Endovascular treatment of distal anterior cerebral artery aneurysms using flow modulation devices: mid- and long-term results from a two-center study.

Purpose: Flow-diverter (FD) stents have become an established treatment for intracranial aneurysms in recent years, but their use for aneurysms in distal cerebral vessels with small carrier vessel diameters remains controversial. This study describes the method and mid- and long-term outcomes of FD treatment of distal anterior cerebral artery aneurysms (DACAAs) at two neurointerventional centers, to elucidate this topic and provide more in-depth data. Methods: Data for all patients at two neurointerventional centers who were treated with FDs for DACAAs in the pericallosal and supracallosal segment of the anterior cerebral artery were retrospectively analyzed. Data on periprocedural complications, and short-, mid- and long-term follow-up findings were recorded. Results: Forty-one patients were eligible for inclusion in the study. Three FD models were used, one of which had an anti-thrombotic coating. Two periprocedural complications (5%) occurred but did not cause a change in the mRS. In the long-term follow-up, at 29 months and beyond, 83% of assessable patients showed complete occlusion of the aneurysms without new neurological deficits. Conclusion: FDs are a safe and effective treatment approach for DACAAs. This study indicated a low risk of complications, and high closure rates in short-, mid- and long-term follow-up.

ANCA-associated refractory vasculitis with multiple systemic involvement: A rare case report.

We report the case of a 65 year old female patient, presenting with a combination of bilateral hearing loss, otalgia, and hyperacusis. Pure tone audiometry revealed mixed bilateral hearing loss. Conventional cranial imaging tests failed to show a significant brain pathology, but fat-suppressed T1-weighted gadolinium-enhanced magnetic resonance imaging scan displayed a diffuse infiltrative skull base process, extending from the nasopharynx to the jugular fossa, and encasing the internal carotid artery. The latter findings, besides elevated inflammatory markers and a positive perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) led to the diagnosis of ANCA-associated vasculitis. Additional disease manifestations sequentially appeared, including a right peripheral nerve palsy, aortitis, hepatitis, peripheral neuropathy, and uveitis. Therapy with corticosteroids, azathioprine, and then cyclophosphamide brought no evident benefit, but rituximab led to impressive clinical and radiologic improvement.

Carotid Artery Stenting in Patients with Atrial Fibrillation: Direct Oral Anticoagulants, Brief Double Antiplatelets, and Testing Strategy.

Carotid endarterectomy is usually preferred over carotid artery stenting (CAS) for patients with atrial fibrillation (AF). We present our experience with short-course periprocedural triple antithrombotic therapy in 32 patients aged >18 years with nonvalvular AF undergoing CAS. There were no deaths, cardiac events, embolic strokes, hyperperfusion syndrome, intracranial hemorrhage, or stent thrombosis within 30 days. Transient intraprocedural hemodynamic instability in 15/32 (47%) and prolonged instability in 4/32 (13%) was managed conservatively. At a mean 16-month follow-up, there were no new neurological events or deterioration. Mean stenosis was reduced from 78.0% ± 9.7% to 17.3% ± 12.2%. This retrospective study included patients AF who were symptomatic (minor stroke (NIHSS ≤ 5)/TIA) with ICA stenosis >50%, or asymptomatic under DOAC therapy with carotid stenosis >80%, who underwent CAS from 6/2014-10/2020. Patients received double antiplatelets and statins. Antiplatelet therapy effectiveness was monitored. Stenting was performed when P2Y12 reaction units (PRU) were <150. DOACs were discontinued 48 h before angioplasty; one 60 mg dose of subcutaneous enoxaparin was administered in lieu. DOAC was restarted 12-24 h after intervention. Patients were discharged under DOAC and one nonaspirin antiplatelet. 32 patients on DOAC were included (26 male, mean age 71). 19 (59.4%) presented with stroke (ICA stenosis-related in 14); 13 (40.6%) were asymptomatic. Stents were deployed under filter protection following pre-angioplasty; post-angioplasty was performed at least once in 12 patients (37.5%). Our experience suggests that CAS can be safely performed in selected patients with CAS and AF requiring DOAC. The role of CAS in AF patients under DOAC warrants study in rigorous trials.

Standalone Flow Diversion Therapy Effectively Controls Rebleeding of Acutely Ruptured Internal Carotid Artery Trunk (Nonbranching) Microaneurysms.

