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1.
J Am Chem Soc ; 2024 Apr 09.
Article En | MEDLINE | ID: mdl-38591952

Oxide ion conductors are attractive materials because of their wide range of applications, such as solid oxide fuel cells. Oxide ion conduction in oxyhalides (compounds containing both oxide ions and halide ions) is rare. In the present work, we found that Sillén oxychlorides, Bi2-xTexLuO4+x/2Cl (x = 0, 0.1, and 0.2), show high oxide ion conductivity. The bulk conductivity of Bi1.9Te0.1LuO4.05Cl reaches 10-2 S cm-1 at 431 °C, which is much lower than 644 °C of yttria-stabilized zirconia (YSZ) and 534 °C of La0.8Sr0.2Ga0.83Mg0.17O2.815 (LSGM). Thanks to the low activation energy, Bi1.9Te0.1LuO4.05Cl exhibits a high bulk conductivity of 1.5 × 10-3 S cm-1 even at a low temperature of 310 °C, which is 204 times higher than that of YSZ. The low activation energy is attributed to the interstitialcy oxide ion diffusion in the triple fluorite-like layer, as evidenced by neutron diffraction experiments (Rietveld and neutron scattering length density analyses), bond valence-based energy calculations, static DFT calculations, and ab initio molecular dynamics simulations. The electrical conductivity of Bi1.9Te0.1LuO4.05Cl is almost independent of the oxygen partial pressure from 10-18 to 10-4 atm at 431 °C, indicating the electrolyte domain. Bi1.9Te0.1LuO4.05Cl also exhibits high chemical stability under a CO2 flow and ambient air at 400 °C. The oxide ion conduction due to the two-dimensional interstitialcy diffusion is considered to be common in Sillén oxyhalides with triple fluorite-like layers, such as Bi1.9Te0.1RO4.05Cl (R = La, Nd, Sm, Eu, Gd, Dy, Ho, Er, Tm, Yb, Lu) and Bi6-2xTe2xO8+xBr2 (x = 0.1, 0.5). The present study opens a new field of materials chemistry: oxide ion-conducting Sillén oxyhalides with triple fluorite-like layers.

2.
Dysphagia ; 2024 Mar 16.
Article En | MEDLINE | ID: mdl-38492048

Patients with pseudobulbar palsy often present with velopharyngeal incompetence. Velopharyngeal incompetence is usually observed during expiratory activities such as speech and/or blowing during laryngoscopy. These patients typically exhibit good velopharyngeal closure during swallowing, which is dissociated from expiratory activities. We named this phenomenon "speech-swallow dissociation" (SSD). SSD on endoscopic findings can help in diagnosing the underlying disease causing dysphagia. This endoscopic finding is qualitative, and the quantitative characteristics of SSD are still unclear. Accordingly, the current study aimed to quantitatively evaluate SSD in patients with pseudobulbar palsy. We evaluated velopharyngeal pressure during swallowing and expiratory activity in 10 healthy subjects and 10 patients with pseudobulbar palsy using high-resolution manometry, and compared the results between the two groups. No significant differences in maximal velopharyngeal contraction pressure (V-Pmax) were observed during dry swallowing between the pseudobulbar palsy group and healthy subjects (190.5 mmHg vs. 173.6 mmHg; P = 0.583). V-Pmax during speech was significantly decreased in the pseudobulbar palsy group (85.4 mmHg vs. 34.5 mmHg; P < 0.001). The degree of dissociation of speech to swallowing in V-Pmax, when compared across groups, exhibited a larger difference in the pseudobulbar palsy group, at 52% versus 80% (P = 0.001). Velopharyngeal pressure during blowing was similar to that during speech. Velopharyngeal closure in patients with pseudobulbar palsy exhibited weaker pressure during speech and blowing compared with swallowing, quantitatively confirming the presence of SSD. Pseudobulbar palsy often presents with SSD, and this finding may be helpful in differentiating the etiology of dysphagia.

