Remission of Tardive Dystonia with ECT

A 30-year-old patient with tardive dystonia, who had failed to respond to cessation of neuroleptics, placebo, diazepam, biperiden, propranolol, and clonidine, had an impressive response to courses of electroconvulsive therapy (ECT) on three successive trials.     

Arthritis and nephritis in patients with systemic lupus erythematosus

Previous publications suggest that in patients with systemic lupus erythematosus (SLE), rheumatoid factor (RF) may be "protective" against nephritis. In our study of 662 patients with SLE, we found that persistent, rheumatoid-like arthritis showed a much stronger inverse correlation with nephritis than RF. Of 186 such patients, 59 developed clinically evident nephritis (32%) compared to 263 of the other 476 patients (55%) (p less than 10(-7). RF showed only a weak inverse relationship to nephritis (p = 0.064). We conclude that the presence of persistent rheumatoid-like arthritis in patients with SLE identifies a clinical subset of patients who are less likely to develop nephritis than those with no arthralgia, no objective arthritis or only episodic arthritis. We hypothesize that such patients represent a genetically determined subset among patients with SLE and that perhaps they are more likely to bear the HLA-DR4 allele.     

Successful therapy of crystalcryoglobulinemia: a case report.

Successful therapy for a case of multiple myeloma with a spontaneously crystallizing cryoglobulin of the IgG2-kappa light chain variety was achieved, using both continuous-flow cell centrifugation plasmapheresis to rapidly lower the M component and combination chemotherapy with phenylalanine mustard, prednisone, procarbazine, and vincristine to control the myeloma process. This resulted in resolution of incapacitating large and small necrotic cutaneous ulcerations of the extremities. Physicochemical studies of the crystalcryoprotein demonstrated that cryoprecipitation was rapid and accompanied by the formation of needle-shaped crystals, yet was completely reversible at 37 degrees C. Cryocrit determinations varied depending upon relative centrifugal forces and temperature and did not always relate linearly to the amount of abnormal protein, thus making these alone unreliable in assessing response to therapy.     

Psychiatric disorders in adolescents exposed to domestic violence and physical abuse

The relationship between abuse and psychiatric diagnoses was investigated in two groups of physically abused adolescents, 57 living in homes with interparental violence and 32 in homes without such violence, and in 96 nonabused adolescents living in nonviolent homes. Adolescents in the first group were found to be at greater risk for depression, separation anxiety disorder, post-traumatic stress disorder, and oppositional defiant disorder than were those in the second group. Adolescents in the first group also appeared more vulnerable to anxiety and depression.     

Acetylcholinesterase inhibition in dementia with Lewy bodies: results of a prospective pilot trial

OBJECTIVE: Dementia with Lewy bodies (DLB) is the second commonest form of dementia. The response to acetylcholinesterase inhibition (AChEI) could be greater in DLB than in Alzheimer's disease (AD) because cholineacetyl-transferase levels are more reduced in the former. This preliminary trial seeks to compare performances in cognitive tasks before and after tacrine administration in DLB and AD subjects. METHODS: Six DLB and 6 AD patients were enrolled in an open, nonrandomized, intervention trial using 80 mg/day tacrine. Patients met ADRDA or DLB consortium criteria for probable diseases. Subjects were matched for Mini Mental State Examination (MMSE) score, age and sex. Mattis Dementia Rating Scale (DRS), Controlled Oral Word Association Test (FAS) and Boston Naming tests were administered at baseline and at 6 months into treatment. RESULTS: AD and DLB groups did not differ in initial mean total DRS scores. In the primary analysis, both groups declined during the course of treatment (-7.3 +/- 4.2 and -16.8 +/- 39.2 DRS points, respectively). Due to the large variability in DLB posttreatment scores, this group was divided post hoc into responders (DLBr) and nonresponders (DLBnr). The DLBr group outperformed the DLBnr group at baseline (p < 0.05) and, notably, in follow-up DRS test scores (p < 0.001). Two-way MANOVA comparing both DLB subgroups with either the entire AD cohort or similarly stratified AD subgroups showed a significant interaction (F = 7.6; p < 0.015), attributed mostly to declines in DLBnr group scores (p < 0.01). Surprisingly, on DRS memory subscale and FAS tests, there were significant improvements in DLBr scores (p < 0.02). A baseline MMSE (or DRS memory) score >/=15 predicted a positive response to tacrine in DLB. Acceleration of parkinsonism occurred in all DLB subjects. CONCLUSION: Results from a primary analysis of the therapeutic effect of 80 mg/day tacrine in DLB and AD were negative. However, post hoc analysis showed that mild to moderate DLB responds favorably to AChEI relative to AD through stabilization of global cognitive decline and improvements in specific cognitive areas. These results could be useful in the planning of a more definitive study.     

