A challenging case of endoscopic third ventriculostomy.

Background: Although controversial, endoscopic third ventriculostomy (ETV) in the management of Myelomeningocele and Chiari type II malformation-related hydrocephalous is gaining wider popularity and use. With variable success rates, it can be proposed as a first or second option after shunt malfunction. ETV in post-infectious hydrocephalus may also be considered as an alternative to shunting. With reported success rates of 50-60%, failure is attributed to anatomical reasons and/or to pathological subarachnoid space scarring that may result from infectious processes. Similarly, ETV in repeated shunt malfunctions is an acceptable option that may offer shunt independency. In all situations, case-by-case selection and discussion are to be considered. Case Description: A 5-year-old boy with a history of surgically treated lumbosacral myelomeningocele and ventriculoperitoneal shunting at six months of age is presented. During the course following the initial surgery, he experienced multiple shunt malfunctions, with two episodes of meningitis, leading to 7 shunt revision surgeries. Lately, the patient presented a large peritoneal cyst formation that needed regular evacuations. With a magnetic resonance imaging (MRI)-scan showing a large bi-ventricular hydrocephalus and a trapped third ventricle with multiple septations, surgical options included either ventriculoatrial shunting or third ventriculostomy. The latter option, offering shunt independency, was chosen after family consent and risk explanation. The expected success rate of the procedure was discussed and evaluated to 40-60% on the ETV success score. The video describes a step-by-step procedure with detailed radiological and correlated anatomical annotations of a completely distorted anatomy of a multifactorial hydrocephalous. No scarring at the prepontine cistern was observed. Shunt independency was achieved. However, the patient died from late postoperative status epilepticus and pulmonary complications. Whether these postoperative events are directly related to the procedure is unclear, although technically and clinically successful in the short term. Conclusion: We believe that ETV should be carefully indicated in selected patients with Chiari II, post-infectious hydrocephalus, by experienced hands, as the surgical anatomy can be extremely complex and misleading.

Surgical Management and Outcome of the Planum Sphenoidale Meningioma: A Single-Center Strobe Compliant Study.

BACKGROUND: The resection of the jugum (planum) sphenoidale meningioma (JSM) is challenging, and there is a scarcity of available data in the literature. This study aimed to describe the surgical management and long-term outcome of jugum sphenoidale meningioma. METHODS: This was a retrospective, single-center, cohort study of 91 JSM patients treated in our department from 2005 to 2022. All the included patients have undergone surgical treatment. Clinical, radiologic, and surgical data were extracted with specific attention to the age at onset, family history, duration from symptoms to diagnosis, clinical presentation, quality of excision, recurrence, and treatment outcome. RESULTS: WHO grade I and grade II meningioma were 83.5% (n = 76) and 16.5% (n = 15) respectively. Fronto-lateral 42.9% (n = 39) and pterional approaches 57.1% (n = 52) were the 2 major surgical approaches used for the management of planum sphenoidale meningioma. Twelve patients needed emergency surgical treatment at admission: 4 (4.4%) External CSF Shunt and 8 (8.8%) VP-Shunt. We have found a statistically significant (P = 0.032) association between the quality of excision and the risk of recurrence. The GTR (Hazard ratio = 1) of the planum meningioma protects the patient from recurrence within 60 months of follow-up. Postoperative complications encountered were CSF-Leak in 2.2%, Meningitis 4.4%, surgical site infection 2.2%, and surgical site hematoma 3.3%. The overall postoperative favorable outcome was 68.1% (n = 62). CONCLUSIONS: The pterional approach has allowed the achievement of GTR for jugum sphenoidale meningioma with protective odds from recurrence risk.

Advocacy for Epilepsy Surgery in Africa: Moroccan Experience of 132 Cases.