3.
Muscle Nerve ; 69(2): 222-226, 2024 Feb.
Article En | MEDLINE | ID: mdl-38018268

INTRODUCTION/AIMS: We have encountered patients with myasthenia gravis (MG) who exhibited palatal prolapse (PP) during nasal expiration in the supine position while awake. This may be an overlooked cause of dyspnea in MG patients. This study aimed to examine and describe the characteristics of MG patients with PP. METHODS: We reviewed the medical records of 183 consecutive patients who were diagnosed with MG in our hospital from 2012 to 2021. Thirty-two patients underwent laryngoscopy because of bulbar symptoms. Eight of these patients (25%) exhibited PP on laryngoscopy. Clinical features of these eight patients were retrospectively characterized. RESULTS: Median age of the eight patients with PP was 70 years. Six were men. Median body mass index was 21.6 kg/m2 . All patients exhibited PP in the supine position but not the sitting position. Although no patient had abnormal findings on spirometry nor chest computed tomography, six reported dyspnea or difficulty with nasal expiration only in the supine position. PP improved in all four patients who underwent edrophonium testing. All eight patients eventually improved after immunotherapy. DISCUSSION: PP during nasal expiration may be a cause of dyspnea in MG patients, along with respiratory muscle impairment, lung disease, and vocal cord paralysis. Laryngoscopy in the supine position is required to confirm.


Myasthenia Gravis , Respiratory Insufficiency , Vocal Cord Paralysis , Aged , Female , Humans , Male , Dyspnea/etiology , Edrophonium/therapeutic use , Myasthenia Gravis/diagnosis , Respiratory Insufficiency/drug therapy , Retrospective Studies
4.
Vasc Endovascular Surg ; 58(3): 287-293, 2024 Apr.
Article En | MEDLINE | ID: mdl-37858317

PURPOSE: In the majority of cases, large vessel occlusion (LVO) in ischemic stroke patients has an embolic origin. Systemic embolism can occur simultaneously with brain thrombosis. This retrospective study evaluated the frequency and locations of systemic embolism in LVO stroke patients receiving revascularization therapy. MATERIALS AND METHODS: In our facility, we use contrast-enhanced computed tomography (CE-CT) to assess suspected stroke patients and routinely perform CE-CT from the chest to the abdomen after brain CT angiography to rule out contraindications like aortic dissection and trauma for thrombolysis. Systemic embolism is also assessed using these images, while myocardial infarction is evaluated based on electrocardiograms and laboratory findings. Other relevant clinical features of each patient are also analyzed. RESULTS: In total, 612 consecutively admitted stroke patients and 32 LVO patients who underwent revascularization therapy were included in the present study. Systemic embolism was identified in four patients (13%). The spleen was the most commonly affected organ, followed by the heart, kidneys, limbs, and lungs. All four patients with systemic embolism exhibited LVO resulting from embolism as the underlying mechanism. CONCLUSION: Systemic embolism was observed in 13% of our LVO patients, all of whom had LVO of embolic origin.


Brain Ischemia , Embolism , Stroke , Humans , Retrospective Studies , Treatment Outcome , Stroke/diagnostic imaging , Stroke/etiology , Stroke/therapy , Tomography, X-Ray Computed , Computed Tomography Angiography , Brain Ischemia/diagnostic imaging , Brain Ischemia/etiology , Brain Ischemia/therapy , Thrombectomy/adverse effects
5.
Intern Med ; 2023 Oct 20.
Article En | MEDLINE | ID: mdl-37866920

A 53-year-old woman was admitted to our hospital because of increasingly frequent transient speech disturbance and left upper limb weakness. Brain magnetic resonance imaging and angiography revealed multiple intracranial stenoses, including the proximal right middle cerebral artery (MCA), without evidence of infarction. The diagnosis of primary angiitis of the central nervous system was established based on circumferential vascular wall thickening with contrast enhancement observed in the right MCA. Following the administration of dual antiplatelet therapy and intravenous methylprednisolone pulse therapy, the patient experienced complete cessation of symptoms, and the stenosis gradually improved without infarction. The risk of infarct development in crescendo transient ischemic attacks may differ between inflammatory vascular stenosis and atherosclerosis.