Relationships: importance for patients with cerebral tumours

OBJECTIVE: To investigate the influence of relationships on psychosocial status in patients with Stage III and IV brain tumours. PATIENTS AND METHODS: Thirty-three outpatients at a university medical centre were referred to the study by their treating physician prior to the initiation of aggressive chemotherapy. All subjects underwent a comprehensive psychosocial assessment. RESULTS: Depressive and anxious complaints were common, but not related to gender. Single/divorced status suggested greater vulnerability to anxiety as 88% of singles and only 48% of patients with partners reported clinical levels of anxiety. Single patients' anxiety was related to inactivity (r = 0.78, p < 0.05) and fears of financial difficulties (r = 0.72, p < 0.05). State and trait anxiety related differently to married patients' complaints. Married patients more frequently reported clinical levels of depressive symptoms (44% partnered vs 28% singles). Depression among married patients was predicted by problems with sex, finances, marital difficulties, and inactivity [R2 = 0.803; F = 20.420, df(4,20), p < 0.0001)]. Problems with relationships were disassociated from problems with sex. Married patients' problematic relationships were predicted by overprotection, inactivity, concerns of bodily deterioration, depression, and anxiety [R2 = 0.775, F = 13.060, df(5, 19) < 0.0001]. CONCLUSIONS: Married neuro-oncology patients may experience depressive symptoms arising from the marriage, but appear to be buffered from anxiety when compared with single/divorced patients. The prevalence of anxiety among single patients suggests modifications of interventions by relationship status.     

Allograft loss in renal transplant recipients with Fabry's disease and activated protein C resistance

INTRODUCTION: Fabry's disease is associated with an increased incidence of thrombotic events and rejection. Spontaneous thrombosis of a functioning cadaveric renal allograft in a recipient with Fabry's disease prompted prospective evaluation of all transplant candidates with Fabry's disease for hypercoagulability. MATERIALS AND METHODS: Transplant candidates with Fabry's disease were tested for hypercoagulability, analyzed for HLA-type and ABO group, and comorbid conditions suggestive of hypercoagulability. RESULTS: A unique association of Fabry's disease with activated protein C Resistance was documented in a cohort of Caucasian male renal transplant recipients with Fabry's disease. Four of five patients were blood group A and had no significant comorbid conditions suggestive of hypercoagulability. The resistance to activation of protein C (APCR)(+) patients shared HLA loci-B8 and Dr3, although the APCR(-) patients shared HLA loci-B27 and -B38. CONCLUSIONS: Due to the observed increase in the incidence of APCR in our Fabry's cohort, we suggest screening all patients with Fabry's disease for APCR. Because factor V and factor Va receptors are found on vascular endothelium and peripheral blood monocytes, APCR in the presence of Fabry's disease may be a nonimmunological stimulus for rejection. Analysis of HLA typing in patients with Fabry's disease may further elucidate HLA-based association of Fabry's disease and resistance to activated protein C with the risk of thrombosis and rejection.     

Reconstruction of a parotidectomy defect using a paraumbilical perforator flap without deep inferior epigastric vessels

A 37-year-old woman who underwent a parotidectomy for acinic cell carcinoma was referred for correction of the resulting defect. As an assistant principal, the patient was often in public and, because she was somewhat self-conscious about her facial deformity, she sought reconstruction. Physical examination revealed a pre-auricular soft-tissue defect that measured approximately 5 x 5 cm. After consultation with the patient, microsurgical transfer of fat from the lower abdomen based on branches of the deep inferior epigastric vessels, rather than the deep inferior epigastric artery and vein itself, was planned. A vertical skin ellipse measuring 6 x 5 cm was raised from the right lower abdomen with a pedicle consisting of only a branch of the deep inferior epigastric artery and vein. The donor vessels were then microsurgically anastomosed to the superficial temporal artery and vein on the left side of the face. Two weeks postoperatively the flap was defatted, with removal of the skin-monitoring island. The patient continues to do well with a normal contour of the face and decreased anxiety secondary to correction of her facial deformity.     

Participation of patients 65 years of age or older in cancer clinical trials.

PURPOSE: Although 61% of new cases of cancer occur among the elderly, recent studies indicate that the elderly comprise only 25% of participants in cancer clinical trials. Further investigation into the reasons for low elderly participation is warranted. Our objective was to evaluate the participation of the elderly in clinical trials sponsored by the National Cancer Institute (NCI) and assess the impact of protocol exclusion criteria on elderly participation. PATIENTS AND METHODS: We conducted a retrospective analysis using NCI data, analyzing patient and trial characteristics for 59,300 patients enrolled onto 495 NCI-sponsored, cooperative group trials, active from 1997 through 2000. Our main outcome measure was the proportion of elderly patients enrolled onto cancer clinical trials compared with the proportion of incident cancer patients who are elderly. RESULTS: Overall, 32% of participants in phase II and III clinical trials were elderly, compared with 61% of patients with incident cancers in the United States who are elderly. The degree of underrepresentation was more pronounced in trials for early-stage cancers than in trials for late-stage cancers (P <.001). Furthermore, protocol exclusion criteria on the basis of organ-system abnormalities and functional status limitations were associated with lower elderly participation. We estimate that if protocol exclusions were relaxed, elderly participation in cancer trials would be 60%. CONCLUSION: The elderly are underrepresented in cancer clinical trials relative to their disease burden. Older patients are more likely to have medical histories that make them ineligible for clinical trials because of protocol exclusions. Insurance coverage for clinical trials is one step toward improvement of elderly access to clinical trials. Without a change in study design or requirements, this step may not be sufficient.