Until recently, cerebral arteriopathy due to heterozygous mutations of the ACTA2 gene was considered a variant of moyamoya disease. However, radiographic analysis of patients with these mutations reveals a distinctive angiographic appearance from that seen in moyamoya disease. Several heterozygous missense ACTA2 mutations have been implicated in the development of this distinct cerebrovascular entity; however, the penetrance and systemic manifestations of these mutations vary based on the location of the amino acid replacement within the α-smooth muscle actin protein. The severity of the phenotype may also differ among patients within a single mutation type. There is limited literature on the safety and efficacy of revascularization procedures for ACTA2 arteriopathy, which have been limited to those patients with known Arg179His mutations. The authors provide a review of the breadth of mutations within the ACTA2 literature and report a case of two siblings with de novo ACTA2 Arg258Cys mutations with differing clinical courses, highlighting the utility of indirect revascularization with 8-year follow-up data. This case highlights the importance of early recognition of the angiographic appearance of ACTA2 cerebral arteriopathy and performance of genetic testing, as the location of the mutation impacts clinical presentation and outcomes.

Morning glory disc anomaly (MGDA) characterizes a congenital dysgenetic disorder of the optic disc, coexisting with arterial intracranial abnormalities, including Moyamoya vascular disease, a significantly rare disease in the European populations. We report a 2.5-year-old female child from Greece previously diagnosed with MGDA, who presented with right-hand paresis, accompanied by focal epileptic spasms, followed by an episode of brief absence seizure, as well as some arm clonic spasms. Magnetic resonance angiography scan revealed the presence of an anomalous origin of the anterior cerebral artery (ACA) from the internal carotid artery (ICA) along with vascular abnormalities, compatible with Moyamoya pattern. To the very best of our knowledge, this is the first reported case of anomalous origin of ACA from the supraclinoid ICA accompanied by severe occlusive intracranial disease (moyamoya-like pattern) in a patient with known MGDA, highlighting the embryonic character of the vascular manifestations in MGDA. It also verifies the association of Moyamoya pattern with MGDA, thus linking vascular dysgenesis as a possible cause of MGDA.

Pulmonary vascular abnormalities are important causes of hemoptysis. Arteriovenous malformation (AVM), pulmonary arterial aneurysms or invasion of the pulmonary arterial structures by the tumor may cause hemoptysis. Pulmonary artery aneurysms (PAA) are an infrequent disease of the pulmonary vasculature. Endovascular coil application is a convenient treatment option for the treatment of hemoptysis due to vascular anomalies. The migration of intravascular coil to another tissue is a rare complication. To review this extremely rare complication, herein we report two unusual cases who had pulmonary artery aneurysm and who had hemoptysis due to tumor invasion to pulmonary artery, initially treated with endovascular coil successfully. In both cases endovascular coil was migrated to the bronchus subsequently. Lobectomy may be performed in such cases with coil migration into the bronchus or conservative therapy with follow-up chest imaging may be a suitable treatment option for selected patients. The choice of treatment should be made individually for each patient considering the characteristics of the patients. In patients with coils, the biopsy can lead to massive hemorrhages that are fatal.

BACKGROUND: Cranial arterial air embolism is a rare but potentially fatal complication after computed tomography (CT)-guided pulmonary interventions.
METHODS: A 64-year-old man was diagnosed with a pulmonary nodule (diameter: approximately 1 cm) in the right lower lobe. The patient developed convulsions after CT-guided hook-wire localization.
METHODS: Cranial CT revealed arborizing/linearly distributed gas in the territory of the right middle cerebral artery.
METHODS: The patient was administered hyperbaric oxygen, antiplatelet aggregation therapy, and dehydration treatment.
RESULTS: Clinical death occurred 55 hours after air embolism.
CONCLUSIONS: Systemic air embolism is a serious complication of lung puncture. Clinicians should improve their understanding of this complication and remain vigilant against air embolism.

Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction (RCVS) are rare neurological disorders with complex physiopathology which is not yet fully understood. We present here the case of a 31-year-old woman with a bi-amniotic bi-chorial pregnancy who developed immediate postpartum eclampsia after vaginal delivery, associated with RCVS and PRES. Although post-partum is a well-known precipitating factors for these diseases, to our knowledge, there are only few similar cases reported with the association of these syndromes. Repeated MRI scans were instrumental in the final diagnosis of RCVS associated with PRES, allowing us to give the patient the appropriate treatment. These two syndromes have similar symptoms but may have different treatments, thus highlighting the importance of a correct diagnosis.

