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BACKGROUND: Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease, the clinical manifestations of which are complex and easily misdiagnosed. NIID clinical characteristics are varied, affecting the central and peripheral nervous systems and autonomic nerves. In this study, we present an NIID case with both stroke-like onset and encephalitic attacks, which is a rare case report. CASE PRESENTATION: A 68-year-old Chinese female presented with sudden aphasia and limb hemiplegia as the first symptoms, as well as fever, cognitive impairment and mental irritability from encephalitic attacks. During hospitalization, a brain magnetic resonance imaging (MRI) examination detected high signal intensity from diffusion-weighted imaging (DWI) of the bilateral frontal grey matter-white matter junction. Electrophysiological tests revealed the main site of injury was at the myelin sheath in the motor nerves. A skin biopsy revealed eosinophilic spherical inclusion bodies in the nuclei of small sweat gland cells, fibroblasts and fat cells, whilst immunohistochemistry revealed that p62 and ubiquitin antibodies were positive. From genetic analyses, the patient was not a carrier of the fragile X mental retardation 1 (FMR1) permutation, but repeated GGC sequences in the NOTCH2NLC gene confirmed an NIID diagnosis. Through antipsychotic and nutritional support therapy, the patient's symptoms were completely relieved within 3 weeks. CONCLUSIONS: This report of an NIID case with both stroke-like onset and encephalitic attacks provides new information for NIID diagnoses, and a comprehensive classification of clinical characteristics.
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Encefalite/etiologia , Doenças Neurodegenerativas/complicações , Doenças Neurodegenerativas/diagnóstico , Acidente Vascular Cerebral/etiologia , Idoso , Encefalite/patologia , Feminino , Humanos , Corpos de Inclusão Intranuclear/genética , Mutação , Doenças Neurodegenerativas/genética , Receptor Notch2/genética , Acidente Vascular Cerebral/patologiaRESUMO
BACKGROUND: Extracranial carotid artery (ECA) tortuosity may influences successful recanalization rates of mechanical thrombectomy in acute ischemic stroke (AIS), yet the relationship between ECA tortuosity and the prognosis of patients with anterior circulation AIS who cannot undergo endovascular treatment remains uncertain. We hypothesized that increased tortuosity of the ECA leads to unfavorable outcomes in such patients. METHODS: Patients with anterior circulation AIS who underwent computed tomography angiography of the head and neck in our hospital between March 2018 and November 2018 were retrospectively analyzed. The tortuosity of the bilateral ECA was measured, and functional outcomes were evaluated by a modified Rankin Scale (mRS) at 90 days. Multivariate logistic regression models were used to determine the association between ECA tortuosity and outcomes of patients. RESULTS: A total of 203 patients were enrolled in our study, including 140 patients (68.97%) with favorable outcomes (mRS, 0-2) and 63 patients (31.03%) with unfavorable outcomes (mRS, 3-6). After adjusting for age, atrial fibrillation, stroke territory, and posthospital antithrombotics/statins therapy in multivariate logistic regression model I, ECA tortuosity (odds ratio, 1.052; 95% confidence interval, 1.010-1.096; Pâ¯=â¯.015) was an independent risk of unfavorable outcomes in enrolled patients. In the other 2 models (II and III) which adjusted for age, sex, baseline National Institutes of Health Stroke Scale score, and with or without posthospital medication, ECA tortuosity was also showed independent relationship to unfavorable outcomes. The optimal cutoff was 12.5 to predict the unfavorable outcomes in a receiver operating characteristic curve. CONCLUSIONS: Our study demonstrated that the ECA tortuosity is an independent predictor of unfavorable outcomes for anterior circulation AIS patients who without undergoing endovascular treatment after hospital admission. ECA tortuosity values greater than 12.5 may indicate an unfavorable outcome.
