Clinical and radiological features of main dementias / Aspectos clínicos e radiológicos das principais demências

Dementia is a syndrome characterized by a decline of two or more cognitive functions, affecting social or professional life. Alzheimer's Disease is a neurodegenerative disorder that represents 53% of dementia cases; memory loss, inability to recognize faces, impaired judgement, disorientation and confusion are possible common symptoms. Vascular Dementia is responsible for 42% of dementia cases, due to cerebrovascular pathologies, and the clinical aspects are related to the extension and location of the brain injury. Lewy Bodies Dementia is a neurodegenerative disorder that represents 15% of dementia cases, and its symptoms include visual hallucinations, parkinsonism and fluctuating cognitive decline. Frontotemporal dementia is a group of clinical syndromes, divided in Behavioral-variant, characterized by disinhibition, compulsions, apathy, aberrant sexual behavior and executive dysfunction; and Primary Progressive Aphasia, which is subdivided in Nonfluentvariant and Semantic-variant. Vitamin B12 deficiency is a reversible cause of dementia, with a wide clinical feature, that includes psychiatric symptoms such as depression and irritability, hematological symptoms related to anemia (e.g. dyspnea and fatigue), and neurological symptoms including dementia and neuropathy. Normal pressure hydrocephalus is also reversible, presenting forgetfulness, changes in mood, decline of executive functions, reduced attention, and a lack of interest in daily activities as symptoms. The radiological findings vary depending on the etiology of dementia. For that reason, understanding neuroimaging and clinical aspects is important to diagnose effectively.

A demência é uma síndrome que consiste em um declínio de um ou mais domínios cognitivos, que afeta o desempenho social ou profissional do indivíduo. A Doença de Alzheimer é um transtorno neurocognitivo que representa 53% dos casos de demência; seus sintomas podem incluir perda de memória, incapacidade de reconhecer rostos familiares, julgamento comprometido desorientação e confusão mental. A Demência Vascular é responsável por 42% dos casos de demência e é causada por doenças cerebrovasculares, seus achados clínicos são relacionados com o local e com a extensão do dano cerebral. Já a Demência por Corpos de Lewy é uma doença neurocognitiva que representa 15% dos casos de demência, cujos sintomas incluem alucinações visuais, parkinsonismo e flutuação cognitiva. A Demência Frontotemporal, por sua vez, é um grupo de síndromes, que se dividem em variante comportamental ­ caracterizada por desinibição, compulsão, apatia, hipersexualidade e disfunções executivas ­ e Afasia Progressiva Primária, subdividida em variante não-fluente e variante semântica, que cursam com disfunções da linguagem. Há, ainda, a Deficiência de Vitamina B12, uma causa reversível de demência. Ela possui um quadro clínico variado, que inclui sintomas psiquiátricos, como depressão e irritabilidade, sintomas hematológicos relacionados a anemia, como dispneia e fadiga) e sintomas neurológicos, que incluem demência e neuropatias. Uma outra causa reversível é a Hidrocefalia de Pressão Normal, que se apresenta com esquecimentos, alterações de humor, perda de função executiva e redução da atenção e do interesse nas atividades cotidianas. Os achados de neuroimagem variam dependendo da etiologia da demência. Assim, compreender os aspectos clínicos e radiológicos é importante para um diagnóstico efetivo..

Feasibility of action observation effect on gait and mobility in idiopathic normal pressure hydrocephalus patients / Viabilidade do efeito de observação da ação na marcha e mobilidade de pacientes com hidrocefalia de pressão normal idiopática