A 31-year-old woman was admitted to the hospital after several episodes of transient numbness in her fingers and dysarthria. No neurological symptoms and signs were observed in this patient on admission; however, blood tests revealed elevated amylase and immunoglobulin G levels, an increased erythrocyte sedimentation rate, and positive anti-Sjögren\'s-syndrome (SjS)-related antigen A antibodies. The cerebrospinal fluid examination revealed a slight increase in the cell count and protein content. Diffusion-weighted and fluid-attenuated inversion recovery images on magnetic resonance imaging showed high-signal-intensity lesions in the cerebral white matter and basal ganglia supplied by the left middle cerebral artery. A part of the lesions was enhanced by the administration of gadolinium contrast medium. Magnetic resonance angiography suggested stenosis in the left anterior, middle, and posterior cerebral arteries. The patient was thus diagnosed with acute cerebral infarction and was then treated with argatroban, edaravone, and clopidogrel. Furthermore, Schirmer\'s test, sialography, and salivary gland biopsy results led to the diagnosis of SjS. A nerve conduction study suggested concomitant mild polyneuritis or mononeuritis multiplex. Collectively, we speculate that cerebral arterial stenosis associated with SjS leads to transient ischemic attack-like episodes, transient neurological symptoms and signs, and cerebral infarction. (Received June 13, 2018; Accepted September 11, 2018; Published December 1, 2018).

Transient Cerebral Arteriopathy (TCA) is one of the main causes of childhood stroke. Here we present an unusual case of Arterial Ischemic Stroke (AIS) caused by a TCA of posterior flow and originally located in the right thalamus. The detection of enterovirus in the cerebrospinal fluid allowed us to suppose a probable post infectious etiology. The course of symptoms was self-limited and the child had a complete clinical recovery after five days. A new ischemic lesion on the antero-inferior paravermian region of the left cerebellum was revealed by a following brain Magnetic Resonance Imaging (MRI) three months later and these findings were reported by further brain MRI control performed after 15 months. Comparing follow up Magnetic Resonance Angiography (MRA) with previous High Resolution Vessel Wall Magnetic Resonance Imaging (HRMI), we found a vessel narrowing at the level of the Posterior Inferior Cerebellar Artery that might explain the arteriopathy process. In conclusion, clinical and radiological course allow us to speculate that this multifocal cerebral arteriopathy might be a transient lesion due to enterovirus infection. To our knowledge, there are only three articles describing TCA enterovirus-related, and brain MRA was performed in only one case; in addition, no one with the involvement of the posterior circulation.

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A 68-year-old right-handed woman with acute-onset inability to stand was admitted to our department. Although left hemiparesis was minor, the neurological examination on admission showed marked body lateropulsion (BL) to the left when she stood or stepped with eyes open and feet closed. Neither ataxia nor sensory disturbance was present. Brain MRI and 3D-CT angiography revealed infarction of the right posterior cingulate and the precuneus due to dissection of the right anterior cerebral artery. BL improved on day 10 and she was discharged without sequelae on day 26. BL caused by cerebral lesions is rare, and we should recognize that infarction of the posterior cingulate and/or the precuneus can cause BL.

BACKGROUND: Intravenous tissue plasminogen activator with or without mechanical thrombectomy during the acute phase are approved therapies for ischaemic stroke. Due to the short treatment time window (<6 hours) and often treatment failure, these patients would still have an intracranial arterial occlusion (IAO). It is unclear whether these patients can benefit from subsequent interventional recanalizationof their occluded artery in the subacute phase. In this retrospective study, we have examined the efficacy and safety in patients who have received either percutaneous transluminal angioplasty (PTA) or percutaneous transluminal angioplasty and stenting (PTAS) for IAO in the subacute phase of their stroke.
METHODS: Patients with subacute symptomatic ischaemic stroke caused by IAO were assessed to identify the responsible artery and low perfusion areas by CT angiography, MR angiography or digital subtraction angiography. In eligible patients, a PTA or PTAS was performed to reopen the occluded artery. Regular antithrombotic therapy, use of statins, control of risk factors and rehabilitation therapy were prescribed after the procedure. All patients had regular follow-up up to 12 months.
RESULTS: PTA or PTAS was performed in 16 patients with cerebral infarction caused by IAO in the subacute phase. After the procedure, 12 cases were recanalized, two were partially recanalized and two failed to open. One patient with left C6 segment occlusion of the carotid artery had a central retinal artery embolism after PTAS. The perioperative adverse events were 6.25%. At 3 months, the distribution of modified Rankin scale scores was 0 (seven cases), 1 (three cases), 2 (five cases) and 3 (one case).
CONCLUSIONS: Selective PTA or PTAS could be performed in ischaemic stroke patients with a small infarct size and large area of hypoperfusion from an occluded large cerebral artery after the acute phase. It may improve neurological dysfunction and reduce the incidence of disability.