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  • 1
    Online Resource
    Online Resource
    Myasthenia gravis with anti-muscle-specific tyrosine kinase antibodies during therapy for multiple myeloma: a case report
    Springer Science and Business Media LLC ; 2020
    In:  BMC Neurology Vol. 20, No. 1 ( 2020-12)
    Details
    In: BMC Neurology, Springer Science and Business Media LLC, Vol. 20, No. 1 ( 2020-12)
    Abstract: The onset of myasthenia (MG) gravis with anti-muscle-specific tyrosine kinase (MuSK) antibodies most commonly peaks in the fourth decade of life, and MG with MuSK antibodies (MuSK-MG) rarely coexists with a malignant tumor. To date, MuSK-MG has not been reported in multiple myeloma (MM). Case presentation A 60-year-old male with MM who was receiving treatment with bortezomib and thalidomide presented diplopia, ptosis, and limb weakness. A diagnosis of MM with Bence-Jones proteinuria was established when he was 56 years old, and he received chemotherapy with four courses of bortezomib and dexamethasone. Although he received thalidomide as maintenance therapy, it was discontinued a year before hospital admission because of sensory neuropathy as a side effect. Six months before hospital admission, he developed mild diplopia. One month before admission, his chemotherapy was interrupted because of viral infection and fatigability. Then he developed neck weakness and bilateral ptosis. A diagnosis of MuSK-MG was made based on neurological and serological examinations. According to the previous relevant literature, this is the first report of MuSK-MG in a patient with MM. Conclusions In patients with MM, the possibility of co-existing of autoimmune disease, including MuSK-MG, should be considered. This case emphasizes the need to still consider testing for anti-MuSK antibodies in older MM patients where there is clinical suspicion for possible MG despite negative anti-acetylcholine receptor antibodies and lacking classic MuSK MG phenotype at onset.
    Type of Medium: Online Resource
    ISSN: 1471-2377
    URL: Article
    DOI: 10.1186/s12883-020-01813-1
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2020
    detail.hit.zdb_id: 2041347-6
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  • 2
    Online Resource
    Online Resource
    Microbleeds after Carotid Artery Stenting: Small Embolism May Induce Cerebral Microbleeds
    S. Karger AG ; 2019
    In:  Cerebrovascular Diseases Extra Vol. 9, No. 2 ( 2019-6-14), p. 57-65
    Details
    In: Cerebrovascular Diseases Extra, S. Karger AG, Vol. 9, No. 2 ( 2019-6-14), p. 57-65
    Abstract: 〈 b 〉 〈 i 〉 Background: 〈 /i 〉 〈 /b 〉 Since the advent of magnetic resonance imaging technology, cerebral microbleeds can be diagnosed in vivo. However, the underlying mechanism of cerebral microbleed formation is not fully understood. 〈 b 〉 〈 i 〉 Objectives: 〈 /i 〉 〈 /b 〉 This study aimed to identify the factors associated with cerebral microbleeds after carotid artery stenting (CAS). 〈 b 〉 〈 i 〉 Method: 〈 /i 〉 〈 /b 〉 We retrospectively examined 125 patients who underwent CAS for carotid stenosis. Cerebral microbleeds were investigated using T2*-weighted gradient-echo (GRE) imaging before and after CAS. We analyzed the possible association of new microbleeds with the following risk factors: the number of baseline microbleeds and ischemic cerebral lesions, the occurrence of cerebral hyperperfusion syndrome, and new ischemic cerebral lesions after CAS. 〈 b 〉 〈 i 〉 Results: 〈 /i 〉 〈 /b 〉 Baseline cerebral microbleeds were detected in 53 patients (42.4%). New cerebral microbleeds after CAS were observed in 13 of 125 patients (10.4%) and were exclusively associated with new ischemic lesions but not with other risk factors. No patient showed a merged image of a new cerebral microbleed on GRE imaging or a new ischemic lesion on diffusion-weighted imaging. Lobar and deep microbleeds were noted in 12/13 (92.3%) and 1 patient (7.7%), respectively. Of 12 patients with new microbleeds, 10 (76.9%) and 2 (15.4%) had a new microbleed in the ipsilateral and contralateral hemispheres, respectively. 〈 b 〉 〈 i 〉 Conclusions: 〈 /i 〉 〈 /b 〉 We found that new cerebral microbleeds developed after CAS and that these might be associated with new ischemic lesions, mostly in the territory of the treated carotid artery. We speculate that these microbleeds result from the deoxygenation of hemoglobin in the embolus or, alternatively, small hemorrhagic transformation of ischemic lesions.
