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Fatal Pneumocystis pneumonia from ACTH therapy for infantile spasms
Author(s) -
Goetting Mark G.
Publication year - 1986
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410190320
Subject(s) - annals , section (typography) , citation , pediatrics , medicine , pneumocystis pneumonia , pediatric neurology , neurology , pneumonia , family medicine , library science , pneumocystis jirovecii , psychiatry , classics , history , computer science , operating system
cautiously. Only a few relatives have been examined , and therefore ascertainment of the correct diagnosis by history alone is difficult. Increased heritability in PD is suggested by the occasional occurrence of PD in large kindreds and in monozygotic twins [4]. We and others have provided evidence that a subgroup of PD patients, particularly those with prominent tremor, have a genetic susceptibility for P D 11, 81. This notion contrasts with the conclusion from the twin study, in which increased genetic susceptibility could not be demonstrated C71. However, it is possible that the patients in the twin study had the postural-instability-gait-difficulty variety of PD which, in contrast to the tremor type, may not be associated with a genetically increased risk for PD. According to Dr Lang and his colleagues, the occurrence of tremor, but not of PD, in first-degree relatives of PD patients is three times that expected. Therefore, it must be inferred that the tremor among the relatives is of the familial essential type. Thus, the 1.3% incidence of coexisting ET and PD is surprisingly low. Also, the 4.4% incidence of ET in the dystonic population is much lower than that previously reported 123. Since the publication of our series, we analyzed the cases of additional patients with typical PD and pre-existing ET. Among 833 typical PD patients and 281 ET patients, we identified 56 with a combination of ET and PD. Thus, 6.7% of all PD and 19.9% of all ET patients had the combination syndrome. (This compares to the expected prevalence of 0.41% for ET and 0.35% for P D {4, 61.) The number of patients with coexisting ET and P D would be higher if Par-kinson's patients who later developed ET were included. While it is difficult to diagnose ET in patients with pre-existing PD because of the frequent occurrence of action tremor in PD, Parkinson's patients are just as likely, if not more so, to develop ET later in life. Finally, because our patients are referred from a large geographic area, it is difficult to determine the prevalence rate of ET in Houston. This, of course, points to one limitation of our study, since our patients may be more unique and perhaps do not reflect the clinical and hereditary characteristics of the general population. However, by using a matched population of PD patients, we have attempted to correct for this possible bias. Therefore, our conclusion …