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ObjectiveThe severity of cerebral small vessel disease (SVD) is assessed through neuroimaging findings, including hypertensive arteriopathy (HA)-SVD and cerebral amyloid angiopathy (CAA)-SVD. HA-SVD and CAA-SVD have been collectively estimated as total scores: the HA-SVD and CAA-SVD scores, respectively. Previous reports suggest that HA-SVD scores are associated with cognitive function; however, the relationship between CAA-SVD scores and cognitive function remains unclear. Therefore, we examined the association between CAA-SVD scores and cognitive function. Furthermore, we developed a modified CAA-SVD score considering cortical microinfarcts and posterior dominant white matter hyperintensities, which are imaging findings of CAA, and examined the association between these scores and cognitive function in the same patient group.DesignProspective study.SettingSingle centre study from a memory clinic.ParticipantsSubjects were diagnosed with mild cognitive impairment (MCI) or mild dementia in our memory clinic between February 2017 and July 2019 and underwent clinical dementia rating scale and brain MRI assessment. A total of 42 patients (aged 75.3±9.12 years) were registered prospectively.Primary and secondary outcome measuresWe evaluated intellectual function, memory, frontal lobe function and constructional ability. Furthermore, the relationship between each score and cognitive function was examined.ResultsThe CAA-SVD score showed significant associations with cognitive function (R2=0.63, p=0.016), but the HA-SVD score did not (R2=0.41, p=0.35). The modified CAA-SVD score was also significantly associated with cognitive function (R2=0.65, p=0.008).ConclusionCognitive function is associated with the CAA-SVD score, and more efficiently with the modified CAA-SVD score, in memory clinic patients. Although we have not validated the weighting of the modified CAA-SVD score, these scores can be a predictor of cognitive deterioration in patients with MCI and mild dementia.

Corticobasal syndrome (CBS) is characterized by unilateral atrophy of the brain. New diagnostic criteria for CBS include intermediate somatosensory dysfunction. Here, we aimed to carefully examine intermediate somatosensory function to identify tests which can assess impairment in CBS patients. Using voxel-based morphometry (VBM), we also aimed to show the anatomical bases of these impairments. Subjects included 14 patients diagnosed with CBS and 14 patients with Parkinson's disease (PD). Patients were evaluated using intermediate somatosensory tests and neuropsychological assessments. VBM was used to analyze differences in gray matter volumes between CBS and PD patients. In the PD group, no tests showed a significant difference between the dominant-side onset and the non-dominant-side onset. In the CBS group, all tests showed worse scores on the affected side. For detecting intermediate somatosensory dysfunction in CBS, two tests are recommended: tactile object naming and 2-point discrimination. VBM analysis showed that the volume of the left post- and pre-central gyrus, and both sides of the supplementary motor area were significantly decreased in the CBS group compared to the PD group. Although CBS remains untreatable, early and correct diagnosis is possible by performing close examination of intermediate somatosensory function.

To assess the long-term effects of tadalafil, a therapeutic agent for fetal growth restriction (FGR), we evaluated the developmental progress of 1.5-year-old infants whose mothers had taken tadalafil during pregnancy. Twenty-four infants were assessed. We evaluated infant body weight, height, and head circumference, and performed the Kyoto Scale of Psychological Development (KSPD) test, a standardized developmental assessment covering Postural&ndash, Motor (P&ndash, M), Cognitive&ndash, Adaptive (C&ndash, A), and Language-Social (L&ndash, S) functions. The sum score was converted to a developmental quotient (DQ). The mean gestational week of the included cases was 36.1 (29&ndash, 39) weeks, and the mean birth weight was 1841 (874&ndash, 2646) g. Twenty-one and 20 out of the 24 cases, respectively, attained body weight and height similar to those of age-matched normal infants (within the 3rd percentile), all cases caught up in head circumference. KSPD was performed for 18 cases at 1.5 years of corrected age. The mean DQ scores were 87 (in total): 82 in P&ndash, M, 90 in C&ndash, A, and 88 in L&ndash, S. The total DQ score in one case (5.6%) was less than 70, and ranged from 70 to 85 in five cases (27.7%), and was more than 85 in 11 cases (61.1%). The growth and development of infants born of tadalafil-treated mothers seem to show good progress at a corrected age of 1.5 years.