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Isquemia Encefálica/terapia , Artérias Carótidas/diagnóstico por imagem , Angiografia Cerebral/métodos , Circulação Cerebrovascular , Angiografia por Tomografia Computadorizada , Procedimentos Endovasculares , Acidente Vascular Cerebral/terapia , Idoso , Idoso de 80 Anos ou mais , Anticoagulantes/uso terapêutico , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/fisiopatologia , Artérias Carótidas/fisiopatologia , Avaliação da Deficiência , Procedimentos Endovasculares/efeitos adversos , Feminino , Fibrinolíticos/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Recuperação de Função Fisiológica , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/fisiopatologia , Fatores de Tempo , Resultado do TratamentoRESUMO
Borna disease virus (BDV) is a non-cytolytic, neurotropic RNA virus that can infect many vertebrate species, including humans. To date, BDV infection has been reported in a range of animal species across a broad global geographic distribution. However, a systematic epidemiological survey of BDV infection in domesticated animals in China has yet to be performed. In current study, BDV RNA and antibodies in 2353 blood samples from apparently healthy animals of eight species (horse, donkey, dog, pig, rabbit, cattle, goat, sheep) from three areas in western China (Xinjiang province, Chongqing municipality, and Ningxia province) were assayed using reverse transcription qPCR (RT-qPCR) and ELISA assay. Brain tissue samples from a portion of the BDV RNA- and/or antibody-positive animals were subjected to RT-qPCR and western blotting. As a result, varying prevalence of BDV antibodies and/or RNA was demonstrated in various animal species from three areas, ranging from 4.4 % to 20.0 %. Detection of BDV RNA and/or antibodies in Chongqing pigs (9.2 %) provided the first known evidence of BDV infection in this species. Not all brain tissue samples from animals whose blood was BDV RNA and/or antibody positive contained BDV RNA and protein. This study provides evidence that BDV infection among healthy domestic animal species is more widespread in western China than previously believed.
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Animais Domésticos/virologia , Doença de Borna/virologia , Vírus da Doença de Borna/fisiologia , Animais , Anticorpos Antivirais/sangue , Doença de Borna/sangue , Doença de Borna/diagnóstico , Doença de Borna/epidemiologia , Vírus da Doença de Borna/genética , Vírus da Doença de Borna/imunologia , Vírus da Doença de Borna/isolamento & purificação , Bovinos , China/epidemiologia , Cães , Equidae , Cabras , Cavalos , Coelhos , Ovinos , SuínosRESUMO
Internal carotid artery congenital absence with acute embolism of the middle cerebral artery trunk is very rare. A 65-year-old female with a history of hypertension and atrial fibrillation was admitted to the neurology department of our hospital. Computed tomography of the head and neck showed no carotid canal of the petrous portion of the temporal bone; digital subtraction angiography (DSA) showed no left internal carotid artery and the right middle cerebral artery trunk occlusion. These results suggested acute embolism of the middle cerebral artery trunk with contralateral internal carotid artery congenital absence. Mechanical thrombectomy was performed, which had a good outcome. This case showed the vascular anatomy features of ICA congenital absence with contralateral large vessel acute occlusion, and it is essential to promptly identify the vascular variation during the interventional procedure.
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Ischemic stroke accounts for 70-80% of all stroke cases. Immunity plays an important role in the pathophysiology of ischemic stroke. Microglia are the first line of defense in the central nervous system. Microglial functions are largely dependent on their pro-inflammatory (M1-like) or anti-inflammatory (M2-like) phenotype. Modulating neuroinflammation via targeting microglia polarization toward anti-inflammatory phenotype might be a novel treatment for ischemic stroke. Mesenchymal stem cells (MSC) and MSC-derived extracellular vesicles (MSC-EVs) have been demonstrated to modulate microglia activation and phenotype polarization. In this review, we summarize the physiological characteristics and functions of microglia in the healthy brain, the activation and polarization of microglia in stroke brain, the effects of MSC/MSC-EVs on the activation of MSC in vitro and in vivo, and possible underlying mechanisms, providing evidence for a possible novel therapeutics for the treatment of ischemic stroke.
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BACKGROUND: Iatrogenic aortic dissection (IAD) is a rare but fatal complication of interventional treatment for the proximal supra-aortic large vessels. Several cases of IAD after endovascular treatment of subclavian artery have been reported. Nevertheless, the pathogenesis of IAD is still unclear. Here we report a patient with IAD following a balloon expandable stent implanted into the left subclavian artery (LSA). CASE SUMMARY: An 84-year-old man with a history of hypertension was admitted to the Neurology Department of our hospital complaining of dizziness and gait disturbance for more than 1 mo. Computed tomography angiography of the head and neck showed severe stenosis at the proximal LSA and the origin of the left vertebral artery. Magnetic resonance diffusion-weighted imaging of the brain revealed subacute infarctions in cerebellum, occipital lobe and medulla oblongata. He suffered a Stanford type B aortic dissection after the proximal LSA angioplasty with a balloon expandable stent. Thoracic endovascular aortic repair was performed immediately with the chimney technique and he was discharged 20 d later. After exploring the pathogenesis with multimodal imaging analysis, an easily neglected focal intramural hematoma (IMH) in the aorta near the junction of the LSA was found to be the main cause of the IAD. The risk of IAD should be sufficiently evaluated according to the characteristics of aortic arch lesions before the proximal LSA angioplasty. CONCLUSION: Focal aortic IMH is a potential risk factor for IAD during a seemingly simple stenting of the proximal LSA.