6.
J Neurol Sci ; 449: 120666, 2023 06 15.
Article En | MEDLINE | ID: mdl-37148775

BACKGROUND AND PURPOSE: Large vessel occlusion (LVO) in hyperacute ischemic stroke occurs mainly by one of two mechanisms, embolism or atherosclerosis. However, the mechanism is difficult to identify prior to treatment. We aimed to investigate the factors associated with embolic LVO in hyperacute ischemic stroke, and to develop a preoperative predictive scale for the event. MATERIALS AND METHODS: This retrospective multicenter study was conducted with consecutive ischemic stroke patients with LVO who underwent thrombectomy, thrombolysis, or both. The embolic LVO was defined as an occlusion that underwent recanalization with no residual stenosis. Multivariate logistic regression analysis for embolic LVO was performed to identity the independent risk factors. With this approach, a novel prediction scale (Rating of Embolic Occlusion for Mechanical Thrombectomy [REMIT] scale) was developed. RESULTS: A total of 162 patients (104 men; median age 76 years; interquartile range 68-83) were included in this study. Embolic LVO was observed in 121 patients (75%). Multivariate logistic regression analysis showed that embolic LVO was independently associated with high brain natriuretic peptide (BNP), high National Institutes of Health Stroke Scale (NIHSS) on admission, and absence of non-culprit stenosis (NoCS). The REMIT scale comprises high BNP (>100 pg/dL), high NIHSS (>14) and absence of NoCS, with one point for each risk factor. The frequencies of embolic LVO for the REMIT scale scores were as follows: score 0, 25%; score 1, 60%; score 2, 87%; score 3, 97% (C-statistic 0.80, P < 0.001). CONCLUSION: The novel REMIT scale has predictive value for embolic LVO.


Brain Ischemia , Embolism , Ischemic Stroke , Stroke , Male , Humans , Aged , Constriction, Pathologic/complications , Stroke/diagnosis , Stroke/diagnostic imaging , Risk Factors , Thrombectomy/adverse effects , Embolism/complications , Retrospective Studies , Brain Ischemia/diagnosis , Brain Ischemia/diagnostic imaging , Treatment Outcome
7.
Cureus ; 15(12): e50201, 2023 Dec.
Article En | MEDLINE | ID: mdl-38192909

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by arterial, venous, or small vessel thromboembolic events. We present here a rare case of APS with repeated multiple cerebral infarctions in the same watershed area without visible arterial stenosis. A 53-year-old woman without a past medical history presented with a headache and numbness of the right fingers. Magnetic resonance imaging (MRI) showed acute ischemic lesions in the left middle cerebral artery (MCA) watershed area. Blood tests revealed positive anticardiolipin (aCL) and aCL beta-2-glycoprotein I antibodies (aCL-ß2GPI). Three months later, aCL and aCL-ß2GPi antibodies were still positive, and APS was confirmed. After four months from the index stroke, she was suddenly affected by right arm and leg weakness under a warfarin prescription. Brain MRI showed a recurrence of acute ischemic stroke in the same left MCA watershed area and the right cerebellar hemisphere without visible intracranial artery stenosis in magnetic resonance angiography. The examination of carotid ultrasonography, electrocardiogram monitoring, as well as transthoracic and transesophageal echocardiography revealed no abnormalities, indicating that the recurrent ischemic stroke was due to APS. Single-photon emission-computed tomography captured wide hypoperfusion beyond the infarction area. Thus, the stroke may have been caused by a repeated thromboembolic mechanism. In conclusion, APS should be considered a differential diagnosis in repeated watershed strokes without obvious intracranial arterial stenosis.

8.
Clin Neurol Neurosurg ; 223: 107504, 2022 12.
Article En | MEDLINE | ID: mdl-36370600

A 51-year-old Japanese man presented with slowly progressing cerebellar ataxia from age 49. Anti-thyroglobulin, anti-thyroid peroxidase, and anti-NAE antibodies were detected. Brain magnetic resonance imaging showed moderate atrophy of the pons, cerebellum, and middle cerebellar peduncle. Dopamine transporter single-photon emission computed tomography showed normal uptake. With the diagnosis of Hashimoto's encephalopathy, repeated steroid pulse therapy and intravenous immunoglobulin therapy were administered; however, they were ineffective for cerebellar ataxia. Afterwards, autonomic failure and parkinsonism became evident. The final diagnosis was MSA of the cerebellar type. Immunotherapies for patients with MSA with anti-NAE antibodies should be carefully considered.


Cerebellar Ataxia , Encephalitis , Hashimoto Disease , Multiple System Atrophy , Male , Humans , Middle Aged , Multiple System Atrophy/diagnostic imaging , Multiple System Atrophy/therapy , Multiple System Atrophy/complications , Cerebellar Ataxia/diagnostic imaging , Cerebellar Ataxia/therapy , Hashimoto Disease/diagnostic imaging , Hashimoto Disease/therapy , Encephalitis/complications , Magnetic Resonance Imaging/adverse effects , Immunologic Factors , Immunotherapy
9.
J Neurol Sci ; 440: 120325, 2022 09 15.
Article En | MEDLINE | ID: mdl-35779417

