A Lower Rate of Hyposmia in Non-Ashkenazi Jewish Patients with Parkinson's Disease: Motor and Non-motor Disease Characteristics in Different Ethnic Groups in Israel.

BACKGROUND: Little is known about phenotypical variations among ethnic groups in patients with Parkinson's disease (PD) in Israel. Clinical characteristics of non-Ashkenazi Jews (NAJ) are scantly described. OBJECTIVES: To describe clinical aspects of PD in ethnic groups in Israel, focusing on NAJ and Ashkenazi Jews (AJ). METHODS: In this cross-sectional retrospective study, we collected demographic, genetic, and clinical characteristics of patients from different ethnic Jewish backgrounds. Ethnic groups included AJ; North African Jews (NAFJ); oriental Jews (OJ) originating from Iran, Iraq, and Buchara; Balkan Jews; Yemenite Jews (YJ); and Jews of mixed origin. Clinical characteristics included hyposmia, urinary complaints, constipation, and rapid eye movement sleep behavioral disorder. Cognitive complaints, motor features, levodopa-induced dyskinesia, and motor fluctuations were collected. Motor part of the MDS-UPDRS and Hoehn and Yahr scores were collected. RESULTS: The study comprised 174 PD Jewish patients (63.2% AJ, 56.4% males). The age at onset was 65.3 ± 10.2 years; 106 patients (60.9%) were genotyped (17 glucocerebrosidase [16.0%], 13 leucine-rich repeat kinase 2 [LRRK2] [12.3%]). Rates of hyposmia were significantly higher in AJ than NAJ (56.6% vs. 39.5%, respectively, P = 0.003). No significant differences were found in motor features in all variables. Of 13 AJ patients carrying the LRRK2 mutation, only one had hyposmia. Three patients with LRRK2 were NAJ. CONCLUSIONS: Hyposmia is less prevalent in PD patients of NAJ origin than in AJ. The rate of hyposmia in NAFJ patients is particularly low. The rate of other non-motor features is similar between NAJ and AJ patients.

Traumatic cerebral dural sinus vein thrombosis/stenosis in pediatric patients-is anticoagulation necessary?

INTRODUCTION: Cerebral dural vein thrombosis/stenosis (CDVT/S) is a condition that affects the venous drainage of the brain. Risk factors and causes associated with CDVT/S include systemic risk factors that cause hypercoagulability, or local factors such as head trauma. While consensus is that non-traumatic sinus vein thrombosis should be treated with anticoagulation therapy, treatment of patients with TBI-induced CDVT is not yet established. METHODS: Retrospective review of clinical data of pediatric patients presented to our medical center from July 2017 to August 2020. Inclusion criteria were age, birth to 18 years, admission due to head trauma, head CT scan with positive traumatic findings, and follow-up in our clinic. Exclusion criteria were a normal head CT on admission and failure to follow-up. Data regarding demographics, clinical presentation, imaging findings, treatment, and status on follow-up were recorded. Study protocol was approved by our institutional ethics committee. RESULTS: One hundred sixty-two patients were enrolled. Falling accident occurred in 90.1%, a minority suffered from direct head trauma or gunshot wound. Of the patients, 95.1% suffered from mild TBI. Forty-two percent suffered from an associated intracranial injury. Fourteen cases with CDVT were included in the cohort. Linear fractures were significantly correlated with CDVT. Additionally, occipital/suboccipital fractures, associated intracranial injury, and proximity of injury to the sinus were correlated with CDVT. From this group, 12 were treated conservatively; one patient was treated surgically due to EDH. All patients with CDVT were neurologically intact at discharge. Only one patient was treated with therapeutic dose of LMWH. A total of 86.7% of patients with CDVT who were treated conservatively had full recanalization on follow-up imaging. Four patients had CDVS; all were neurologically intact at admission and discharge, and all were treated conservatively and had full recanalization on follow-up. DISCUSSION: Treatment with ACT is established in pediatric CDVT but not in the sub-group of TBI. While ACT prevents progression of thrombosis, it might cause worsening of extra-axial hemorrhage. In our study, no clinical deterioration was noted with expectant management; thus, we present an algorithm for diagnosis and treatment of trauma-induced CDVT/S in children with frequent clinical and radiologic imaging while avoiding anticoagulation. CONCLUSION: In most cases, anticoagulation therapy is not necessary in traumatic CDVT/S. Initial expectant management in children is safe. However, each case should be evaluated individually and further studies should be performed.

