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Dementia & Neuropsychologia

Print version ISSN 1980-5764

Dement. neuropsychol. vol.3 no.1 São Paulo Jan./Mar. 2009

http://dx.doi.org/10.1590/S1980-57642009DN30100003 

Views & Reviews

Normal pressure hydrocephalus: Diagnostic and predictive evaluation

Hidrocefalia de pressão normal: avaliação diagnóstica e preditiva

Benito Pereira Damasceno1 

1Unidade de Neuropsicologia e Neurolinguística, Departamento de Neurologia, Faculdade de Ciências Médicas, Universidade Estadual de Campinas, SP, Brazil.


Abstract

In typical cases, normal pressure hydrocephalus (NPH) manifests itself with the triad of gait disturbance, which begins first, followed by mental deterioration and urinary incontinence associated with ventriculomegaly (on CT or MRI) and normal cerebrospinal fluid (CSF) pressure. These cases present minor diagnostic difficulties and are the most likely to improve after shunting. Problems arise when NPH shows atypical or incomplete clinical manifestations (25-50% of cases) or is mimicked by other diseases. In this scenario, other complementary tests have to be used, preferentially those that can best predict surgical outcome. Radionuclide cisternography, intracranial pressure monitoring (ICP) and lumbar infusion tests can show CSF dynamics malfunction, but none are able to confirm whether the patient will benefit from surgery. The CSF tap test (CSF-TT) is the only procedure that can temporarily simulate the effect of definitive shunt. Since the one tap CSF-TT has low sensitivity, it cannot be used to exclude patients from surgery. In such cases, we have to resort to a repeated CSF-TT (RTT) or continuous lumbar external drainage (LED). The most reliable prediction would be achieved if RTT or LED proved positive, in addition to the occurrence of B-waves during more than 50% of ICP recording time. This review was based on a PubMed literature search from 1966 to date. It focuses on clinical presentation, neuroimaging, complementary prognostic tests, and differential diagnosis of NPH, particularly on the problem of selecting appropriate candidates for shunt.

Key words: normal pressure hydrocephalus; neuropsychological tests; cerebrospinal fluid tap test; shunt surgery

Resumo

Em casos típicos, a hidrocefalia de pressão normal (HPN) manifesta-se com a tríade: distúrbio da marcha, que começa primeiro, seguido de deterioração mental e incontinência urinária associados a ventriculomegalia (na TC ou RM) e pressão liquórica normal. Esses casos conferem pouca dificuldade diagnóstica e são os que mais provavelmente melhoram após a derivação liquórica. O problema é quando a HPN manifesta-se de forma incompleta ou atípica (25-50% dos casos) ou é mimetizada por outras doenças. Então, outros testes complementares têm que ser usados, preferencialmente aqueles que melhor predizem o resultado cirúrgico. A cisternocintilografia, o monitoramento da pressão intracraniana e o teste de infusão lombar podem realmente mostrar disfunção da dinâmica liquórica, mas nenhum deles pode confirmar se o paciente vai beneficiar-se da cirurgia. O teste de punção liquórica é o único que pode temporariamente simular o efeito definitivo da derivação. Uma vez que o teste de (uma única) punção liquórica tem baixa sensibilidade, ele não pode ser usado para excluir pacientes da cirurgia. Em tais casos, temos que apelar para o teste da punção liquórica repetida (PLR) ou da drenagem liquórica lombar externa contínua (DLE). Um diagnóstico preditivo mais seguro seria conseguido se a PLR ou a DLE é positiva, juntamente com a ocorrência de ondas B em mais que 50% do tempo de monitoramento da pressão intracraniana. Este artigo de revisão baseou-se em uma busca na literatura, via PubMed, desde 1966. Ele focaliza a apresentação clínica, diagnóstico diferencial, testes prognósticos complementares e a questão da seleção de candidatos apropriados para a derivação.

Palavras-chave: hidrocefalia de pressão normal; testes neuropsicológicos; teste da punção liquórica; cirurgia de derivação ventriculoperitoneal

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Received: December 31, 2008; Accepted: February 12, 2009

Benito Pereira Damasceno - Universidade Estadual de Campinas, Box 6111 - 13083-970 Campinas SP - Brazil. E-mail: damascen@unicamp.br

Disclosure:

The author reports no conflicts of interest.

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