OBJECTIVE: Bulbar symptoms in amyotrophic lateral sclerosis (ALS) are variable, reflecting bulbar and pseudobulbar palsy. The current study sought to characterize the pharyngeal findings in ALS using a fiberoptic laryngoscope and compare them with the findings of general neurological examination. METHODS: We enrolled ALS patients with bulbar symptoms who were admitted between 2014 and 2020. All participants were evaluated on salivary status, velopharyngeal movement during speech and swallowing, pharyngeal constriction, and vocal cord movement using fiberoptic laryngoscopy. The laryngoscopic findings were compared with general neurological examination results. RESULTS: A total of 50 patients (31 men; median age: 69 years) were enrolled. Salivary residue in the hypopharynx was the most common abnormal finding on laryngoscopy (40 patients; 80%). Twenty-three patients (46%) exhibited velopharyngeal insufficiency, 18 of which exhibited good velopharyngeal closure in swallowing and poor velopharyngeal closure in speech. Thus, these patients presented speech-swallow dissociation (SSD) in velopharyngeal closure. Five patients (10%) exhibited unilateral weakness of the pharyngeal constrictor muscles in phonation. Compared with general neurological examinations, emotional incontinence was more frequent in patients who presented with SSD in velopharyngeal movement, compared with those who did not. None of the five patients with unilateral weakness of pharyngeal constriction showed curtain movement on examination via the mouth. CONCLUSION: The specific findings of laryngoscopy in ALS patients, such as SSD in velopharyngeal closure and laterality in pharyngeal constriction, could not be evaluated by general neurological examination via the mouth.


Amyotrophic Lateral Sclerosis , Laryngoscopes , Respiration Disorders , Aged , Amyotrophic Lateral Sclerosis/complications , Amyotrophic Lateral Sclerosis/diagnosis , Deglutition/physiology , Humans , Male , Neurologic Examination , Pharyngeal Muscles
10.
Respir Investig ; 60(5): 684-693, 2022 Sep.
Article En | MEDLINE | ID: mdl-35871065

BACKGROUND: Anti-aquaporin-4 (AQP4) antibody is an autoantibody marker often observed in patients with neuromyelitis optica spectrum disorder (NMOSD). The pathological relevance of complicated pulmonary disorders in anti-AQP4 antibody-positive NMOSD remains unclear. We aimed to assess the clinical and histological relevance of complicated pulmonary disorders in anti-AQP4 antibody-positive NMOSD. METHODS: We retrospectively reviewed the medical records of 52 patients with anti-AQP4 antibody-positive NMOSD and conducted immunohistochemical evaluations of the lung biopsy specimens. RESULTS: Among 52 patients with anti-AQP4 antibody-positive NMOSD, 4 patients showed pulmonary involvement with a diagnosis of organizing pneumonia (OP). The proportion of males was larger (75% vs. 12.5%; p = 0.013) and creatine kinase levels were higher (458.3 U/L vs. 83.9 U/L; p = 0.003) in patients with OP than in those without OP. OP development preceded or coincided with the NMOSD symptoms. Chest computed tomography findings were consistent with OP in all four patients. Bronchoalveolar lavage fluid predominantly contained lymphocytes. Transbronchial lung biopsy revealed intraluminal plugs of inflammatory debris within the alveoli. Alveolar epithelial cells covering the OP lesions exhibited AQP4 loss, immunoglobulin G deposition, and complement activation. Corticosteroid treatment resulted in clinical improvement of OP. CONCLUSION: OP may be considered a pulmonary manifestation of anti-AQP4 antibody-positive NMOSD beyond the central nervous system. Complement-dependent cytotoxicity of the lung epithelial cells caused by anti-AQP4 antibody is at least partly involved in OP development. When diagnosing NMOSD, the possibility of OP should be carefully evaluated based on the detailed history and chest imaging findings.


Neuromyelitis Optica , Pneumonia , Aquaporin 4/therapeutic use , Autoantibodies , Humans , Male , Neuromyelitis Optica/complications , Neuromyelitis Optica/drug therapy , Pneumonia/complications , Retrospective Studies
11.
J Neurol Sci ; 436: 120247, 2022 05 15.
Article En | MEDLINE | ID: mdl-35381404