BACKGROUND: In Africa, epilepsy is a real burden. Temporal lobe epilepsy is the most common drug-resistant focal epilepsy disorder, and temporal lobectomy is the most common effective treatment for patients with drug-resistant epilepsy. OBJECTIVE: We aim to highlight the Moroccan experience in epilepsy surgery and to ascertain its long-term outcome. Through the results of surgical treatment in our series, we hope to raise awareness of the need for epilepsy surgery in Africa and contribute to its development. METHODS: We present a retrospective study of 132 patients who underwent surgery for epilepsy from January 2005 to December 2021 at our institution. The presurgical evaluation was based on clinical screening, interictal electroencephalography, video-electroencephalography, neuropsychological tests, magnetic resonance imaging, and positron emission tomography in some cases. Data are presented as the median and ranges. For all analyses, P values <0.05 were considered statistically significant. RESULTS: Our series includes 132 patients (69 males; 52.27%); the median age at surgery was 24 years (range, 1-64). One hundred and fifteen patients (87%) were operated on for temporal lobe epilepsy, of whom 98 (85%) had anterior temporal lobectomy and 17 (15%) had lesionectomy. Seventeen patients (13%) were operated on for extratemporal epilepsy, of whom 4 had lesionectomy, 7 functional hemispherotomy, and 5 Gamma Knife stereotactic radiosurgery. Our postoperative outcomes 3 months after surgery found 113 patients (85.6%) seizure-free (Engel class I), 16 with Engel class II (12.1%), and 3 with Engel class III (2.3%) in temporal lobe epilepsy. In extratemporal lobe epilepsy, 12 patients (70.5%) showed Engel class I, seizure-free, 4 Engel class II (23.5%), and 1 Engel class III (6%). CONCLUSIONS: These results confirm that most patients with drug-resistant epilepsy may benefit from surgical treatment without submitting to preoperative invasive explorations. This finding should help develop epilepsy surgery widely in Africa.

Outcome of Brain Metastases Treated with Gamma Knife Stereotactic Radiosurgery: A Retrospective Cohort Study of 205 Cases.

BACKGROUND: Brain metastases (BMs) are the most common expansive intracranial lesions in adults. Approximately 50% of patients diagnosed with new BMs will have >1 BM at the diagnosis. We report our experience with BMs treated with Leksell Gamma Knife stereotactic radiosurgery (GKSR) and evaluate the outcomes. METHODS: Patients treated by GKSR in our institution between 2008 and 2021 for BMs were evaluated retrospectively. RESULTS: A total of 205 patients (56.6% women) were included, with a median age of 59 years (range, 25-83 years). The breast (n = 85; 42.5%) and lung (n = 76; 38%) were the most common original locations for the primary tumors. Of the 205 patients, 103 (50.3%) had a single BM and 102 (49.7%) had ≥2 BMs. The median number of multiple BMs treated was 4 (range, 2-43). The mean overall survival (OS) time was 6.00 months (95% confidence interval [CI], 5.07-6.93 months) for all BMs. The median rate of tumor control after radiosurgery was 65% (range, 20%-99%) during a median follow-up of 6.00 months (95% CI, 3-84 months). In the overall population, the 1-, 2-, and 5-year OS rate was 37.55%, 25.12%, and 18.51%, respectively. The mean OS time was longer for those with multiple BMs than for those with a single BM (mean, 10 months [95% CI, 6.67-13.33 months] vs. mean, 5 months [95% CI, 4.21-5.70 months]; P = 0.03). Retreatment, tumor stability (control), and progression influenced the OS of patients with BMs, whether single or multiple (P < 0.001). CONCLUSIONS: GKSR provides good results in terms of OS and better quality of life for patients with BMs, whether single or multiple. Tumor stability and retreatment influenced the OS of patients with BMs.

Outcomes of single brain metastasis treated with gamma knife stereotaxic radiosurgery(GKSR). Our experience on 103 cases.