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.

Distal shunt revision: a simple method to avoid repeat laparotomy.

Hydrocephalus is usually treated by placement of a ventriculo-peritoneal (VP) shunt. Distal VP shunt failure is a common complication of this procedure, especially in the paediatric population. Distal shunt revisions are often made more technically difficult by challenging abdominal anatomy. In this technical note, we describe a simple technique utilizing tenting sutures and the previous shunt tract for placement of the distal abdominal catheter accurately into the peritoneal space during distal shunt revision.

[INSPIRATORY STRIDOR OF A NON-TRADITIONAL CAUSE].

INTRODUCTION: Stridor is a respiratory sound caused by turbulent air flow through narrow airways. The most common cause for acute inspiratory stridor is a viral infection of the upper airways, causing laryngotracheitis (Croup). Other common causes include an allergic reaction, foreign body aspiration and trauma. BACKGROUND: A nine week old infant with a history of meningomyelocele repair perinatally presented to the emergency room with alternating inspiratory stridor. The infant did not have a history of a recent febrile illness, and he did not suffer from cough or any other symptoms suggesting a foreign body aspiration. After the common causes of stridor were ruled out, an MRI was performed - demonstrating Chiari malformation Type 2, with herniation of the cerebellar tonsils and medullary compression. Bronchoscopy demonstrated the rare cause of the intermittent stridor - intermittent vocal cord paralysis, caused by the raised intracranial pressure and medullary compression. Due to rapid clinical deterioration, emergent surgical decompression was performed with complete recovery. CONCLUSIONS: This case report raises a rare and important cause of inspiratory stridor. Physicians should be alert to the symptoms suggesting a Chiari malformation in every child with a relevant medical history presenting with stridor. DISCUSSION: Intermittent stridor is mostly caused by an upper respiratory infection in a child with narrowed airways, allergic laryngotracheitis, or laryngomalacia. A less common cause of stidor of this kind is vocal cord paralysis, which may be caused by a symptomatic Chiari malformation.

Spinal intradural microsurgery in a nascent neurosurgical department: Lessons learned from the first 25 cases.

BACKGROUND: Between 2017 and 2021, the newly established Department of Neurosurgery at Shaare Zedek Medical Center in Jerusalem, a high volume metropolitan hospital, operated on 25 intradural lesions in 24 patients (one patient had multiple tumors). In this retrospective study, we review results and lessons learned as experienced surgeons opened a new service line. METHODS: A multidisciplinary team was assembled and led by experienced neurosurgeons with skills in both microneurosurgery and complex spine care. Standard operative techniques were used. A chart review was done to assess complications and outcome. RESULTS: 25 lesions were reviewed in 24 patients (14 female; 10 male) between the ages of 11-82 years of age. In 14 cases, gross total resection (GTR) was achieved; 11 cases underwent partial resection. Of the 11 non-GTR cases, 3 were initially planned as biopsies. In one case, there was a significant neurologic decline directly related to surgery. In a separate case, there was iatrogenic instability, necessitating further treatment. CONCLUSIONS: We identify six lessons learned in a nascent neurosurgical department, noting that surgical excellence is of paramount importance, but that the surgeon must also expand his/her role from master technician to team leader. Both microsurgical expertise for neural anatomy and understanding of spinal biomechanics for osseous anatomy is mandatory for surgery of SIDT. This retrospective analysis of our case series demonstrates experienced neurosurgeons can successfully deploy a new service line for challenging cases to the benefit of the hospital and local community.