Flow diversion is a promising option in selected patients with acutely ruptured microaneurysms. In this article, we reviewed our experience. Patients with acute spontaneous subarachnoid hemorrhage (SAH) after rupture of a blister-like or saccular microaneurysm (≤2 mm maximal diameter) at a nonbranching ICA site treated from January 2016 to June 2019 using flow diversion as standalone therapy were included in this study. An EVD was usually placed preventively. Antiplatelet effects of pre-procedure DAPT were evaluated (target PRU, 80-160). After the intervention, DAPT was continued for ≥6 months, aspirin-indefinitely. Angiographic controls were obtained. Fifteen patients (12 female; mean age, 46.4 years) with 15 ruptured ICA microaneurysms (mean diameter, 1.8 mm) were included. An EVD was placed in 12 patients (75%) before DAPT administration and stenting. PRU values immediately before FDS were 1-134 (mean, 72.1). One patient died 27 days after flow diversion due to a suspected fulminant pulmonary embolism. Aneurysms were completely occluded at the 6-12-month angiographic follow-up in 14/14 surviving patients, with no rebleeding at a mean of 14 months. Late mRS was 0-2 in 13/14 patients and 3 in one due to sequelae of the original hemorrhage. Flow diversion provided robust aneurysm rebleeding control. Angiographic follow-up confirmed complete aneurysm occlusion in all the cases.

Neurological variability in chemotherapy-induced posterior reversible encephalopathy syndrome associated with thrombotic microangiopathy: Case reports and literature review.

Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome characterized by headaches, seizures, a confusional state and visual disturbances associated with transient predominantly bilateral posterior white mater magnetic resonance imaging lesions. It is primarily reported in the setting of hypertension, acute renal failure, peripartum eclampsia, autoimmune disease, immunosuppression and chemotherapy. Thrombotic microangiopathy (TMA), including hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) has also been reported as potential PRES inducer. The present study reviews two cases of patients with PRES, associated with TMA caused by chemotherapy. Their clinical and imaging data, and the relevant literature were reviewed. Patient 1 presented with TMA-induced PRES following mitomycin-C for metastatic colon adenocarcinoma. Treatment with steroids, plasma exchange, intravenous immunoglobulins, aspirin, antihypertensive drugs, and diuretics resulted in resolution of the neurological and imaging deficits. Patient 2 presented with TMA-induced PRES following gemcitabine for metastatic breast carcinoma. Treatment was ineffective and the patient deteriorated despite verapamil, dexamethasone, and plasma exchange. In this report, the relevant literature regarding pathogenesis, treatment and prognosis of chemotherapy-induced PRES associated with TMA was reviewed. We conclude that several chemotherapy agents may cause PRES through various pathogenic mechanisms, leading to clinical variability and divergent response to therapy.

Clinical and angioarchitectural factors influencing the endovascular approach to galenic dural arteriovenous fistulas in adults: case series and review of the literature.

BACKGROUND: Galenic dural arteriovenous fistulas (DAVF) are rare; however, they are the most frequent type of DAVF to manifest aggressive clinical behavior and usually represent a diagnostic and therapeutic challenge for clinicians. METHODS: We retrospectively reviewed clinical and imaging data of patients managed with neuroendovascular techniques for the treatment of galenic DAVFs from 2000 to 2016. We searched the 2000-2016 English-language literature for papers discussing neuroendovascular management of galenic DAVFs, with or without companion surgical procedures. RESULTS: Five patients were treated for galenic DAVFs during the study period (four males; mean age, 61 years). Three presented with progressive neurological deterioration due to venous congestion, two with acute intracranial hemorrhage. Three were treated by staged transarterial embolization procedures (three procedures in two, four procedures in one); two underwent a single transvenous embolization procedure. Four out of five fistulas were completely occluded. All patients improved clinically; the patient whose fistula was partially occluded remains angiographically stable at 2-year follow-up. Six reports describing 17 patients are reviewed. Embolization was performed via transvenous approach in 1/17 and transarterial approach in 16/17 with additional open surgery in 9/16. The trend toward the use of transarterial approaches is based primarily on advances on embolization techniques that allow better and more controllable penetration of the embolizing agents with improved clinical and angiographic results, as well as the technical complexity of the transvenous approach. CONCLUSIONS: Although transarterial embolization is the preferred endovascular route for the management of most galenic DAVFs, selected cases can be successfully treated by transvenous approach.