    Type of Medium: Online Resource
    ISSN: 1664-5456
    URL: Article
    DOI: 10.1159/000500112
    Language: English
    Publisher: S. Karger AG
    Publication Date: 2019
    detail.hit.zdb_id: 2651613-5
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  • 3
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    Hypertensive Arteriopathy and Cerebral Amyloid Angiopathy in Patients with Cognitive Decline and Mixed Cerebral Microbleeds
    IOS Press ; 2020
    In:  Journal of Alzheimer's Disease Vol. 78, No. 4 ( 2020-12-08), p. 1765-1774
    Details
    In: Journal of Alzheimer's Disease, IOS Press, Vol. 78, No. 4 ( 2020-12-08), p. 1765-1774
    Abstract: Background: Hypertensive arteriopathy (HA) and cerebral amyloid angiopathy (CAA) may contribute to the development of mixed cerebral microbleeds (CMBs). Recently, the total small vessel disease (SVD) scores for HA and CAA were proposed, which are determined by a combination of MRI markers to reflect overall severity of these microangiopathies. Objective: We investigated whether or not total HA-SVD and CAA-SVD scores could be used to predict overlap of HA and CAA in patients with mixed CMBs. Methods: Fifty-three subjects with mixed CMBs were retrospectively analyzed. MRI markers (CMBs, lacunes, perivascular space, white matter hyperintensity [WMH] and cortical superficial siderosis [cSS] ) were assessed. The HA-SVD score and CAA-SVD score were obtained for each subject. Anterior or posterior WMH was also assessed using the age-related white matter changes scale. Results: The two scores were positively correlated (ρ= 0.449, p  〈  0.001). The prevalence of lobar dominant CMB distribution (p  〈  0.001) and lacunes in the centrum semiovale (p  〈  0.001) and the severity of WMH in the parieto-occipital lobes (p = 0.004) were significantly higher in the high CAA-SVD score group. cSS was found in four patients with high CAA-SVD score who showed lobar-dominant CMB distribution and severe posterior WMH. Conclusion: Mixed CMBs are mainly due to HA. Assessing both two scores may predict the overlap of HA and CAA in individuals with mixed CMBs. Patients with a high CAA-SVD score may have some degree of advanced CAA, especially when lobar predominant CMBs, severe posterior WMH, lobar lacunes, or cSS are observed.
    Type of Medium: Online Resource
    ISSN: 1387-2877 , 1875-8908
    URL: Article
    DOI: 10.3233/JAD-200992
    Language: Unknown
    Publisher: IOS Press
    Publication Date: 2020
    detail.hit.zdb_id: 2070772-1
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  • 4
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    Online Resource
    Association between cortical microinfarcts and total small vessel disease burden in cerebral amyloid angiopathy on 3‐Tesla magnetic resonance imaging
    Wiley ; 2021
    In:  European Journal of Neurology Vol. 28, No. 3 ( 2021-03), p. 794-799
    Details
    In: European Journal of Neurology, Wiley, Vol. 28, No. 3 ( 2021-03), p. 794-799
    Abstract: Cortical microinfarcts (CMIs) are frequently found in the brains of patients with advanced cerebral amyloid angiopathy (CAA) at autopsy. The small vessel disease (SVD) score for CAA (i.e., the CAA‐SVD score) has been proposed to evaluate the severity of CAA‐associated vasculopathic changes by a combination of magnetic resonance imaging (MRI) markers. The aim of this study was to examine the association between total CAA‐SVD score and features of CMIs on in vivo 3‐Tesla MRI. Methods Eighty patients with probable CAA were retrospectively analyzed. Lobar cerebral microbleeds, cortical superficial siderosis, enlargement of perivascular space in the centrum semiovale and white matter hyperintensity were collectively assessed, and the total CAA‐SVD score was calculated. The presence of CMI was also examined. Results Of the 80 patients, 13 (16.25%) had CMIs. CMIs were detected more frequently in the parietal and occipital lobes. A positive correlation was found between total CAA‐SVD score and prevalence of CMI ( ρ  = 0.943; p  = 0.005). Total CAA‐SVD score was significantly higher in patients with CMIs than in those without ( p  = 0.009). In a multivariable logistic regression analysis, the presence of CMIs was significantly associated with total CAA‐SVD score (odds ratio 2.318 [95% confidence interval 1.228–4.376]; p  = 0.01, per each additional point). Conclusions The presence of CMIs with a high CAA‐SVD score could be an indicator of more severe amyloid‐associated vasculopathic changes in patients with probable CAA.
