伴抗-p200类天疱疮自身抗体及剥脱性食管炎的获得性大疱性表皮松解症1例

Background:Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported.Observation:A 52-year-old Japanese woman had pruritic blisters on almost the whole body as well as erosive lesions in the oral cavity and esophagus.A histological finding was subepidermal bullae.Direct immunofluorescence(IF)revealed a linear deposition of IgG,IgM and C3 at the epidermal basement membrane zone(BMZ).Indirect IF using human skin split by 1 M NaCl as a substrate showed IgG antibody reactive with the dermal side.By immunoblot analysis using normal human dermal extract,the 200-kDa and 290-kDa bands were detected.Indirect IF did not show any anti-BMZ antibody activity,when using the skin of the patient with recessive dystrophic epidermolysis bullosa as a substrate.Conclusion:We regarded our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen.This is the second case in the literature associated with autoantibodies to these two antigens.

年轻男性患者的黏蛋白囊性瘤

A 25-year-old Japanese man was admitted to our hospital with a history of recurrent pancreatitis and a pseudocyst of the pancreas. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed an encapsulated multilocular cystic mass 5 cm in diameter in the pancreatic tail. Endoscopic ultrasonography demonstrated a mural nodule, and endoscopic retrograde pancreatography showed a communication of the lesion with the main pancreatic duct. A neoplastic cystic tumor was suspected, and a resection of the body tail of the pancreas was performed. The lesion was a multilocular cyst having a common fibrous capsule and viscous content. Histologically, the cystic lesion was lined with a single layer of columnar cells with low-grade atypia. Ovarian-type stroma (OS) was confirmed, and it showed positive for antiestrogen receptor and antiprogesteron receptor staining. Based on these findings, the lesion was diagnosed as mucinous cystic neoplasm (MCN), an adenoma that shows extraordinarily high prevalence in women. Further study on the pathogenesis of MCN in male patients should be undertaken to elucidate the process of development.

前额和颞叶边缘病理对精神病机制的不同影响

Common abnormalities within the schizophrenia spectrum may be essential for the pathogenesis of schizophrenia, but additional pathological changes may be required for the development of full-blown schizophrenia. Clarifying the neurobiological similarities and differences between established schizophrenia and a milder form of schizophrenia spectrum disorder would potentially discriminate the pathophysiological mechanisms underlying the core features of the schizophrenia spectrum from those associated with overt psychosis. High-resolution MRIs were acquired from 25 patients with schizotypal disorder, 53 patients with schizophrenia and 59 healthy volunteers matched for age, gender, handedness and parental education. Volumetric measurements of the medial temporal structures and the prefrontal cortex subcomponents were performed using consecutive 1-mm thick coronal slices. Parcellation of the prefrontal cortex into subcomponents was performed according to the intrinsic anatomical landmarks of the frontal sulci/gyri. Compared with the controls, the bilateral volumes of the amygdala and the hippocampus were reduced comparably in the schizotypal and schizophrenia patients. The parahippocampal gyrus volume did not differ significantly between diagnostic groups. Total prefrontal grey matter volumes were smaller bilaterally in the schizophrenia patients than in the controls and the schizotypal patients, whereas the schizotypal patients had larger prefrontal grey matter than the controls in the right hemisphere. In the schizophrenia patients, grey matter volumes of the bilateral superior frontal gyrus, left middle frontal gyrus, bilateral inferior frontal gyrus and bilateral straight gyrus were smaller than those in the controls. The schizophrenia patients also had reduced grey matter volumes in the right superior frontal gyrus, bilateral middle frontal gyrus and right inferior frontal gyrus relative to the schizotypal patients. Compared with the controls, the schizotypal patients had larger volumes of the bilateral middle frontal gyrus and smaller volumes of the right straight gyrus. There were no significant between-group differences in volumes of the ventral medial prefrontal cortex or the orbitofrontal cortex. These findings suggest that volume reductions in the amygdala and hippocampus are the common morphological substrates for the schizophrenia spectrum, which presumably represent the vulnerability. Additional widespread involvement of the prefrontal cortex in schizophrenia may lead to the loss of inhibitory control in other brain regions and suggests (although it is not specifically be related to) its critical role in the manifestation of overt psychosis.

枕叶区α波活动改善是一氧化碳中毒进行反复高压氧治疗效果的潜在预测指标

Objectives: The main aim of this study was to evaluate the efficacy of repetitive hyperbaric oxygen (HBO) treatments with monitoring of quantitative electroencephalography (qEEG) for symptoms of acute carbon monoxide (CO) poisoning and prevention of delayed neuropsychiatric sequelae. Methods: Eight patients with acute CO poisoning received repetitive HBO treatments five times a week and qEEG was recorded once a week. Peak alpha frequency, peak alpha power, and absolute and relative alpha power in the occipital region were evaluated. The repetitive HBO treatments were discontinued when the peak alpha frequency increased to and maintained a maximum in each patient. Results: The mean number of HBO treatments was 20.3. The peak alpha frequency and the relative alpha power significantly increased after repetitive HBO treatments. The absolute alpha power and the peak alpha power insignificantly improved. Total numbers of HBO treatments were not correlated with age, duration of CO exposure, initial level of COHb, or interval to the first HBO treatment. After the completion of repetitive HBO treatments, no patient developed delayed neuropsychiatric sequelae. Conclusions: These results suggest that repetitive HBO treatments may prevent the delayed neuropsychiatric sequelae of CO poisoning when applied individually with monitoring of the peak alpha frequency as an indicator of efficacy.