A case report of autoimmune GFAP astrocytopathy presenting with abnormal heart rate variability and blood pressure variability.

BACKGROUND: Autonomic dysfunctions including bladder dysfunction, gastrointestinal dysfunction and orthostasis are common symptoms of autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A); however, cardiac autonomic dysfunction and abnormal circadian rhythm of blood pressure, which can lead to poor prognosis and even sudden cardiac death, has never been reported in A-GFAP-A patient. CASE PRESENTATION: A 68-year-old male Chinese patient presented to our hospital with headache, fever, progressive disturbance of consciousness, dysuria, and limb weakness. Abnormal heart rate variability and non-dipper circadian rhythm of blood pressure gradually developed during hospitalization, which is rare in A-GFAP-A. He had positive GFAP IgG in cerebrospinal fluid (CSF). Enhanced brian MRI showed uneven enhancement and T2 hyperintense lesions of medulla oblongata; Cervical spine MRI showed T2 hyperintense lesions in medulla oblongata and upper margin of the T2 vertebral body. A contrast-enhanced thoracic spine MRI showed uneven enhancement and T2 hyperintense lesions of T1 to T6 vertebral segments. After treatment with intravenous immunoglobulin and corticosteroids, the patient's symptoms, including autonomic dysfunction, alleviated dramatically. Finally, his heart rate variability and blood pressure variability became normal. CONCLUSIONS: Our case broadens the spectrum of expected symptoms in A-GFAP- A syndromes as it presented with heart rate variability and blood pressure variability.

A unique temporary collateral pathway between carotid-vertebrobasilar arteries in a carotid dissection patient.

BACKGROUND: In adults, the anastomosis between carotid and vertebrobasilar arteries is usually the posterior communicating artery, sometimes the primitive trigeminal artery. In this case, the basilar artery fed the internal carotid artery through the pontine-to-tentorial artery anastomosis after severe stenosis from traumatic carotid dissection. CASE PRESENTATION: A 32-year-old female was diagnosed with ischemic stroke caused by traumatic carotid artery dissection. Aspirin (100 mg/day) and clopidogrel (75 mg/day) were prescribed. Digital subtraction angiography performed 6 days after stroke onset showed a dissection in the cervical segment of left internal carotid artery with severe local stenosis, and a collateral pathway from BA to the cavernous segment of internal carotid artery through the lateral pontine and tentorial artery. Without interventional therapy, clinical symptoms improved significantly within 10 days after onset. At 3-month follow-up, left common carotid artery angiography showed the stenosis had been significantly improved with a residual aneurysm. There was no collateral pathway between carotid-vertebrobasilar arteries, and a residual small artery originated from the posterior vertical segment of cavernous internal carotid artery. The small artery was clearly visualized by 3-dimensional rotational angiography and identified the tentorial artery. CONCLUSION: To the author's knowledge, this is the first report of a collateral pathway between carotid vertebrobasilar arteries through the pontine-to-tentorial artery anastomosis. Meanwhile, tentorial artery origination directly from the cavernous segment of internal carotid artery is rare and easily mistaken for persistent primitive trigeminal artery. 3-dimensional rotational angiography can provide sensitive and accurate diagnostic assessment of the small artery, and may be a useful tool for screening of abnormal small arteries.

Effectiveness and safety of high dose clopidogrel plus aspirin in ischemic stroke patients with the single CYP2C19 loss-of-function allele: a randomized trial.

BACKGROUND: Dual antiplatelet aggregation therapy leads to better outcomes in patients with carotid artery stenosis, intracranial artery stenosis, minor strokes, or transient ischaemic attacks. However, carriers of the CYP2C19 loss-of-function allele may not experience the desired effects. We attempted to increase the clopidogrel dose to determine whether it would improve the outcomes of stroke patients who carry a single loss-of-function allele. METHODS: We recruited 131 patients with minor ischaemic stroke, within less than 7 days of stroke onset and a CYP2C19 loss-of-function allele, who had moderate-to-severe cerebral artery stenosis. Patients were divided into the high dose group (clopidogrel 150 mg per day + aspirin 100 mg per day over 21 days.) and a normal dose group (clopidogrel 75 mg per day + aspirin 100 mg per day over 21 days). The reported outcomes included any vascular or major bleeding events as the primary and safety endpoints, respectively. RESULTS: One and six vascular events occurred in the high dose and normal dose groups during the 3-months follow-up period, respectively. However, no significant difference was found between the two groups when adjusted for history of diabetes (hazard ratio, 5482; 95% confidence interval, 0.660 to 45.543; P = 0.115). No major bleeding events occurred. CONCLUSIONS: In patients with ischaemic stroke who had a single CYP2C19 loss-of-function allele and moderate to severe cerebral stenosis, fewer vascular events occurred within 3 months with high dose of clopidogrel and aspirin than with normal dose of clopidogrel and aspirin. However, the difference between the two groups was not significant. TRIAL REGISTRATION: Clinical study of clopidogrel in the treatment of patients with symptomatic moderate to severe cerebral artery stenosis with intermediate metabolites of CYP2C19, URL: http://www.chictr.org.cn/ . Unique identifier: ChiCTR1800017411 , 07/28/2018.