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A spontaneous spinal epidural hematoma without any identified etiology is a very rare entity. We report here a 44 years old woman admitted within our department for 04 days of severe back pain complicated rapidly of bilateral lower-limbs weakness and urinary retention. Neurological examination found a complete paraplegia (0/5 stergh), with incomplete sensory deficit below the T-10 level, bowel and bladder dysfunction, decreased deep and superficial reflex. MRI with different sequences showed a large epidural mass lesion with slightly high signal intensity on T1-weighted images and heterogeneous low signal intensity on T2-weighted images from T11 to L1 suggesting an epidural hematoma. She underwent an urgent total laminectomy from T11 to L1 following by a complete removal of a large dark and compact epidural hematoma compressing the spinal cord. Operatively, there’s no sign suggesting an AVM. Postoperative course was uneventful and she was discharged ten days after surgery with complete recovery.

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.

Cerebellar liponeurocytoma (CL) described under many different names, is a rare WHO grade I or II well differentiated neurocytic tumor of the cerebellum with focal lipomatous differentiation. Mainly reported in adulthood, it is fought to be a posterior fossa benign tumor. In this paper, we talk about a 64-year-old woman, following up for Parkinson’s disease presented in our department for 7 months’ history of headache and gait disturbance. MRI showed a right cerebellopontine angle (CPA) heterogeneous unlimited tumor mimicking an epidermoid cyst. She underwent a lateral suboccipital craniectomy procedure that permitted obtaining the whole gross total resection of this tumor. The histopathological diagnosis was a cerebellar liponeurocytoma. She was discharged from hospital the 8th day after surgery and was free of symptoms since 5 years.

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, angiography

Resume Le syndrome de Sneddon est une affection rare definie par l’association d’accidents ischemiques cerebraux (AIC) et d’un livedo racemosa. Nous rapportons une serie retrospective de 15 patients, comportant 13 femmes et 2 hommes, âges en moyenne de 37,93 +/- 9,77 ans. Tous les malades ont presente un AIC unique ou multiple. Six patients avaient un syndrome dementiel. L’imagerie par resonance magnetique a note des infarctus corticaux multiples avec une atteinte de la substance blanche. L’angiographie cerebrale faite dans tous les cas a montre des signes d’arteriopathie distale dans 12 cas et une thrombose de la carotide interne droite dans un cas. Des anticorps anti-phospholipides etaient presents a un taux significatif chez un seul patient. Dix patients ont ete traites par des anti-agregants plaquettaires et cinq par des anti-coagulants. L’evolution etait favorable dans 8 cas, stationnaire dans trois cas. Quatre patients ont presente ulterieurement de nombreux AIC, une des l’arret des anticoagulants, une sous anti-agregants plaquettaires et deux n’avaient aucun traitement.

Resume Introduction L’intoxication oxycarbonee est a l’origine de sequelles cognitives severes peu etudiees dans la litterature. Methodes Nous en rapportons 5 cas avec etude neuropsychologique et neuroradiologique par imagerie par resonance magnetique (IRM) cerebrale. Resultats Tous les malades avaient une atteinte severe de la memoire a long terme, une alteration moderee des fonctions executives, intellectuelles, visuospatiales et constructives. Une patiente a installe une alexie-agraphie, des troubles visuoperceptifs severes et une apraxie de l’habillage. Quatre patients avaient un syndrome depressif et un autre presentait un syndrome de perte de l’auto-activation psychique. L’IRM cerebrale a trouve dans tous les cas, une necrose pallidale bilaterale, une atrophie hippocampique bilaterale et une atrophie corticale moderee. Une atteinte de la substance blanche a ete notee chez un malade, une atrophie du fornix chez deux malades et des corps mamillaires chez trois autres. Des lesions bilaterales cerebelleuses etaient presentes dans deux cas et corticales dans trois cas. Un traitement par bromocriptine a entraine une amelioration des troubles neuropsychologiques chez trois patients.

BACKGROUND Hydrocephalus and cerebellar abscesses as the principal manifestations of posterior fossa dermoid cyst are rare. In addition, extradural dermoid cyst of the posterior fossa has been described in only 9 cases in the literature. We present an unusual case of obstructive hydrocephalus due to cerebellar abscesses induced by an adjacent extradural dermoid cyst with complete occipital dermal sinus. CASE DESCRIPTION A 14-month-old child presented with acute raised intracranially pressure, seizures, and meningitis. Neuroradiological studies revealed cerebellar cysts with ring enhancement associated with a contiguous occipital cyst, with compression of the adjacent cisterns and the fourth ventricle causing hydrocephalus. The diagnosis of cerebellar abscesses with congenital occipital defect was briefly entertained. The patient was treated by radical excision of the occipital cyst with hair contents, the dermal sinus, and the abscesses through a suboccipital approach, followed by systemic antibiotic therapy with a good outcome. Pathologic examination revealed a dermoid cyst. CONCLUSION Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT scan and MRI are the methods of choice for further investigation of suspect congenital dermal lesions. Neurosurgical treatment of these malformations should be planned early to prevent the high incidence of infections such as bacterial meningitis and cerebellar abscess. Clinical presentation, diagnostic evaluation, and treatment of these rare lesions are reviewed.

La Presse Medicale - In Press.Proof corrected by the author Available online since mercredi 4 mars 2015

Cerebrovenous thrombosis is quite rare in infammatory bowel disease. There are only a few reports of this association in the literature. We report 2 cases of patients with Crohn's disease (CD) who developed cerebral thrombophlebitis confirmed by neuroimaging. The first case was a 35-year-old man with a history of CD who presented with acute confusion. Brain magnetic resonance imaging demonstrated a left temporoparietal infarction and thrombosis of the left lateral sinus. Coagulation studies showed a marked protein S deficiency. His condition improved significantly after initiation of anticoagulant therapy. The second case was a 38-year-old woman who was admitted for a sudden loss of consciousness with tetraplegia. Brain computed tomography revealed a profound cerebrovenous thrombosis. She died within a few days after admission. Inflammatory bowel disease carries an increased risk of venous and arterial thrombosis. Although the pathogenic mechanisms of this predisposition are unclear, a possible role of inherited risk factors for thrombosis in determining this predisposition has been suggested. In these cases, both fibrinolysis and coagulation are activated as well.

Auteur(s) : Najwa Ech-Cherif El Kettani1, Mohamed Chihab2, Anas Benbouzid2, My Rachid El Hassani1, Noureddine Chakir1, Mohamed Jiddane1 1Hopital des Specialites, CHU Ibn-Sina, Service de neuroradiologie, Rabat, Maroc 2Hopital des Specialites, CHU Ibn-Sina, Service d’ORL, Rabat, Maroc Observation Mme A.T., 66 ans, sans antecedents notables, se plaint d’une dysphagie chronique lentement evolutive depuis deux ans. Une tomodensitometrie (TDM) cervicale a ete [...]

In four human left cerebral hemispheres the cortical speech areas were identified, having been marked by differential color code before transverse sectioning. The results from the anatomic dissection specimens were compared with thin CT sections in normal individuals, the CT plane being aligned to the orbitomeatal plane as an external reference, and thus closely parallel to the bicommissural plane, as an internal reference. The same cortical structures were identified in the CT sections. A strategic approach to CT examination of the cortical speech areas is formulated, based on the external-internal system of reference.