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Epilepsy has been rarely reported in patients with neurofibromatosis type 1 (formally known as von Recklinghausen disease), which may occur in 3 to 6% of cases. This condition is generally related to neuronal migration anomalies or cortical malformations. We report a case of temporal epilepsy secondary to temporal meningocele due to sphenoidal dysplasia in a patient who presented with neurofibromatosis type 1 and also discuss this association., (© 2013 Elsevier Masson SAS. All rights reserved.)

Introduction: Cephalic tetanus is the most serious form of localized tetanus. It associates trismus with impairment of one or more cranial nerves. It was a rare condition, whose diagnosis can raise several problems., Case Report: A 49-year-old-man presented multiple and unilateral cranial nerve involvement revealing cephalic tetanus., Conclusion: This case illustrates the importance of considering cephalic tetanus when patients present cranial nerve palsy associated with injury., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Introduction: Tuberous sclerosis is an autosomal dominant inherited phakomatosis. It is associated with a wide variety of central nervous system abnormalities, but intracranial aneurysms are rare., Case Report: We report a 34-year-old patient fulfilling the diagnostic criteria of tuberous sclerosis in association with intracranial aneurysm., Discussion: This association has been reported in only 17 other cases of tuberous sclerosis. We discuss the etiopathogenic mechanisms, preferential localizations and the various therapeutic propositions., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Introduction: Segmental neurofibromatosis 1 (segmental NF-1) is a rare genodermatosis caused by somatic mutations in the NF-1 gene. It consists of localized characteristic skin lesions. A serial study using magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) of a brain tumor in a 16-year-old patient with segmental NF-1 is reported., Case Report: A 16-year-old boy with congenital dorsal scoliosis and segmental NF-1 was evaluated for bilateral optic atrophy. Neurological examination showed an isolated tetra pyramidal syndrome. The cerebral MRI showed a bilateral brain lesion involving the basal ganglia, optic pathways, temporal lobes, and the midbrain. Serial MRSs showed a decreased N-acetylaspartate (NAA)/creatine ratio and increased choline/creatine ratio. An increase in the myoinositol (MYO)/creatine ratio and the presence of a lipid/lactate peak were also recorded. A neuroimaging follow-up with MRI and MRS performed 2 years later showed similar findings., Comments and Conclusion: We describe an MRS study of a brain tumor in a patient with segmental NF-1 for the first time. The MRS study showed similar findings, described earlier in rare studies of patients with the classic form of NF-1. MRS is a noninvasive technique for detecting the presence of tumor tissue in the brain through its metabolic activity. MRS plays an important role in clinical studies and it can be used to differentiate malignant and nonmalignant brain lesions from normal brain tissue., (Copyright (c) 2010 Elsevier Masson SAS. All rights reserved.)

We describe the case of a 70-year-old woman, with type 1 diabetes mellitus, who suddenly developed a movement disorder on the left side of her body that rapidly extended to the right side, evoking biballism. There was no facial involvement and no vascular lesions on cerebral MRI but non-ketotic hyperglycaemia was present. A combination of a reduction in glucose levels and the use of neuroleptic drugs resulted in the disappearance of the abnormal movements. In this report, we discuss the association between non-ketotic hyperglycaemia and ballism along with a review of the literature.

Introduction: Multiple sclerosis is one of the most common diseases of the central nervous system with a variety of clinical and radiological presentations. Several cases have been reported of demyelinating processes mimicking a tumour of the central nervous system., Observation: A 45-year-old man was admitted with acute right hemiparesis associated with intracranial hypertension syndrome. Initial CT scan and magnetic resonance imaging of the brain revealed a mass lesion in the left hemisphere. Combined, careful history taking, assessment of the clinical course and magnetic resonance imaging findings led to the final diagnosis of multiple sclerosis., Commentary and Conclusion: This case report illustrates the wide variety of multiple sclerosis presentation. Recognition of the demyelinating tumor like lesions is essential; the diagnosis of multiple sclerosis should be considered in young adults with similar presentations.

Osteogenesis imperfecta (OI) is a group of hereditary disorders most often due to an anomaly of collagen biosynthesis. Divers clinical manifestations are reported. Neurological manifestations are exceptional. A 40-year-old man with a history of multiple bone fractures was admitted for a generalized tonic-clonic seizure. There was no metabolic disorder, the patient however complained of bilateral shoulder pain. Standard radiography and shoulder MRI revealed bilateral humeral fractures. The electroencephalogram and the brain MRI showed no abnormalities. He was given valproate acid and eight months later was free of crises. Search for an etiological favored the diagnosis of Lobstein disease.

Introduction: Celiac disease (CD) is an immune-mediated disease triggered by the ingestion of gluten in genetically susceptible individuals. Neurological manifestations are rare and severe and must be sought systematically., Clinical Cases: Two non related patients each from a consanguineous marriage developed progressive spastic paraplegia 2 and 8 years respectively after onset of CD. The radiological and biological findings were normal except for the presence of abnormalities related to CD., Conclusion: The relationship between spastic paraplegia and CD is not well established. Autoimmune, metabolic and genetic mechanisms could be considered but the probability of a fortuitous association should not be ruled out.

Introduction: Cardiac involvement is described as one of the most frequent multisystemic manifestations of Steinert myotonic dystrophy (DM1). This study was performed to determine the frequency of cardiac abnormalities in Steinert myotonic dystrophy and to decipher the correlation between the severity of cardiac involvement and the degree of neurologic deficit., Patients and Results: Thirty-four DM1 patients 23 men and 11 women, aged 13-61 years (mean 37.3+/-13.2 years) underwent neurological and cardiac evaluations. According to the MDRS scale, 32.5 percent were classified in the second stage, 23 percent in stage 3; 32.5 percent in stage 4 and 12 percent in stage 5. There was a positive correlation between neurological symptoms duration and the MRDS scale. Cardiac involvement was detected in 77.4 percent of patients. Electrocardiographic conduction abnormalities were the most frequent, represented by first-degree atrioventicular block in 64 percent of patients and bundle-branch block in 32 percent. From 5 patients having an invasive electrophysiology testing, subhisien block was observed in 3 patients. We respectively found alterations in systolic and diastolic left ventricular function in 22 percent and 30 percent of patients and a cardiac pacemaker was implanted in 3 patients. The frequency of cardiac manifestations was correlated to the degree of the neurological involvement assisted by MDRS scale, but it seam that the severity of cardiac abnormalities is not correlated to the degree of neurological deficit., Conclusion: We recommend that patients with DM1 undergo 24-h electrocardiogram monitoring and echocardiography at least yearly. Long-term prospective follow-up is required to determine the prognostic value of the observed abnormalities.

Central nervous system involvement in patients with hepatitis C infection have been rarely reported. Stroke and encephalopathic syndromes have been frequently attributed to the ischemia or hemorrhage associated with mixed cryoglobulinemia and anticardiolipin antibodies. We describe the case of a 31-year-old woman with cerebral ischemia who had hepatitis C infection confirmed by polymerase change reaction detection of HCV RNA but who was negative for cryoglobulinemia. Cerebral involvement may be the initial manifestation of hepatitis C infection.