Background: Brain metastases (BM) occur in the natural course of malignant tumors in 18-40% of cases. Their management has changed considerably over the past decade thanks to the advent of Gamma knife Stereotactic Radiosurgery (GKSR). Objective: We report our experience on Single Brain metastasis treated with (GKSR). Methods: Patients treated by Gamma Knife stereotaxic radiosurgery (GKSR) in our institution between 2009 and 2021 for Single BM were recorded retrospectively. Results: A total of 103 patients (n = 52; 50.5% females) were included, with a mean age of 56.33 ± 11.33. Breast (n = 39, 37.9%) and lung (n = 36, 35%) were the common original location for the primary tumors. GKSR alone without prior surgery, radiotherapy, or chemotherapy was achieved in 81.5% (n = 84). Thirteen patients (15.1%) progressed in BM volume while finding the appearance of de novo BM in 5 (5.8%) patients. The median percentage of tumor control after radiosurgery treatment was 70% (IQR: 65-78) and only 26.2% (n = 27) of patients had > 80% tumor control and stability over the median follow-up time of 5 (95% CI, 4-6) months. We found only two cases of radionecrosis (1.9%). The median survival time was 5.21 (IQR, 3-8) months. Retreatment, recursive partitioning analysis (RPA) class, and tumor stability influenced the overall survival of BM respectively (Hazard Ratio adjust (HRa)= 5.610,p = 0.045; HRa= 6.133,p = 0.031; HRa= 22.463, p = 0.036). Conclusion: Stereotaxic Radiosurgery provides good results in terms of Overall survival with fewer neurocognitive disorders.RPA class and tumor control (stability) influenced the overall survival of single BM.

Papillary tumor of the pineal region: Is stereotactic radiosurgery efficient for this rare entity?

BACKGROUND: Papillary tumors of the pineal region are rare neuroepithelial lesions that were described for the 1st time in the WHO 2007 classification. Management of such lesions remains controversial. CASE DESCRIPTION: We describe the case of a 26-year-old female who presented with intracranial hypertension syndrome secondary to a 1.9 cm3 lesion of the pineal region causing hydrocephalus. The patient benefited from an endoscopic third ventriculocisternostomy and a biopsy of her lesion in favor of a papillary tumor of the pineal region. After discussion of the surgical risks, the patient refused the surgical option and a stereotactic radiosurgery (SRS) was performed. She improved both clinically (allowing her to regain autonomy) and radiologically (reduction of 60% of tumor volume) at 1 year follow-up. CONCLUSION: Because of the rarity of the lesion, literature is yet not able to find consensus concerning management of such lesion, but SRS has proven efficiency for these Grades II or III lesions with high recurrence rates. Therefore, it should be considered as a primary therapeutic option allowing good outcome with low risks for the patient.

Cerebrospinal fluid fistula in a patient with chronic constipation related to an autonomic dysfunction and revealed by bacterial meningitis - A case report.

BACKGROUND: Cerebrospinal fluid (CSF) fistula represents a rare neurosurgical entity that can be defined as a communication between the subarachnoid space and nasal fossa or less commonly the ear cavity. It can be spontaneous without an evident etiology or secondary following a skull base surgery or trauma. The early diagnosis of spontaneous forms remains a challenge as clinical signs (e.g., unilateral rhinorrhea) can be absent or neglected by patients and can result in meningitis. CASE DESCRIPTION: Here, we report the case of a 31-year-old man with chronic constipation complicated by chronic intracranial hypertension, and resulting in rhinorrhea with bacterial meningitis. The etiological assessment of chronic constipation retained an autonomic dysfunction with sympathetic hyperactivity (e.g., pure autonomic failure) as an underlying cause. Beta-2 transferrin testing associated with cerebral magnetic resonance imaging and computed tomography scan confirmed the diagnosis and localization of the fistula at the cribriform plate. The patient underwent an endoscopic endonasal approach with a repair of fistula. He presented with recurrent rhinorrhea 17 months later which required a surgical revision along with CSF diversion with a ventriculoperitoneal shunt. CONCLUSION: Although rare, autonomic dysfunction can result in chronic constipation in young patients, with intermittent or permanent intracranial hypertension, leading to CSF leaks. The early identification and treatment of the underlying etiology may prevent severe complications and improve the management and outcome of CSF fistula patients.

