Futaba Maki

Progression of Intracranial Major Artery Stenosis is Associated with Baseline Carotid and Intracranial Atherosclerosis

AIM Intracranial atherosclerotic major artery stenosis (IMAS) is associated with a high risk of ischemic stroke. Carotid ultrasound (US) has been widely used to evaluate an individuals atherosclerotic burden, but no information is available on whether the carotid US findings are associated with IMAS progression. The aim of the present study was to identify the associations among traditional risk factors, the duplex carotid US findings and IMAS progression in patients with varying degrees of carotid atherosclerosis. METHODS All patients who underwent a set of imaging studies (MRI, MRA and carotid US) in our outpatient clinic were screened. A total of 101 patients with a mean age of 75.0±10.6 years, who received the same imaging studies with a mean interval of two years, were studied. In each patient, the extent of stenosis of three arteries (both middle cerebral arteries [MCAs] and the basilar artery [BA]) was classified into five grades. The total score of the three arteries was calculated as the global stenosis score (GSS). The progression of IMAS was defined as worsening of stenosis by ≥1 grade on final MRA. The maximum IMT (maxIMT), plaque findings and carotid stenosis were measured by carotid US. A multivariate stepwise logistic regression analysis was used to identify independent predictors of IMAS progression. RESULTS Follow-up MRA revealed IMAS progression in 12 patients (11.9%). The logistic regression analysis demonstrated that the baseline GSS (p=0.008) and carotid stenosis ≥70% on the baseline carotid US (p=0.023) were significantly associated with IMAS progression. CONCLUSIONS The baseline severity of intracranial and extracranial atherosclerosis was significantly associated with the progression of IMAS.

MRI-based cerebellar volume measurements correlate with the International Cooperative Ataxia Rating Scale score in patients with spinocerebellar degeneration or multiple system atrophy

BackgroundProgression of clinical symptoms and cerebellar atrophy may vary among subtypes of spinocerebellar degeneration and multiple system atrophy. The aim of this cross-sectional study was to demonstrate the relationship between the International Cooperative Ataxia Rating Scale (ICARS) score and cerebellar volume derived from magnetic resonance imaging (MRI) in a broad spectrum of Japanese patients with cerebellar ataxia.MethodsA total of 86 patients with cerebellar ataxia (18 with cortical cerebellar atrophy, 34 with spinocerebellar ataxia, and 34 with multiple system atrophy) and 30 healthy subjects were studied. MRI-based cerebellar volume measurements were performed in all subjects using T1-weighted images acquired with a 1.5-T MRI scanner. The cerebellar volume/cranial anteroposterior (AP) diameter was used for statistical analysis.ResultsStepwise multiple regression analyses demonstrated that cerebellar volume/cranial AP diameter and midbrain AP/cranial AP diameter were significantly associated with the total score and domain I sub-score of ICARS. We found no interactions between these two anatomical factors in the ICARS total and domain I sub-scores. The main effects of these two predictors were statistically significant both in total and domain I sub-scores (p = 0.001 and 0.022, respectively).ConclusionsCerebellar volume and midbrain AP diameter normalized to the cranial AP diameter were significantly correlated with the ICARS total and domain I sub-scores. Further longitudinal studies are warranted to explore the role of these MRI biomarkers for predicting disease progression.

Familial Adult-Onset Alexander Disease with a Novel GFAP Mutation

The patient was a 65‐year‐old woman who became gradually more prone to falling from age 30 and who was visiting the hospital on an outpatient basis following a diagnosis of multiple system atrophy, cerebellar type. While eating, she started choking as a result of aspiration and was transported to our hospital by ambulance. Head magnetic resonance imaging (MRI) revealed tadpole‐like atrophy of the brainstem, i.e. marked atrophy of the medulla oblongata and cervical spinal cord with disproportionately slight atrophy of the pons. Her eldest son also had the same symptoms, suggesting Alexander disease. A search of the glial fibrillary acidic protein gene revealed the previously unreported mutation Y242N. The same MRI findings and genetic mutation were confirmed in her 38‐year‐old son. Adult onset Alexander disease is a rare condition with very few reported familial cases. We hereby report this case with a discussion of the literature.

