The purpose of this study was to clarify the association of HLA-DRB1 and -DPB1 alleles with multiple sclerosis (MS) in Japanese, to determine whether optico-spinal MS (OS-MS) and conventional MS are immunogenetically distinct, and to verify the role of gender difference in HLA associations of MS. We studied HLA-DRB1 and -DPB1 polymorphisms in 166 Japanese patients with MS. Forty-seven patients were classified as having the optico-spinal MS (OS-MS) and 119 as having conventional MS. A lack of DPB1*0301 and a higher frequency of DPB1*0501 compared with controls (corrected P<0.0074; odds ratio=9.48) were found in OS-MS. By contrast, we found for the first time an association of DPB1*0301 with conventional MS in Japanese (corrected P=0.0444; odds ratio=3.28). Logistic analysis, adjusted for sex and age, revealed independent associations of DPB1*0301 (P=0.0004, adjusted odds ratio (aOR)=4.70), DPB1*0501 (P=0.0081, aOR= 2.50) and DRB1*1501 (P=0.0252, aOR=2.21) with conventional MS. However, the frequencies of DRB1*1501 and DPB1*0501 in male patients with conventional MS were equal to those in male controls while the DPB1*0301 frequency was increased in both male and female patients. We did not find any association of these HLA alleles with disease course and severity. In conclusion, OS-MS is a DPB1*0501-associated distinct subtype of MS, and DPB1*0301 is the most strongly associated allele with conventional MS in Japanese. In addition, gender plays an important role in HLA association with MS.

Medicine-To examine associations between interpersonal relationships in work settings and burnout, a cross-sectional survey was conducted on home care workers in Sapporo, Japan, by using the Maslach Burnout Inventory (MBI) and scales of interpersonal conflict and social support developed by the authors. Questionnaires were distributed among 303 subjects and returned by 243 subjects (80%). Complete answers were obtained from 106 subjects and were used for analysis. In multiple regression analyses, conflict with clients and their families significantly related to emotional exhaustion and depersonalization of the MBI (p<.05). Supervisory conflict significantly related to emotional exhaustion (p<.05), whereas coworker conflict significantly associated with depersonalization (p<.01). It is suggested that conflicts with clients' families, as well as clients, are important i n d i c a t o r s f o r e m o t i o n a l e x h a u s t i o n a n d depersonalization of home care workers. (J Occup Health 2003; 45: 313-320)

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A 59 year old woman developed pronounced thirst, increased water intake, and increased urinary output followed by slowly progressive cerebellar symptoms. Brain MRI showed abnormal hyperintensity on T2 weighted studies in the region of both dentate nuclei without atrophy of the cerebellum or the brainstem. A 99mTC diphosphonate bone scan showed bone lesions in the distal parts of both femurs as well as distal and proximal parts of both tibias. The diagnosis of Erdheim-Chester disease was made by bone biopsy. This is the first case of Erdheim-Chester disease presenting as a slowly progressive cerebellar syndrome and diabetes insipidus, and also showing high signal lesions in deep cerebellar nuclei on MRI. Skeletal surveys are indicated for patients with otherwise unexplained slowly progressive cerebellar symptoms.

A 37-year-old man developed encephalopathy with prominent eosinophilia. Magnetic resonance imaging (MRI) revealed multiple T2-weighted high signal intensity lesions with dimeglumine gadopentetate (Gd-DTPA) enhancement on Tl-weighted images, which were distributed in the cerebral cortex, thalamus, deep white matter and cerebellum. He was diagnosed as having systemic mastocytosis on the basis ofproliferating mast cells in the bone marrowand peripheral eosinophilia. Following steroid administration, there was a rapid improvementof his symptomsand laboratory data. To our knowledge, this was the first reported case of systemic mastocytosis provoking encephalopathy with serial MRIfindings. (Internal Medicine 33: 23-26, 1994)

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