COVID-19 and SARS-Cov-2 Infection: Pathophysiology and Clinical Effects on the Nervous System.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is an infectious disease caused by SARS-Cov-2, resulting in severe acute respiratory syndrome, with high potential of spreading and infecting humans worldwide. Since December 2019, when the virus was identified in humans, the literature on COVID-19 has grown exponentially and extrarespiratory symptoms including neurologic symptoms are increasingly highlighted. METHODS: Given the high and increasing number of publications reporting neurologic involvements of SARS-Cov-2, we thought that providing an update for neurologic complications of COVID-19 would be useful for physicians and especially young trainees in neurology and neurosurgery. Indeed, in this review we discuss several neurologic aspects reported in the literature to date including the evidence and pathways of neuroinvasion in COVID-19 and the main neurologic disorders reported in the literature to date, as well as future perspectives and the potential long-term consequence of current neuroinfection in COVID-19 patients. RESULTS: Currently, there is convincing evidence that SARS-CoV-2, the etiologic agent of COVID-19, can affect the nervous system, with damage and neurologic alterations. These neurologic disorders are grouped into several categories, ranging from nonspecific and moderate symptoms such as headache, myalgia, and hyposmia to severe symptoms including cerebrovascular disease and intracranial infections. Severe neurologic symptoms such as acute cerebrovascular disease occur only in a minority of patients with usual risk factors and are associated with poor outcome. However, most COVID-19 patients exhibit only minor or mild neurologic symptoms. CONCLUSIONS: Management of COVID-19 patients should include early clinical, radiologic, and laboratory neurologic assessment, with a close follow-up, especially in severe forms. Future studies should assess late and long-term consequences of current COVID-19 patients with neurologic involvement.

Diagnosis, Management, and Outcome in 9 Children with Unilateral Posterior Synostotic Plagiocephaly.

BACKGROUND: Posterior synostotic plagiocephaly represents a rare challenging type of craniosynostosis, often misdiagnosed as a simple posterior positional plagiocephaly. Underdiagnosed forms may result in delayed diagnosis and neurosurgical management, with potential ophthalmologic, cognitive, and aesthetic sequelae in children. METHODS: Here we retrospectively analyzed data of 9 posterior synostotic plagiocephaly infants treated in our center over a 10-year period (January 2000-December 2009). RESULTS: Patients averaged 10 months of age, and there was a clear male predominance (8 males/1 female). Cerebral computed tomography scan was performed in all patients, and the abnormal suture was located at the right side in 7 cases (77.77%). Ipsilateral occipitomastoid bulge and occipital flattening were found in all cases (100%). Posterior ear displacement was found in 7 cases (77.77%), and there was minimal facial asymmetry in 4 cases (44.44%). The ophthalmoscopic examination found a papillary edema grade 1 in 2 cases and grade 2 in 4 cases. There were no deaths or reoperation in our series. All of our patients underwent a neurosurgical correction, with total ophthalmic recovery in all patients with preoperative papillae edema. Eight patients had an early satisfactory aesthetic aspect. Long-term follow-up in treated children found a normal childhood, with no major school dysfunctions and normal social integration. CONCLUSIONS: Interesting findings in our series were male gender predominance and predilection of the right-side synostotic lambdoid suture. We think that early correct diagnosis and appropriate neurosurgical treatment may prevent potential complications such as neurocognitive and aesthetic sequelae in children with posterior synostotic plagiocephaly.

Novel pathogenic VPS13A mutation in Moroccan family with Choreoacanthocytosis: a case report.

BACKGROUND: Choreoacanthocytosis (ChAc), is a rare neurodegenerative disease, characterized by movement disorders and acanthocytosis in the peripheral blood smears, and various neurological, neuropsychiatric and neuromuscular signs. It is caused by mutations in VPS13A gene with autosomal recessive pattern of inheritance. CASE PRESENTATION: Here we report two patients belonging to a consanguineous Moroccan family who present with movement disorder pathology. They were suspected to have choreoacanthocytosis according to biological, clinical and radiological finding. Thus, whole-exome sequencing was performed for precise diagnosis and identified a homozygous novel nonsense mutation c.337C > T (p.Gln113*) in exon 5 of VPS13A in the two affected siblings. CONCLUSION: Here, we report a novel nonsense p.Gln113* mutation in VPS13A identified by whole-exome sequencing, which caused ChAc in a Moroccan family. This is the first description of ChAc in Morocco with genetic confirmation, that expands the mutation diversity of VPS13A and provide clinical, neuroimaging and deep brain stimulation findings.

