A rare neuronal tumor of the cerebellum with myloid features

We report an extremely rare tumor presenting with myoid features in the left cerebellar hemisphere in a 62- year-old man. This tumor consisted of medium to large round cells with focal lipomatous and myoid differentiation. Immunohistochemically, the tumor cells expressed synaptophysin, GFAP (glial fibrillary acidic protein) and focally desmin. From these findings, we concluded that this tumor was a liponeurocytoma with myoid features. To the best of our knowledge, this report constitutes the second described case of liponeurocytoma with myoid differentiation in the cerebellum

Paragangliome malin orbitaire, à propos d’un cas

Les paragangliomes sont des tumeurs neuroendocrines développées aux dépens du système nerveux parasympathique. Ils peuvent se localiser n’ importe où dans l’organisme depuis la tète et cou jusqu’au pelvis. La localisation orbitaire de cette tumeur est très rare. Nous présentons le cas d’un patient âgé de 37 ans qui présente depuis 4 mois une exophtalmie unilatérale droite, d’installation progressive, sans douleur ni baisse de l’acuité visuelle associés. L’examen général montre une tuméfaction sous le cuire chevelu, sans adénopathies locorégionales ni hépato ou splénomégalie. La tomodensitométrie retrouve un processus tumoral occupant le cadran supéro-externe de l’orbite droite, mesurant 38 mm de grand axe, envahissant la paroi supérieure et externe de l’orbite avec une importante ostéolyse. Un body scan révèle alors une métastase pulmonaire. L’examen histopathologique complétés par l’immunohistochimie, réalisé après biopsie, révèle un marquage cytoplasmique par l’anticorps anti-chromogranin, l’anticorps anti-synaptophysine et un marquage des vaisseaux par l’anticorps anti-CD31 soulignant l'architecture en zellbalen des nids tumoraux. Cet aspect est en faveur d’un paragangliome malin. Une exérèse chirurgicale incomplète suivie d’une radiothérapie adjuvante, sont alors réalisés. L’origine exacte de cette tumeur au sein de l’orbite reste très controversée. L’exophtalmie reste le principal signe révélateur. La tomodensitométrie, l’imagerie par résonnance magnétique et la scintigraphie au Metaiodobenzylguanidine radioinonisée à l’iode (MIBG-I131) permettent d’orienter le diagnostic et faire un bilan d’extension de la tumeur. Le diagnostic de certitude repose sur l’histopathologie et l’immunohistochimie. L'excision totale de la lésion est le traitement de choix pour les lésions bien délimitées. Dans les formes plus étendues le traitement repose sur l’excision incomplète associée à une radiothérapie adjuvante ou au MIBG I 131. La localisation orbitaire du paragangliome reste très rare. Son diagnostic est difficile et repose essentiellement sur l’immunohistochimie. Son pronostic dépend essentiellement de l’extension locale et de la présence de métastases à distance qui signe le caractère malin du paragangliome.Pan African Medical Journal 2012; 12:3

Spontaneous pneumorrhachis and transverse myelitis complicating purulent meningitis

Pneumorrhachis is the presence of air in the spinal canal; mostly, it has an iatrogenic origin. The association of this entity with spontaneous pneumomediastinum without any pneumothorax is rarely reported in the literature. The spontaneous resorption is the usual evolution. The association to acute transverse myelitis is discussed by the authors. The patient is a 21-year-old male with pneumorrhachis associated to a spontaneous pneumomediastinum was admitted at the emergency department for bacterial meningitis. The antibiotherapy has marked the clinical profile by disappearance of the meningeal signs in the 48 h after admission. In contrast, the neurological symptoms were of marked aggravation by appearance of a tetraparesis with a respiratory distress syndrome having required artificial ventilation. The computed tomography (CT) scan showed a typical hypodensity corresponding to paramedullary air extending to several thoracic segments. The spinal magnetic resonance imaging (MRI) showed a high cervical medullary edema without signs of compression. The patient died within 15 days with a profile of vasoparalysis resistant to vasoactive drugs. Pneumomediastinum associated to pneumorrhachis and transverse myelitis complicating purulent meningitis is a rare entity. Although the usual evolution is favorable, the occurrence of serious complications is possible

Multisystem Sarcoidosis in a Patient on Interferon-α Therapy for Chronic Hepatitis C