Overnight Monitoring of Turnover Movements in Parkinsons DiseaseUsing A Wearable Three-Axis Accelerometer

Background: In patients with Parkinsons disease (PD), the impairment of voluntary and involuntary movement during sleep might affect their natural sleep and quality of life; however, there is no reliable method to evaluate turnover movements during sleep. We aimed to clarify whether overnight monitoring of turnover movements in bed using a wearable three-axis accelerometer is a feasible and reliable tool for evaluating the impact of motor complications during sleep in PD. Methods: The number of turnover movements in bed was counted based on the graphic pattern in the X, Y, and Z axis using threshold values in each axis to discriminate turnover movements from other movements mainly associated with respiration or cough. These threshold values were defined by the recordings of various turnover movements in normal volunteers. Overnight monitoring of turnover movements in bed from 9:00 pm to 7:00 am was performed in 7 normal volunteers and 5 patients (mean age, 76.4 ± 4.6 yrs, Hoehn-Yahr stage, 3.6 ± 0.5; duration of disease, 8.8 ± 5.6 yrs). In patients with PD, monitoring was performed before and after adjustment of anti-Parkinson medications. Results: The number of turnover movements was significantly more restricted in PD before drug control than in control subjects (p=0.005). The median number of overnight turnover movements increased from 0 to 5 times after drug control in patients with PD. The number of overnight turnover movements increased significantly in all 5 patients after adjusting their anti-Parkinson medications (p=0.041). Conclusion: Overnight monitoring of turnover movements in bed using a wearable three-axis accelerometer is feasible. Further studies are warranted to evaluate the impact of movement disorders during sleep on patients with PD.

Cognitive impairment of Japanese multiple sclerosis patients: Follow-up study using BRB-N assessment tool

OBJECTIVES The purpose of this study was to evaluate cognitive function in patients with multiple sclerosis (MS), compared with control subjects, and to establish whether decline of cognitive function continues in the patients during remission. METHODS The Japanese version of the Brief Repeatable Battery of Neuropsychological tests (BRB-N), which includes the selective reminding test (SRT), spatial recall test (SPART), symbol digit modalities test (SDMT), paced auditory serial addition test (PASAT), and the word list generation test (WLG), was performed in 34 Japanese patients with MS (8 males, 26 females; mean age, 42 years) and in 37 age- and education-matched healthy controls (20 males, 17 females; mean age, 36 years). BRB-N was conducted at intervals of two years for MS patients who remained in remission, and the cognitive changes were evaluated by comparing the results with those of the initial examination at entry into the study. RESULTS The MS patients showed lower BRB-N scores than controls, with high significance in the SRT, SRT-D, SDMT and PASAT tests (p<0.01). The BRB-N scores of the MS patients who remained in remission were not significantly changed for at least 2 years. CONCLUSIONS The Japanese version of the BRB-N is useful to clarify the nature of cognitive impairment in Japanese MS patients. Based on this neuropsychological assessment, we suggest that working memory and information-processing speed are key deficits. Patients who remained in remission showed little or no further impairment of cognitive functions for at least two years.

Therapeutic efficacy of transcranial magnetic stimulation for amyotrophic lateral sclerosis and spinocerebellar degeneration

Abstract We treated four patients with amyotrophic lateral sclerosis (ALS) and nine patients with spinocerebellar degeneration (SCD) using transcranial magnetic stimulation (TMS) with an SMN-1200 transcranial magnetic stimulator. The stimulus strength was below 1.5 T and the interpulse interval was greater than 5 s. The stimulus coil was placed on P3, P4 and the bilateral cervical and lumbar root in the ALS patients, and tangentially over Iz, 4 cm lateral to the right from Iz and 4 cm lateral to the left from Iz in the SCD patients. Ten consecutive pulses were delivered to each region in the ALS patients and 15 in the SCD patients. Transcranial magnetic stimulation (TMS) was applied for a total of 20 days for in-patients and once or twice a week as ongoing therapy for outpatients. Patients with ALS were assessed according to the Barthel index, and a respiratory function test was performed before and after TMS. Patients with SCD were neurologically assessed according to the International Cooperative Ataxia Rating Scale (ICARS). In addition, the patients were evaluated for regional cerebral blood flow (rCBF) by 99 m Tc-ECD SPECT using a modified Patlak-plot method before and after the TMS trial. No change in the Barthel index or rCBF was observed in patients with ALS. The respiratory function test results improved in one patient in the ALS group. The ICARS rating of seven patients with SCD improved after TMS. The rCBF of patients with SCD in the bilateral cerebellar hemispheres was significantly faster compared to their flow rate before TMS. These data suggest the potential usefulness of TMS as a therapeutic tool for ALS and SCD.