Indocyanine green video angiography-assisted surgical management of coil migration resulting from the endovascular treatment of anterior communicating artery aneurysm: technical case report and instrumentation assessment.

We describe surgical management of herniated coil using indocyanine green video angiography (ICGA) following failure of endovascular retrieval methods in a patient who had endovascular treatment for anterior communicating artery aneurysm. The interdisciplinary cooperation between the endovascular and neurosurgical teams proved to be crucial for obtainment of a desirable outcome in this case. ICGA technology applied in such difficult cases may improve the surgical outcome.

The Downside of Botulinum Injections for Anterocollis: A Case Series and a Review of the Literature.

OBJECTIVES: This is a case series and a review of the literature of therapeutic outcomes of botulinum toxin (BT) injections for anterocollis. METHODS: Data collected included gender, age, age at onset, muscles targeted, and doses injected. Routine forms were filled out during each visit: Patient Global Impression of Change, Clinician Global Impression of Severity, Tsui scale. The effect duration and side effects (SEs) of the previous treatment were noted. RESULTS: We described 4 patients (3 men, 13 visits) with anterocollis, as primary postural abnormality of the neck, emphasizing the therapeutic response to BT injection. Mean age at onset was 75.3 ± 7.0 years, age at first injection was 80.7 ± 3.5 years. The mean total dose per treatment was 290.0 ± 95.6 units. Patient Global Impression of Change with any grade of favorable effect was reported in 27.3% of the treatments. In objective assessment, Global Impression of Severity and Tsui scores did not show a consistent tendency of improvement. Neck weakness was prevalent in 18.2% of the visits of the anterocollis group while no other SEs were noted. We found 15 articles describing experience with BT for anterocollis in 67 patients (19 in deep and 48 in superficial neck muscles). CONCLUSIONS: This case series describes the poor outcome of BT treatment for anterocollis, with low efficacy and bothersome SE. Levator scapulae injection for anterocollis is not effective and is highly associated with head drop and should perhaps be abandoned. Injection to the longus colli might give some benefit in non-responders.

Therapeutic Approach to Botulinum Injections for Hemifacial Spasm, Synkinesis and Blepharospasm.

The aim of this study was to show our therapeutic outcome of botulinum injection to the facial muscles and thereby to find the best therapeutic concept which should be embraced. The decision to treat the lower eyelid with 1-point or 2-points injection was randomly taken as there is no consensus regarding this debate. Injections of the lateral end of the upper eyelid were performed more laterally to the conventional injection point, just lateral to the conjunction of the upper and lower eyelids. Twenty-three patients (12 hemifacial spasm, 6 blepharospasm, 5 post facial palsy synkinesis) were enrolled. Data were retrieved from 112 visits between 2019 and 2022. Overall, 84.9% of the treatments had moderate or marked improvement. The most common side effect was facial weakness (11.8%). Neither ptosis nor diplopia were noted. Two-points regimen in the lower eyelid was associated with a lower risk of facial weakness (p = 0.01), compared to 1-point regimen, with a better therapeutic outcome as reflected by more favorable PGI-C scores (p = 0.04). Injection of the pretarsal segment of the upper eyelid, just onto or even lateral to the conjunction of the upper and lower eyelids, lowers the risk of ptosis.

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.

Conservative management for pediatric patients with chiari 1 anomaly: A retrospective study.

OBJECTIVE: To better understand the natural history of non-surgical management of chiari 1 anomaly. PATIENTS AND METHODS: After obtaining approval of the institutional review board, medical records and radiological exams of patients treated for CM1 at our institution between the years 2010 and 2016 were reviewed. Twenty-nine patients total were included in our study. RESULTS: The average age of our patient population was 8.5 years old at the time of diagnosis. The average tonsillar herniation on first MRI was 9.4 mm (+/- 4.6) and the average tonsillar herniation on second MRI was 10.4 mm (+/- 4.8). The average follow up time of our sample of patients was 26 months. Of the 29 patients in our study 9 (31 %) had symptomatic presentation. Interestingly, four of our patients (13.8 %) presented with epilepsy. CONCLUSIONS: Our findings support the previous work that nonoperative management is best in asymptomatic or mildly symptomatic chiari patients.

