Pathogenesis of aquaporin-4 immunoglobulin G positive neuromyelitis optica spectrum disorder / 中华神经科杂志

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease of the central nervous system characterized by the involvement of the optic nerve and spinal cord. The main clinical features are optic neuritis, acute myelitis, and area postrema syndrome. Aquaporin-4 (AQP4)-IgG-positive patients accounted for the majority and compared with AQP4-IgG-negative patients, the clinical symptoms were more severe, the recurrence was more frequent, and the disability rate was higher. The pathogenesis of AQP4-IgG-positive NMOSD is still not clear. This article reviews the research progress of the pathogenesis of AQP4-IgG-positive NMOSD.

Pathophysiological mechanisms of futile recanalization following endovascular therapy for acute ischemic stroke due to large vessel occlusion and potential targeted therapeutic strategy / 中华神经科杂志

Endovascular therapy is the standard treatment for acute ischemic stroke due to large vessel occlusion. However, an increasing number of studies have demonstrated that about half of patients had poor functional recovery despite successful recanalization, namely futile recanalization. Previous studies suggested that tissue no-reflow (lack of reperfusion despite recanalized large vessel), early re-occlusion, poor collateral status, hemorrhagic transformation, impaired cerebral autoregulation and large hypoperfusion volume may all contribute to futile recanalization. Targeted treatment strategies for these mechanisms have been attempted in pre-clinical researches but failed to translate into clinical practices. This review aimed to summarize the mechanisms of futile recanalization and potential targeted treatment strategies, particularly focus on the no-reflow phenomenon and its mechanisms, clinical evaluation, and treatment approaches, hoping to promote translational research and improve the status of stroke treatment.

Nasu-Hakola disease due to a homozygous triggering receptor expressed on myeloid cell 2 gene mutation / 中华神经科杂志

Nasu-Hakola disease is an extremely rare genetic disorder with cognitive dysfunction and fractures as the main clinical manifestations. The clinical characteristics, laboratory, imaging, and genetic data of a Nasu-Hakola case from a consanguineous Chinese family were analyzed. The patient was a 40-year-old female complaining about progressive forgetting and behavior change of three years and urinary incontinence of eight months. Neurological examination of the patient showed tetra-pyramidal signs. Neuropsychological testing revealed severe cognitive and behavioral impairment. Head magnetic resonance imaging showed generalized brain atrophy predominantly involving the frontal lobe, caudate nucleus, and anterior corpus callosum, and head computer tomography showed bilateral basal ganglia calcification. The patient had no history of bone pain or fracture and a skeletal survey showed no abnormalities. Whole exome sequencing identified a novel homozygous triggering receptor expressed on myeloid cells 2 gene mutation (c.523delA) in the patient and confirmed the heterozygous status of her parents and sisters. The patient showed no signs of improvement during the last six months after discharge. Although the patient′s clinical presentations mimicked the behavioral variant of frontotemporal dementia, reduced 42-amino acid form of amyloid-β protein level in the cerebrospinal fluid suggested amyloid deposition in the brain, which might be related to astrocytic dysfunction.

The research progress of carotid web and cryptogenic stroke / 中华神经科杂志

Carotid web can be defined as an endoluminal shelf-like projection often noted at the origin of the internal carotid artery just beyond the bifurcation.In recent years,it has been found that the carotid web is one of possible risk factors for cryptogenic stroke.Understanding the pathogenesis and pathological characteristics of carotid artery web,and accurately diagnosing the carotid artery web will help to implement targeted intervention for cryptogenic stroke,and reduce the recurrence of stroke events.In this paper,we will review the research progress of relationship between the carotid artery web and cryptogenic stroke,the common imaging features,pathological research,pathogenesis,diagnosis and treatment.

Expression of nNOS in the hippocampus in vascular dementia rats / 临床神经病学杂志

Objective To explore the expression of neuronal nitric oxide synthase (nNOS) in the hippocampus in vascular dementia rats.Methods 60 rats were randomly divided into five groups: control group, model 12 h group,model 1 d group, model 3 d group and model 7 d group.An animal model of vascular dementia (VD) was established through repeated cerebral ischemia-reperfusion in rats with repeated bilateral common carotid arteries occlusion. The number of neurons in CA 1 area of hippocampus in every group was observed using HE staining. The expression of nNOS in the hippocampus in VD rats was detected by immunohistochemical staining and western blot method.Results The number of neurons in CA 1 area of hippocampus decreased significantly in 12 h, 1 d, 3 d and 7 d group. The expression of nNOS in CA 1 area of hippocampus was weak in control group and up-regulated in 12 h group and increased further in 1 d group. However, its expression decreased gradually in 3 d and 7 d group.Conclusion nNOS may be related to the injury of hippocampus at early phase in VD rats. It might be regarded as one of mechanisms to cause VD.