Effectiveness and safety of MR-guided focused ultrasound thalamotomy in patients with essential tremor and low skull density ratio: a study of 101 cases.

OBJECTIVE: The objective was to investigate the effectiveness and safety of MR-guided focused ultrasound (MRgFUS) treatment in patients with essential tremor, particularly those with low skull density ratio (SDR) and including those with very low SDR, and to identify the factors influencing treatment effectiveness and to provide insights into therapeutic approaches for patients with lower SDR. METHODS: Real-world data from 101 patients who underwent MRgFUS between July 2019 and March 2022 at a single institution were analyzed. Tremor severity was assessed using the Fahn-Tolosa-Marin Clinical Rating Scale for Tremor (CRST). The patients were categorized into quartile groups based on their mean SDR, and the characteristics, treatment effectiveness, treatment parameters, and adverse events were evaluated among these subgroups. RESULTS: Patients were classified into 4 quartiles based on the mean SDR: quartile 1 (Q1) (SDR 0.26-0.37), Q2 (SDR 0.38-0.42), Q3 (SDR 0.43-0.49), and Q4 (SDR 0.50-0.75). MRgFUS significantly improved total CRST and tremor score across all SDR subgroups. Additionally, there were no significant differences in the improvement rates among the 4 subgroups. Analysis of the treatment parameters revealed that lower mean SDR was associated with lower target maximum temperature and smaller coagulation volume after focused ultrasound (FUS). Regarding adverse events, headache and nausea during FUS and facial and head edema on the day after surgery were more frequent in the Q1 subgroup (very low-SDR group). In contrast, numbness was more common in the Q4 subgroup. However, all these adverse events had resolved by the 3-month follow-up except numbness. CONCLUSIONS: This study suggested that MRgFUS is effective and safe for patients with medication-resistant essential tremor, including those with very low mean SDR. However, the very low-SDR group had insufficient temperature elevation at the target site compared with the high-SDR group, suggesting the need for a different strategy. Notably, with careful adjustments and considerations, positive outcomes can still be achieved in patients with very low SDR. Therefore, very low SDR should not be considered an absolute exclusion criterion because it is expected to increase the number of patients who benefit from MRgFUS.

Video Motion Analysis as a Quantitative Evaluation Tool for Essential Tremor during Magnetic Resonance-Guided Focused Ultrasound Thalamotomy.

The Clinical Rating Scale for Tremor (CRST) is commonly used to evaluate essential tremor (ET) during focused ultrasound (FUS) thalamotomy. However, it faces challenges such as the ceiling effect and test-retest variability. This study explored the utility of videographic motion analysis as an evaluation index for ET. Forty-three patients with ET performed postural tremor and line-drawing tasks recorded on video, and the data were analyzed using motion analysis software. The test-retest and inter-rater reliability, correlations with the CRST and tremor scores, and pre/post-FUS treatment comparisons were analyzed. The video motion analysis showed excellent test-retest and inter-rater reliability. In the postural tremor tasks, video parameter amplitude significantly correlated with the CRST and tremor scores. Similarly, for the line-drawing task, video parameter amplitude showed significant correlations with CRST and tremor scores, effectively addressing the ceiling effect. Regarding post-FUS treatment improvements, changes in the CRST and tremor scores were significantly associated with changes in video parameter amplitude. In conclusion, quantitative analysis of the video motion of ET enables precise evaluation of kinematic characteristics and effectively resolves the ceiling effect and test-retest variability. The video motion analysis score accurately reflected the tremor severity and treatment effects, demonstrating its high clinical utility.

Cavitation with low-energy sonication during focused ultrasound thalamotomy for a patient with tremor-dominant Parkinson's disease: a potential risk.

We report a patient with tremor-dominant Parkinson's disease who had a mild cavitation bioeffect during magnetic resonance-guided focused ultrasound thalamotomy. During the aligning phase with low-energy sonication, cavitation caused mild dysarthria and paresthesia, prompting treatment cessation. At the same time, tremor and rigidity improved. MRI revealed extensive high-intensity lesions in the thalamus 1 day after the procedure followed by steroid infusion, which resulted in resolution of adverse events. Tremor and rigidity improved 1.5 years after the procedure. Although cavitation can relieve tremors and rigidity, it should be carefully monitored due to potential permanent adverse events by unpredictable and unknown behaviors.

The Forward and Lateral Tilt Angle of the Neck and Trunk Measured by Three-Dimensional Gait and Motion Analysis as a Candidate for a Severity Index in Patients with Parkinson's Disease.

