A case of unusual renal manifestation in a patient with neuronal intranuclear inclusion disease treated with steroids.

Neuronal intranuclear inclusion disease (NIID) is a progressive neurodegenerative disorder characterized by intranuclear inclusions. Kidney injury involvement and successful treatment for NIID have rarely been reported. A NIID patient developed crescentic IgA nephropathy. Steroid therapy resolved digestive symptoms and recovered renal function. Steroids are considered for concomitant symptoms of NIID.

The Usefulness of Thin-section Iso-Voxel Diffusion Weighted Imaging for Stroke Subtype Classification: Case Series and Review.

BACKGROUND: Determining stroke subtypes on initial clinical evaluation is a prerequisite for the selection of appropriate initial treatment. Although diffusion-weighted imaging (DWI) is a powerful tool for detection of acute cerebral infarction, its diagnostic accuracy is not always sufficient particularly in the hyperacute phase. METHODS: Patients admitted within 2 weeks from the symptom onset with the diagnosis of acute ischemic strokes were analyzed with thin-section iso-voxel DWI, namely 3-dimension DWI (3D-DWI), to obtain axial, coronal, and sagittal sections in order to elucidate stroke characteristics. In this case series, we introduce the effectiveness of 3D-DWI. RESULTS: 3D-DWI uncovered stroke subtypes and distribution more precisely compared with conventional DWI. While previous studies indicated the utility of thin section DWI in detecting infratentrial infarctions, 3D-DWI is beneficial for the detection of not only infratentrial but also supratentorial lesions. Furthermore, since both 3D-DWI and magnetic resonance angiography (MRA) are multiplanar reconstruction images, the fusion image of 3D-DWI with MRA is available, enabling cross-reference of spatial cerebrovascular configuration and ischemic lesions. CONCLUSIONS: 3D-DWI is applicable to standard 1.5 T MRI by slight modification of data acquisition protocols, and becomes a key modality to solve the diagnostic puzzle of acute ischemic strokes.

A case of amyloid myopathy diagnosed during the treatment of myopathy associated with anti-signal recognition particle antibodies.

A 78-year-old man presented with subacute progressive proximal weakness and dysphagia. A biopsy specimen from the left biceps femoris revealed evidence of necrotic and regenerating muscle fibers, but lymphocyte infiltration was not noted. The patient was diagnosed with necrotizing myopathy with anti-signal recognition particle (SRP) antibodies. Concomitant therapy with prednisolone and azathioprine caused the serum CK level to return to normal and it caused clinical manifestations to abate. One year later, however, muscle weakness worsened. Immunoelectrophoresis of serum revealed IgG M protein, and muscle pathology revealed amyloid deposits in numerous blood vessels and at the periphery of a few muscle fibers, and deposits stained positive for anti-λ light chain antibody. The patient was diagnosed with amyloid myopathy, and therapy for systemic amyloid light chain amyloidosis caused muscle weakness to diminish. Amyloidosis is believed to be the primary pathology in this case based on the patient's response to treatment reaction, but the significance of a case involving both amyloid myopathy and necrotizing myopathy warranted examination.

Peripheral Vitamin C Levels in Alzheimer's Disease: A Cross-Sectional Study.

We previously reported lower lymphocyte vitamin C levels in individuals with type 2 diabetes mellitus and in individuals with severe Parkinson's disease. Oxidative stress has been proposed to play a key role in the progression of Alzheimer's disease. Thus, the objective of this study was to investigate the association between peripheral levels of vitamin C and the progression of cognitive dysfunction in Alzheimer's disease. Fifty individuals with Alzheimer's disease being treated at Shizuoka General Hospital were consecutively enrolled in this study from December 2009 to March 2015 (76.0±9.7 y of age [mean±SD]; 32 men and 18 women; Mini-Mental State Examination Japanese version (MMSE-J) score range, 8-27). Plasma and lymphocyte vitamin C levels in fasting blood samples were measured. The association between the MMSE-J scores and vitamin C levels was estimated using Spearman's rank correlation coefficient (ρ) and the criteria defined by Swinscow. Spearman's ρ for the relationship between peripheral vitamin C levels and the MMSE-J score was ρ=0.17 for plasma vitamin C and ρ=0.26 for lymphocyte vitamin C. Thus, the associations were relatively weak based on the criteria. In contrast with type 2 diabetes mellitus and Parkinson's disease, lymphocyte vitamin C levels in the peripheral blood may not directly reflect the progression of cognitive dysfunction in Alzheimer's disease. Additional longitudinal studies are needed to evaluate the clinical importance of changes of peripheral vitamin C status in Alzheimer's disease.

Lymphocyte vitamin C levels as potential biomarker for progression of Parkinson's disease.

