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Possível parentesco entre as moléstias familiares com lesões predominantes na medula e moléstias familiares com lesões no neurônio periférico

After the authors had described on lines carefully written, the type of Charcot-Marie-Tooth's progressive and familiar muscular atrophy, they point out its similarity with that of the type of Dejerine-Sottas's and Werdnig-Hoffmann's muscular atrophies. They recall the attention to remember the old tendency of uniting the classic familiar diseases in only one group, because its transition ways between one another are enough frequent. About to this subject they remember that Austregésilo defends this theory with plenty documentation from 1918, looking to unite them together, and he names them Great Abiotrophic Family, which central group is Friedreich's disease. The contribution presented in this subject is the observation of theree brothers members of a family whose ancestors suffered from progressive paraplegia, with the typic neurologic symptoms that of Charcot-Marie-Tooth's disease, added with a bilateral pyramidal syndrome (with exalted deep reflexes, rotula and foot clonus and Babinski's signs) in the second brother, and exalted patellar reflexes in the third one. This association of a degenerative alteration in the central nervous system, in one familiar degenerative disease from the peripheric neuron, is one more favorable argument to add to the nosologic unity of such systematized familiar diseases. If we must approach at clinic views about a superposition of Char-cot-Marie's and Strumpell-Lorrain's diseases or Charcot-Marie's and Friedreich's diseases, it will be a purely speculative question that it will not change the basic idea that they try to defend on this work.


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