ABSTRACT. Action observation (AO) has been proved to be of benefit in several neurological conditions, but no study has previously been conducted in idiopathic normal pressure hydrocephalus (iNPH). Objective: This study aimed to investigate the feasibility of AO in iNPH patients. Methods: A single-group pretest-posttest design was conducted in twenty-seven iNPH patients. Gait and mobility parameters were assessed using the 2D gait measurement in the timed up and go (TUG) test for two trials before and after immediate AO training. The outcomes included step length and time, stride length and time, cadence, gait speed, sit-to-stand time, 3-m walking time, turning time and step, and TUG. In addition, early step length and time were measured. AO consisted of 7.5 min of watching gait videos demonstrated by a healthy older person. Parameters were measured twice for the baseline to determine reproducibility using the intraclass correlation coefficient (ICC3,1). Data between before and after immediately applying AO were compared using the paired t-test. Results: All outcomes showed moderate to excellent test-retest reliability (ICC3,1=0.51 0.99, p<0.05), except for the step time (ICC3,1=0.19, p=0.302), which showed poor reliability. There were significant improvements (p<0.05) in step time, early step time, gait speed, sit-to-stand time, and turning time after applying AO. Yet, the rest of the outcomes showed no significant change. Conclusions: A single session of AO is feasible to provide benefits for gait and mobility parameters. Therapists may modify this method in the training program to improve gait and mobility performances for iNPH patients.

RESUMO. A observação de ação (OA) teve benefícios comprovados em diversas condições neurológicas, mas nenhum estudo foi conduzido anteriormente em Hidrocefalia de Pressão Normal idiopática (HPNi). Objetivo: O presente estudo teve como objetivo investigar a viabilidade da OA em pacientes com HPNi. Métodos: Um projeto de pré-teste e pós-teste de grupo único foi realizado em 27 pacientes com HPNi. Parâmetros de marcha e mobilidade foram avaliados por meio de parâmetros 2D para a medida da marcha com o teste timed up and go (TUG) com duas tentativas antes e imediatamente depois do OA. Os resultados incluíram comprimento e tempo do passo, comprimento e tempo da passada, cadência, velocidade da marcha, tempo para sentar-e-levantar, tempo de caminhada de 3 metros, tempo de virada e passo, e tempo do teste (TUG). Além disso, o comprimento do passo inicial e o tempo da etapa inicial foram medidos. A OA consistia em assistir 7,5 minutos de vídeos de marcha demonstrados por um idoso saudável. Os parâmetros foram medidos duas vezes para a linha de base para determinar a reprodutibilidade usando o coeficiente de correlação intraclasse (CCI3,1). Os dados entre antes e depois da aplicação imediata de OA foram comparados com o teste t pareado. Resultados: Todos os resultados mostraram confiabilidade teste-reteste moderada a excelente (CCI3,1=0,51 0,99, p<0,05), exceto para o tempo do passo (CCI3,1=0,19, p=0,302), que apresentou confiabilidade pobre. Houve melhorias significativas (p<0,05) no tempo do passo, tempo do passo inicial, velocidade da marcha, tempo sentar-e-levantar e tempo de virar após a aplicação de OA. Os demais resultados não mostraram nenhuma mudança significativa. Conclusões: Uma única sessão de aplicação de OA é viável para proporcionar benefícios aos parâmetros de marcha e mobilidade. Os terapeutas podem modificar esse método no programa de treinamento para obter desempenho de marcha e mobilidade para pacientes com HPNi.

Normal pressure hydrocephalus: Diagnostic delay / Hidrocefalia de presión normal: demora en el diagnóstico

Abstract. Introduction: Normotensive hydrocephalus is a differential diagnosis in the evaluation of the dementia syndrome. The diagnostic protocols would allow detecting this pathology that has more effective treatment than other dementias. Objective: To describe a population with clinical suspicion of normal pressure hydrocephalus evaluated in a Colombian psychiatric hospital and discuss the possible reasons for its diagnostic and therapeutic delay. Materials and methods: We conducted a retrospective study of medical records to identify patients with suspected normal pressure hydrocephalus during a 5-year period. Results: Thirty-five patients with suspected normal pressure hydrocephalus underwent diagnostic lumbar puncture and five of them were considered candidates for a peritoneal-venous shunt, but none underwent this surgical procedure. After three to six months of the lumbar puncture, the gait pattern improved in 22.8% of the patients, cognition in 22.8%, and sphincter control in 11.4%. Improvement was not sustained in the long term (1 year) in any of them. Conclusion: This study suggests the poor implementation of the protocols for evaluating patients with cognitive deficits and delays in the diagnosis of normal pressure hydrocephalus. A small number of patients were identified as candidates for treatment. Normal pressure hydrocephalus is a potentially reversible clinical entity with the placement of a peritoneal ventricular shunt, but delays in diagnosis and treatment have deleterious consequences for patients and their families.