BACKGROUND AND PURPOSE: To diagnose atherosclerotic occlusion mechanism in acute ischemic stroke patients with large vessel occlusion prior to revascularization therapy is challenging. The aim was to verify the relationship between atherosclerotic occlusion and non-culprit stenosis detected in urgent neuroimaging prior to interventional procedure. MATERIALS AND METHODS: This study collected hyperacute stroke with large vessel occlusion, who underwent revascularization therapy (intravenous thrombolysis, thrombectomy, or both). An atherosclerotic occlusion was defined as an occlusion that did not recanalize or had residual stenosis at the initially occluded lesion, ensured in the second angiographic imaging performed after 1 week. The remaining patients who did not fulfill the definition of atherosclerotic occlusion was classified as embolic occlusion. A non-culprit stenosis was defined as a ≥ 50%-99% stenosis located other than the culprit occluded artery. Logistic regression analyses were performed to determine the factors independently associated with atherosclerotic occlusion. RESULTS: A total of 162 patients (104 men, median age 76 years old) were enrolled in our study. Forty one patients (25%) was atherosclerotic occlusion. Non-culprit stenosis was frequently observed in the atherosclerotic occlusion group than the embolic occlusion group (68% vs. 26%, P < 0.001). The presence of non-culprit stenosis was independently associated with atherosclerotic occlusion (OR, 11.00; 95% CI, 3.96-30.52; P < 0.001). CONCLUSION: In hyperacute stroke receiving endovascular therapy, non-culprit stenosis identification may be needed in order to perform an adequate revascularization, especially for atherosclerotic occlusion.


Atherosclerosis , Ischemic Stroke , Stroke , Aged , Atherosclerosis/complications , Constriction, Pathologic/complications , Female , Humans , Male , Neuroimaging , Retrospective Studies , Stroke/complications , Stroke/diagnostic imaging , Thrombectomy/methods , Treatment Outcome
12.
Intern Med ; 61(6): 801-810, 2022 Mar 15.
Article En | MEDLINE | ID: mdl-34483213

Objective Limited data exist regarding the comparative detailed clinical characteristics of patients with ischemic stroke (IS)/transient ischemic attack (TIA) and intracerebral hemorrhage (ICH) receiving oral anticoagulants (OACs). Methods The prospective analysis of stroke patients taking oral anticoagulants (PASTA) registry, a multicenter registry of 1,043 stroke patients receiving OACs [vitamin K antagonists (VKAs) or non-vitamin K antagonist oral anticoagulant (NOACs)] across 25 medical institutions throughout Japan, was used. Univariate and multivariable analyses were used to analyze differences in clinical characteristics between IS/TIA and ICH patients with atrial fibrillation (AF) who were registered in the PASTA registry. Results There was no significant differences in cardiovascular risk factors, such as hypertension, diabetes mellitus, dyslipidemia, smoking, or alcohol consumption (all p>0.05), between IS/TIA and ICH among both NOAC and VKA users. Cerebral microbleeds (CMBs) [odds ratio (OR), 4.77; p<0.0001] were independently associated with ICH, and high brain natriuretic peptide/N-terminal pro B-type natriuretic peptide levels (OR, 1.89; p=0.0390) were independently associated with IS/TIA among NOAC users. A history of ICH (OR, 13.59; p=0.0279) and the high prothrombin time-international normalized ratio (PT-INR) (OR, 1.17; p<0.0001) were independently associated with ICH, and a history of IS/TIA (OR, 3.37; 95% CI, 1.34-8.49; p=0.0101) and high D-dimer levels (OR, 2.47; 95% CI, 1.05-5.82; p=0.0377) were independently associated with IS/TIA among VKA users. Conclusion The presence of CMBs, a history of stroke, natriuretic peptide and D-dimer levels, and PT-INR may be useful for risk stratification of either IS/TIA or ICH development in patients with AF receiving OACs.


Atrial Fibrillation , Hemorrhagic Stroke , Stroke , Administration, Oral , Anticoagulants/adverse effects , Atrial Fibrillation/complications , Atrial Fibrillation/drug therapy , Humans , Stroke/epidemiology , Stroke/etiology , Vitamin K/therapeutic use
13.
J Stroke Cerebrovasc Dis ; 30(9): 105973, 2021 Sep.
Article En | MEDLINE | ID: mdl-34271277