    Type of Medium: Online Resource
    ISSN: 1351-5101 , 1468-1331
    URL: Issue
    URL: Article
    DOI: 10.1111/ene.v28.3
    DOI: 10.1111/ene.14610
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2020241-6
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  • 5
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    Online Resource
    Vertebral artery dissection associated with familial Mediterranean fever and Behçet's disease
    Wiley ; 2019
    In:  Annals of Clinical and Translational Neurology Vol. 6, No. 5 ( 2019-05), p. 974-978
    Details
    In: Annals of Clinical and Translational Neurology, Wiley, Vol. 6, No. 5 ( 2019-05), p. 974-978
    Abstract: Vertebral artery dissection and recurrent meningitis are rare complications in Behçet's disease. Behçet's disease may be associated with familial Mediterranean fever. Here, we describe a 52‐year‐old woman with severe headache who exhibited recurrent meningitis and vertebral artery dissection. Cerebrospinal fluid showed high levels of interleukin‐6. Magnetic resonance imaging revealed right vertebral artery dissection. The patient had three heterozygous mutations in the familial Mediterranean fever gene ( MEFV ) gene. She fulfilled criteria for diagnosis of Behçet's disease and familial Mediterranean fever. In conclusion, mutations of the MEFV gene may cause neuro‐inflammatory disorders and cerebrovascular disorders by reducing anti‐inflammatory activity of pyrin.
    Type of Medium: Online Resource
    ISSN: 2328-9503 , 2328-9503
    URL: Issue
    URL: Article
    DOI: 10.1002/acn3.2019.6.issue-5
    DOI: 10.1002/acn3.773
    Language: English
    Publisher: Wiley
    Publication Date: 2019
    detail.hit.zdb_id: 2740696-9
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  • 6
    Online Resource
    Online Resource
    Micro-MRI improves the accuracy of clinical diagnosis in cerebral small vessel disease
    Oxford University Press (OUP) ; 2021
    In:  Brain Communications Vol. 3, No. 2 ( 2021-04-05)
    Details
    In: Brain Communications, Oxford University Press (OUP), Vol. 3, No. 2 ( 2021-04-05)
    Abstract: Even with postmortem pathological examination, only limited information is provided of the foci of in vivo clinical information. Cerebral small vessel disease, which is associated with ageing, dementia and stroke, highlights the difficulty in arriving at a definitive diagnosis of the lesions detected on in vivo radiological examination. We performed a radiological−pathological comparative study using ex vivo MRI to examine small cerebral lesions. Four patients with small vessel disease lesions detected on in vivo MRI were studied. Exact pathological findings of in vivo MRI-detected lesions were revealed. The ischaemic lesion after 17 days from onset showed positivity for peroxiredoxin, cluster of differentiation 204 and glial fibrillary acidic protein, indicating sterile inflammation and neuroprotective reaction. Cortical microinfarcts beneath the cortical superficial siderosis were associated with inflammation from the superficial layer in a patient with cerebral amyloid angiopathy; in this patient, a bilinear track-like appearance of the cortical superficial siderosis on the ex vivo MRI was compatible with iron deposition on the pia matter and within cortical layers II–III. An in vivo MRI-detected cerebral microbleed was revealed to be heterogeneous. An in vivo MRI-detected cerebral microbleed was revealed to be a venous angioma. Furthermore, a neuropathologically confirmed embolic cerebral microbleed was firstly detected using this method. Our results suggest that in vivo MRI-detected lobar cerebral microbleeds can be caused by non-cerebral amyloid angiopathy aetiologies, such as microembolism and venous angioma. Venous angioma and embolic microbleeds may mimic cerebral amyloid angiopathy markers on in vivo MRI. To clarify the clinical importance of these lesions, we should investigate their rate and frequency in a large cohort of healthy individuals and patients with cardiac risk factors. Thus, we provide evidence that ex vivo micro-MRI improves the clinical diagnosis of small vessel diseases.
