Preliminary experience in Awake Surgery: Functional recovery profile

Background. Until the advent of new exploration techniques: functional magnetic resonance imaging (fMRI) and surgical protocols such as exeresis in awake mode, the functional recovery potential of postoperative deficits was limited by conventional tumor surgery. The use of these methods simultaneously improves the quality of life and survival medians, mainly for removing low-grade gliomas massively infiltrating subcortical networks in eloquent regions where surgery is historically not associated with high functional recovery rates. Accordingly, the results from the awake brain surgery literature motivate us to establish a new baseline on the relationship between electrical stimulation mapping (ESM) threshold, the extent of resection (EOR), neuroplastic typology, and functional recovery after intraoperative crises or postoperative care deficits using induced neuroplasticity. Materials and patients. This is a retrospective analytical study of 35 brain tumor cases of gliomas, operated by common craniotomy in awake conditions from September 2016 to July 2022. Before entering awake resection mode, all patients underwent brain mapping (ESM) by direct electrical stimulation (DES) according to standard conditions and Helsinki ethical guidelines. Analysis according to ESM was done for two groups (group 1 and group 2) of different intensities of DES. Outcomes. The ESM by threshold intensity expressed in mean ± deviation standard was: 2,45 ± 0.125 mA for sensorimotor functions against 1.35 ± 0.175 mA for cognitive mapping. These stimulation currents were optimum thresholds which allowed us during control mapping to overcome all boundary conditions, mostly false negative results. The functional recovery time (FRT) following stimulation-induced seizures was varied from 2 s to 6.26 s, marking the intraoperative neuroplasticity operated mainly by synaptic remodeling during the functional reactivation. The EOR was better for group 1 with 82.35 % gross total resection (GTR) with only 8.75% of the occurrence of transient seizures against 45.7% for group 2 and only 2.86% suffered from neurological permanent deficits in group 1 against 11.42% in group 2. Conclusions. ESM in the range of [2.35 - 2.45 mA] improved DES sensitivity without false negatives. We had a compromise of improved results between these stimulation thresholds, the duration of the craniotomy, the functional recovery time, the EOR and overall the occurrence of neurological deficits, which explain the processes involved in the success of awake surgery

An autosomal recessive leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa maps to chromosome 17q24.2-25.3

Background Single-gene disorders related to ischemic stroke seem to be an important cause of stroke in young patients without known risk factors. To identify new genes responsible of such diseases, we studied a consanguineous Moroccan family with three affected individuals displaying hereditary leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa that appears to segregate in autosomal recessive pattern. Methods All family members underwent neurological and radiological examinations. A genome wide search was conducted in this family using the ABI PRISM linkage mapping set version 2.5 from Applied Biosystems. Six candidate genes within the region linked to the disease were screened for mutations by direct sequencing. Results Evidence of linkage was obtained on chromosome 17q24.2-25.3. Analysis of recombination events and LOD score calculation suggests linkage of the responsible gene in a genetic interval of 11 Mb located between D17S789 and D17S1806 with a maximal multipoint LOD score of 2.90. Sequencing of seven candidate genes in this locus, ATP5H, FDXR, SLC25A19, MCT8, CYGB, KCNJ16 and GRIN2C, identified three missense mutations in the FDXR gene which were also found in a homozygous state in three healthy controls, suggesting that these variants are not disease-causing mutations in the family. Conclusion A novel locus for leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa has been mapped to chromosome 17q24.2-25.3 in a consanguineous Moroccan family

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

Incidental optic nerve sheath arachnoid cyst: A rare case finding with literature review

Arachnoid cysts are the most common benign cystic abnormalities formed due to congenital splitting of the arachnoid layer. They comprise 1% of intracranial masses, and the orbital location is even more rarely reported in history especially in the pediatric population. They might be discovered as an asymptomatic finding on imaging performed for a concomitant condition or, in most reported cases, as a result of ophthalmic impairment. They can be isolated or associated with gliomas, neurofibromas, empty sella syndrome, and frontotemporal porencephalic cysts. Computed tomography scan shows a non-enhancing liquid cystic lesion, and magnetic resonance imaging remains the best assessment tool confirming the similarity of the fluid to cerebrospinal fluid and evaluating the optic nerves. Herein, we report the case of an incidental discovery of an intraorbital arachnoid cyst on magnetic resonance imaging in a 53-year-old woman with a history of epilepsy. No treatment was performed as the cystic formation was asymptomatic

Moya moya: étiologie rare d’accident vasculaire cérébral ischémique chez l’enfant: à propos d’un cas

La maladie de Moya Moya est une maladie angiogénique, caractérisée par un rétrécissement de l'artère carotide interne distale qui s'étend aux segments proximaux des artères cérébrales moyennes et antérieures, induisant la formation de vaisseaux de suppléance. Ces derniers proviennent des collatérales parenchymateuses, perforantes, leptoméningées et autres anastomoses  transdurales. Ces vaisseaux collatéraux ont un aspect caractéristique à l'angiographie formant un nuage de fumée : réseau Moya Moya. Son étiologie reste encore mal élucidée et représente 10 à 15% des causes d'accidents vasculaires cérébraux (AVC), avec 2 pics d'âge où l'atteinte est plus fréquente: les enfants autour de 5 ans et les adultes autour de 40 ans. Son évolution peut être lente avec des symptômes intermittents ou être fulminante avec un déclin neurologique rapide. Les données actuelles montrent l'importance du traitement chirurgical comme méthode de référence pour la prise en charge du syndrome de Moya en particulier chez les patients avec des symptômes progressifs et récidivants.Mots clés: Moya, vascularite, AVC, angioscanner, angio-IRM, angiographieEnglish Title: Moya moya disease: a rare cause of ischemic stroke in children: about a caseEnglish AbstractMoya moya disease is an angiogenic disease characterized by the narrowing of the distal internal carotid artery extending to the proximal segments of the middle and anterior cerebral arteries, inducing collateral vessels formation. These vessels come from the collateral parenchymal vessels, the perforating vessels, leptomeningeal vessels and other transdural anastomoses. These collateral vessels have a characteristic appearance on angiography, forming a cloud of smoke: net-like moyamoya. Its etiology is still poorly understood. Moyamoya disease accounts for 10-15% of the causes of stroke, with 2 age peaks at which its occurrence is more frequent: children around 5 years old and adults around 40 years old. Its evolution can be slow with intermittent symptoms or fulminant with fast neurological deterioration. The current data show the role of surgery as the gold standard for the treatment of moyamoya syndrome, in particular in patients with progressive and recurrent symptoms.Keywords: Moya, vasculitis, stroke, angioscanner, angio-MRI, angiograph

A spinal cord compression syndrome revealing neurofibromatosis type 1: A case report

Neurofibromatosis type 1 (NF1), formerly known as von Recklinghausen disease is an autosomal dominant disease with multisystem involvement. In the peripheral nervous system, it leads to the development of benign tumors from the tissue of the spinal or cranial nerve sheaths, known as “neurofibromas.” We report the case of a 40-year-old patient with spinal cord compression syndrome in whom spinal MRI revealed cervical, dorsal and lumbosacral neurofibromas revealing neurofibromatosis type 1