Resumen. Introducción. La hidrocefalia normotensiva es un diagnóstico diferencial en la evaluación del síndrome demencial. Los protocolos diagnósticos permitirían detectar esta condición, cuyo tratamiento es más efectivo que el de otras demencias. Objetivo. Describir una población con sospecha clínica de hidrocefalia normotensiva evaluada en un hospital psiquiátrico colombiano y discutir las posibles razones de la demora en el diagnóstico y en la terapia de esta condición clínica. Materiales y métodos. Se hizo un estudio retrospectivo de los registros médicos para detectar pacientes con sospecha de hidrocefalia normotensiva durante un período de cinco años. Resultados. A 35 pacientes con sospecha de hidrocefalia normotensiva se les hizo una punción lumbar diagnóstica. Cinco de ellos se consideraron candidatos para una derivación ventrículo-peritoneal, pero ninguno se sometió a este procedimiento quirúrgico. A los 3-6 meses de la punción lumbar, se observó una mejoría del patrón de la marcha en el 22,8 % de los pacientes, de la cognición en el 22,8 % y del control del esfínter en el 11,4 %. La mejoría no se mantuvo a largo plazo (un año) en ningún paciente. Conclusión. Se encontró una implementación deficiente de los protocolos de evaluación de los pacientes con déficit cognitivos y demoras en el diagnóstico de la hidrocefalia normotensiva, así como un número reducido de pacientes clasificados como candidatos para el tratamiento. La hidrocefalia normotensiva es una condición clínica potencialmente reversible con la colocación de una derivación ventrículo-peritoneal. Los retrasos en el diagnóstico y en el tratamiento tienen consecuencias perjudiciales para los pacientes y sus familias.

Retraso del diagnóstico en un paciente con Hakim Adams / Delayed diagnosis in a patient with Hakim Adams

La hidrocefalia normotensiva es una patología caracterizada por alteración en la marcha, incontencia vesical, déficit cognitivo, hallazgos imagenológicos de ventriculomegalia comunicante y, presión normal de líquido cefalorraquídeo. Por la ambigüedad de sus síntomas y por la falta de criterios imagenológicos muy concluyentes es una patología de difícil diagnóstico inicial; el paciente muchas veces acude a distintos especialistas por síntomas aislados hasta que se relacionan los mismos y, se deriva el caso a Neurología / Neurocirugía. Se presenta el caso de un paciente de 65 años que presentó un cuadro de 2 años de evolución de incontinencia urinaria tratado como alteración de la micción secundaria a una hiperplasia prostática benigna sin mejoría alguna en ese intervalo de tiempo. Luego se agregó al cuadro una alteración en la marcha que fue poco valorada por el paciente y su entorno, sin ser conscientes del déficit cognitivo hasta que se realizó la evaluación neuropsicológica. Si bien el paciente presentaba la triada clásica que caracteriza a la hidrocefalia de presión normal, no fue hasta dos años luego del inicio del cuadro que se llegó al diagnóstico y, fue intervenido quirúrgicamente. Se colocó una válvula de derivación ventrículo peritoneal de presión media, con la cual presentó una evolución favorable y, resolución absoluta de los síntomas.

