Progressive cerebellocerebral atrophy: a new syndrome with microcephaly, mental retardation, and spastic quadriplegia.
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OBJECTIVES: Chronic toxic encephalopathy (CTE) caused by long term occupational exposure to organic solvents is still a controversial disorder. Neuropsychological testing is the cornerstone for diagnosing the syndrome, but can be negatively influenced by motivational problems. In this nationwide study, we investigated the neuropsychological functioning and psychological symptoms of a large group of patients with suspected CTE, and ruled out alternative explanations for their complaints, including suboptimal performance due to insufficient effort. METHODS: We studied participants with suspected CTE (n = 386) who were referred for further diagnosis to the Netherlands Centre of Occupational Diseases in the period 1998-2003 and who had completed the entire diagnostic protocol. Patients were excluded if there was the slightest suspicion that test performance had been negatively influenced by insufficient effort (n = 221), or if comprehensive assessment identified an alternative diagnosis (n = 80). Insufficient effort was defined by a combination of three indices. The neuropsychological test scores of the patient group (n = 85) were compared with those of a control group of building trade workers matched for sex, age, and educational level (n = 35). RESULTS: The patient group had significantly more psychological complaints and performed significantly worse than the control group on tests of speed of information processing and memory and learning. However, only a small percentage of the patients had clearly abnormal scores for cognitive speed (9%) or memory (8%). Attention, verbal abilities, and constructional functions were not disturbed. Exposure duration and cognitive complaints were significantly correlated, whereas the correlation between exposure duration and neuropsychological domain scores was not significant. CONCLUSIONS: Insufficient effort was present in a substantial part of the patient group. After minimising the likelihood that insufficient effort negatively influenced neuropsychological scores, we still found neuropsychological deficits in speed of cognitive processing and memory; however, these scores were clearly abnormal only in a minority of patients with suspected CTE. Screening instruments should focus on these domains.
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A patient who presented with "hyperventilation syndrome" was initially mis-treated as severe crush injury, illustrating the need for thorough assessment of all casualties whilst on exercise prior to arranging casualty treatment and evacuation.
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Research on posttraumatic stress disorder (PTSD) has been notable for controversy as well as progress. This article concerns the evidence bearing on the most contentious issues in the field of traumatic stress: broadening of the definition of trauma, problems with the dose-response model of PTSD, distortion in the recollection of trauma, concerns about "phony combat vets," psychologically toxic guilt as a traumatic stressor, risk factors for PTSD, possible brain-damaging effects of stress hormones, recovered memories of childhood sexual abuse, and the politics of trauma.
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The aim of the study was to determine the impact of midtrimester abortion with extra-amniotic PGE(2) on future fertility and reproduction. Two hundred and fifteen women were enrolled. The mean induction-to-abortion interval was 21.3 h. The complication rate was 11.7%. Twenty women (9.3%) were lost to follow-up, 82 patients (38.1%) used contraception. Spontaneous pregnancy occurred in 110 out of 113 women who desired fertility (97%). Three patients were defined as infertile but conceived following treatment. The average time between the abortion and the next conception was 15.9 months (range 1-77 months). Seventy patients (63.7%) delivered at term. Premature delivery occurred in 18 patients (16.4%). Eighteen women aborted spontaneously in the first and 3 in the second trimester. One extrauterine pregnancy occurred. Second-trimester abortion with extra-amniotic PGE(2) is a safe procedure with a low rate of complication. Apparently it does not affect future fertility, but may be associated with an increased rate of premature deliveries in future gestations.
A Pain Rating Scale [PRS] and a Pain Behavior Checklist [PBC] were developed for use during a clinical pain interview. Norms were based on 395 chronic pain patients referred for pain management. The PRS obtained ratings of present pain, and worst and least pain during the previous 30 days. The PBC recorded 16 pain behaviors during the 45- to 60-min interview. Normative data were given for seven measures: (1) worst pain rating, past month; (2) least pain rating, past month; (3) present pain rating; (4) difference between worst and least pain ratings; (5) pain behavior score; (6) total pain score (based on Nos. 1, 2, 3, and 5), and (7) consistency score. The consistency score reflects the agreement (or discrepancy) between the pain behavior and present pain ratings. A moderate overall relationship was found between pain behavior and present pain ratings (r = 0.46, p < 0.001). The frequency of good agreement between pain behavior and present pain ratings was unaffected by sex, race, age, pain site, type of injury, duration of pain, legal representation, and evaluating psychologist; but it varied markedly with conscious symptom magnification. Patients seen as consciously exaggerating pain (n = 127) gave higher pain ratings (all p values < 0.001); and had frequent (64.6%) discrepancies between their pain report and pain behavior compared to others (14.2%). The report of present pain was unrelated to pain behavior for conscious exaggerators (r = 0.04, NS); in contrast there was a moderately high relationship for other patients (r = 0.68, p < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)
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Certain aetiological factors related to prognosis of the post-traumatic syndrome are presented. The organic vs. psychological controversy and the principal risk factors, namely the pre-traumatic personality, psychological state, social and occupational influences and the compensation issue are discussed in relation to management and prevention.
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The genes for acetylcholinesterase (ACHE) and butyrylcholinesterase (CHE) are expressed in multiple tumor tissues, including ovarian carcinomas. Both CHE and ACHE genes coamplify in leukemias. To examine the relationship of gene amplification to the expression of these genes in tumors, ACHE and CHE genes and their expression were studied in primary ovarian carcinomas. DNA blot hybridization demonstrated a significant amplification and mutagenesis of both genes in 6 of 11 malignant tumors studied. This was greater or of the same order of magnitude as the amplification of the oncogenes c-rafi, v-sis, and c-fes in these tumors. No amplification was found in normal ovarian tissues or benign ovarian cysts. Xenopus oocyte microinjections, blot and in situ hybridizations, and immuno- and cytochemical staining revealed translatable CHEmRNA and its active protein product in discrete tumor foci. The frequent coamplification in ovarian carcinomas of ACHE and CHE genes implicates cholinesterases in neoplastic growth and/or proliferation.