OBJECTIVES: Vitamin C is a major antioxidant and also is known as a neuromodulator in dopaminergic neurons. The aim of this study was to investigate the association between lymphocyte and plasma vitamin C levels in various stages of Parkinson's disease (PD). METHODS: Sixty-two individuals with PD (age 71 ± 8.8 y [mean ± SD]) being treated at Shizuoka General Hospital from December 2007 to August 2013 were consecutively recruited. PD severity was classified using the Hoehn-Yahr scale for staging PD. Fasting blood samples were collected, and plasma and lymphocyte vitamin C levels were measured. The association between PD severity and vitamin C levels was estimated by ordinal logistic regression with confounding variables. RESULTS: The distribution of Hoehn-Yahr stages in patients was as follows: stage I, 7; II, 28; III, 16; and IV, 11. Lymphocyte vitamin C levels in patients with severe PD were significantly lower (odds ratio [OR], 0.87; 95% confidence interval [CI], 0.80-0.97; P < 0.01) compared with those at less severe stages. Plasma vitamin C levels also tended to be lower in patients with severe PD; however, this was not significant (OR, 0.98; 95% CI, 0.96-1.00; P = 0.09). CONCLUSIONS: Our findings suggest that lymphocyte vitamin C levels in the peripheral blood may be a potentially useful biomarker for the progression of PD.

Thalamocortical disconnection in the orbitofrontal region associated with cortical thinning in schizophrenia.

CONTEXT: Dysfunction of the thalamocortical pathway has been proposed as a putative underlying pathology of schizophrenia. Although the mechanisms involved remain unclear, postmortem studies suggest the involvement of altered neural projections from the thalamus to layers within the prefrontal cortex. OBJECTIVES: To investigate thalamocortical connectivity in schizophrenia and to examine its possible association with cortical thinning in vivo. DESIGN: Case-control cross-sectional study. SETTING: Department of Psychiatry at Kyoto University Hospital, Japan. PATIENTS AND OTHER PARTICIPANTS: A total of 37 patients with schizophrenia and 36 age-, sex-, and education-matched healthy controls recruited from the local community underwent diffusion-weighted imaging and T1-weighted 3-dimensional magnetic resonance imaging. MAIN OUTCOME MEASURES: Probabilistic tractography was performed to investigate thalamocortical pathways. Group differences in mean fractional anisotropy (FA) values were examined in the entire thalamocortical pathway, the thalamolateral prefrontal pathway, the thalamomedial prefrontal pathway, and the thalamo-orbitofrontal pathway. Surface-based analysis was performed to investigate cortical thickness, and the correlation between FA values and cortical thickness was examined. RESULTS: The patient group exhibited reduced FA values within the right thalamo-orbitofrontal pathway (P < .05 for the 8 group comparisons of FA, Bonferroni correction). In the patient group only, the mean FA value for this pathway was positively correlated with thickness of the right frontal polar and lateral orbitofrontal cortices (P < .05, clusterwise correction). CONCLUSIONS: These results suggest that, in schizophrenia, regional thalamocortical white matter pathology is specifically associated with cortical pathology in regions where fibers connect.

Age-related cortical thinning in schizophrenia.

Although the effects of aging on the neural correlates of schizophrenia have been researched for many years, no clear conclusion has been reached. While some studies have demonstrated progressive age-related gray matter reductions in schizophrenia, other studies have not found evidence of progression. Moreover, it remains unclear whether the influence of aging on global or regional cortical thickness differs between schizophrenia patients and healthy controls. This study aimed to confirm previous reports of reduced cortical thickness in schizophrenia, and to investigate the effects of age on global and regional cortical thickness. Eighty-three patients with schizophrenia (six first-episode patients and 77 chronic patients; age range=18-55 years) and 90 age-, gender- and education-matched healthy controls (age range=19-56 years) underwent structural magnetic resonance imaging (MRI) using a 3-Tesla scanner. Surface-based analysis was applied to assess cortical thickness in the whole brain. The patient group exhibited both global and regional cortical thinning in regions including the prefrontal and temporal cortices. The correlation between age and cortical thickness showed a similar pattern in patients and controls, both globally and regionally. These results suggest that the reduction of cortical thickness in schizophrenia might not be progressive over the course of the illness, indicating that pathological processes occur in a relatively limited period of time around the onset of illness.

Correlation between cognitive deficits and glucose hypometabolism in mild cognitive impairment.

BACKGROUND: In patients with mild cognitive impairment (MCI), poor performances on delayed recall and executive function are risk factors of progression to dementia. The aim of the present study was to clarify neural correlates of these neuropsychological deficits. METHODS: Thirty patients with amnestic MCI and 15 control subjects underwent neuropsychological tests including three-word delayed recall, visual delayed recall of Rey complex figure (RCF), and two-relational reasoning of Raven's colored progressive matrices (RCPM) with a (18)F-fluorodeoxyglucose (FDG)-position emission tomography (PET) measurement of resting state. We evaluated a relationship between performance of neuropsychological tests and regional cerebral glucose metabolism using voxel-based analysis. RESULTS: Poor performance in three-word delayed recall was related to glucose hypometabolism in the right medial temporal, right prefrontal, and left superior parietal cortices. The deficit in visual delayed recall of RCF correlated positively with hypometabolism in the bilateral posterior cingulate. The impairment in two-relational reasoning was associated with hypometabolism in the right prefrontal cortex. CONCLUSIONS: The present findings suggest that hypometabolism in the right medial temporal cortex, right prefrontal cortex, left superior parietal cortex, and bilateral posterior cingulate reflects impairments in delayed recall while hypometabolism in the right prefrontal cortex mirrors deficits in executive function in MCI.