BACKGROUND AND PURPOSE: The association between the cerebral microbleed (CMB) count and outcomes in ischemic stroke has not been fully clarified. The aim of this study was to investigate the relationship between the CMBs count and functional outcomes in patients with a minor ischemic stroke treated with antiplatelet therapy METHODS: Non-cardiogenic minor ischemic stroke (NIHSS score < 4 on admission) patients who were treated with antiplatelet therapy were enrolled. The patients were divided into four groups based on the number of CMBs (absent, 1, 2-4, and > 4), and their clinical outcomes were compared. A poor outcome was defined as a modified Rankin scale (mRS) score of 3-6 90 days after symptom onset. Logistic regression analysis was performed to evaluate whether the CMBs count contributes to poor outcomes with well-known risk factors such as age, NIHSS score on admission, ischemic stroke recurrence, large artery atherosclerosis stroke subtype, and DWMHs. RESULTS: A total of 240 patients were enrolled, and their pre mRS scores were matched based on CMB presence. A higher burden of CMBs was linearly correlated with the incidence of poor outcomes (4% in the absent group, 8% in the 1 CMB group, 13% in the 2-4 CMB group, and 20% in the > 4 CMB group, P = 0.002). Multivariate logistic regression analysis showed that CMBs count was one of the independent factor associated with poor outcomes (odds ratio 1.07, 95% confidence interval 1.02-1.12, P = 0.003). CONCLUSION: The CMBs count contributes independently to poor outcomes in minor ischemic stroke patients treated with antiplatelet therapy.


Cerebral Hemorrhage/etiology , Ischemic Stroke/drug therapy , Platelet Aggregation Inhibitors/therapeutic use , Aged , Aged, 80 and over , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/physiopathology , Disability Evaluation , Female , Functional Status , Humans , Ischemic Stroke/complications , Ischemic Stroke/diagnosis , Ischemic Stroke/physiopathology , Magnetic Resonance Imaging , Male , Middle Aged , Platelet Aggregation Inhibitors/adverse effects , Recovery of Function , Retrospective Studies , Risk Assessment , Risk Factors , Time Factors , Treatment Outcome
14.
JAMA Neurol ; 78(7): 853-863, 2021 07 01.
Article En | MEDLINE | ID: mdl-34047774

Importance: Repeat expansion of CGG in LRP12 has been identified as the causative variation of oculopharyngodistal myopathy (OPDM). However, to our knowledge, the clinicopathologic features of OPDM with CGG repeat expansion in LRP12 (hereafter referred to as OPDM_LRP12) remain unknown. Objective: To identify and characterize the clinicopathologic features of patients with OPDM_LRP12. Design, Setting, and Participants: This case series included 208 patients with a clinical or clinicopathologic diagnosis of oculopharyngeal muscular dystrophy (OPDM) from January 1, 1978, to December 31, 2020. Patients with GCN repeat expansions in PABPN1 were excluded from the study. Repeat expansions of CGG in LRP12 were screened by repeat primed polymerase chain reaction and/or Southern blot. Main Outcomes and Measures: Clinical information, muscle imaging data obtained by either computed tomography or magnetic resonance imaging, and muscle pathologic characteristics. Results: Sixty-five Japanese patients with OPDM (40 men [62%]; mean [SD] age at onset, 41.0 [10.1] years) from 59 families with CGG repeat expansions in LRP12 were identified. This represents the most common OPDM subtype among all patients in Japan with genetically diagnosed OPDM. The expansions ranged from 85 to 289 repeats. A negative correlation was observed between the repeat size and the age at onset (r2 = 0.188, P = .001). The most common initial symptoms were ptosis and muscle weakness, present in 24 patients (37%). Limb muscle weakness was predominantly distal in 53 of 64 patients (83%), but 2 of 64 patients (3%) had predominantly proximal muscle weakness. Ptosis was observed in 62 of 64 patients (97%), and dysphagia or dysarthria was observed in 63 of 64 patients (98%). A total of 21 of 64 patients (33%) had asymmetric muscle weakness. Aspiration pneumonia was seen in 11 of 64 patients (17%), and 5 of 64 patients (8%) required mechanical ventilation. Seven of 64 patients (11%) developed cardiac abnormalities, and 5 of 64 patients (8%) developed neurologic abnormalities. Asymmetric muscle involvement was detected on computed tomography scans in 6 of 27 patients (22%) and on magnetic resonance imaging scans in 4 of 15 patients (27%), with the soleus and the medial head of the gastrocnemius being the worst affected. All 42 muscle biopsy samples showed rimmed vacuoles. Intranuclear tubulofilamentous inclusions were observed in only 1 of 5 patients. Conclusions and Relevance: This study suggests that OPDM_LRP12 is the most frequent OPDM subtype in Japan and is characterized by oculopharyngeal weakness, distal myopathy that especially affects the soleus and gastrocnemius muscles, and rimmed vacuoles in muscle biopsy.