(1) Objective: To evaluate the usefulness of a three-dimensional motion-analysis system (AKIRA®) as a quantitative measure of motor symptoms in patients with Parkinson's disease (PD). (2) Method: This study included 48 patients with PD. We measured their motion during 2 m of walking using AKIRA®, we calculated the tilt angles of the neck and trunk, ankle height, and gait speed, then we compared these parameters with the MDS-UPDRS and the Hoehn and Yahr scale. Furthermore, we measured these AKIRA indicators before and after 1 year of observation. (3) Results: The forward tilt angle of the neck showed a strong correlation with the scores on parts II, III, and the total MDS-UPDRS, and the tilt angle of the trunk showed a moderate correlation with those measures. The lateral tilt angle of the trunk showed a moderate correlation with a freezing of the gait and a postural instability. Regarding changes over the course of 1 year (n = 34), the total scores on part III of the MDS-UPDRS and the forward tilt angle of the neck improved, while the lateral tilt angle of the trunk worsened. (4) Conclusion: Taken together, the forward and lateral tilt angles of the neck and trunk as measured by AKIRA® can be a candidate for quantitative severity index in patients with PD.

Cervical Transverse Myelitis Following COVID-19 Vaccination.

Various COVID-19 vaccines are associated with numerous adverse side effects. Associations between vaccinations and neurological disorders, such as transverse myelitis, stroke, Bell's palsy, acute disseminated encephalomyelitis, and Guillain-Barré syndrome, have been reported. A 27-year-old Japanese woman presented with paresthesia four days after receiving a second dose of the COVID-19 vaccine. One month after vaccination, she started to feel left lower limb weakness, and her symptoms almost improved after two steroid pulse therapies. Spinal cord tumor biopsy could potentially help make a definitive diagnosis in clinical situations. However, it is very important to review the patient's medical history, including vaccinations received, before performing a direct spinal cord biopsy, which is invasive and does not guarantee a definitive diagnosis.

Paraneoplastic cerebellar degeneration with anti-Yo antibodies and an associated submandibular gland tumor: a case report.

BACKGROUND: As a debilitating syndrome, paraneoplastic cerebellar degeneration (PCD) remains challenging to treat. Further, anti-Yo antibody (directed against human cerebellar degeneration-related protein 2) detection in patients with PCD is associated with unsatisfactory responses to existing therapies. Here, we present the case of a 60-year-old woman who developed PCD with anti-Yo antibodies and a submandibular gland tumor. CASE PRESENTATION: A 60-year-old woman presented with a 5-day history of unsteadiness of gait and inadequate coordination of her extremities, along with truncal instability. Although walking without aid was possible, dysmetria of all four limbs, trunk, and gait ataxia was observed. While routine biochemical and hematological examinations were normal, the patient's blood was positive for anti-Yo antibodies. When the neurological symptoms deteriorated despite administration of intravenous methylprednisolone, fluorodeoxyglucose-positron emission tomography (FDG-PET) and computed tomography (CT) images with contrast enhancement were performed, which showed a tumor in the left submaxillary gland. She underwent total left submandibular gland resection, including the tumor; histological and immunohistochemical results revealed a salivary duct carcinoma. She was administered intravenous methylprednisolone, followed by 10 plasma exchange sessions, intravenous immunoglobulins, and cyclophosphamide therapy. Following treatment, her symptoms were not alleviated, even after the reduction of anti-Yo titers. CONCLUSIONS: Although tumor detection was delayed, early tumor detection, diagnosis, and PCD treatment are essential because any delay can result in the progression of the disorder and irreversible neurological damage. Therefore, we recommend that the possibility of a salivary gland tumor should be considered, and whole-body dual-modality CT, including the head and neck, and FDG-PET should be performed at the earliest for patients with well-characterized paraneoplastic antibodies when conventional imaging fails to identify a tumor.

Predictors of thermal increase in magnetic resonance-guided focused ultrasound treatment for essential tremor: histogram analysis of skull density ratio values for 1024 elements.

