The age at initial rheumatic involvement is considered especially important in determining which symptom pattern will appear, for as Roth26 indicates, the state of a tissue at the time of initial rheumatic involvement may determine whether or not it will be affected, but, once affected, the tissue may for some time remain in a state which disposes it to further rheumatic attack. It is possible 4,8 that rheumatic attacks are not truly discrete events, but simply exacerbations of a continuous, underlying process (or tissue sensitivity). Which tissues will be involved may be almost completely determined at the time of the initial "sensitization," but the appearance of clinical manifestations may vary widely for different tissues.
Age-specificity can be used to explain cases of "pure" Sydenham's chorea. These would hypothetically be cases where rheumatic disease attacked a child at a time when his particular developmental pattern was "ripe" for the appearance of chorea as a symptom, but already "overripe" for the (typically earlier-occurring) symptom of rheumatic heart disease. This hypothesis permits "pure" chorea to be seen as a true rheumatic symptom, yet anticipates the empirical finding that such cases do not seem to develop subsequent rheumatic heart disease.16 The fact that both chorea and heart symptoms do frequently occur in the same rheumatic child presumably indicates that many hosts go through an age when they are susceptible to both symptoms.
While the grimacing of schizophrenia may be somewhat different from that observed in typical chorea, it is assumed here that a similar brain process could underlie both, and that this process might in each case be a sign of rheumatic brain involvement. In his discussion of the "spasmodic phenomena" in dementia praecox, Kraepelin18 (p. 83) mentions that the grimacing and twitching observed "remind one of the corresponding disorders of choreic patients."
There is a sex difference (not shown in Table 1) which suggests that schizophrenic grimacing may be related to the occurrence of a rheumatic attack during puberty, rather than to its occurrence at a particular age, as such. Schizophrenic grimacing was most common (46% of 13 cases) in the female patients whose first rheumatic attack was noted between 9 and 16. Rheumatic onset at other ages rarely "produced" grimacing (12% of 26 cases). For males, schizophrenic grimacing was most commonly noted when the first rheumatic attack occurred a little later, between ages 13 and 18 (41% of 17 cases). Only 24% of the remaining 59 males with overt rheumatic history showed grimacing. This sex difference runs roughly parallel to the sex difference in age at puberty.
Definition A appears to tap largely those "rheumatic" schizophrenics whose rheumatic lesions were initiated in childhood (Section II,B). Definition C presumably taps cases with later rheumatic onset (Section I). Thus the irreversible lesions (poor prognosis) seem largely associated with later rheumatic onset—a finding also consistent with the very different outcomes seen in chorea and in grimacing schizophrenia (Section III,F).
Chapman,5 Gerstley,9 Leys,19 and Shaskan 27 all present data at least somewhat suggestive of excess representation of first siblings in chorea, although their data were not gathered or analyzed primarily with this in mind.
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