The Utility of Early Postoperative Neuroimaging in Elective/Semielective Craniotomy Patients: A Single-Arm Prospective Trial.

BACKGROUND: The necessity and timing of early postoperative imaging (POI) are debated in many studies. Despite the consensus that early POI does not change patient management, these examinations are routinely performed. This is the first prospective study related to POI. Our aims were to assess the necessity of early POI in asymptomatic patients and to verify accuracy of the presented algorithm. METHODS: This was an algorithm-based prospective single-center study. The algorithm addressed preoperative, perioperative, and postoperative considerations, including estimated pathology type, device placement, and postoperative neurologic change. Early computed tomography scans were obtained in all patients, but if postoperative algorithm indications did not recommend a scan, the treating team was blinded to them, and patient management was conducted based on clinical examinations alone. A neuroradiologist and study-independent neurosurgeon reviewed all the scans. RESULTS: Of 103 enrolled patients, 88 remained asymptomatic, and 15 experienced symptoms postoperatively. Pathology was present on POI in 1% of the asymptomatic patients and 53% of the symptomatic patients (P < 0.001). In the asymptomatic group, no treatment modifications were made postoperatively. Blinding of the surgical team was not removed, and 20% of the symptomatic patients returned to the operating room because of imaging and neurologic findings. The goal of <5% algorithm failure was reached with statistical significance. CONCLUSIONS: In asymptomatic postoperative patients in whom early imaging is not performed for oncologic indications, device placement verification, or similar reasons, POI is unnecessary and does not change the management of these patients.

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.

Urgent endovascular stent-graft placement for traumatic penetrating subclavian artery injuries.

Penetrating injuries may infrequently cause pseudoaneurysms, lacerations and arteriovenous fistulas involving the subclavian artery. These injuries present with life-threatening bleedings, associated regional injuries and critical limb ischemia and although surgery has been considered the treatment of choice, subclavian injuries pose a real surgical challenge. We prospectively examined data of six patients presenting with penetrating subclavian artery injuries that were treated by urgent endovascular stent-graft placements. All stent-grafts were deployed successfully achieving complete exclusion of the pseudoaneurysm, control of bleeding and reconstruction of the injured artery. No procedural complications, stent thrombosis or stent infections occurred during hospitalization. One patient developed stenosis at 7 months, which required angioplasty. The series mean clinical and ultrasound-CTA follow-up is 38+/-19.7 months (range 11-60 months) and 28+/-19.1 months (range 6-58 months), respectively. This series shows the feasibility of endovascular repair by means of stent-grafts for selected patients with acute penetrating injuries of the subclavian arteries. This approach proved to be safe and effective in restoring the arterial lumen and patency, excluding the pseudoaneurysms and controlling the bleeding caused by subclavian lacerations. Mid-term follow-up on stent-graft patency rates are encouraging.

Endovascular treatment of congenital carotid-cavernous fistulas in infancy.

Congenital dural arteriovenous fistulas have been infrequently described in pediatric population and they generally affect the transverse, sigmoid, straight or sagittal sinuses. Congenital carotid-cavernous fistulas in infancy are considered very rare and to our knowledge, only four cases have been reported in literature. Currently, the preferred treatment is endovascular embolization through trans-arterial route. We review the literature on congenital carotid cavernous fistulas in infancy focusing on the endovascular treatment of this rare entity and present our experience.

Single-staged sequential endovascular stenting in patients with in tandem carotid stenoses.