OBJECTIVE: Sufficient thermal increase capable of generating thermocoagulation is indispensable for an effective clinical outcome in patients undergoing magnetic resonance-guided focused ultrasound (MRgFUS). The skull density ratio (SDR) is one of the most dominant predictors of thermal increase prior to treatment. However, users currently rely only on the average SDR value (SDRmean) as a screening criterion, although some patients with low SDRmean values can achieve sufficient thermal increase. The present study aimed to examine the numerical distribution of SDR values across 1024 elements to identify more precise predictors of thermal increase during MRgFUS. METHODS: The authors retrospectively analyzed the correlations between the skull parameters and the maximum temperature achieved during unilateral ventral intermediate nucleus thalamotomy with MRgFUS in a cohort of 55 patients. In addition, the numerical distribution of SDR values was quantified across 1024 elements by using the skewness, kurtosis, entropy, and uniformity of the SDR histogram. Next, the authors evaluated the correlation between the aforementioned indices and a peak temperature > 55°C by using univariate and multivariate logistic regression analyses. Receiver operating characteristic curve analysis was performed to compare the predictive ability of the indices. The diagnostic performance of significant factors was also assessed. RESULTS: The SDR skewness (SDRskewness) was identified as a significant predictor of thermal increase in the univariate and multivariate logistic regression analyses (p < 0.001, p = 0.013). Moreover, the receiver operating characteristic curve analysis indicated that the SDRskewness exhibited a better predictive ability than the SDRmean, with area under the curve values of 0.847 and 0.784, respectively. CONCLUSIONS: The SDRskewness is a more accurate predictor of thermal increase than the conventional SDRmean. The authors suggest setting the SDRskewness cutoff value to 0.68. SDRskewness may allow for the inclusion of treatable patients with essential tremor who would have been screened out based on the SDRmean exclusion criterion.

Utility of transoral motion-mode ultrasonography to detect tongue fasciculation in patients with amyotrophic lateral sclerosis.

INTRODUCTION: Increasing evidence suggests the utility of the submandibular approach for ultrasonography to detect tongue fasciculation in amyotrophic lateral sclerosis (ALS). We hypothesized that transoral motion-mode ultrasonography (TOMU) would be useful to detect tongue fasciculation in patients with ALS. METHODS: Patients with sporadic ALS showing clinically definite tongue fasciculation were enrolled, and the ultrasonography findings of patients' tongues on TOMU and ultrasonography by the conventional submandibular approach were analyzed. RESULTS: Six patients with clinically definite ALS were enrolled in this study. Although small, irregular muscle movements of 5 to 10 mm in amplitude and 0.1 to 0.2 second in duration were detected in all patients by TOMU, similar muscle movements were detected in only two of the six patients by the submandibular approach. DISCUSSION: TOMU appeared to be useful for detecting tongue fasciculation in ALS patients. Further study is needed to better determine its role as a diagnostic tool for ALS.

An Investigation of Factors Related to Food Intake Ability and Swallowing Difficulty After Surgery for Thoracic Esophageal Cancer.

Swallowing difficulty is among the major complications that can occur after surgery for thoracic esophageal cancer. Recurrent laryngeal nerve paralysis (RLNP) has been considered the most significant cause of a postoperative swallowing difficulty, but association between the two has not been adequately explained. We investigated the relation between postoperative RLNP and swallowing difficulty by means of video fluoroscopy. Our study included 32 patients who underwent subtotal esophagectomy for thoracic esophageal cancer at St. Marianna University School of Medicine between April 2014 and March 2017. We evaluated patients' age and sex, disease stage, preoperative presence of a swallowing difficulty, nutritional status, extent and duration of surgery, blood loss volume, and postoperative presence of RLNP and/or hoarseness. Patients were divided into two groups according to whether oral food intake was possible when video fluoroscopy was performed on postoperative day (POD) 7, and we analyzed the associated factors. Postoperative RLNP occurred in 21 patients (65.6%); hoarseness occurred in 19 (59.4%). Eleven patients (34.4%) suffered swallowing difficulty that prevented food intake. No significant association was found between postoperative swallowing difficulty and postoperative RLNP or hoarseness, but a significant relation was found between the prognostic nutritional index and intraoperative lymph node dissection. Multivariable analysis revealed a significant relation between postoperative swallowing difficulty and only one factor: cervical lymph node dissection (P = 0.0075). There appears to be no relation between RLNP pursuant to esophageal cancer surgery and swallowing difficulty that prevents oral food intake.

Usefulness of the combination of iodine-123-metaiodobenzylguanidine scintigraphy and iodine-123-ioflupane scintigraphy in new-onset Parkinson's disease.