Normotensive hydrocephalus is a pathology characterized by impaired gait, bladder incontinence, cognitive deficit, images findings of communicating ventriculomegaly and normal cerebrospinal fluid pressure. Due to the ambiguity of its symptoms and the lack of conclusive images criteria, it is difficult initial diagnosis pathology; the patient often goes to different specialists for isolated symptoms until they are related, and the case is referred to Neurology / Neurosurgery. We present the case of a patient who presented a 2-year history of urinary incontinence treated as an alteration of urination secondary to a benign prostatic hyperplasia without any improvement in that time interval. Then a gait disturbance was added to the picture that was poorly valued by the patient and his environment, without being aware of the cognitive deficit until a neuropsychological evaluation. Although the patient presented the classic triad that characterizes normal pressure hydrocephalus, it was not until two years after the onset of the condition that the diagnosis was reached and underwent surgery. A peritoneal ventricular shunt of medium pressure was placed, presenting a favorable evaluation and complete resolution of symptoms.

The Impact of Comorbid Alzheimer's Disease in a Patient with Normal Pressure Hydrocephalus?

No abstract available.

Indicación de estimulación cerebral profunda en casos complejos de enfermedad de Parkinson. Experiencia en el Hospital Clínico Universidad de Chile / Indication of deep brain stimulation in complex cases of Parkinson's disease. Experience at the Hospital Clínico Universidad de Chile

Introduction: Deep brain stimulation (DBS) is a standard surgical procedure for the treatment of advanced Parkinson Disease (PD) with motor complications that cannot be adequately managed by medical treatment. Currently available literature can guide physicians on basic aspects of patients' selection and indications for DBS. However, there is a range of real-world clinical settings where the indications of DBS for Parkinson disease are debatable. Objective: to present the experience on PD patients with complex clinical manifestations treated with DBS in our hospital. Method: Report of four PD cases treated with DBS. Case 1: 63-year old woman with advanced PD and severe motor complications; Case 2: 60-year old man with 5 years of disease duration and mild motor complications; Case 3: 67-year old man with severe ventriculomegaly that may have precluded direct electrode passage to the surgical target; Case 4: 67- year-old woman with putative severe axial disability. Results: After one year of follow-up, all patients showed improvement on motor symptoms as well as quality of life. Discussion: We provide a brief rationale for the patient selection in each case to support the decision-making in the management of PD patients with complex clinical cases. (AU)

Home physical exercise program: analysis of the impact on the clinical evolution of patients with normal pressure hydrocephalus / Programa de exercícios físicos domiciliares: análise do impacto na evolução clínica de pacientes com hidrocefalia de pressão normal

ABSTRACT Objective: To present a program of home physical exercises for patients with normal pressure hydrocephalus (NPH) and to evaluate adherence, acceptance and applicability; to verify possible changes in patients with NPH in the home physical exercise program, comparing patients with, and without, a ventriculoperitoneal shunt, regarding gait, quality of life, activities of daily living, static and dynamic balance and its impact on the risks of falling. Methods: This was a controlled clinical trial, with assessments in three moments (0, 10 and 18 weeks) at the home, from October 2015 to November 2017. Fifty-two patients (30 women and 22 men) participated in the study. Results: There was a statistically significant improvement with 10 weeks of home physical exercises for the groups with and without ventriculoperitoneal shunt, respectively, in the sub-items: activities of daily living p = 0.032*, p = 0.003*; static balance p < 0.001*, p < 0.001*; functional capacity p < 0.001*, p = 0.027*; and dynamic balance and gait p = 0.009*, p < 0.001*. There was no statistically significant difference for the subitems: quality of life p = 0.695, p = 1.000; and NPH grading scale p = 0.695, p = 1.000, respectively. Conclusion: The developed program of home physical exercise was easily applied and there was good acceptance by most patients with NPH included in the research. There was a statistically significant improvement with the 10 weeks of home physical exercises in the sub-items: activities of daily living, static balance and functional capacity, for both groups. In the sub-item dynamic balance and gait, there was a statistically significant improvement for both groups, but with a higher score for the group with a ventriculoperitoneal shunt. There was no statistically significant difference for the sub-items: quality of life, NPH grading scale and risk of falls, based on the Berg scale.

