Objective To assess the long-term effects of hybrid assistive limb (HAL) treatment on gait in patients with amyotrophic lateral sclerosis (ALS). Methods Three courses of treatment with HAL were ...administered to three women with ALS. Each course had a four- to five-week duration, during which the treatment was performed nine times, with a rest period of at least two months between each course. Gait ability (2-minutes-walk and 10-m-walk tests), ALS Functional Rating Scale-Revised, and respiratory function tests were performed before and after each treatment course. Patients Patients diagnosed with ALS, according to the updated Awaji criteria, by board-certified neurologists in the Department of Neurology and Department of Rehabilitation Medicine, Toho University Omori Faculty of Medicine between January and December 2019 were recruited. Results The average time from the start to the end of the 3 courses was 319.7±33.7 days. A multiple regression analysis was performed for the 2-minutes-walk and 10-m-walk tests, using the baseline value, each participant's ID, and time point as covariates. Changes after each course were considered outcomes. Following the 3 treatment courses, the 2-minutes walk distance improved by 16.61 m (95% confidence interval, -9.33-42.54) compared with the baseline value, but this improvement was not statistically significant (p=0.21). However, cadence significantly improved by 1.30 steps (95% confidence interval, 0.17-2.42; p=0.02). Conclusion Long-term, repetitive HAL treatments may help patients with ALS maintain their gait.
The efficacy of mechanical thrombectomy (MT) for acute ischemic stroke has been established, but there are few reports on the effectiveness of MT for stroke patients with collagen disease. We report ...the case of a systemic lupus erythematosus (SLE) patient with cerebral infarction who underwent MT. A 48-year-old woman had been diagnosed with SLE for 30 years. She visited our hospital because of dizziness from the day before, but when she arrived at the hospital parking lot, she developed vomiting and impaired consciousness. An MRI revealed increased cerebellar hemisphere infarction and magnetic resonance angiography (MRA) did not visualize the right vertebral artery or basilar artery. Urgent cerebral angiography was performed, and angiography of the right vertebral artery revealed occlusion of the V4 segment of the vertebral artery. In addition to these angiographic findings, the patient also had impaired consciousness and was judged to be in need of emergency revascularization treatment. We performed an MT using a stent retriever. Immediately after the angiography examination, reperfusion to the basilar artery and severe stenosis of the right vertebral artery were noted. Therefore, percutaneous transluminal angioplasty (PTA) and stent placement for vertebral artery stenosis were done. This procedure successfully maintained the patency of the vertebral artery and blood flow to the basilar artery. Her consciousness improved; she only had mild nausea and no remarkable neurological findings.
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory condition of an unknown etiology. Stroke is a rare complication associated with AOSD; most of these are cerebral infarctions due to ...the occlusion of small blood vessels. Here, we report the first case of mechanical thrombectomy in a patient with cerebral infarction due to a large vessel occlusion associated with AOSD. A 60-year-old man with no underlying disease was diagnosed with AOSD. Sixteen days after admission, he suddenly lost consciousness and was found to have right hemiplegia and aphasia. Head CT showed early signs of ischemic infarction in the left insular cortex, and head CT angiography demonstrated occlusion in a part of the left middle cerebral artery (MCA). Therefore, we decided that mechanical thrombectomy was an indication of revascularization. We performed mechanical thrombectomy using a Trevo NXT 4 × 28 mm (Stryker, Kalamazoo, USA) and obtained reperfusion of the MCA. The results of the cerebral angiography were indicative of an embolic cerebral infarction, and we investigated the source of the embolism including an insertable cardiac monitor (ICM) (Reveal LINQ, Medtronic, Minneapolis, USA). However, no disease other than AOSD that could be a source of embolism was observed. Therefore, AOSD was assumed to be associated with embolisms. AOSD may cause embolic cerebral infarction and may be indicated for mechanical thrombectomy.