OBJECTIVES: The aim of this study was to study the significance of combining iodine-123-cardiac metaiodobenzylguanidine scintigraphy (I-MIBG scintigraphy) and iodine-123-ioflupane (I-ioflupane) dopamine transporter scintigraphy (I-ioflupane scintigraphy) in patients suspected of having Parkinson's disease (PD). PATIENTS AND METHODS: We carried out a retrospective study from April 2014 to December 2015 in 48 patients suspected of having new-onset PD who underwent both I-MIBG and I-ioflupane scintigraphies within 3 months. Cases included 37 patients diagnosed as having PD. Controls included 11 patients who had never been diagnosed as having PD or other diseases showing parkinsonism. The cutoff for diagnosing PD was a heart to mediastinum ratio (H/M ratio) of less than or equal to 2.2 for I-MIBG scintigraphy in the delayed phase and a specific binding ratio (SBR) of less than or equal to 3.8 for I-ioflupane scintigraphy. The combined use of both scintigraphies was studied using the formula SBR×H/M ratio as a marker for the logistic regression model. RESULTS: Sixteen (33.3%) patients had SBR of greater than 3.8: eight with PD; eight were controls. Five of eight patients had an H/M ratio of less than or equal to 2.2 (62.5%) and had PD. In the receiver-operating characteristic analysis, the SBR×H/M ratio cutoff was 12.5, with an area under the curve of 0.844 (95% confidence interval: 0.619-1). In an age-adjusted regression analysis in patients with SBR of greater than 3.8, the SBR×H/M ratio was associated significantly with an odds ratio of 0.69 (95% confidence interval: 0.48-0.98, P=0.041). CONCLUSION: If SBR is greater than 3.8, the SBR×H/M ratio can help diagnose PD. The combined use of the two scintigraphies can improve the diagnosis of PD.

Rheumatoid meningitis presenting with a stroke-like attack treated with recombinant tissue plasminogen activator: a case presentation.

BACKGROUND: Rheumatoid meningitis presenting with a stroke-like attack (RMSA) is a rare manifestation of rheumatoid arthritis (RA). When the patients arrive within the time-window for recombinant tissue plasminogen activator (rt-PA) infusion therapy, no diagnostic protocol has been established. CASE PRESENTATION: A 55-year-old woman was brought by ambulance to our hospital with complaints of sudden-onset dysarthria and left arm numbness. The National Institutes of Health Stroke Scale (NIHSS) score was 5, and the Alberta Stroke Program Early CT Score was 8. She was diagnosed with acute embolic stroke. At 4 h, 6 min after onset, intravenous administration of rt-PA (alteplase, 0.6 mg/kg) was started. Her neurological deficits improved rapidly, and her NIHSS score was 1. Brain MRI was then performed. There was no hemorrhagic transformation, but the MRI findings were not compatible with ischemic stroke. She had a past history of RA diagnosed 6 months earlier, and she had been treated with methotrexate (10 mg daily). She was diagnosed with RMSA, and continuous infusion of methylprednisolone 1000 mg daily was started for 3 days. The high signal intensity on the FLAIR image disappeared. CONCLUSION: CT-based decision-making for rt-PA injection is reasonable, but MRI is needed for the early diagnosis of RMSA. In this case, it is particularly important that neither adverse events nor bleeding complications were observed, suggesting the safety of CT-based thrombolytic therapy in RMSA.

Stratification of disease progression in a broad spectrum of degenerative cerebellar ataxias with a clustering method using MRI-based atrophy rates of brain structures.

BACKGROUND: The rate of disease progression differs among patients with degenerative cerebellar ataxia. The uncertain natural course in individual patients hinders clinical trials of promising treatments. In this study, we analyzed atrophy changes in brain structures with cluster analysis to find sub-groups of patients with homogenous symptom progression in a broad spectrum of degenerative cerebellar ataxias. METHODS: We examined 48 patients including 21 cases of spinocerebellar ataxia (SCA), 17 cases of the cerebellar type of multiple system atrophy (MSA-C), and 10 cases of cortical cerebellar ataxia (CCA). In all patients, at least two sets of evaluations including magnetic resonance imaging (MRI) and the International Cooperative Ataxia Rating Scale (ICARS) scoring were performed. The median number (min-max) of follow-up studies in each patient was three (2-6), and the mean follow-up period was 3.1 ± 1.6 years. The area of the corpus callosum on midsagittal images and the cerebellar volume were measured using MRI, and these values were divided by the cranial antero-posterior diameter of each patient to correct for individual head size differences as an area index (Adx) and a volume index (Vdx), respectively. The annual changes in Adx, Vdx, and ICARS score were calculated in each patient, and atrophy patterns in patients were categorized with cluster analysis. RESULTS: The annual atrophy rates for the corpus callosum (Adx) and cerebellum (Vdx) and symptom progression differed significantly by subtype of cerebellar ataxia (p = 0.026, 0.019, and 0.021, respectively). However, neither the annual atrophy rate of Adx nor Vdx was significantly correlated with the annual increase in the ICARS score. When the patients were categorized into three clusters based on the annual changes in Adx and Vdx, the annual increase in the ICARS score was significantly different among clusters (2.9 ± 1.7/year in Cluster 1, 4.8 ± 3.2/year in Cluster 2, and 8.7 ± 6.1/year in Cluster 3; p = 0.014). CONCLUSIONS: The annual increase in the ICARS score can be stratified by cluster analysis based on the atrophy rates of the corpus callosum and cerebellum. Further studies are warranted to explore whether these simple MRI methods could be used for random allocation of a broad spectrum of patients with degenerative cerebellar ataxia in clinical trials.