Sandfly virus seroconversion associated with neurologic presentation.

OBJECTIVE: To describe the clinical presentation and unique neurologic manifestations of sandfly viruses (SFVs) in the Jerusalem area. METHODS: We identified all patients with acute seroconversion to SFV at the Hadassah-Hebrew University Medical Centers during the years 2008-2013 and retrospectively collected and analyzed the clinical and imaging data. RESULTS: Nine patients (ranging from 1.5 to 85 years old) were identified. Presentation included acute neurologic disease, mostly with fever, change in consciousness and behavior, seizures, headache, meningitis, limb paresis, or myelitis. Eight patients had clinical signs of meningitis, meningoencephalitis, or encephalitis alone. Four patients had myelitis. MRI identified pathologic symmetrical changes in the basal ganglia, thalami, and other deep structures in 5 patients, and additional myelitis of the spine was noted on imaging in 3 patients. Seven patients had long-term follow-up: 4 completely recovered and 3 had remaining neurologic sequelae, among them 1 with permanent severe brain damage. CONCLUSION: Neurologic involvement associated with acute SFV infections is considered to be benign. However, in this series, all 9 patients presented with significant neurologic pathology associated with a unique finding of myelitis and symmetrical basal ganglia, thalami, or white matter involvement. Thus, acute SFV infection should be included in the differential diagnosis in febrile onset of neurologic manifestations and neuroradiologic changes.

Devastating recurrent brain ischemic infarctions and retinal disease in pediatric patients with CD59 deficiency.

Identification of CD59 p.Cys89Tyr mutation in 5 patients from North-African Jewish origin presenting with chronic inflammatory demyelinating polyradiculoneuropathy like disease and chronic hemolysis, led us to reinvestigate an unsolved disease in 2 siblings from the same origin who died 17 years ago. The two patients carried the same CD59 gene mutation previously described by our group. These children had quiet similar disease course but in addition developed devastating recurrent brain infarctions, retinal and optic nerve involvement. Revising the brain autopsy of one of these patients confirmed the finding of multiple brain infarctions of different ages. CD59 protein expression was missing on brain endothelial cells by immunohistochemical staining. This new data expands the clinical spectrum of CD59 mutations and further emphasizes the need for its early detection and treatment.

Influence of common fixed retainers on the diagnostic quality of cranial magnetic resonance images.

INTRODUCTION: Orthodontists are often asked to remove fixed retainers before magnetic resonance imaging (MRI). This study was undertaken to assess the effects of 2 commonly used fixed retainers on MRI distortion and whether they should be removed. METHODS: MRI scans were performed on a dry skull with Twistflex (Dentaurum, Ispringen, Germany) and Ortho Flex Tech (Reliance Orthodontic Products, Itasca, Ill) retainers. Two neuroradiologists independently ranked the distortions. The influence of the fixed retainers' alloys, their distance to the area of diagnosis, location, strength of the magnetic field, and the spin-echo sequence were examined. Statistical analysis included kappa and Pearson chi-square tests. RESULTS: Ortho Flex Tech retainers caused no distortion. Twistflex retainers caused distortion in 46% of the tests in areas close to the retainer (tongue and jaws). Maxillary fixed retainers and the combination of maxillary and mandibular fixed retainers further increased the distortion. Greater distortion was observed with 3-T magnetic fields and T1-weighted spin-echo sequences. CONCLUSIONS: Removal of the Ortho Flex Tech retainer is unnecessary before MRI. Removal of the Twistflex should be considered if the MRI scans are performed to diagnose areas close to the fixed retainers, when 3-T magnetic fields and T1-weighted sequences are used, and when both maxillary and mandibular fixed retainers are present.

Dynamic MRI testing of the cervical spine has prognostic significance in patients with progressive upper-limb distal weakness and atrophy.

