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Abstract: Introduction: Spontaneous low cerebrospinal fluid pressure syndrome is a spontaneous intracranial hypotension pressure due to a cerebrospinal fluid leak without any known dural effraction. It is clinically characterised by postural headaches relieved by supine position. We report a 38-year-old patient with this syndrome and review the literature. Exegesis: The diagnosis is sometimes difficult in atypical presentation of the syndrome and can lead to incapacitating chronic headache and rarely to complications. Cerebral magnetic resonance imaging has dramatically improved identification, diagnosis and management of this syndrome. Treatment is mainly based on blood patch realisation. Cerebrospinal fluid leak probably due to a spontaneous defect in the dural mater is suspected to be the main mechanism of this syndrome without any history of lumbar puncture or penetrating trauma. Conclusion: Early diagnosis, often easy on the basis of clinical characteristics of the headache may avoid complications. [Copyright &y& Elsevier]

Abstract: Idiopathic intracranial hypertension is a rare disorder characterized by elevated intracranial pressure without hydrocephaly or intracranial process. Its mechanism is poorly understood. Most cases of benign intracranial hypertension are presumed to be idiopathic but some of them may be related to some treatment. We report a 26-year-old female with benign intracranial hypertension due to tetracycline, revealed by headaches and gradual visual loss. Standard investigations were unremarkable and favourable outcome after therapeutic lumbar puncture confirmed the diagnosis. [Copyright &y& Elsevier]

Migrainous infarction is a rare ischemic stroke developing during an attack of migraine, which is more prevalent in young woman and preferentially affects the posterior cerebral circulation. The case of a migrainous 35-old patient with patent foramen ovale admitted in neurology department for a typical migrainous infarction is reported and compared with a literature review. Furthermore, this case report highlights the existence of complex epidemiologic and physiopathologic links between cerebral ischemia, migraine and patent foramen ovale.

The FXTAS syndrome (Fragile X-associated tremor/ataxia syndrome) is a specific neurodegenerative syndrome affecting subjects carrying a premutation of the FMR1 (fragile X mental retardation 1) gene. It affects mainly men with the premutation and aged more than 50 years. This syndrome is separate and distinct from the fragile X syndrome. The FXTAS syndrome remains underestimated today. It should be considered in patients older than 50 years with tremors and cerebellar ataxia, especially when Parkinson disease or cognitive disorders are present or when there is a family history of infertility, early menopause, or mental retardation. In these patients, hyperintense signals of mid-cerebellar peduncle images on T2 and FLAIR MRI justify genetic testing for the FMR1 premutation., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)

Introduction: Paramyotonia congenita is an autosomal dominant sodium channelopathy, caused by mutations in gene coding for muscle voltage-gated sodium channel alpha subunit., Case Report: We report the case of a 38-year-old man who described since childhood muscle stiffness with attacks ok weakness induced by two provocative stimuli: cold exposure and exercise. It primarily concerned eyelids and hands, occasionally limbs. Family history suggested an autosomal dominant mode of transmission. Clinical examination revealed myotonia at the thenar eminence percussion. Generalized myotonic discharges were observed on electromyography. Molecular diagnosis reported an Arg1448Cys mutation in exon 24 in gene coding for muscle voltage-gated sodium channel alpha subunit (SCN4A) in chromosome 17., Conclusion: Paramyotonia congenita is not evolutive. Treatment is essentially preventive. Some medications could be proposed: membrane stabilizing agents like antiarrhythmic drugs (mexiletine, tocainide), or the carbonic anhydrase inhibitor (acetazolamide). Precautions may be taken during general anaesthesia because of diaphragm myotonia risk.