Somatosensory functions are subdivided into two large groups: the elementary somatosensory functions, which consist of light touch, pain, thermal sensation, joint position sense and vibration sense, and the intermediate somatosensory functions, which include two-point discrimination, tactile localisation, weight, texture and shape perception. It is generally accepted that the functional localisation of somatosensory function is the postcentral gyrus, but some researchers suggested that the insula is also involved in somatosensory function.1 Insula is a multimodal area and has a major role as a convergence zone implicated in the coordination between internal and external information through emotional subjective awareness. However, the subregional specificity of somatosensory functions in the insula has not been fully elucidated.2 We experienced three cases of stroke restricted exactly to the subregions of the insula which showed heterogeneous somatosensory dysfunction. For these patients, we performed a close examination of the intermediate somatosensory function, and discussed the relation between the insula and intermediate somatosensory function. ### Patient 1 An 81-year-old, right-handed woman who had difficulty speaking in February 2017 was transferred to our hospital. MRI revealed a lesion in the right middle insula (figure 1A). The symptom improved at the time of hospital arrival and she underwent the best medical treatment. The symptom disappeared on the next day. Intelligence and elementary somatosensory functions were normal. The results of the intermediate somatosensory examinations showed that two-point discrimination, tactile localisation and graphesthesia were significantly impaired. Figure 1 MRI findings of the cases presented. (A) Patient 1 had a cortical lesion in the right middle insula. (B) Patient 2 had a cortical lesion in the …

We reported a 60 year-old man with Churg-Strauss syndrome (CSS). Three months later, he presented with dysarthria, dysphagia and severe headache. We detected glossopharyngeal and vagal nerve palsy, and made a diagnosis of cranial nerve involvement comorbid with CSS. Intravenous administration of methypredonisolone was effective for alleviating clinical signs and symptoms. Two months later, he complained of headache and facial numbness, but symptoms improved with an escalating dose of prednisolon. As compared to previously reported cases, our case was characteristic because of involvement of lower cranial nerve with CSS, which has been reported previously in only one case.

Background The Kii peninsula of Japan is one of the foci of amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in the world. The purpose of this study is to clarify the neuropsychological features of the patients with ALS/PDC of the Kii peninsula (Kii ALS/PDC). Methods The medical interview was done on 13 patients with Kii ALS/PDC, 12 patients with Alzheimer’s disease, 10 patients with progressive supranuclear palsy, 10 patients with frontotemporal lobar degeneration and 10 patients with dementia with Lewy bodies. These patients and their carer/spouse were asked to report any history of abulia-apathy, hallucination, personality change and other variety of symptoms. Patients also underwent brain magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT), and neuropsychological tests comprising the Mini Mental State Examination, Raven’s Colored Progressive Matrices, verbal fluency, and Paired-Associate Word Learning Test and some of them were assessed with the Frontal Assessment Battery (FAB). Results All patients with Kii ALS/PDC had cognitive dysfunction including abulia-apathy, bradyphrenia, hallucination, decrease of extraversion, disorientation, and delayed reaction time. Brain MRI showed atrophy of the frontal and/or temporal lobes, and SPECT revealed a decrease in cerebral blood flow of the frontal and/or temporal lobes in all patients with Kii ALS/PDC. Disorientation, difficulty in word recall, delayed reaction time, and low FAB score were recognized in Kii ALS/PDC patients with cognitive dysfunction. Conclusions The core neuropsychological features of the patients with Kii ALS/PDC were characterized by marked abulia-apathy, bradyphrenia, and hallucination.