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      Hipotensão intracraniana secundária a fístula liquórica radicular espontânea: relato de caso Translated title: Spontaneous nerve root cerebrospinal fluid leaks and intracranial hypotension: case report

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          Abstract

          A hipotensão intracraniana espontânea é síndrome rara, caracterizada pela diminuição da pressão no líquido cefalorraquidiano (LCR), nos valores de 50 a 70 mm H2O, e cefaléia postural. O diagnóstico é feito através do quadro clínico, da medida da pressão do LCR e do estudo radiológico do encéfalo e da coluna vertebral. O reconhecimento dessa patologia tem sido crescente em razão de suas características próprias que permitem distingui-la de processos meníngeos inflamatórios ou de tumores, evitando assim investigações desnecessárias. Relatamos um caso de hipotensão intracraniana secundária a fístula liquórica radicular espontânea em um homem de 34 anos e fizemos revisão da literatura sobre os aspectos clínicos, diagnósticos e terapêuticos dessa entidade de incidência incomum.

          Translated abstract

          Spontaneous intracranial hypotension is a rare syndrome, characterized by pressure in the cerebrospinal fluid ranging between 50 and 70 mmH2O and postural headache. Its diagnosis is made through the clinical presentation, measurement of the cerebrospinal fluid pressure and neurorimage features. The clinical recognition of this pathology has been increasing and the differential diagnosis must be made with inflammatory meningeal processes and tumor. We report a case of spontaneous nerve root cerebrospinal fluid leaks in a 34 year-old man and intracranial hypotension. A literature review was performed evaluating the clinical, diagnostic and therapeutic aspects of this unusual pathology.

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          Most cited references28

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          Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.

          Spontaneous intracranial hypotension is caused by spontaneous spinal cerebrospinal fluid (CSF) leaks and is known for causing orthostatic headaches. It is an important cause of new headaches in young and middle-aged individuals, but initial misdiagnosis is common. To summarize existing evidence regarding the epidemiology, pathophysiology, diagnosis, and management of spontaneous spinal CSF leaks and intracranial hypotension. MEDLINE (1966-2005) and OLDMEDLINE (1950-1965) were searched using the terms intracranial hypotension, CSF leak, low pressure headache, and CSF hypovolemia. Reference lists of these articles and ongoing investigations in this area were used as well. Spontaneous intracranial hypotension is caused by single or multiple spinal CSF leaks. The incidence has been estimated at 5 per 100,000 per year, with a peak around age 40 years. Women are affected more commonly than men. Mechanical factors combine with an underlying connective tissue disorder to cause the CSF leaks. An orthostatic headache is the prototypical manifestation but other headache patterns occur as well, and associated symptoms are common. Typical magnetic resonance imaging findings include subdural fluid collections, enhancement of the pachymeninges, engorgement of venous structures, pituitary hyperemia, and sagging of the brain (mnemonic: SEEPS). Myelography is the study of choice to identify the spinal CSF leak. Treatments include bed rest, epidural blood patching, percutaneous placement of fibrin sealant, and surgical CSF leak repair, but outcomes have been poorly studied and no management strategies have been studied in properly controlled randomized trials. Spontaneous intracranial hypotension is not rare but it remains underdiagnosed. The spectrum of clinical and radiographic manifestations is varied, with diagnosis largely based on clinical suspicion, cranial magnetic resonance imaging, and myelography. Numerous treatment options are available, but much remains to be learned about this disorder.
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            Syndrome of Orthostatic Headaches and Diffuse Pachymeningeal Gadolinium Enhancement

            To characterize clinical and imaging features, biopsy findings, etiologic factors, and outcome in the syndrome of intracranial hypotension, headaches, and diffuse pachymeningeal gadolinium enhancement on magnetic resonance imaging (MRI).
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              Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak.

              Spontaneous intracranial hypotension has been considered a rare clinical entity, with less than 75 cases reported in the medical literature. With the advent of magnetic resonance imaging (MRI), spontaneous intracranial hypotension currently is being recognized more frequently. The authors report the neurovisual manifestations of this disorder in a consecutive series of three patients. Each patient underwent clinical examination, computerized visual field testing, and MRI. After treatment, each patient was re-examined, and MRI was repeated. Two patients had transient visual obscurations and unusual binasal visual field defects on automated perimetry. A third patient had diplopia from an abducens nerve paresis. After treatment of spontaneous intracranial hypotension, these findings improved or resolved. This case series, and a review of previously reported cases, indicates that neurovisual problems are common in patients with spontaneous intracranial hypotension. Findings may include diplopia from sixth nerve paresis, transient visual obscurations, blurred vision, visual field defects, photophobia, and nystagmus.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                anp
                Arquivos de Neuro-Psiquiatria
                Arq. Neuro-Psiquiatr.
                Academia Brasileira de Neurologia - ABNEURO (São Paulo )
                1678-4227
                March 2003
                : 61
                : 1
                : 134-136
                Affiliations
                [1 ] Fundação Universidade de Caxias do Sul Brasil
                [2 ] Universidade Federal de São Paulo Brazil
                [3 ] Universidade Federal de São Paulo Brazil
                [4 ] Universidade de Caxias do Sul
                Article
                S0004-282X2003000100028
                10.1590/S0004-282X2003000100028
                ce21c304-f29a-4b33-9d8e-ec9b546c7541

                http://creativecommons.org/licenses/by/4.0/

                History
                Product

                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=0004-282X&lng=en
                Categories
                NEUROSCIENCES
                PSYCHIATRY

                Neurosciences,Clinical Psychology & Psychiatry
                spontaneous intracranial hypotension,postural headache,perineural cyst,hipotensão intracraniana espontânea,cefaléia postural,cisto perineural

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