DNA Repeat Expansion , Low Density Lipoprotein Receptor-Related Protein-1 , Muscular Dystrophies/diagnosis , Adolescent , Adult , Female , Humans , Japan , Magnetic Resonance Imaging , Male , Middle Aged , Muscle Weakness , Muscle, Skeletal/pathology , Pedigree , Young Adult
15.
Heliyon ; 7(1): e06106, 2021 Jan.
Article En | MEDLINE | ID: mdl-33553762

A 49-year-old woman developed vomiting, hiccups, double vision, and bilateral ptosis, after which tinnitus and deafness appeared. Head magnetic resonance imaging (MRI) showed a brainstem lesion focused on the midbrain and pons. Anti-aquaporin 4 (AQP4) antibody was positive, and there was no evidence of optic neuritis or myelitis, leading to the diagnosis of neuromyelitis optica spectrum disorder (NMOSD). The auditory brainstem response (ABR) showed no derivation of wave V on left stimulation and extended latency between waves III and V on right stimulation, so impairment between the midbrain and pons was suspected. It was useful to evaluate head MRI and the ABR for identification of the location of auditory pathway dysfunction.

16.
J Neurol ; 268(2): 680-688, 2021 Feb.
Article En | MEDLINE | ID: mdl-32909093

OBJECTIVE: The purpose of the present study is to report the clinical characteristics of polyradiculoneuropathy induced by immune checkpoint inhibitors (ICIs). METHODS: We retrospectively reviewed lists of all inpatients with neurological immune-related adverse events (irAEs) treated at the neurology departments of three hospitals in January 2017 and December 2019. We also performed a review of the previous case reports with polyradiculoneuropathy induced by ICI therapy. RESULTS: We had 4 patients with polyradiculoneuropathy following ICI therapy. We comprehensively reviewed our 4 patients and 32 previous case reports. There were 28 men and 8 women with a mean onset age of 61 years. ICI monotherapy was performed in 27 patients, whereas the combination of ICIs was administered in 9 patients. All patients except 2 showed limb weakness, which was observed symmetrically and predominantly in the legs rather than the arms. Bulbar involvement was observed in 7 patients. The laboratory findings were demyelination in electrophysiological studies and elevated protein with lymphocytes in the cerebrospinal fluid. Disease severity was ranked on the Hughes functional scale; 17 patients were grade 4 or greater. The treatment responses to corticosteroid and intravenous methylprednisolone were favorable. Intravenous immunoglobulin was also used in combination with steroids. Seven patients died, including 4 who on mechanical ventilation. CONCLUSION: Polyradiculoneuropathy induced by ICIs has a distinct subset of neurological irAEs and requires early recognition.


Immune Checkpoint Inhibitors , Polyradiculoneuropathy , Female , Humans , Immunoglobulins, Intravenous , Male , Methylprednisolone , Middle Aged , Polyradiculoneuropathy/chemically induced , Retrospective Studies
17.
J Stroke Cerebrovasc Dis ; 29(11): 105267, 2020 Nov.
Article En | MEDLINE | ID: mdl-33066915

Thrombolytic agents are infusion formulations, and some patients cannot be cannulated by a peripheral venous route. This report describes a patient with acute ischemic stroke who was administered alteplase following central venous catheter placement. An 82-year-old man with paroxysmal atrial fibrillation presented with left unilateral spatial neglect and left hemiparesis. Magnetic resonance imaging showed acute cerebral infarction located in the right cerebrum without occlusion of the main artery. The infarction was considered appropriately indicated for thrombolysis. However, no peripheral venous access could be secured, even by trained emergency room physicians. A central venous catheter was therefore placed in the right jugular vein and thrombolysis was performed. After treatment, neurological deficits completely resolved without any clinically serious bleeding. Venous catheter placement may be a safe alternative to peripheral vein access in such a circumstance.


Brain Ischemia/drug therapy , Catheterization, Central Venous , Fibrinolytic Agents/administration & dosage , Jugular Veins , Stroke/drug therapy , Thrombolytic Therapy , Tissue Plasminogen Activator/administration & dosage , Aged, 80 and over , Brain Ischemia/diagnostic imaging , Humans , Male , Stroke/diagnostic imaging , Treatment Outcome
18.
J Neurol Sci ; 417: 117068, 2020 10 15.
Article En | MEDLINE | ID: mdl-32745720