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OBJECTIVE: The objective was to explore the ability of head impulse-nystagmus-test of skew (HINTS) combined with ABCD2 score to identify cerebrovascular causes of dizziness. MATERIALS AND METHODS: We prospectively recruited 85 patients with acute onset of dizziness from September 2016 to December 2018 and analyzed their clinical characteristics, ABCD2 scores, HINTS, and neuroimages data. RESULTS: Acute stroke was identified by MRI in 21 of 85 patients. The mean ± SD ABCD2 scores were significantly higher among patients with acute stroke than those without acute stroke (4.0 ± 0.8 h vs. 2.5 ± 0.7 h, p < 0.01). The majority (71.4%) of patients with cerebrovascular causes had central pattern of nystagmus at the initial 48 h from symptoms onset. The sensitivity and specificity of HINTS were 100% and 87% for the presence of stroke in patients with nystagmus. When combined central pattern of nystagmus and ABCD2 ≥ 4, the sensitivity increased to 100% for identifying cerebrovascular causes. Nystagmus were absence at time of examination in 16.5% of our patients, and ABCD2 scores in patients who had cerebrovascular diagnoses were all ≥ 4. CONCLUSION: HINTS examinations could efficiently differentiate stroke from nonstroke under the condition that patients remaining symptomatic, including spontaneous or gaze-evoked nystagmus. It is more practical to apply the combination of central pattern of nystagmus and ABCD2 ≥ 4 in ED setting. If patients were absence of central nystagmus at admission, cerebrovascular event should be a priority diagnosis when their ABCD2 ≥ 4.
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Nistagmo Patológico , Acidente Vascular Cerebral , Tontura/diagnóstico , Tontura/etiologia , Humanos , Imageamento por Ressonância Magnética , Nistagmo Patológico/diagnóstico , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/diagnóstico , VertigemRESUMO
Previous studies have shown that vestibular migraine (VM) is a cerebral disease with recurrent vertigo. Vestibular rehabilitation (VR) is an effective type of physical therapy for minimizing vestibular symptoms, as it improves vestibular compensation in patients with VM. Currently, the cerebral regions that are associated with the pathogenesis of VM are largely unknown. To further understand the underlying mechanisms of VM, we performed resting-state functional magnetic resonance imaging (fMRI) before and after 1 month of VR in 14 patients with VM. The Dizziness Handicap Inventory (DHI), the 36-Item Short-Form Health Survey (SF-36), the Hamilton Depression Scale (HAMD) and the Hamilton Anxiety Scale (HAMA) scores were included as clinical outcomes. The amplitude of low-frequency fluctuation (ALFF) was assessed to characterize spontaneous brain activity. The correlations between the clinical characteristics and ALFF values were assessed. After 1 month of VR training, the DHI scores in patients with VM were significantly lower than those at baseline (p = 0.03), as were the HAMA scores (p = 0.02). We also found that the ALFF values in the left posterior cerebellum of VM patients increased significantly after 1 month of VR training. Moreover, the ALFF values in the left cerebellum were inversely correlated with the patients' DHI scores. Overall, this study showed that VR exercise for 1 month has a positive effect on vestibular symptoms in patients with VM. Asymmetric cerebellar hyperactivity might be a functional compensation for vestibular dysfunction in patients with VM.
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BACKGROUND: Germinal matrix hemorrhage (GMH) is a leading cause of mortality and lifelong morbidity in preterm infants. Posthemorrhagic hydrocephalus (PHH) is a common complication of GMH. A sodium-coupled bicarbonate exchanger (NCBE) encoded by solute carrier family 4 member 10 gene is expressed on the choroid plexus basolateral membrane and may play a role in cerebrospinal fluid production and the development of PHH. Following GMH, iron degraded from hemoglobin has been linked to PHH. Choroid plexus epithelial cells also contain iron-responsive element-binding proteins (IRPs), IRP1, and IRP2 that bind to mRNA iron-responsive elements. The present study aims to resolve the following issues: (1) whether the expression of NCBE is regulated by IRPs; (2) whether NCBE regulates the formation of GMH-induced hydrocephalus; and (3) whether inhibition of NCBE reduces PHH development. METHODS AND RESULTS: GMH model was established in P7 rat pups by injecting bacterial collagenase into the right ganglionic eminence. Another group received iron trichloride injections instead of collagenase. Deferoxamine was administered intraperitoneally for 3 consecutive days after GMH/iron trichloride. Solute carrier family 4 member 10 small interfering RNA or scrambled small interfering RNA was administered by intracerebroventricular injection 24 hours before GMH and followed with an injection every 7 days over 21 days. NCBE expression increased while IRP2 expression decreased after GMH/iron trichloride. Deferoxamine ameliorated both the GMH-induced and iron trichloride-induced decrease of IRP2 and decreased NCBE expressions. Deferoxamine and solute carrier family 4 member 10 small interfering RNA improved cognitive and motor functions at 21 to 28 days post GMH and reduced cerebrospinal fluid production as well as the degree of hydrocephalus at 28 days after GMH. CONCLUSIONS: Targeting iron-induced overexpression of NCBE may be a translatable therapeutic strategy for the treatment of PHH following GMH.
