A case of sporadic adult-onset neuronal intranuclear inclusion disease presented with pure autonomic dysfunction / 中华神经科杂志

Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous system, and also in the visceral organs. Although in recent years skin biopsy is useful for the antemortem diagnosis of this disease, it is often misdiagnosed due to its highly variable clinical manifestations. A case of NIID is reported here. The patient had a long course of disease, mainly presenting as dysfunction of autonomic nervous system. No significant cognitive impairment was found. Thus, a new idea is provided for the diagnosis of this disease.

Retrospective study of stroke mechanism and lesion patterns in middle cerebral artery territory / 中华神经科杂志

Objective To identify lesion patterns and stroke mechanisms in middle cerebral artery (MCA) territory using early diffusion-weighted imaging (DWI) combined with CTA as well as EKG and echocardiography.Methods One hundred and forty-eight acute ischemic stroke patients who had (1) symptomatic lesions located in the unilateral MCA territory on DWI performed within 1 week of symptom onset,and (2) either corresponding MCA disease,internal carotid artery (ICA) disease,MCA & ICA disease or cardio embolism (CE),or (3) neither corresponding MCA disease,ICA disease,nor CE which were taken as group of negative results (NR),were reviewed.Acute DWI lesion patterns were classified as (1) single (small perforator < 2 cm;large perforator ≥2 cm;pial;large territorial;border-zone) and (2) multiple according to principle of single-blind.Results There were 12 types of lesions in MCA territory.Distribution of lesion patterns in different stroke subtypes might be different (χ2= 55.88,P = 0.004).No specific pattern could be found in patients with MCA disease,ICA disease,MCA & ICA disease or CE.Big perforator infarcts might be more common in patients with MCA disease than with ICA disease and CE.Compared with negative group,concomitant perforator and pial infarcts were more common in patients with ICA disease (7/27,χ2=6.61,P <0.05),especially with severe stenosis or occlusion (5/16,χ2=7.32,P < 0.05);No specific pattern could be found in patients with MCA disease or CE.Concomitant perforator,pial,with border-zone infarcts (6/30,χ2= 6.41,P <0.05),and concomitant perforator with border-zone infarcts (4/30,χ2= 5.59,P < 0.05) were more often in patients with severe stenosis or occlusion of MCA.Conclusion Different lesion patterns may indicate different mechanisms of stroke such as hypoperfusion and arterial embolism could be coexistent in MCA territory.The relationship has not been identified perfectly.