PURPOSE: This study aimed to evaluate the risk factors, etiology, and outcomes of ischemic stroke (IS) in Japanese young adults. METHODS: This was a prospective multicenter study. We enrolled patients aged 16 to 55 years with IS within seven days of the onset of symptoms. We assessed the demographic data, risk factors, stroke etiology, and outcome at discharge. The clinical characteristics were compared between sexes and among age groups. RESULTS: We prospectively enrolled 519 patients (median age, 48 years: 139 females). The mean National Institute of Health Stroke Scale score was 3.6 ± 0.2. The most common risk factors were hypertension (HT) (55%), dyslipidemia (DL) (47%), and current smoking (42%). Body mass index, incidence of current smoking, and heavy alcohol consumption were higher in males. The prevalence of current smoking, HT, DL, and diabetes mellitus increased with aging. The most common etiologic subgroup of IS was small vessel disease (145/510, 28%). Intracranial arterial dissection (IAD) was the most common among the other determined causes (56/115, 49%). The outcome at discharge was relatively good (mRS 0-1, 71.7%); however, poor outcome (mRS ≥ 4) was observed at an incidence of 9.5%. CONCLUSIONS: Most young adults with IS had modifiable risk factors, of which prevalence increased with age. This emphasizes lifestyle improvement to prevent IS in the young population. Furthermore, we indicated that the incidence rate of IAD was high among the other determined causes.


Brain Ischemia , Ischemic Stroke , Stroke , Adolescent , Adult , Brain Ischemia/epidemiology , Female , Humans , Japan/epidemiology , Male , Middle Aged , Prospective Studies , Risk Factors , Stroke/epidemiology , Stroke/etiology , Young Adult
19.
Prog Rehabil Med ; 5: 20200003, 2020.
Article En | MEDLINE | ID: mdl-32789271

BACKGROUND: Associated laryngeal paralysis (ALP) is defined as vagus nerve impairment combined with other lower cranial nerve paralysis. Traumatic ALP is reported infrequently. CASE: A 72-year-old man was injured on the back of the head when a large tree fell on him; he was admitted to a general hospital, where he was diagnosed with brain concussion and Guillain-Barre syndrome (GBS). The patient developed aspiration pneumonia due to severe dysphagia. Although he underwent treatment and rehabilitation for 6 months, some disabilities persisted, and a percutaneous endoscopic gastrostomy tube was placed. Six months after the accident, the patient was transferred to our rehabilitation hospital. Videoendoscopic examination and videofluoroscopy revealed persistent upper esophageal sphincter (UES) opening, left dominant bilateral IX and X nerve paralysis, and left XII nerve paralysis; moreover, these examinations showed that the swallowing reflex was absent, although a bolus could pass through the UES. We suspected that the patient's condition was not GBS and performed head computed tomography and magnetic resonance imaging; these revealed a bone fracture at the skull base. Consequently, the patient's diagnosis was changed to bilateral ALP. He received swallowing rehabilitation for 2 months and could orally consume alternative nutrition. Finally, the patient was able to eat orally texture-modified foods (Food Intake LEVEL Scale level 8). DISCUSSION: While post-trauma dysphagia due to bilateral ALP might be severe, patients can regain the ability to eat orally if clinicians understand the etiology of dysphagia and provide appropriate swallowing rehabilitation techniques, including patient position adjustment while eating and selection of food textures.

20.
Front Neurol ; 11: 522, 2020.
Article En | MEDLINE | ID: mdl-32612570

Background and Purpose: The relationship between cerebral microbleeds (CMBs) and prognosis in patients with ischemic stroke is still unclear. Our aim here was to verify the relationship between CMBs and functional outcomes in patients with minor ischemic stroke treated with antiplatelet therapy. Methods: We retrospectively reviewed consecutive patients with a non-cardiogenic minor ischemic stroke (NIHSS <4 on admission) who underwent initial brain magnetic resonance imaging within the first 48 h following symptom onset. The patients were divided into two groups based on the presence or absence of CMBs and the two groups were adjusted using the pre-stroke modified Rankin scale (mRS). Poor outcome was defined as an mRS score in the 3-6 range measured 90 days after symptom onset. Logistic regression analyses were performed to determine the factors independently associated with poor outcome. Results: A total of 240 patients (187 men, median age 66 years old) were enrolled in our study. There was a non-significant trend toward a worsening shift of 3-month mRS score distribution in the CMB group compared with the no-CMB group. Multivariate analysis revealed that the presence of CMBs was independently predictive of poor outcome (OR, 3.44; 95% CI, 1.08-10.93; P = 0.036). Conclusion: Our findings suggest that the presence of CMBs is the predicting factor of poor outcome in minor ischemic stroke patients.

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