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Hemorragia Cerebral/terapia , Plexo Corióideo/efeitos dos fármacos , Desferroxamina/farmacologia , Hidrocefalia/prevenção & controle , RNA Interferente Pequeno/administração & dosagem , Terapêutica com RNAi , Sideróforos/farmacologia , Simportadores de Sódio-Bicarbonato/metabolismo , Animais , Animais Recém-Nascidos , Comportamento Animal/efeitos dos fármacos , Hemorragia Cerebral/genética , Hemorragia Cerebral/metabolismo , Hemorragia Cerebral/fisiopatologia , Líquido Cefalorraquidiano/metabolismo , Cloretos , Plexo Corióideo/metabolismo , Plexo Corióideo/fisiopatologia , Cognição/efeitos dos fármacos , Modelos Animais de Doenças , Compostos Férricos , Hidrocefalia/genética , Hidrocefalia/metabolismo , Hidrocefalia/fisiopatologia , Injeções Intraventriculares , Proteína 1 Reguladora do Ferro/genética , Proteína 1 Reguladora do Ferro/metabolismo , Proteína 2 Reguladora do Ferro/genética , Proteína 2 Reguladora do Ferro/metabolismo , Atividade Motora/efeitos dos fármacos , Ratos Sprague-Dawley , Simportadores de Sódio-Bicarbonato/genéticaRESUMO
OBJECTIVE: To investigate the epidemiological pattern of Borna disease virus (BDV) among different canine breeds in Ili, China, and to analyze its potential phylogeny. METHODS: BDV p24 RNA fragments were detected from peripheral blood mononuclear cells (PBMCs) of canine by modified nested RT-PCR (nRT-PCR). Possible false positives were excluded by determination of both BDV p40 RNA fragments and PMD19 plasmid standards. Analysis were performed on genetic sequence, homologous comparison, amino acid sequence and phylogeny after p24 positive products were validated. RESULTS: BDV p24 RNA fragments were found only in Kazakh Tobet (a shepherd dog) in 8 breeds of 150 cases and their overall positive rate was 11.0% (10/91). Compared with the strain of He/80 from horse and that of S6 from sheep in Germany, the homologous similarities of Kazakh Tobet was 99.2% and 95.7%, and that of amino acid as 100% and 89.3%, respectively. The kinship of Kazakh Tobet was close to He/80 and next to S6. CONCLUSION: There was potential natural BDV infection in Kazakh Tobet in Ili, and its endemic strain was concerned with He/80 infecting Ili horse and S6 of German Merino sheep introduced into the region from Germany.
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Doença de Borna/epidemiologia , Vírus da Doença de Borna/isolamento & purificação , Cães/virologia , Sequência de Aminoácidos , Animais , Doença de Borna/virologia , Vírus da Doença de Borna/genética , China/epidemiologia , Alemanha/epidemiologia , Doenças dos Cavalos/epidemiologia , Doenças dos Cavalos/virologia , Cavalos/virologia , Leucócitos Mononucleares/virologia , Filogenia , Plasmídeos/genética , RNA Viral/sangue , RNA Viral/genética , RNA Viral/isolamento & purificação , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Ovinos/virologia , Doenças dos Ovinos/epidemiologia , Doenças dos Ovinos/virologia , Especificidade da EspécieRESUMO
OBJECTIVE: To investigate the epidemiological pattern of Borna disease virus (BDV) infection in horses and to analyze the phylogenetic tree of derived BDV in Yili, Xinjiang. METHODS: We established a modified nested RT-PCR (nRT-PCR) to detect BDV p24 segment in peripheral blood mononuclear cells (PBMCs) and brain tissues of 120 horses in Yili, Xinjiang. Positive products were analyzed by sequencing and homology analysis. RESULTS: The positive rate of BDV infection was 2.5% in both PMBCs and brain tissues at the same time. The gene sequence revealed in positive PCR samples was more than 93%, identical to that of BDV derived from horses in other countries. We also noticed a high degree of identity (> 98%) to standard strain He/80 in gene sequence of positive PCR samples. CONCLUSION: Our study found the presence of BDV natural infection in horses in Yili. The endemic BDV had a high degree of identity to standard strain He/80.