A neuropathologic study of long-term, Economo-type postencephalitic parkinsonism with a prolonged clinical course

Abstract In this report, the neuropathologic features of five autopsied cases of postencephalitic parkinsonism of the Economo-type (PEPE) with a mean age of 66.6 years and a mean duration of the illness of 53.6 years are described. All five patients had presented with personality changes and severe parkinsonism. In addition, four patients had also had ocular symptoms. A pronounced chronic progression of the symptoms characterized all five cases. Active degenerating lesions were found in the substantia nigra (patients 3, 4 and 5) and the oculomotor nucleus (patient 5) which might explain the clinical observation of chronic active disease in these patients. We found that the intraneuronal neurofibrillary tangles (NFT) were immunoreactive to paired helical filaments (PHF), tau and ubiquitin; but ghost tangles demonstrated immunoreactivity only to glial fibrillary acid protein (GFAP). The ghost tangles consisted of dispersed bundles of abnormal tubules, and electron-dense glial filaments would surround and occasionally invade the ghost tangles. The present study suggests that NFT in PEPE are similar in their immunohistochemistry and ultrastructure to those observed in the case of Alzheimer-type dementia.     

A specific chromosome abnormality of t(4;12)(qll-12;pl3) in CD7+ acute leukaemia

Summary. Three cases of acute leukaemia with t(4;12) (qll-12;pl3) karyotypic abnormalities were analysed. They had the following common clinical and biological characteristics: (1) dysplasia of three haemopoietic lineages; (2) absent or low myeloperoxidase activity; and (3) retention of platelets in the peripheral blood and megakaryocytes in the bone marrow. There were increased numbers of basophils in the bone marrow and peripheral blood in two of the cases. In all, the blast cells displayed the unique immunophenotype CD7⁺CD13⁺CD34⁺HLA-DR⁺. The blasts analysed in one case expressed c-kit on the membrane surface. These findings suggest that the t(4:12) (qll-12:pl3) abnormality is associated with a particular type of acute leukaemia, one in which the morphology and immunophenotype suggest that the translocation may have occurred at an early stage of haemopoiesis.     

A case of intractable diarrhea firmly suspected to have autoimmune enteropathy

Protracted diarrhea with insulin dependent diabetes mellitus (DM) and hypothyroidism in a 9 month old Japanese girl who was firmly suspected to have autoimmune enteropathy (AIE) is reported. Her severe secretory diarrhea failed to respond to intensive antidiarrheic treatment and was gradually improved with steroid therapy. The circulating autoantibodies to enterocytes in her serum were detected by indirect immunofluorescence technique and the impaired suppressor T (Ts) cell function was proved by plaque forming assay using bead-separated CD4 or CD8 T cells together with CD19 B cells. The anti-enterocyte antibodies were exclusively of immunoglobulin M (IgM) class and were detected with the progress of the protracted diarrhea. Maximum antibody titer was obtained at the onset of DM and the disappearance of autoantibodies was associated with the resolution of the clinical symptoms and signs. The helper functions of adult CD4 T cells to induce Ig-secreting cells from adult and the patient were strikingly suppressed by adult CD8 T cells. However, the CD8 T cells from the patient lost the ability to inhibit the induction of these Ig-secreting cells when stimulated with adult CD4 T cells. Moreover, the patient's CD8 T cells stimulated rather than suppressed the induction of Ig-secreting cells from the patient when stimulated with the patient's CD4 T cells. These results suggest that the impaired Ts cell function in this patient might play some immunological role in the pathogenesis of AIE     

A case of common variable immunodeficiency associated with cyclic thrombocytopenia

A 12 year old boy was found to be deficient in immunoglobulins (Ig) A, G₂ and G₄, and common variable immunodeficiency was diagnosed. He also had cyclic thrombocytopenia at intervals of approximately 28–30 days. His bone marrow revealed normocellular with slightly decreased megakaryocytes. In vitro colony assays showed markedly imparied megakaryocytopoiesis, erythropoiesis and granulopoiesis. Platelet-associated IgG was elevated at his thrombocytopenic phase. Direct Coombs' test was repeatedly positive. Although not defined at present, we suggest the autoimmune nature of the disease.     

