Biomedical subjects
Publications and source records attributed to D Soffer.
A patient with idiopathic hypoparathyroidism presenting with spells of paroxysmal choreoathetosis is described. The possible mechanisms by which hypoparathyroidism induces choreoathetosis and other extrapyramidal motor dyfunctions are discussed. The need for screening patients with extrapyramidal disease for hypoparathyroidism is stressed.
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Subacute sclerosing panencephalitis (SSPE), a disease related to measles (rubeola) infection, was more common in Arabs and Sephardi Jews than in Ashkenazi Jews in Israel. There were no familial aggregates and it is unlikely that genetic differences account for this selectivity. Among several non-genetic factors which might explain the selectivity, family size emerged as a possible risk factor. Family size has not previously been suspected as influencing the risk of SSPE. Preponderance of SSPE in rural areas and among the poor would also be compatible with this idea as family size tends to be larger in rural and lower socioeconomic groups. In large families, there may be a greater change that older siblings will transmit measles to very young siblings. In small families, measles may be acquired from peers at about school age when risk of SSPE may be lower.
Three cases of primary brain tumors simulating purulent meningitis have been described. Two patients were infants suffering from ependymoma of the posterior fossa, while the third was a 35-year-old man with astrocytoma of the temporal lobe. All cases were characterized by acute onset with fever, signs of meningeal irritation without any other neurological signs, and marked CSF pleocytosis. The diagnosis in 2 cases was made only at necropsy, and the third case was correctly diagnosed only after a delay. The possible occurrence of brain tumor and meningitis simultaneously was considered and seemed unlikely in our cases. A possible explanation for the clinical and CSF findings was irritation of the leptomeninges by the tumor and its breakdown products.
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Central nervous system manifestations developed in a patient suffering from the chronic, adult form of Gaucher's disease. At autopsy, massive systemic fat embolism was found, involving mainly the brain and the lungs. As there was no evidence of bone fracture, or trauma to the adipose tissues, it is presumed that the severe skeletal involvement by the Gaucher's disease led to the development of the fat embolization.
During the period 1968-73, 46 patients with subacute sclerosing panencephalitis (SSPE), a fatal childhood disease related to rubeola, were encountered in Israel. The incidence per million population was 3.4 for Sephardic Jews, 3.2 for Arabs and only 0.5 for Ashkenazic Jews. An environmental factor which sharply demarcated Arabs and Sephardic Jews from Ashkenazic Jews, and which might account for the differences in incidence of SSPE, was family size: 42.3% of Arab families and 21.5% of the families of Sephardic Jews but only 1.1% of Ashkenazic Jewish families had five or more children. It is postulated that older siblings in large families might constitute vectors which introduce rubeola to the younger siblings at a time when they are at unusual risk of developing SSPE (i.e. before two years of age), whereas in small families, rubeola tends to be acquired later in childhood when the risk of SSPE is reduced.
In a research study, the vocational status of 172 patients with multiple sclerosis who were from 20 to 50 years of age was evaluated. These patients constituted 35% of the total population having multiple sclerosis in Israel. Ninety-four are working and 78 are unemployed. In the unemployed group, 41 are severely disabled but 37 have working potential. No rational explanation could be found in the demographic, vocational or educational backgrounds for the unemployment of the 37 patients. It is suggested that a vocational rehabilitation program at the onset of the patient's disability may prevent eventual unemployment.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A 40-year-old male, with established AIDS developed cachexia and dementia. At autopsy opportunistic infections were found. An additional neuropathological finding was Wernicke's encephalopathy. Although malnutrition is common in AIDS patients, it seems that Wernicke's encephalopathy is rare in AIDS. Nevertheless, it is suggested that a therapeutic trial with parenteral thiamine may be useful in AIDS patients with amnestic syndrome and dementia.