Small orbitofrontal traumatic lesions detected by high resolution MRI in a patient with major behavioural changes.

We report a case of a male patient who showed personality changes and marked social problems after a traumatic brain injury. Although suspected to have lesions in the orbitofrontal cortex because of the typical characteristics of his behavioural change, lesions were not apparent using conventional imaging techniques. However, investigation using high-resolution MRI revealed lesions in the orbitofrontal cortex. Our case suggests that standard MRI scanning techniques may have only limited power. Hence, we stress the important role played by qualitative assessments of emotion, personality, and social behaviour in evaluating sequelae of traumatic orbitofrontal injuries.

Quantification of nicotinic acetylcholine receptors in Parkinson's disease with (123)I-5IA SPECT.

We quantified in vivo brain nicotinic acetylcholine receptor (nAChR) distributions in patients with Parkinson's disease (PD) and evaluated correlations between nAChR distributions and clinical variables of the patients, especially dopaminergic medications. Ten patients with PD without dementia underwent 5-(123)I-iodo-3-(2(S)-azetidinylmethoxy)pyridine ((123)I-5IA) single photon emission computed tomography (SPECT) and the data were compared with those of 10 age-matched healthy volunteers. Correlation analyses between (123)I-5IA distribution volumes (DVs) in each brain region and clinical variables of the patients were also performed. The PD group showed a statistically significant decrease (20-25%) in the brainstem and frontal cortex as compared with the control group. Although age, duration of disease, daily dose of levodopa, duration of PD medication use, and scores on the motor section of Unified Parkinson's Disease Rating Scale were not significantly correlated with DV values in any brain regions, high daily doses of dopamine agonist showed a significant negative correlation with DVs in the cerebellum, and temporal, parietal and occipital cortices. These findings suggest that patients with PD without dementia can show reductions especially in the brainstem and frontal cortex. They also suggest that dopamine agonists can have a negative influence on the distribution of nAChRs.

Efficient plant regeneration from leaves of rapeseed (Brassica napus L.): the influence of AgNO3 and genotype.

Factors influencing reliable shoot regeneration from leaf explants of rapeseed (Brassica napus L.) were examined. Addition of AgNO(3) to callus induction medium was significantly effective for shoot regeneration in all three genotypes initially tested. When 48 genotypes subsequently were surveyed, a large variation of shoot regenerability was observed, ranging from 100 to 0% in frequency of bud formation and from 7.5 to 0 in the number of buds per explant. A significant correlation (r=0.84) was observed between the frequency of bud formation and the number of buds per explant. The shoot regenerability from leaf explants was not related to that from cotyledonary explants (r=0.28). Histological observations showed that an organized structure developed from calluses produced at vascular bundle tissues after 7 days of culture on callus induction medium, and they developed shoot apical meristems one week after transfer onto shoot induction medium. Regenerated plantlets were obtained 2 months after the initiation of culture and they normally flowered and set seeds. No alterations of morphology or DNA contents were observed in regenerated plants and their S1 progenies.

Prediction of psychiatric response to donepezil in patients with mild to moderate Alzheimer's disease.

Donepezil is a selective acetylcholinesterase inhibitor approved for the symptomatic treatment of mild to moderate Alzheimer's disease (AD). Since behavioral symptoms severely affect quality of life for AD patients and their caregivers, predicting behavioral responses to donepezil will be useful in managing patients with AD. In this study, we analyzed 70 consecutive cases with mild to moderate AD. Caregivers were interviewed with the Neuropsychiatric Inventory for behavioral assessment and 4-point improvement at week 12 was accepted as a treatment response. Twenty-one (30.0%) patients showed a behavioral response, while 42 (60.0%) showed no behavioral change and 7 (10.0%) worsened. Dysphoria, anxiety and apathy significantly improved after treatment among the responder group. The baseline profile including age, sex, Mini-Mental State Examination (MMSE), the Alzheimer's Disease Assessment Scale (ADAS-cog) and the Geriatric Depression Scale did not differ significantly among the three groups. Statistical Parametric Mapping analysis of single photon emission computed tomography (SPECT) images at baseline showed that cerebral blood flow in the premotor and parietotemporal cortices was significantly higher in the responder group than in the worse group. The present study suggested usefulness of SPECT imaging in the prediction of behavioral response to donepezil among AD patients even with similar psychiatric symptoms and cognitive functions.