Introduction:Branch atheromatous disease (BAD) is a type of cerebral infarction caused by stenosis or occlusion at the entrance of the penetrating branch due to the presence of plaque. Despite its ...clinical significance, it is not clear how these plaques are formed. Focal geometrical characteristics are expected to be as important as vascular risk factors in the development of atherosclerosis. This study aimed to analyze the association between middle cerebral artery (MCA) geometric features and the onset of BAD. Shear stress results from the blood flow exerting force on the inner wall of the vessels and places with low wall shear stress may be prone to atherosclerosis. At the curvature of blood vessels, the shear stress is weak on the inside of the curve and plaque is likely to form. When this is applied to the MCA M1 segment, downward type M1 is likely to form plaques on the superior side. Because the lenticulostriate artery usually branches off from the superior side of the MCA M1 segment, in downward type M1, a plaque is likely to be formed at the entrance of the penetrating branch, and for that reason, BAD is likely to onset. Methods:We retrospectively reviewed hospitalized stroke patients with BAD and investigated the morphology of their MCA using magnetic resonance imaging. The M1 segment was classified as straight or curved. Additionally, we compared the difference between the symptomatic and the asymptomatic side. Data regarding patients’ medical history were also collected. Results:A total of 56 patients with lenticulostriate artery infarctions and BAD were analyzed. On the symptomatic side, downward type M1 accounted for the largest proportion at 44%, whereas on the asymptomatic side, it was the lowest, at 16%. Conclusion:A downward type MCA may be associated with the onset of BAD and the morphological characteristics might affect the site of plaque formation. J. Med. Invest. 70 : 411-414, August, 2023
Objective The coronavirus disease 2019 (COVID-19) pandemic has resulted in a shortage of medical resources, including ventilators, personal protective equipment, medical staff, and hospital beds. We ...investigated the impact of COVID-19 in amyotrophic lateral sclerosis (ALS) patients, their families, caregivers, and medical experts. Methods We conducted a nationwide ALS webinar about COVID-19 in May 2020 and sent a questionnaire to those enrolled. Results A total of 135 participants (31 ALS patients; 23 families and caregivers of ALS patients; 81 medical experts) responded to this cross-sectional self-report questionnaire. The results showed that tracheostomy and invasive ventilation (TIV) was used in 22.6% of ALS patients, whereas 77.4% of ALS patients were not under TIV. Among non-TIV patients (n=24), 79.2% did not want TIV in the future. However, 47.4% of non-TIV patients not wanting a tracheostomy in advanced stages replied that they would want an emergency tracheostomy if they developed COVID-19-related pneumonia. These results suggest that ALS patients may be receptive to emergency treatments for reasons other than ALS. In addition, approximately half of the ALS patients agreed with the policy of not ventilating the elderly or ALS patients in case of a ventilator shortage. Furthermore, compared with medical experts, few ALS patients reported that the chance for ALS patients to obtain work was higher due to the increasing availability of remote work. Conclusions This survey indicates that the COVID-19 pandemic might be associated with increased distress about access to care and work, inducing contradictory responses and potential hopelessness among ALS patients.
Abstract Background Primary lateral sclerosis (PLS) is an extremely rare condition; therefore, to date no clinical studies have been conducted. The Primary Lateral Sclerosis Functional Rating Scale ...(PLSFRS) was developed in the United States of America. The PLSFRS is a crucial assessment scale for international collaborative research and future clinical trials for PLS. It is useful for evaluating medical conditions through face-to-face assessments and telephone interviews such as when a face-to-face assessment is not possible due to disasters or the burden of hospital visits. This study assessed the reliability and consistency of in-person and telephone interviews using the Japanese version of the PLSFRS. Methods We enrolled 19 Japanese patients who met the specific criteria for inclusion at the six collaborating institutions. The PLSFRS assessments were performed by two evaluators at defined time points and analyzed for intra-rater and inter-rater reliability and consistency between the in-person and telephone interviews. Results The Japanese version of the PLSFRS was developed by a specialized company and translator, and modified to consider the Japanese lifestyle through a consensus among motor neuron specialists. The quadratic-weighted kappa coefficients for the intra-rater and the inter-rater agreement were substantial (intra-rater: 0.691-1.000, inter-rater: 0.634-1.000). Moreover, the intraclass correlation coefficient for the PLSFRS total score was 0.997 (95% confidence interval, 0.992–0.999). Conclusions This study provides results regarding the Japanese version of the PLSFRS intra-rater and inter-rater reliability and consistency between in-person and telephone interviews.
