A case of aseptic meningitis caused by relapsing polychondritis]

We report a patient of relapsing polychondritis (RP) with antecedent aseptic meningitis. A 65-year-old man has developed headache and fever. Neurological examination showed meningeal signs, and cerebrospinal fluid (CSF) examination revealed meningeal inflammation which contained 450 polymorphonuclear cells/microl, 302 mononuclear cells/microl, and 0 red cells/microl, with 79 mg protein/dl. Serologic testing for autoimmune disease as well as the culture and cytology of CSF were negative. He admitted our hospital as having aseptic meningitis and experienced antibiotic therapy. However, his pyrexia continued and he developed repeating visual and hearing impairment reacting to steroid. Three months later, he became behaviorally deaf, and bilateral auricular chondritis occurred with nonerosive seronegative inflammatory polyarthritis. The result of condral biopsy was consistent with the diagnosis of RP showing cartilage surrounded by an intense inflammatory cell response with a decreased number of chondrocytes. A clinical diagnosis was made and prednisolone 60 mg/day was begun with the result of resolution of the auricular chondritis, and slight improvement of his deafness. Aseptic meningitis is a rare complication of RP. Only one report detailed RP patient who had preceding meningitis. RP is a potentially lethal disease resulting from suffocation by airway collapse, the complications of a cardiac large vessel, and so on. For improvement of a life prognosis, an early diagnosis and treatment are indispensable. Although RP is a rare discovery, it is necessary that RP should be taken into consideration and be differentiated as a cause of relapsing aseptic meningitis.     

Evaluation of gender differences in caregiver burden in home care: Nagoya Longitudinal Study of the Frail Elderly (NLS-FE)

Background: Japan is presently experiencing a growth in the number of male caregivers and this situation has given rise to some concerns over gender differences. Previous studies have suggested that there are gender differences in caregiver burden in home care, however, it is still unclear whether or not gender differences exist. We therefore conducted this study to attain a better understanding of the Japanese male caregiver burden in home care, using data from the Nagoya Longitudinal Study of Frail Elderly (NLS‐FE). Methods: NLS‐FE is a large prospective study of community‐dwelling elderly persons eligible for public long‐term care insurance who live in Nagoya city and use the services of the Nagoya City Health Care Service Foundation for Older People, which comprises 17 visiting nursing stations and corresponding care‐managing centers, from November to December 2003. Data used in this study included the Japanese version of the Zarit Caregiver Burden Interview, caregivers’ and dependents’ characteristics, and the caregiving situation. The differences in dependent and caregiver characteristics between male and female caregiver groups were assessed using the χ²‐test for categorical variables or the unpaired t‐test for continuous variables. Multiple logistic regression was used to examine the association between dependent and caregiver characteristics and caregiver burden. Results: A total of 399 male caregivers and 1193 female caregivers were included in our analysis. Before and after controlling baseline variables, we did not detect a difference between male and female caregivers with respect to caregiver burden. Conclusion: Our study suggests that differences in caregiver burden may not necessarily exist between male and female caregivers in Japan.     

Clinical features of chromosome 16q22.1 linked autosomal dominant cerebellar ataxia in Japanese

Chromosome 16q22.1-linked autosomal dominant cerebellar ataxia (16q-ADCA) is strongly associated with a substitution in the puratrophin-1 gene. This locus overlaps with spinocerebellar ataxia type 4 (SCA4) which shows ataxia with prominent sensory axonal neuropathy. We found that 16q-ADCA is a common ADCA subtype in the Tohoku District of Japan. The clinical feature of Japanese 16q-ADCA is characterized as late-onset pure cerebellar ataxia.     

Neuroradiological study of a possible progressive multifocal leukoencephalopathy using diffusion tensor imaging and proton magnetic resonance spectroscopy]

We report a possible case of progressive multifocal leukoencephalopathy (PML) that was attempted to evaluate the pathogenesis by a novel brain MRI techniques. A 72-year-old woman had developed subacute visual disturbance, right hemiparesis and sensory disturbance. Laboratory examinations revealed liver dysfunction and pancytopenia due to liver cirrhosis (type C) and preclinical status of multiple myeloma. Thus, this patient had these two underlying diseases, while anti-HIV antibody was negative. She was suspected with PML by detection of JCV-DNA in cerebrospinal fluid using with PCR. MRI showed multifocal T2-high signals in the bilateral parieto-occipital deep white matter, basal ganglia and right cerebellar hemisphere. No gadolinium enhancement was found. On FLAIR and diffusion weighted images (DWI), the lesion showed hyperintensity. The hyperintense areas on DWI showed various pattern on apparent diffusion coefficient (ADC) and fractional anisotropy (FA). In particular white matter changes, the course of FA reflected the clinical course more than ADC. Proton magnetic resonance spectroscopy (1H-MRS) in deep brain white matter showed ratios of reduced N-acetyl aspartate (NAA) and increased choline (Cho) to creatine. 1H-MRS by chemical shift imaging were undergone three times between 4 and 6 months after the onset. The change of these chemical markers correlated with her clinical course. We conclude that the approach of diffusion tensor imaging (DTI) and 1H-MRS are useful for evaluating neuropathologic observations and clinical course.     

