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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.38 no.3 São Paulo Sept. 1980

http://dx.doi.org/10.1590/S0004-282X1980000300009 

Leucodistrofia metacromatica: registro de um caso

 

Metachromatic leucodystrophy: a case report

 

 

Osvaldo J. M. NascimentoI; Marcos R. G. FreitasI; Alexandre A. AlencarII; Bernardo H. N. CoutoIII

IProfessor Auxiliar de Ensino. Instituto de Neurologia Deolindo Couto da U.F.R.J. (Serviço do Professor Bernardo Couto)
IIProfessor Adjunto (Neuropatologista). Instituto de Neurologia Deolindo Couto da U.F.R.J. (Serviço do Professor Bernardo Couto)
IIIProfessor Titular. Instituto de Neurologia Deolindo Couto da U.F.R.J. (Serviço do Professor Bernardo Couto)

 

 


RESUMO

É relatado um caso de leucodistrofia metacromática em paciente com três anos de idade. O exame histopatológico revelou inclusões metacromáticas dentro do citoplasma dos neurônios, o que permite comentar sobre a fisiopatogenia desta doença, grupando-a entre as lipidoses.


SUMMARY

A case of metachromatic leucodystrophy in a three years old female patient is reported. Histopathological examination revealed metachromatic inclusions within the cytoplams of the neurons, which led the authors to comment on the physiopathogenesis of this disease, grouping it in cathegory of the lipidoses.


 

 

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REFERENCIAS

1. AUSTIN, J. H. - Metachromatic form of diffuse cerebral sclerosis, diagnosis during life by urine sediment examination. Neurology (Minneapolis) 7:415, 1957.         [ Links ]

2. AUSTIN, J. H. - Metachromatic form of diffuse cerebral sclerosis: diagnosis during life by isolation of metachromatic lipide from urine. Neurology (Minneapolis) 7:716, 1957.         [ Links ]

3. CANELAS, H. M.; ESCALANTE, O. D.; ISIDYA, K. & De JORGE, F.B. - The diagnosis of metachromatic leucodystrophy during life: metachromatic lipids in saliva ani cerebrospinal sediments and in the parotid glands. Arq. Neuro-Psiquiat. (São Paulo) 22:122, 1964.         [ Links ]

4. ESCALANTE, O. D.; AMARAL, A. D. & CANELAS, H. M. - Metachromatic leucodystrophy: study of the free aminoacids in blood, urine, salive and cerebrospinal fluid. Arq. Neuro-Psiquiat. (São Paulo) 22:195, 1964.         [ Links ]

5. GREENFIELD, J. C. - A form of progressive cerebral sclerosis in infants associated wits primary degeneration of the interfascicular glia. J. Neurol. & Psychiat. 13:289, 1933.         [ Links ]

6. HAGBERG, B. & SUENNERHOLM, L. - Laboratory diagnostic tests in metachromatic leucodystrophy. Acta paediat. (Uppsala) 48:632, 1959.         [ Links ]

7. HAGBERG, B. & SUENNERHLM, L. - Metachromatic leucodystrophy, a generalized lipidosis: determination of sulfatide in urine, blood plasma and cerebrospinal fluid. Act. paediat. (Uppsala) 49:690, 1960.         [ Links ]

8. HAGBERG, B.; SOURANDER, P. & SUENNERHOLM, L. - Sulfatide lipidosis in childhood: report of a case investigated during life and autopsy. Am. J. Dis. Child. 104:644, 1962.         [ Links ]

9. JATZKEMITZ, H. & MEHL, E. - Cerebroside-sulphatase and aryl-sulphatase: a deficiency in metachromatic leucodistrophy. J. Neurochem. 16:19, 1969.         [ Links ]

10. JERVIS, G. A. - Infantile metachromatic leucodystrophy (Greenfield's disease) J. Neuropath. Exp. Neurol. 19:323, 1960.         [ Links ]

 

 

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