Reversible cerebral vasoconstriction syndrome with basilar artery stenosis: A case report.

INTRODUCTION: Acute severe headaches in young patients may be associated with fatal neurological complications that necessitate imaging examinations. Among acute severe headaches, a thunderclap headache may indicate the rupture of a cerebral aneurysm or the onset of reversible cerebral vasoconstriction syndrome for which emergent evaluation is required. PATIENT CONCERNS: We report the case of a 36-year-old man who presented to our hospital with an acute severe headache after excessive exercise the previous day. He was prescribed a pain reliever and discharged under the suspicion of vestibular migraine but returned to the emergency room after 4 hours due to right hemiparesis, right facial palsy, severe dysarthria, and a mild drowsy mental status. DIAGNOSIS: After cerebral angiography, we diagnosed basilar artery stenosis with acute infarction in the posterior circulation due to reversible cerebral vasoconstriction syndrome. INTERVENTIONS: Brain computed tomography angiography revealed complete occlusion of the vertebrobasilar artery. Transfemoral cerebral angiography showed spontaneous improvement in the occlusion before thrombectomy. OUTCOMES: Ten months later, high-resolution vessel wall magnetic resonance angiography showed persisting severe stenosis of the basilar artery. CONCLUSIONS: A headache in young patients with risk factors of atherosclerosis, such as smoking history, uncontrolled hypertension, and dyslipidemia may be caused by reversible cerebral vasoconstriction syndrome or ischemic stroke, which has fatal neurological complications. Therefore, reversible cerebral vasoconstriction syndrome or ischemic stroke should be suspected and appropriately evaluated in such patients, even if the headache is not the thunderclap type.

Bilateral, vertical supranuclear gaze palsy following unilateral midbrain infarct.

A 60-year-old man recently admitted for bipedal oedema, endocarditis and a persistently positive COVID-19 swab with a history of anticoagulation on rivaroxaban for atrial fibrillation, transitional cell carcinoma, cerebral amyloid angiopathy, diabetes and hypertension presented with sudden onset diplopia and vertical gaze palsy. Vestibulo-ocular reflex was preserved. Simultaneously, he developed a scotoma and sudden visual loss, and was found to have a right branch retinal artery occlusion. MRI head demonstrated a unilateral midbrain infarct. This case demonstrates a rare unilateral cause of bilateral supranuclear palsy which spares the posterior commisure. The case also raises a question about the contribution of COVID-19 to the procoagulant status of the patient which already includes atrial fibrillation and endocarditis, and presents a complex treatment dilemma regarding anticoagulation.

Intravenous thrombolysis for pure pontine infarcts caused by cardiac myxoma: a case report and literature review.

Purpose: Cardiac myxoma (CM) is a rare but important cause of ischemic stroke, and typically involves the middle cerebral artery and rarely affects the brainstem only. The safety and efficacy of intravenous thrombolysis (IVT) for CM-related acute cerebral embolism are not clear.Methods: We report a case of a 55-year-old woman who suffered a CM-related acute cerebral embolism presented with pure pontine infarcts and achieved a favorable prognosis by IVT with urokinase. We summarized the clinical data of this entity and performed a literature review of 21 previous reports of patients with CM-related acute cerebral embolism who were treated with IVT.Results: In combination with previous reports, we found that the majority of patients (81.8%) obtained improvements in symptoms after IVT, including 63.6% in remarkable clinical improvement. The total rate of IVT-induced intracerebral hemorrhage was 22.7% and all occurred within 36 h, including hemorrhagic infarction type 1 (4.5%) and parenchymal hematoma type 2 (18.2%). Most of the cases had relatively good outcomes and no case died due to IVT.Conclusion: Taken together, our findings support the use of IVT as an effective and safe tool for the ultra-early treatment of CM-related acute phase ischemic stroke.

Brain stem infarction associated with familial Mediterranean fever and central nervous system vasculitis.

Familial Mediterranean fever (FMF) is an autoinflammatory autosomal recessive disease caused by mutations of the Mediterranean fever (MEFV) gene on chromosome 16p. Clinically, it is characterized by recurrent episodes of fever and painful polyserositis. An association of FMF with systemic vasculitis, namely Henoch-Schönlein purpura, polyarteritis nodosa and Behçet's disease has been described. Neurological manifestations of FMF occur rarely and include demyelinating (MS-like) lesions, posterior reversible encephalopathy syndrome, and pseudotumour cerebri. Hitherto hardly known, we herein present a young patient with a genetically proven FMF who suffered a brain stem infarction during a typical FMF attack. After a careful diagnostic workup including cerebrospinal fluid analysis, intra-arterial angiography and leptomeningeal biopsy, a FMF-associated central nervous system vasculitis was identified as the cause of stroke. The pathophysiological background and potential therapeutic strategies are discussed.

Use of intravenous tissue plasminogen activator in a 16-year-old patient with basilar occlusion.

Intravenous tissue plasminogen activator (t-PA) is currently approved by the US Food and Drug Administration (FDA) for the treatment of ischemic stroke in patients > 18 years of age who present within 3 hours of stroke onset and meet certain criteria. We report a case of a 16-year-old, previously healthy female who presented with a basilar artery occlusion and pontine ischemic stroke. She was treated with intravenous t-PA approximately 4 hours after the onset of symptoms. The patient demonstrated a remarkable recovery 6 hours after onset of her symptoms and had minimal deficits on discharge from the hospital 1 week later. She was found to have a lupus anticoagulant and was heterozygous for the prothrombin gene G2010A mutation. These were likely contributing causes for her stroke. She was also homozygous for plasminogen activator inhibitor 1 (PAI-1) 4G/4G, which at present is a controversial stroke risk factor.

Uveitis associated with Primary Angiitis of the Central Nervous System.

BACKGROUND: In Primary Angiitis of the Central Nervous System (PACNS), disease is typically limited to the brain and spinal cord although other organs may be affected. Uveitis is occasionally seen in systemic vasculitides but is not a recognized manifestation of PACNS. We describe two patients who developed PACNS following the onset of uveitis. CASE DESCRIPTIONS: Case 1--a 47-year-old male suffered multiple TIAs and left pontine stroke shortly after two episodes of diffuse uveitis. A cerbral angiogram demonstrated multiple caliber changes within several intracranial vessels. Cyclophosphamide was added after his stroke occurred during pulse methylprednisolone therapy. Case 2--a 35-year-old male suffered a spinal cord TIA followed by hemispheric and brainstem infarctions two months after an episode of uveitis and Bell's palsy treated with oral prednisone. A cerebral angiogram demonstrated multiple caliber changes within several intracranial vessels. He was successfully treated with oral prednisone and cyclophosphamide. CONCLUSIONS: Uveitis should be considered a recognized feature of PACNS. Combination immunosuppressive therapy with prednisone and cyclophosphamide may be necessary for successful treatment.