BACKGROUND: The syndrome of isolated progressive upper-limb distal weakness and atrophy results from disease processes affecting lower motor neurons originating in the cervical anterior horn gray matter. Lower motor neuron dysfunction restricted to the C7-T1 myotomes in the absence of neuropathy, upper motor neuron signs, sphincter disturbances or abnormality on conventional MR imaging is suggestive of either Hirayama disease (HD), or the initial manifestation of a progressive motor neuron disease such as amyotrophic lateral sclerosis (ALS). In HD the supposed etiologic mechanism is a mechanical compression of the cervical spinal cord during neck flexion; therefore, dynamic MRI (dMRI) of the cervical cord might help differentiate between these possibilities. METHODS: This was a multi-center observational cohort study. Over a 4-year period between 8/2009 and 8/2013, 22 patients were identified as having a disease consistent with HD. We identified a subgroup of patients suspected of suffering from active progressive disease and prospectively followed them after performing dynamic MRI studies of the cervical spine. RESULTS: Twenty-two patients were identified as having a disease consistent with HD, of whom 8 were defined as having actively progressive disease. Seven of these 8 patients demonstrated clear dynamic compression of the cervical spine during neck flexion. The patient who did not demonstrate the typical MRI changes associated with HD went on to develop generalized ALS. CONCLUSIONS: dMRI has a practical role in patients presenting with progressive upper-limb distal weakness and atrophy, and the presence of characteristic changes typical of HD may suggest a more optimistic prognosis.

Delayed complications after flow-diverter stenting: reactive in-stent stenosis and creeping stents.

We assessed the frequency and severity of changes in stent configuration and location after the treatment of intracranial aneurysms, and patterns of in-stent stenosis. We retrospectively reviewed data for consecutive aneurysm patients managed with endovascular implantation of flow-diverter stents (Silk Flow Diverter [Balt Extrusion, Montmorency, France] and Pipeline Embolization Device [ev3/Coviden, Minneapolis, MN, USA]) from October 2011 to July 2012. Routine 2, 6, 9-12, and 16-20 month follow-up angiograms were compared, with a focus on changes in stent configuration and location from immediately after deployment to angiographic follow-up, and the incidence and development of in-stent stenosis. Thirty-four patients with 42 aneurysms met inclusion criteria. The Silk device was implanted in 16 patients (47%, single device in 15), the Pipeline device in 18 (53%, single device in 16). On first follow-up angiography, in-stent stenosis was observed in 38% of Silk devices and 39% of Pipeline devices. In-stent stenosis was asymptomatic in 12 of 13 patients. One woman presented with transient ischemic attacks and required stent angioplasty due to end tapering and mild, diffuse in-stent stenosis. Configuration and location changes, including stent creeping and end tapering were seen in 2/16 patients (13%) with Silk devices, and 0/18 patients with Pipeline devices. We describe stent creeping and end tapering as unusual findings with the potential for delayed clinical complications. In-stent stenosis, with a unique behavior, is a frequent angiographic finding observed after flow-diverter stent implant. The stenosis is usually asymptomatic; however, close clinical and angiographic monitoring is mandatory for individualized management.

Stiletto stabbing: penetrating injury to the hypothalamus with hyperacute diabetes insipidus.

Diabetes insipidus (DI) is a well documented complication observed after traumatic head injuries. We report a case of hyperacute onset DI in a 19-year-old male who sustained a hypothalamic-pituitary injury when he was stabbed in the head with a 30-cm long thin-bladed knife. At CT, our patient showed significant hemorrhagic contusions of the lower hypothalamus. He developed polydipsia, polyuria, and mild hypernatremia in the Emergency Department. Diagnostic digital subtraction angiography showed a hypervascular congestive pituitary gland with prominent draining veins. On the third day his hypernatremia became severe (183mEq/L). He was managed with parenteral fluids and a regimen of intranasal DDAVP (1-desamino 8-d-arginine vasopressin), leading to improved plasmatic sodium levels, urine output, and urinary specific gravity. In patients presenting with hyperacute posttraumatic DI, emergency room physicians and neurosurgeons should rule out direct injury to the hypothalamus and/or the posterior lobe of the pituitary, and initiate early pharmacological treatment.

Therapeutic modalities in symptomatic intracranial arterial occlusive disease.

Intracranial atherosclerotic disease (ICAD) is a frequent cause of stroke that is sometimes underdiagnosed. In this review, we survey the literature concerning ICAD and present the prognostic factors and the therapeutic options pertinent to it by comparing medical, surgical and endovascular approaches.

Joubert syndrome 2 (JBTS2) in Ashkenazi Jews is associated with a TMEM216 mutation.