Subjective assessments of the quality of life, well-being and self-efficacy in patients with schizophrenia

Aim:  The present study examined three kinds of subjective assessment scales in the same patient group with schizophrenia to analyze the correlations among scores obtained in relation to the background data.
Method:  Thirty-six patients with schizophrenia were examined with the 26-item short form of the World Health Organization Quality of Life (WHO-QOL 26), Subjective Well-being under Neuroleptic drug treatment: Short Japanese version (SWNS) and Self-Efficacy for Community Life scale (SECL) for subjective assessment scales, five kinds of neurocognitive tests, Positive and Negative Syndrome Scale (PANSS) for clinical symptom, Social Functioning Scale (SFS), and Global Assessment of Functioning (GAF) scale for social functioning.
Result:  The scores for delusions (components of positive syndrome), anxiety and depression (components of general psychopathology) on the PANSS significantly correlated with QoL and subjective well-being scores. In contrast, the scores for components of negative syndrome were not correlated with the subjective assessment scores. Furthermore, none of the clinical symptom scores were correlated with the score in self-efficacy scale. The SFS and GAF scores were significantly correlated with the subjective assessment scores. There were significant correlations among the scores on the three subjective assessment scales.
Conclusion:  Each scale has different features and should be utilized depending upon the expected effect of treatment or the purpose of assessment. The treatments provided to patients must be directed at improving both psychological and social impairments, in order to enhance the social functioning and QoL of patients.     

Gliovascular disruption and cognitive deficits in a mouse model with features of small vessel disease

Cerebral small vessel disease (SVD) is a major cause of age-related cognitive impairment and dementia. The pathophysiology of SVD is not well understood and is hampered by a limited range of relevant animal models. Here, we describe gliovascular alterations and cognitive deficits in a mouse model of sustained cerebral hypoperfusion with features of SVD (microinfarcts, hemorrhage, white matter disruption) induced by bilateral common carotid stenosis. Multiple features of SVD were determined on T2-weighted and diffusion-tensor magnetic resonance imaging scans and confirmed by pathologic assessment. These features, which were absent in sham controls, included multiple T2-hyperintense infarcts and T2-hypointense hemosiderin-like regions in subcortical nuclei plus increased cerebral atrophy compared with controls. Fractional anisotropy was also significantly reduced in several white matter structures including the corpus callosum. Investigation of gliovascular changes revealed a marked increase in microvessel diameter, vascular wall disruption, fibrinoid necrosis, hemorrhage, and blood–brain barrier alterations. Widespread reactive gliosis, including displacement of the astrocytic water channel, aquaporin 4, was observed. Hypoperfused mice also demonstrated deficits in spatial working and reference memory tasks. Overall, gliovascular disruption is a prominent feature of this mouse, which could provide a useful model for early-phase testing of potential SVD treatment strategies.     

Tympanoplasty on only hearing ears

The management of only hearing ears remains controversial because of the risk of postoperative hearing deterioration. We reviewed 12 cases of hearing ears alone operated on at the Hyogo College of Medicine Department of Otolaryngology from 1999 to 2002. All ears were dry at the final examination. Postoperative hearing results were evaluated based on the guideline of the Japan Otological Society (2000). An air bone gap within 15 dB was found in 9 cases (75%), hearing gain exceeding 15 dB was found in 6 cases (50%), the hearing level was within 30 dB in 4 cases (33.3%), and total successful cases numbered 10 (83.3%). Eight patients wore hearing aids before surgery, and 4 patients (50%) did not need them after the surgery. We suggest that tympanoplasty by skillful surgeons should be done to obtain a dry ear, to avoid a progressive hearing loss, to preserve or improve hearing, and to improve the quality of life. We also suggest that handling of ossicles should minimized in chronic otitis media and cholesteatoma.