ATRT of lateral ventricle in a child: A Rare Tumor at a Very Rare Location.

Atypical teratoid/rhabdoid tumors (AT/RTs) of infancy are highly malignant central nervous system neoplasms that are most commonly seen during the first 2 years of life with limited therapeutic options. To date, only two cases have been described in the lateral ventricle. A 4-year-old boy presented with a 4-month history of increased intracranial pressure. Cerebral magnetic resonance imaging (MRI) revealed a huge intraventricular tumor, occupying the entire temporal horn and the body of the left lateral ventricle. The boy was operated through a left temporal transventricular approach with gross total removal of the lesion. The histopathological diagnosis was an AT/RT. The infant underwent adjuvant chemotherapy and radiation therapy. The 1-year MRI of control showed a local recurrence of the tumor. Then after, Gamma Knife radiosurgery was performed because of the small volume and the deep location of the lesion. At the 3-month follow-up, the MRI showed a significant growth of the tumor volume, and the child was given additional adjuvant chemotherapy. Unfortunately, he died 9 months later. AT/RT of the lateral ventricle is a very rare tumor in children, associated with a poor prognosis in spite of multimodal treatment. Gamma knife surgery (GKS) was rarely reported as a treatment modality of AT/RT. The aim of this work is to discuss about the rarity of this tumor and the best treatment strategy to improve prognosis.

Traumatic cervical spine injury: Short-and medium-term prognostic factors in 102 patients.

BACKGROUND: Traumatic cervical spine injuries (CSIs) can be defined as osteodiscoligamentous lesions and are frequent in the young and active population. These lesions are often associated with significant devastating neurological deficits. Here, we sought to establish short-and medium-term prognostic factors that could help predict future outcomes. METHODS: We retrospectively reviewed 102 adults admitted for traumatic CSI over an 11-year period (January 2004-December 2014). Patients were graded using Frankel scale as exhibiting good or poor outcomes. RESULTS: Two risk factors that significantly predicted results for CSI included original poor Frankel grades (e.g., A and B) and initial neurovegetative disorders (e.g., respectively, P = 0.019 and P = 0.001). However, we did not anticipate that two other risk factors, operative delay and mechanism of trauma, would not significantly adversely impact outcomes. CONCLUSION: Here, we identified two significant risk factors for predicting poor outcomes following CSI; poor initial Frankel Grades A and B and neurovegetative disorders at the time of original presentation.

Integration of Three-dimensional Magnetic Resonance Imaging Spectroscopy with the Leksell GammaPlan Radiosurgical Planning Station for the Treatment of Brain Tumors.

Introduction MRI multivoxel spectroscopy mapping is helpful in surgical decision-making. Unfortunately, in daily practice, MRI multivoxel spectroscopy mapping is not always compatible with the current version of Leksell GammaPlan (LGP) (Elekta, Stockholm, Sweden). The aim of this study is to develop a tool to allow the use of this modality in radiosurgical treatments using LGP. Material and methods Multivoxel spectroscopy digital imaging and communications in medicine (DICOM) images were analyzed to identify tags to be modified to make the images compatible with LGP. We identify four important tags to be modified for compatibility with LGP. Using Python language, a new software was designed to modify the identified tags and allow the automatic conversion of images to meet LGP requirements. Results By modifying the tags of DICOM images, we could use spectroscopic cartography images in radiosurgical planning using LGP. We created a software to reproduce these modifications using a simple and rapid interface. This software executes all the protocols established in the methodology. Conclusion The new software, "GP Adapting Solution", can convert any DICOM image and make it compatible with LGP. The integration of multivoxel spectroscopic images was feasible and could be used for radiosurgical planning. This work is the first step in allowing the potential use of new MRI modalities in radiosurgical planning using LGP. The next steps are to evaluate the impact of these modalities in radiosurgical treatments and to develop methods for integrating other imaging modalities.

Deep Brain Stimulation in Moroccan Patients With Parkinson's Disease: The Experience of Neurology Department of Rabat.