Patients with Joubert syndrome 2 (JBTS2) suffer from a neurological disease manifested by psychomotor retardation, hypotonia, ataxia, nystagmus, and oculomotor apraxia and variably associated with dysmorphism, as well as retinal and renal involvement. Brain MRI results show cerebellar vermis hypoplasia and additional anomalies of the fourth ventricle, corpus callosum, and occipital cortex. The disease has previously been mapped to the centromeric region of chromosome 11. Using homozygosity mapping in 13 patients from eight Ashkenazi Jewish families, we identified a homozygous mutation, R12L, in the TMEM216 gene, in all affected individuals. Thirty individuals heterozygous for the mutation were detected among 2766 anonymous Ashkenazi Jews, indicating a carrier rate of 1:92. Given the small size of the TMEM216 gene relative to other JBTS genes, its sequence analysis is warranted in all JBTS patients, especially those who suffer from associated anomalies.

Automatic segmentation and classification of multiple sclerosis in multichannel MRI.

We introduce a multiscale approach that combines segmentation with classification to detect abnormal brain structures in medical imagery, and demonstrate its utility in automatically detecting multiple sclerosis (MS) lesions in 3-D multichannel magnetic resonance (MR) images. Our method uses segmentation to obtain a hierarchical decomposition of a multichannel, anisotropic MR scans. It then produces a rich set of features describing the segments in terms of intensity, shape, location, neighborhood relations, and anatomical context. These features are then fed into a decision forest classifier, trained with data labeled by experts, enabling the detection of lesions at all scales. Unlike common approaches that use voxel-by-voxel analysis, our system can utilize regional properties that are often important for characterizing abnormal brain structures. We provide experiments on two types of real MR images: a multichannel proton-density-, T2-, and T1-weighted dataset of 25 MS patients and a single-channel fluid attenuated inversion recovery (FLAIR) dataset of 16 MS patients. Comparing our results with lesion delineation by a human expert and with previously extensively validated results shows the promise of the approach.

Complete and persistent occlusion of arteriovenous malformations of the mandible after endovascular embolization.

OBJECTIVE: Arteriovenous malformation (AVM) of the mandible is a rare but potentially life-threatening entity. Traditional treatment involved complex surgical procedures that usually failed to completely cure the malformation without disfigurement and functional difficulties. We report our experience in transarterial and transvenous embolizations of mandibular AVMs using different embolization agents and discuss the potential use of Onyx and new detachable-tip microcatheters. CLINICAL PRESENTATION: Patients presented with progressive mandibular swelling, pain, soft-tissues discoloration and dental misalignment with tooth loosening. INTERVENTION: The AVMs were completely and persistently occluded by endovascular transarterial and transvenous approaches. CONCLUSION: Less invasive endovascular approaches proved to be highly effective in curing certain types of mandibular AVMs. Every malformation requires a tailored endovascular strategy in terms of approach and selection of an embolizing agent.

Mutations in the fatty acid 2-hydroxylase gene are associated with leukodystrophy with spastic paraparesis and dystonia.

Myelination is a complex, developmentally regulated process whereby myelin proteins and lipids are coordinately expressed by myelinating glial cells. Homozygosity mapping in nine patients with childhood onset spasticity, dystonia, cognitive dysfunction, and periventricular white matter disease revealed inactivating mutations in the FA2H gene. FA2H encodes the enzyme fatty acid 2-hydroxylase that catalyzes the 2-hydroxylation of myelin galactolipids, galactosylceramide, and its sulfated form, sulfatide. To our knowledge, this is the first identified deficiency of a lipid component of myelin and the clinical phenotype underscores the importance of the 2-hydroxylation of galactolipids for myelin maturation. In patients with autosomal-recessive unclassified leukodystrophy or complex spastic paraparesis, sequence analysis of the FA2H gene is warranted.

Prenatal thrombosis of torcular Herophili with spontaneous resolution and normal outcome.

BACKGROUND: Prenatal thrombosis of torcular Herophili is a rare condition. It may be suspected during the routine ultrasonographic follow-up of the fetus, but MRI is necessary to establish the diagnosis. There are 7 reported cases with various results. METHODS: We report a case of prenatal torcular Herophili thrombosis. We present 2 series of fetal MRIs. The first one was performed at 21 and the second at 37 weeks' gestation. RESULTS: The second MRI revealed a total resolution of the thrombus. The product was a neurologically intact infant with normal development. CONCLUSIONS: This is the fourth reported case of fetal torcular Herophili thrombosis with normal outcome. There are no sufficient data regarding the best management of this rare condition; however, following up with fetal MRIs seems to be the most rational choice.