Kenny JF, Wilfert CM. Progressive multifocal leukoencephalopathy in pediatric acquired immunodeficiency syndrome. Pediatr Infect Dis J 1992;11:232-237.
25. Weber T, Turner RW, Frye S, Ruf B, Haas J, Schielke E, Pohle HD, Luke W, Luer W, Felgenhauer K. Specific diagnosis of progressive multifocal leukoencephalopathy by polimerase chain reaction. J Infect Dis 1994;169:1138-1141.
26. Zurhein GM, Padgett BL, Walker DL, Chun RWM, Chorowitz SD, Hong R. Progressive multifocal leukoencephalopathy in a child with severe combined immunodeficiency. N Engl J Med 1978;299:256-257.

Publication Dates

Publication in this collection
10 Nov 2010
Date of issue
1997

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

[1] 1. Araujo APQC. Neurologic findings in Brazilian children with human immunodeficiency virus infection. Pediatric Aids 1994;5:33-34.

[2] 2. Araujo AQC, Araujo APQC, Novis SAP. A neuropatogenia do vírus da imunodeficiência humana. Arq Neuropsiquiatr 1996;54:335-345.

[3] 3. Behnan AL, Diamond G, Dickson D, Houroupian D, Llena J, Lantos G, Rubinstein A. Pediatric acquired immunodeficiency syndrome: neurologic syndromes. Am J Dis Child 1988;142:29-35.

[4] 4. Berger JR, Coocha M. Progressive multifocal leukoencephalopathy: the evolution of a disease once considered rare. J Neurovirol 1995;1:5-18.

[5] 5. Berger JR, Kaszovitz B, Post MJD, Dickinson G. Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection: a review of the literature with a report of sixteen cases. Ann Intern Med 1987;107:78-87.

[6] 6. Berger JR, Mucke L. Prolonged survival and partial recovery in AIDS-associated progressive multifocal leukoencephalopathy. Neurology 1988;38:1060-1066.

[7] 7. Berger JR, Scott G, Albrecht J, Behnan AL, Tornetore C, Major EO. Progressive multifocal leukoencephalopathy in HIV-infected children. AIDS 1992;6:837-841.

[8] 8. Brooks BR, Walker DL. Progressive multifocal leukoencephalopathy. Neurol Clin 1984;2:299-313.

[9] 9. Butel J. Papova viruses. In Kendall RR, Stroop WG. Handbook of neurovirology. New York: Marcel Decker, 1994:339-354.

[10] 10. Chimelli L, Rosemberg S, Hahn MD, Lopes MB. Pathology of the central nervous system in patients infected with the human immunodeficiency virus (HIV): a report of 252 autopsy cases from Brazil. Neuropathol Appl Neurobiol 1992;5:478-488.

[11] 11. Epstein LG, Sharer LR, Oleske JM, Connor EC, Goudsmit J, Bagdon L, Robert-Guroff M, Koenigsberger R. Neurologic manifestations of human immunodeficiency virus infection in children. Pediatrics1986;78:678-687.

[12] 12. Greenle JE. Papovavirus diseases. In Kendall RR, Stroop WG. Handbook of neurovirology. New York: Marcel Decker, 1994:355-367.

[13] 13. Henson J, Rosemblum M, Armstrong D, Furneaux H. Amplification of JC virus DNA from brain and cerebrospinal fluid of patients with progressive multifocal leukoencephalopathy. Neurology 1991 ;41:1967-1971.

[14] 14. Jakobsen J, Diemer NH, Gaub J, Brun B, Helweg-Larsen S. Progressive multifocal leukoencephalopathy in a patient without other clinical manifestations of AIDS. Acta Neurol Scand 1987;75:209-213.

[15] 15. Katz DA, Berger JR, Hamilton B, Major EO, Post MJD. Progressive multifocal leukoencephalopathy complicating Wiskott-Aldrich syndrome. Arch Neurol 1994;51:422-426.

[16] 16. Knight RS, Hyman NM, Gardner SD, Gibson PE, Esiri MM, Warlow CP. Progressive multifocal leukoencephalopathy and viral antibody litres. J Neurol 1988;235:458-461.

[17] 17. Krasinski K, Borkowsky W, Holzman RS. Prognosis of human immunodeficiency virus infection in children and adolescents. Pediatr Infect Dis J 1989;8:216-220.

[18] 18. Levy JD, Cottingham KL, Campbell RJ, Moore GK, Gyorkey F, Ashizawa T, Goldman AM. Progressive multifocal leukoencephalopathy and magnetic resonance imaging. Ann Neurol 1986;19:399-401.

[19] 19. Major EO, Ault GS. Progressive multifocal leukoencephalopathy: clinical and laboratory observations on a viral induced demyelinating disease in the immunodeficient patient. Curr Op Neurol 1995;8:184-190.

[20] 20. Mark AS, Atlas SW. Progressive multifocal leukoencephalopathy in patients with AIDS: appearance on MR images. Radiology 1989;173:517-520.

[21] 21. Suhrland MJ, Koslow M, Perchick A, Weiner E, Greco MA, Colquhoun F, Muller WD, Burstein DE. Cytologic findings in progressive multifocal leukoencephalopathy: report of two cases. Acta Cytologica 1987;31:505-511.

[22] 22. Stoner GL, Walker DL, Webster HE Age distribution of progressive multifocal leukoencephalopathy. Acta Neurol Scand 1988;78:307-311

[23] 23. Taguchi F, Kajioka J, MiyamuraT. Prevelance rate and age of acquisition of antibodies against JC virus and BK virus in human sera. Microbiol Immunol 1982;26:1057-1064.

[24] 24. Vandersteenhoven JJ, Ddaibo G, Boyko OB, Hulette CM, Anthony DC, Kenny JF, Wilfert CM. Progressive multifocal leukoencephalopathy in pediatric acquired immunodeficiency syndrome. Pediatr Infect Dis J 1992;11:232-237.

[25] 25. Weber T, Turner RW, Frye S, Ruf B, Haas J, Schielke E, Pohle HD, Luke W, Luer W, Felgenhauer K. Specific diagnosis of progressive multifocal leukoencephalopathy by polimerase chain reaction. J Infect Dis 1994;169:1138-1141.

[26] 26. Zurhein GM, Padgett BL, Walker DL, Chun RWM, Chorowitz SD, Hong R. Progressive multifocal leukoencephalopathy in a child with severe combined immunodeficiency. N Engl J Med 1978;299:256-257.