Neuronal intranuclear inclusion disease (NIID) has highly variable clinical manifestations. Sone et al. describe the clinical and pathological features of 57 adult-onset cases diagnosed by postmortem dissection/antemortem skin biopsy. They report ‘dementia dominant’ and ‘limb weakness’ subtypes, and recommend consideration of NIID in the differential diagnosis of leukoencephalopathy and neuropathy.

The rate-limiting step in ribosome biogenesis is the transcription of ribosomal RNA, which is controlled by environmental conditions. The JmjC enzyme KDM2A/ JHDM1A/FbxL11 demethylates mono-and dimethylated Lys 36 of histone H3, but its function is unclear. Here, we show that KDM2A represses the transcription of ribosomal RNA. KDM2A was localized in nucleoli and bound to the ribosomal RNA gene promoter. Overexpression of KDM2A repressed the transcription of ribosomal RNA in a demethylase activity-dependent manner. When ribosomal RNA transcription was reduced under starvation, a cellpermeable succinate that inhibited the demethylase activity of KDM2A prevented the reduction of ribosomal RNA transcription. Starvation reduced the levels of mono-and dimethylated Lys 36 of histone H3 marks on the rDNA promoter, and treatment with the cell-permeable succinate suppressed the reduction of the marks during starvation. The knockdown of KDM2A increased mono-and dimethylated Lys 36 of histone H3 marks, and suppressed the reduction of ribosomal RNA transcription under starvation. These results show a novel mechanism by which KDM2A activity is stimulated by starvation to reduce ribosomal RNA transcription.

Objectives-To determine the relation between severity of obstructive sleep apnoea (OSA) and degree of cerebral metabolic impairment. Methods-Fifty five patients with habitual snoring and excessive daytime sleepiness underwent standard overnight polysomnography and magnetic resonance spectroscopy separately. Proton MR spectra were measured with two dimensional chemical shift imaging (repetition time; 1500 ms, echo time; 135 ms). Severity of cerebral metabolic impairment was assessed by the N-acetylaspartate (NAA)/choline ratios for the cerebral cortex and white matter. Severity of OSA was assessed by the apnoea-hypopnoea index (AHI) and the minimum value of peripheral oxyhaemoglobin saturation. All patients were evaluated for the presence or absence of comobidities including hypertension, cardiac disease, diabetes mellitus, and hyperlipidaemia. Univariate analysis of variance (ANOVA) and mulitple linear regression analysis were used for statistical analyses. Results-Univariate ANOVA disclosed significant eVects of AHI, age, and the presence or absence of hypertension on the NAA/choline ratio for cerebral white matter (p=0.011, p=0.028, p=0.0496, respectively). The AHI had a significant negative association with the NAA/choline ratio for cerebral white matter, independent of age and the presence or absence of cardiac disease, in the final multivariate regression model (standardised partial regression coeYcient=−0.417, p<0.001). No significant relation was found between severity of OSA and the NAA/choline ratio for the cerebral cortex. Age alone had a significant eVect on the NAA/choline ratio for the cerebral cortex on univariate ANOVA (p<0.001) and a significant negative association with the NAA/choline ratio for the cerebral cortex in the regression model (r=−0.552, p<0.001). Conclusions-A significant relation exists between AHI and the degree of metabolic impairment in cerebral white matter in patients with OSA. (J Neurol Neurosurg Psychiatry 2001;71:334-339) Keywords: magnetic resonance spectroscopy; sleep apnoea syndromes; white matter disease Repeated sleep apnoeic episodes may lead to CNS impairment in patients with obstructive sleep apnoea (OSA). Excessive daytime sleepiness and cognitive and emotional deficits are common daytime symptoms of OSA.1-6 A multiple sleep latency test and maintenance of wakefulness test have been used to quantify daytime sleepiness.7 8 Neuropsychological and electrophysiological tests have been used to quantify cognitive dysfunction.1-6 9-11 Hypoxic brain damage and fragmentation of sleep are generally thought to be causes of these deficits. 2-6 8 9Magnetic resonance spectroscopy (MRS) enables non-invasive evaluation of focal metabolic changes in various conditions aVecting the CNS. We previously reported cerebral metabolic changes in patients with OSA.12 The N-acetylaspartate (NAA)/choline ratio for cerebral white matter was significantly lower in patients with moderate to severe OSA than in patients with mild OSA and healthy subjects, indicating axonal injury or gli...

Background: With the striking increase in the number of elderly people in Japan, dementia has not only become a medical but also a social issue. Methods: We studied the prevalence of dementing disorders in a rural island town of Japan (Ama-cho), using a door-to-door 2-phase design. Results: Of the 120 persons screened as having cognitive impairment, 104 people were diagnosed as having dementia. The prevalence (cases/100 persons aged 65 years and older) was 11.0 for all types of dementia, 7.0 for Alzheimer’s disease, 1.7 for vascular dementia, 0.53 for dementia with Lewy bodies, 0.74 for Parkinson’s disease dementia, 0.21 for progressive supranuclear palsy, 0.11 for frontotemporal lobar degeneration and 0.74 for other dementia. The overall prevalence was higher in women for Alzheimer’s disease and Parkinson’s disease dementia, and in men, for vascular dementia and dementia with Lewy bodies. Conclusion: We confirmed the overall prevalence of dementia in the elderly population aged 65 years and older to be 11.0. This finding is higher compared with previous reports in Japan.

A new method for the catalytic aldol reaction to ketones, using CuF.3PPh3.2EtOH complex as the catalyst and (EtO)3SiF as the additive, is described. The reaction can be applied to a wide range of ketones and trimethylsilyl enolates. On the basis of mechanistic studies, a working hypothesis for the catalytic cycle is proposed, in which the dynamic ligand exchange mediated by copper silicates produces the active copper enolate. Moreover, the present reaction can be extended to the catalytic enantioselective reaction using tol-BINAP as a chiral ligand.