Abstract
Background
Myasthenia gravis (MG) is an autoimmune disease caused by antibodies that block or destroy nicotinic acetylcholine receptors at the neuromuscular junction. Most of MG patients ...need immunosuppression agents in addition to treatments that alleviate the symptoms. Intravenous immunoglobulin (IVIg) and plasma exchange are specific treatments given to patients with severe MG and myasthenia gravis crisis. IVIg therapy can cause an increase in serum viscosity; therefore, the risk for thromboembolic events, such as stroke, myocardial infarction, and pulmonary embolism, are reported after IVIg therapy.
Case presentation
An MG patient was treated with pyridostigmine bromide and prednisolone. The patient’s symptoms worsened 26 days after the commencement of treatment and was presented with head drop and dyspnea. The patient was diagnosed with MG crisis and IVIg was initiated. However, the patient reported chest pain and dyspnea 3 days after IVIg had started. An electrocardiogram (ECG) revealed ST elevations in leads II, III, and aVF. A cardiac catheterization was performed and stenosis, obstruction, and sclerosis were ruled out. Glyceryl trinitrate relieved the patient’s symptoms, suggesting coronary spastic angina (CSA).
Conclusions
We report the first case of CSA after IVIg. Practitioners should be aware of the potential risks of CSA when administering IVIg for MG patients, in particular in old patients with vascular risk factors.
Prosopagnosia is a cognitive disorder in which facial recognition is severely impaired despite normal vision and intelligence. Prosopagnosia was first reported in the 1800s, but its cause remains ...unclear. Although other neurological symptoms are often present, some patients have pure prosopagnosia. The bilateral occipital lobes are believed to be associated with symptoms. Recent brain imaging techniques have identified the right fusiform gyrus (rFG), located at the junction of the right occipital temporal lobe, as the affected region. In this report, we present a case of associative prosopagnosia with no concomitant symptoms in a 76-year-old man. Brain magnetic resonance imaging detected a subcortical hemorrhage in the right temporal lobe. Using tractography based on diffusion tensor imaging, we visualized atrophy of the right inferior longitudinal fasciculus (ILF). This is the first time tractography has been used to show a clear association between associative prosopagnosia and ILF damage projecting from the rFG.
Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive sterol storage disease caused by a mutated sterol 27-hydroxylase (CYP27A1) gene. Patients with typical CTX show neurological ...dysfunction including bilateral cataracts, paresis, cerebral ataxia, dementia, and psychiatric disorders, and magnetic resonance imaging (MRI) has revealed symmetrical lesions in the cerebellar white matter.
We report the case of a patient with late-onset spinal form CTX without brain lesion. He showed pyramidal tract signs, and impaired joint position and vibration sensation in the lower limbs. Cervical sagittal MRI demonstrated a longitudinally extensive white matter abnormality in the dorsal column of the C2-C7 spinal cord; however, a brain MRI revealed an absence of lesions, including in the cerebellar white matter. Genetic analysis of CYP27A1 revealed that the patient was compound heterozygous for p.Gln85Arg in exon 1, a novel mutation, and p.Arg405Gln in exon 7, a previously reported mutation.
This is the first report of late-onset spinal form CTX without typical neurological symptoms, and the first report of p.Gln85Arg in CYP27A1. We speculate that spinal form CTX without brain lesion is a clinically and radiologically rare variation of CTX. Therefore, spinal xanthomatosis should be included in the differential diagnosis of chronic myelopathy even with late-onset and/or no other typical neurological findings.
We herein report an 81-year-old woman with Alzheimer's disease (AD) in who donepezil, a cholinesterase inhibitor (ChEI), caused cervical dystonia. The patient had a two-year history of progressive ...memory disturbance fulfilling the NINCDS-ADRDA criteria for probable AD. Mini-Mental State Examination score was 19/30. The remaining examination was normal. After a single administration of donepezil (5 mg/day) for 10 months, she complained of dropped head. Neurological examination and electrophysiological studies supported a diagnosis of cervical dystonia. Antecollis disappeared completely at 6 weeks after cessation of donepezil. Dystonic posture can occur at various timings of ChEI use. Physicians should pay more attention to rapidly progressive cervical dystonia in ChEI-treated AD patients.
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