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

BACKGROUND: Progression 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. METHODS: A 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. RESULTS: Stepwise 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). CONCLUSIONS: Cerebellar 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.

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.

Vim thalamotomy in a patient with Holmes' tremor and palatal tremor - Pathophysiological considerations.

BACKGROUND: We peformed a ventral intermediate nucleus (Vim) thalamotomy in a patient with Holmes' tremor and palatal tremor. The frequencies of these movement disorders were 4 Hz and 3 Hz, respectively. Vim thalamotomy stopped the Holmes' tremor but not the palatal tremor. Our observations suggest different mechanisms for these two involuntary movements. CASE PRESENTATION: A 57-arm 11 months after a pontine hemorrhage. Transoral carotid ultrasonography revealed periodic motion of her posterior pharyngeal wall with a frequency of 3 Hz. Recording of neuronal activities in the thalamus revealed a 4Hz rhythmic discharge time that was associated with her tremor in the contralateral arm. A left Vim thalamotomy was performed. The resting tremor of the upper limb stopped, but the kinetic tremor recurred 6 months after the thalamotomy. No effect was observed on her palatal tremor. CONCLUSIONS: The different effects of Vim thalamotomy on the Holmes' tremor and palatal tremor suggest different oscillation sources for these two involuntary movements.

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 individual's 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.

[A case report of cerebral amyloid angiopathy-related inflammation treated with cyclophosphamide].

A 78-year-old woman was admitted to another hospital with progressive cognitive dysfunction and right hemiparesis. Magnetic resonance imaging (MRI) of the brain revealed a high-intensity area in bilateral frontal lobes on T2-weighted imaging (T2WI) and fluid-attenuated inversion recovery (FLAIR). Brain biopsy revealed no significant findings. She was transferred to our hospital for further examination. Although the etiology of the white matter lesion was not specified, the lesion and symptoms improved with steroid pulse therapy. Three months later, disturbance of consciousness and right hemiparesis worsened again, and the white matter lesion was expanded on MRI. Steroid pulse therapy was again performed, but her condition gradually deteriorated and akinetic mutism developed. T2-based imaging showed microbleeds and analysis of the apolipoprotein E (ApoE) genotype showed ε4/ε4; therefore, cerebral amyloid angiopathy-related inflammation was diagnosed. Two cycles of cyclophosphamide pulse therapy were performed, resulting in improvement of the white matter lesion, disorder of consciousness, and hemiparesis after the first cycle. The patient was discharged from our hospital in an ambulatory state. Steroid therapy for cerebral amyloid angiopathy-related inflammation is relatively well-known in Japan, but cyclophosphamide therapy appears useful for steroid-resistant amyloid angiopathy-related inflammation in the early stage of the disease.

An atypical case of "Takotsubo cardiomyopathy" during alcohol withdrawal: abnormality in the transient left ventricular wall motion and a remarkable elevation in the ST segment.

A 64-year-old man was admitted due to hypokalemia-related myopathy. He was heavy drinker. He felt the stress of alcohol withdrawal during his hospitalization. The patient suffered a cardiopulmonary arrest lasting approximately 5 minutes on the fifth hospital day. One day later, ST-segment elevation was observed in leads I, aV(L), and V(2-6). Emergent cardiac catheterization was performed for suspicion of acute myocardial infarction. Normal coronary arteries with anterior akinesis of the left ventricle were revealed during the procedure. The present case may be an atypical form of "Takotsubo cardiomyopathy" in which the left ventricular contraction is due to focal anterior wall motion abnormalities.