    Type of Medium: Online Resource
    ISSN: 2632-1297
    URL: Article
    DOI: 10.1093/braincomms/fcab070
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2021
    detail.hit.zdb_id: 3020013-1
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  • 7
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    Abstract WP553: Differentiation of Cortical Microinfarctions Due to Cerebral Amyloid Angiopathy From Those Caused by Microembolism on 3T MRI
    Ovid Technologies (Wolters Kluwer Health) ; 2019
    In:  Stroke Vol. 50, No. Suppl_1 ( 2019-02)
    Details
    In: Stroke, Ovid Technologies (Wolters Kluwer Health), Vol. 50, No. Suppl_1 ( 2019-02)
    Abstract: Background: Cortical microinfarctions (CMI) are often detected in patients with cerebral amyloid angiopathy (CAA) and cerebral microembolism. Purpose: To evaluate whether the size, location and distribution of CMIs differ between CAA and microembolism on 3T MRI. Methods: We retrospectively screened 657 patients who consulted our memory clinic from Jan 2011 to Mar 2018 and underwent detection protocol for CMIs (3-dimensional double inversion recovery (DIR), 3-dimensional fluid attenuated inversion recovery (FLAIR) and susceptibility-weighted imaging (SWI) on 3T MRI). There were 120 patients which had DIR-positive and SWI-negative lesion less than 10 mm in diameter. These patients were classified to embolic stroke group and CAA group which fulfilled the modified Boston criteria for possible or probable CAA. We analyzed the size, location and distribution of small cortical lesions on each group. Result: We enrolled 44 patients as embolic stroke group and 27 patients as CAA group. A total of 175 small cortical lesions were identified in the embolic stroke group and 63 in the CAA group. Mean number of small cortical lesions in the embolic stroke group was significantly higher than those of the CAA group (4.0 vs 2.3; p = 0.001). In the CAA group, the frequency of cortical lesions less than 5mm and localized in the cortical gray matter; ie. CMI, was higher than in the embolic stroke group (92% vs 69%; p 〈 0.001). By contrast, the frequency of cortical lesions larger than 5mm was significantly higher in the embolic stroke group than in the CAA group (21% vs 8%; p = 0.004). Furthermore, the frequency of cortical lesions involving subcortical area was significantly higher in the embolic stroke group than in the CAA group (30% vs 8%; p 〈 0.001). Regarding their distribution, the CAA group showed occipital lobe dominance compared to the embolic stroke group (30% vs 10%; p 〈 0.01). Conclusion: Our results suggest that CMIs caused by CAA tend to be less numerous, localized in the cortical gray matter and smaller than 5mm, and show occipital dominance compared to those by microembolism. These characteristics may be helpful for differentiating CMIs due to CAA from those caused by microembolism.