Introduction: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is known as a therapy of choice of advanced Parkinson's disease. The present study aimed to assess the beneficial and side effects of STN DBS in Moroccan Parkinsonian patients. Material and Methods: Thirty five patients underwent bilateral STN DBS from 2008 to 2016 in the Rabat University Hospital. Patients were assessed preoperatively and followed up for 6 to 12 months using the Unified Parkinson's Disease Rating Scale in four conditions (stimulation OFF and ON and medication OFF and ON), the levodopa-equivalent daily dose (LEDD), dyskinesia and fluctuation scores and PDQ39 scale for quality of life (QOL). Postoperative side effects were also recorded. Results: The mean age at disease onset was 42.31 ± 7.29 years [28-58] and the mean age at surgery was 54.66 ± 8.51 years [34-70]. The median disease duration was 11.95 ± 4.28 years [5-22]. Sixty-three percentage of patients were male. 11.4% of patients were tremor dominant while 45.71 showed akinetic-rigid form and 42.90 were classified as mixed phenotype. The LEDD before surgery was 1200 mg/day [800-1500]. All patients had motor fluctuations whereas non-motor fluctuations were present in 61.80% of cases. STN DBS decreased the LEDD by 51.72%, as the mean LEDD post-surgery was 450 [188-800]. The UPDRS-III was improved by 52.27%, dyskinesia score by 66.70% and motor fluctuations by 50%, whereas QOL improved by 27.12%. Post-operative side effects were hypophonia (2 cases), infection (3 cases), and pneumocephalus (2 cases). Conclusion: Our results showed that STN DBS is an effective treatment in Moroccan Parkinsonian patients leading to a major improvement of the most disabling symptoms (dyskinesia, motor fluctuation) and a better QOL.

The Transcranial Stab Wound and the Life-Saving Zygomatic Arch Clinical Report.

A 58-year-old man presented to the neurosurgical emergencies for a transzygomatic transcranial stab wound with a retained broken knife. The patient was neurologically intact. After radiographic evaluation the knife was found to be penetrating the temporal lobe, neighboring the intracavernous portion of the carotid artery. The patient was successfully managed in a conservative way. No abnormalities were seen at 12 months of follow-up. Dealing with penetrating head injuries is a usual condition in neurosurgical practice. Some situations are though really challenging, especially when the offending object is still in place, with a close connection to vital structures. This clinical reports an unusual penetrating head injury, highlighting the importance of careful radiographic evaluation and trying to discuss clear management options.

Role of gamma knife radiosurgery in the management of pituitary adenomas and craniopharyngiomas.

INTRODUCTION: Radical microsurgical removal of pituitary adenomas (PAs) and craniopharyngiomas (CPHs) is often difficult. In such cases radiosurgery can be used as a second-line treatment option. MATERIALS AND METHODS: Our series included 436 PAs and 164 CPHs. The majority of patients had large or giant tumors and were treated with microsurgery. Additionally, between June 2008 and August 2011, a total of 29 PAs and 10 CPHs underwent radiosurgery using Leksell Gamma Knife PerfeXion. At the time of treatment the volume of the PAs varied from 0.6 to 26.0 cm3 (mean 5.9 cm3) and that of the CPHs from 0.19 to 17.0 cm3 (mean 6.6 cm3). The marginal doses ranged from 12 to 15 Gy (mean 14.5 Gy) for nonsecreting PAs, from 22 to 25 Gy (mean 24 Gy) for hormone-secreting PAs, and from 8 to 14 Gy (mean 11 Gy) for CPHs. RESULTS: The postoperative mortality rates after surgical removal of PAs via the transspenoidal approach and craniotomy were 2.4 % and 8.0 %, respectively, whereas after surgery for CPH it was 5.9 %. No major complications were noted in our limited number of patients after radiosurgical treatment. Taking into consideration only cases with radiological follow-up of at least 12 months, shrinkage of the tumor was demonstrated in 5 of 11 patients with a PA and in 4 out of 6 patients with a CPH. CONCLUSION: Radiosurgery is safe and effective second-line management option in cases of recurrent or residual PA or CPH. Occasionally, it can be applied even as a primary treatment in selected patients.