Application of the relaxation technique in general hospital psychiatry

Abstract The present article demonstrates the clinical application and the efficacy of relaxation techniques in general hospital psychiatry in Japan. During April to December 1993, 20 patients were treated with progressive muscle relaxation and 'modified' autogenic training therapies. The targeted symptoms were anxiety attacks (or panic attacks), neurotic insomnia, hyperventilation syndrome, agoraphobia, chronic pain, and distress. Most patients (18/20) improved rapidly with the use of these techniques and their excellence encourages increased use in general hospital psychiatry for the following reasons. First, physically ill patients are likely to show unexpected reactions to psychotropic medications. Second, some patients prefer these treatment methods rather than medications because they feel that they are participating voluntarily in their own treatment and also because they are fearful of being addicted to tranquilizers. Third, these treatments have produced rapid improvement especially for patients with panic attacks and/or hyperventilation. This study strongly suggests that progressive muscle relaxation and 'modified' autogenic training are simple and useful methods which can be easily employed in the clinical practice of general hospital psychiatry.     

Surgical management of urinary incontinence in children with anatomical bladder-outlet anomalies

BACKGROUND: Pediatric urinary incontinence secondary to anatomical anomalies of the bladder outlet is relatively rare, and there is no consensus on its surgical treatment. We reviewed the clinical courses and surgical management of children with this pathology. METHODS: From 1991 to 2001, we performed bladder neck reconstruction on seven children (three boys and four girls). The mean age at the first intervention was 7 years (range 5-12 years). The underlying diagnosis was bladder exstrophy in two children, pseudoureterocele-type ectopic ureter in three, bladder neck incompetence from unknown etiology in one boy with hypospadias, and bilateral single ectopic ureter associated with cloacal malformations in one child. Lower urinary tract was evaluated through various imagings, endoscopy, and urodynamic studies. RESULTS: The methods used to reconstruct the bladder neck was the Young-Dees-Leadbetter procedure (performed on four children); the Kropp procedure (two children); and the Pippi-Salle procedure (one child). Bladder augmentation with intestinal segment was performed on four children; Mitrofanoff continent stoma was created in all patients. Urethral catheterization could not be routinely used in all patients, because of pain, stricture, or complicated urethral anatomy. All patients achieved urinary continence. CONCLUSION: The treatment of pediatric urinary incontinence from anatomical anomalies is technically challenging. Depending on the underlying disorders and anatomy, one needs to decide which method of reconstruction to perform. It would be safer to employ simultaneous augmentation and to create catheterizable continent stoma.     

A Comparative Study of DCMP versus DCcMP Combination Chemotherapy for Adult Acute Leukemia

An experimental study using L-1210 mouse leukemia indicatedthat daunomycin, cyclocytidine, and 6-mercaptopurine (DCcM)combination chemotherapy was more effective than daunomycin,cytosine arabinoside, and 6-mercaptopurine (DCM). Based on theseresults, a clinical study comparing daunomycin, cytosine arabinoside,6-mercaptopurine, and predni-solone (DCMP) combination chemotherapywith daunomycin, cyclocytidine, 6-mercaptopurine, and prednisolone(DCcMP) in the treatment of adult acute nonlymphocytic leukemiawas conducted. Patients were randomly allocated to receive eitherregimen. Ten of 15 patients treated with DCMP had complete remissionfor a median duration of 43 weeks and one had partial remission.Seven of 15 patients treated with DCcMP had complete remissionfor a median duration of 18 weeks and two had partial remission.The patient characteristics before chemotherapy, the toxicityof the chemotherapeutic regimens and the causes for differencesin antitumor activity between the experimental study and theclinical study are discussed.