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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.37 no.2 São Paulo June 1979

http://dx.doi.org/10.1590/S0004-282X1979000200004 

Paraplegia espastica familiar com amiotrofia: estudo clinico, eletromiografico, histoquimico e microdissecção

 

Familial spastic paraplegia with amyotrophy: a study of 4 cases with electromyography, muscle histochemistry and teased fiber preparation of sural nerves

 

 

Lineu Cesar WerneckI; Maria Cristina Arrua SanchesII

IProfessor Assistente de Neurologia. Disciplina de Neurologia do Departamento de Clínica Médica, Universidade Federal do Paraná (Curitiba)
IIResidente de Neurologia. Disciplina de Neurologia do Departamento de Clínica Médica, Universidade Federal do Paraná (Curitiba)

 

 


RESUMO

Relato de quatro casos de paraplegia, espástica familiar com amiotrofia, em irmãos provenientes de um matrimônio consanguíneo. A investigação laboratorial rotineira estava normal. A eletromiografia e biópsia muscular com histoquímica demonstraram denervação com reinervação. As conduções nervosas motoras estavam diminuídas em três casos no nervo peroneiro. A análise por microdissecção do nervo sural foi anormal nos quatro casos, revelando desmielinização e remielinização.
Após revisão da literatura, os autores acreditam que as alterações encontradas são decorrentes de degeneração axonal distai, com regeneração secundária e levantam a hipótese de defeito no fluxo axoplásmico do sistema nervoso central e periférico.


SUMMARY

Four cases of familial spastic paraplegia with amyotrophy in siblings from a consanguineous married are reported. The routine laboratory examination were normal. The electromiography and muscle biopsy processed by histochemistry showed signs of denervation with reinervation. The motor nerve conduction velocity was decreased in the peroneal nerve in 3 cases. The teased fiber preparation of sural nerves was abnormal in four cases. It was found increased of C, D and G fibers suggesting demyelination with secondary remyelination.
The authors believe the abnormalities found could be due the distal axonal degeneration, with secondary regeneration and suggest the hypothesis that the fact is an axoplasmic flow defect in the central and peripheral nervous system.


 

 

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Trabalho realizado na Disciplina de Neurologia do Departamento de Clínica Médica, Universidade Federal do Parang (Curitiba).
Disciplina de Neurologia - Hospital de Clinicas da Universidade Federal do Paraná - Rua General Carneiro 180 - 80000 Curitiba - PR. - Brasil.

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