Antall József szerk.: Orvostörténeti közlemények 87-88. (Budapest, 1979)

TANULMÁNYOK - Czeizel, Endre: A heredodegeneraciós tan történeti értékelése (angol nyelven)

the title "Spastic paralysis and hereditary neurological diseases in general". In his opinion "the clinical picture of spastic paralysis described by Charcot and Erb... generally speaking cannot be considered an independent disease, but only a valuable and characteristic group of symptoms, the primary cause of which is looked for in anatomical deformities." Accordingly, spastic spinal paralysis should be divided at least into three groups of disease of different origin, such as (i) symptomatic spastic paralysis (with different manifestations of myelitis in the background), (ii) spastic diplegia observed mainly in premature infants due to some damage acquired at birth and (iii) the "inherited form". Earlier, in 1895, this latter possibility was thought of only by Erb when analysing the cases of some families collected from literature and his own case of two sisters. After referring to the case of the Hungarian brother and sister published by Arthur Schwartz in 1886, Jendrassik analyses the case history of three families where this clinical picture accumulates. He comes to the conclusion that "// appears with regular constancy from the relevant cases mentioned so far that the clinical pictures of the respective families differ from each other.

.." "And if we glance over so far rather long series of such familial or hereditary neurological diseases, we see such a variation in the clinical pictures that makes a precise outlining of the respective diseases rather illusory.

.." and so "we must now sum up all kinds of chronic degenerations developing on a hereditary basis." "In my opinion it would be suitable to mark these affections with the name of familial degenerations, and summarized in this way to integrate these clinical patterns into the pathology of the nervous system." By the term "heredodegeneration" as a contrast to heredo-anomalies that meant inherited structural anomalies conspicuous at birth already, he wanted to define primary degenerations developing in the nervous and muscular systems more or less time after birth due to "congenital weaker constitution and reduced viability". (Apart from these "physical" anomalies later he mentions also "chemical affection" on the basis of which ,,hereditary diseases of metabolism e.g. familial diabetes appear".) His second series of communications in the subject appeared in three parts also in the Orvosi Hetilap, in 1898, under the title "On hereditary neurological diseases". As he writes, since the first communication "... / have had the possibility to observe again some related cases and I presume that they not only prove my earlier opinion, but furnish information on some other points, too". Then he sums up in four points the general regularities of heredodegenerative diseases. (Later the number of rules mounts to seven.) Jendrassik's next work on the subject appeared as a chapter ("Hereditary neurological diseases [Primary degenerations]") in the bulky book "Diseases of the nervous system and the musculature" that appeared in 1899 as volume 6 of "A handbook of internal medicine" edited by Árpád Bókay, Károly Kétli and Frigyes Korányi. Here he makes a summary of his earlier observations related to the clinical pictures of spastic spinal paralysis, amyotrophic lateral sclerosis and progressive muscular atrophy. Besides he makes essential statements such as "The diagnosis of hereditary diseases have been based so far nearly exclusively on the multiple occurrence of cases in the respective families. On this basis, however, one cannot recognize this clinical picture when seeing only one case (except for some better known patterns), either because there was only one child in the family or because only one fell ill." Although