    Type of Medium: Online Resource
    ISSN: 0039-2499 , 1524-4628
    URL: Article
    DOI: 10.1161/str.50.suppl_1.WP553
    RVK:
    XA 10000
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2019
    detail.hit.zdb_id: 1467823-8
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  • 8
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    P1-17-08. Clinical and neurophysiological analysis in two patients of facial onset sensory and motor neuronopathy (FOSMN syndrome)
    Elsevier BV ; 2019
    In:  Clinical Neurophysiology Vol. 130, No. 10 ( 2019-10), p. e214-
    Details
    In: Clinical Neurophysiology, Elsevier BV, Vol. 130, No. 10 ( 2019-10), p. e214-
    Type of Medium: Online Resource
    ISSN: 1388-2457
    URL: Article
    DOI: 10.1016/j.clinph.2019.06.183
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2019
    detail.hit.zdb_id: 1499934-1
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  • 9
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    Online Resource
    Association between behavioral and psychological symptoms and cerebral small vessel disease MRI findings in memory clinic patients
    Frontiers Media SA ; 2023
    In:  Frontiers in Aging Neuroscience Vol. 15 ( 2023-3-24)
    Details
    In: Frontiers in Aging Neuroscience, Frontiers Media SA, Vol. 15 ( 2023-3-24)
    Abstract: Cerebral small vessel disease (SVD) is commonly observed among elderly individuals with cognitive impairment and has been recognized as a vascular contributor to dementia and behavioral and psychological symptoms (BPS), however, the relationship between BPS and SVD burden remains unclear. Methods We prospectively recruited 42 patients with mild cognitive impairment (MCI) or mild dementia from the memory clinic in our hospital, who were assigned to either a clinical dementia rating (CDR) of 0.5 or 1.0, respectively. The presence of BPS was determined through interviews with caregivers. The patients underwent brain MRI and three types of SVD scores, total, cerebral amyloid angiopathy (CAA), and modified CAA, were assigned. Patients were also evaluated through various neuropsychological assessments. Results The CDR was significantly higher in patients with BPS ( p  = 0.001). The use of antihypertensive agents was significantly higher in patients without BPS ( p  = 0.038). The time taken to complete trail making test set-A was also significantly longer in patients with BPS ( p  = 0.037). There was no significant difference in total SVD and CAA-SVD score ( p  = 0.745, and 0.096) and the modified CAA-SVD score was significantly higher in patients with BPS ( p  = 0.046). In addition, the number of total CMBs and lobar CMBs was significantly higher in patients with BPS ( p  = 0.001 and 0.001). Receiver operating characteristic curves for BPS showed that for modified CAA-SVD, a cutoff score of 3.5 showed 46.7% sensitivity and 81.5% specificity. Meanwhile, for the total number of cerebral microbleeds (CMBs), a cut-off score of 2.5 showed 80.0% sensitivity and 77.8% specificity and for the number of lobar CMBs, a cut-off score of 2.5 showed 73.3% sensitivity and 77.8% specificity. Conclusion Overall, patients with BPS showed worse CDRs, reduced psychomotor speed, higher modified CAA-SVD scores, larger numbers of total and lobar CMBs. We propose that severe modified CAA scores and higher numbers of total and lobar CMBs are potential risk factors for BPS in patients with mild dementia or MCI. Therefore, by preventing these MRI lesions, the risk of BPS may be mitigated.
    Type of Medium: Online Resource
    ISSN: 1663-4365
    URL: Article
    DOI: 10.3389/fnagi.2023.1143834
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2023
    detail.hit.zdb_id: 2558898-9
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  • 10
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    Impairment of intermediate somatosensory function in corticobasal syndrome
    Springer Science and Business Media LLC ; 2020
    In:  Scientific Reports Vol. 10, No. 1 ( 2020-07-07)
    Details
    In: Scientific Reports, Springer Science and Business Media LLC, Vol. 10, No. 1 ( 2020-07-07)
    Abstract: Corticobasal syndrome (CBS) is characterized by unilateral atrophy of the brain. New diagnostic criteria for CBS include intermediate somatosensory dysfunction. Here, we aimed to carefully examine intermediate somatosensory function to identify tests which can assess impairment in CBS patients. Using voxel-based morphometry (VBM), we also aimed to show the anatomical bases of these impairments. Subjects included 14 patients diagnosed with CBS and 14 patients with Parkinson's disease (PD). Patients were evaluated using intermediate somatosensory tests and neuropsychological assessments. VBM was used to analyze differences in gray matter volumes between CBS and PD patients. In the PD group, no tests showed a significant difference between the dominant-side onset and the non-dominant-side onset. In the CBS group, all tests showed worse scores on the affected side. For detecting intermediate somatosensory dysfunction in CBS, two tests are recommended: tactile object naming and 2-point discrimination. VBM analysis showed that the volume of the left post- and pre-central gyrus, and both sides of the supplementary motor area were significantly decreased in the CBS group compared to the PD group. Although CBS remains untreatable, early and correct diagnosis is possible by performing close examination of intermediate somatosensory function.
    Type of Medium: Online Resource
    ISSN: 2045-2322
    URL: Article
    DOI: 10.1038/s41598-020-67991-7
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2020
    detail.hit.zdb_id: 2615211-3
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