Sarcoidosis is a chronic multisystemic granulomatous disease that is triggered by an autoimmune process. Nowadays, this pathology represents a well-recognized but uncommon complication for antiviral treatment in hepatitis C virus (HCV) infection. Herein, we report a remarkable case of 47-year-old woman treated for chronic HCV infection; the patient has developed interferon alfa-induced sarcoidosis involving the central nervous system. The evolution was fatal despite disrupting the antiviral therapy and initiating a high-dose corticotherapy. This complication of interferon alfa treatment was reported in the literature in only one case. Through this case and a review of the literature, we aim to underline the importance of screening for sarcoidosis before and during the follow-up of HCV patients undergoing antiviral therapy

Intracranial ependymal cyst with unusual presentation: Case report and review of literature

Background: Ependymal cysts remain a very rare cause of neurological symptoms among intracranial cystic lesions. It differs from an arachnoid cyst, as it does not communicate with subarachnoid space. Case description: A 33-year-old woman was admitted in our neurosurgery department with history of headache, vomiting and right unilateral blindness. Neuroimaging studies showed an intraparenchymal cystic lesion in the right frontal lobe with radiologic characteristic as seen in the ependymal cyst or hydatic cyst. She underwent the neurosurgical procedure of marsupialization and was discharged on day 6 post op. At one month follow up, the patient presented with a decrease vision acuity on the left eye, and head CT scan showed a recurrence of the cyst hence, we performed a second surgery with shunt procedure. Histological examination of the resected tissue demonstrated that, it was an ependymal cyst. Conclusion: Since there is often times a clinico-radiological discordance they are generally incidental findings in majority of cases. While complete surgical excision is the treatment of choice for the symptomatic cysts, cystic fluid diversion presents a better compromise nevertheless in cases where this may not be achieved. Keywords: Ependymal cyst, Brain, MRI, Surger

Emotion processing in Parkinson’s disease: a blood oxygenation level-dependent functional magnetic resonance imaging study

Parkinson’s disease is a neurodegenerative disorder caused by loss of dopamine neurons in the substantia nigra pars compacta. Tremor, rigidity, and bradykinesia are the major symptoms of the disease. These motor impairments are often accompanied by affective and emotional dysfunctions which have been largely studied over the last decade. The aim of this study was to investigate emotional processing organization in the brain of patients with Parkinson’s disease and to explore whether there are differences between recognition of different types of emotions in Parkinson’s disease. We examined 18 patients with Parkinson’s disease (8 men, 10 women) with no history of neurological or psychiatric comorbidities. All these patients underwent identical brain blood oxygenation level-dependent functional magnetic resonance imaging for emotion evaluation. Blood oxygenation level-dependent functional magnetic resonance imaging results revealed that the occipito-temporal cortices, insula, orbitofrontal cortex, basal ganglia, and parietal cortex which are involved in emotion processing, were activated during the functional control. Additionally, positive emotions activate larger volumes of the same anatomical entities than neutral and negative emotions. Results also revealed that Parkinson’s disease associated with emotional disorders are increasingly recognized as disabling as classic motor symptoms. These findings help clinical physicians to recognize the emotional dysfunction of patients with Parkinson’s disease

EGFR Amplification and IDH Mutations in Glioblastoma Patients of the Northeast of Morocco

Glioblastomas are the most frequent and aggressive primary brain tumors which are expressing various evolutions, aggressiveness, and prognosis. Thus, the 2007 World Health Organization classification based solely on the histological criteria is no longer sufficient. It should be complemented by molecular analysis for a true histomolecular classification. The new 2016 WHO classification of tumors of the central nervous system uses molecular parameters in addition to histology to reclassify these tumors and reduce the interobserver variability. The aim of this study is to determine the prevalence of IDH mutations and EGFR amplifications in the population of the northeast region of Morocco and then to compare the results with other studies. Methods. IDH1 codon 132 and IDH2 codon 172 were directly sequenced and the amplification of exon 20 of EGFR gene was investigated by qPCR in 65 glioblastoma tumors diagnosed at the University Hospital of Fez between 2010 and 2014. Results. The R132H IDH1 mutation was observed in 8 of 65 tumor samples (12.31%). No mutation of IDH2 was detected. EGFR amplification was identified in 17 cases (26.15%). Conclusion. A systematic search of both histological and molecular markers should be requisite for a good diagnosis and a better management of glioblastomas