BACKGROUND AND PURPOSE: Simultaneous stenoses at the origin of the internal carotid artery and the carotid siphon, also known as 'in tandem stenoses', are not uncommon. However, the clinical importance of this condition is still a subject of controversy. Surgical and endovascular approaches have been proposed to manage symptomatic patients who fail antithrombotic therapy. METHODS: We present a series of eight patients with symptomatic in tandem carotid artery stenoses treated by sequential endovascular stent-assisted angioplasty. In all the cases, the intracranial stenosis was equal to, or greater, than the extracranial stenosis. RESULTS: Procedural success, defined as residual stenosis of less than 30% in extracranial and intracranial lesions, was obtained in all the cases. No patient sustained myocardial infarction, stroke or transient ischemic attack (TIA) during the procedure or hospital stay. During a mean clinical follow-up of 12 +/- 3.4 months (range: 7-20 months), there were no neurological events and on angiographic follow-up after 6 months, no patient presented in-stent de novo stenosis. CONCLUSION: Endovascular stent-assisted angioplasty appears to be a valid alternative for selected patients with symptomatic in tandem carotid stenoses that are refractory to medical treatment. In most of the cases, the characteristics of intracranial stenoses determine the feasibility of the procedure.

Exsanguinating tracheoinnominate artery fistula repaired with endovascular stent-graft.

BACKGROUND: Tracheoinnominate artery fistula is a relatively rare but life-threatening complication of tracheostomy. Peristomal bleed or hemoptysis may be massive and usually fatal if treatment is not instituted immediately. CASE DESCRIPTION: We report the case of a 40-year-old woman who sustained surgical evacuation of a brainstem cavernoma and developed a massive hemoptysis 13 days after a percutaneous tracheostomy. Because of the patient's poor clinical condition, endovascular repair of the arterial injury was decided. The innominate artery was successfully repaired by means of urgent stent-graft placement. CONCLUSIONS: We believe that, if technically feasible, stent-graft placement can be a valuable therapeutic alternative for this dramatic condition. Physicians in charge of tracheostomized patients and neurointerventionalists should be familiar with this management strategy.

Dural arteriovenous fistula of the greater sphenoid wing region in neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) has been infrequently associated with different cerebrovascular conditions that may lead to either ischemic or hemorrhagic stroke. Intracranial dural arteriovenous fistulas have not been described in NF1 patients. In this paper we present a unique case of an 8-year-old child with florid NF1 that presented a greater sphenoid wing dural arteriovenous fistula draining directly through the superior ophthalmic vein and causing exophthalmos. The fistula was cured by direct trans-superior ophthalmic vein approach, by means of detachable coils. Only a few cases of dural fistulas in the lesser sphenoidal wing region have been described in the literature but a fistula of the greater sphenoid wing has not been previously described.

Spontaneous thrombosis of cerebral aneurysms presenting with ischemic stroke.

Complete spontaneous thrombosis of an unruptured cerebral aneurysm is a rare event that can be discovered incidentally on advanced neuroradiologic studies. Occasionally, this phenomenon may be symptomatic and can present as an ischemic stroke. The presumed mechanism is probably due to extension of the thrombi to the parent vessel, embolization of intra-aneurysmatic thrombi to distal arteries or arterial compression due to increased aneurysm mass effect. We present documented cases of this unusual entity and review the literature.

Paradoxical ventriculomegaly due to low-pressure hydrocephalus, a rare complication of the treatment of a trapped fourth ventricle: Case report.

In this paper, we describe the rare phenomena of descending transtentorial herniation and paradoxical ventriculomegaly due to low pressure hydrocephalus. This resulted as a complication of treatment in a 14year old male patient, who had undergone multiple ventriculo-peritoneal shunt placements for hydrocephalus after resection of pilocytic astrocytoma. We discuss the etiology of this rare complication and our strategy for treatment. We emphasize the need for strategic placement of programmable shunts to avoid over shunting and associated complications such as tentorial herniation.