RESUMO Objetivo: Apresentar um programa de exercícios físicos domiciliares para pacientes com hidrocefalia de pressão normal e avaliar a adesão, aceitação e sua aplicabilidade; verificar possíveis alterações nos pacientes com HPN com o programa de exercícios físicos domiciliares, comparando os pacientes com e sem derivação ventriculoperitoneal, no que diz respeito à marcha, qualidade de vida, atividades de vida diária, equilíbrio estático e dinâmico e sua repercussão nos riscos de queda. Métodos: Trata-se de um Ensaio clínico controlado, com avaliações em três momentos (0.10 e 18 semanas) em nível domiciliar, no período de outubro/2015 a novembro/2017 Participaram do estudo 52 pacientes (30 mulheres e 22 homens). Resultados: Houve melhora estatisticamente significante com as dez semanas de exercícios físicos domiciliares para os grupos sem e com derivação ventriculoperitoneal respectivamente, nos subitens: atividades de vida diária p = 0,032* p = 0.003*, equilíbrio estático p < 0.001*, p < 0.001*; capacidade funcional p < 0.001*, p = 0,027*; equilíbrio dinâmico e marcha p = 0.009*, p < 0.001*. Não houve diferença estatística significante para os subitens: qualidade de vida p = 0,695, p = 1,000 e escala de graduação de HPN p = 0,695, p = 1,000. Conclusão: O programa de exercícios físicos domiciliares desenvolvido mostrou-se de fácil aplicabilidade e houve boa aceitação para a maioria dos pacientes com Hidrocefalia de Pressão Normal inseridos na pesquisa. Houve melhora estatisticamente significante com as dez semanas de exercícios físicos domiciliares nos subitens: atividades de vida diária, equilíbrio estático e capacidade funcional para ambos os grupos. No subitem equilíbrio dinâmico e marcha houve melhora estatisticamente significante para ambos os grupos, mas com escore maior para o grupo com derivação ventriculoperitoneal. Não houve diferença estatisticamente significante para os subitens: qualidade de vida, escala de graduação de Hidrocefalia de Pressão Normal e risco de quedas baseado na escala de Berg.

Salomón Hakim: the man behind normal pressure hydrocephalus / Salomón Hakim: o homem por trás da hidrocefalia de pressão normal

ABSTRACT The illustrious Colombian Professor Salomón Hakim provided the annals of neurology with one of the most brilliant and original bodies of research on record, developing the concept of normal pressure hydrocephalus, as well as proving that ventricular shunting is an effective treatment. Thus, Professor Hakim proved that some of the dementias, at that time considered senile, could be successfully treated. Here the authors present an historical review of his main contributions, which continue to influence the study of dementia to this day.

RESUMO O ilustre professor colombiano Salomón Hakim deixou como legado nos anais da neurologia uma das mais brilhantes e originais séries de pesquisa da história, desenvolvendo o conceito de hidrocefalia de pressão normal, bem como introduzindo a derivação ventricular como tratamento efetivo. Assim, Hakim provou que algumas das demências até então consideradas senis tinham possibilidade de tratamento bem-sucedido. Aqui os autores apresentarão uma revisão histórica de suas maiores contribuições, que continuam a influenciar o estudo de demências até os nossos dias.

Normal-pressure hydrocephalus: A critical review / Hidrocefalia de pressão normal: uma revisão crítica

ABSTRACT. Normal-pressure hydrocephalus (NPH) is a potentially reversible syndrome characterized by enlarged cerebral ventricles (ventriculomegaly), cognitive impairment, gait apraxia and urinary incontinence. A critical review of the concept, pathophysiology, diagnosis, and treatment of both idiopathic and secondary NPH was conducted. We searched Medline and PubMed databases from January 2012 to December 2018 using the keywords "normal-pressure hydrocephalus" / "idiopathic normal-pressure hydrocephalus" / "secondary normal-pressure hydrocephalus" / "NPH" / "ventriculoperitoneal shunt". The initial search produced 341 hits. After careful selection, a total of 54 articles were chosen and additional relevant studies were included during the process of writing this article. NPH is an important cause of potentially reversible dementia, frequent falls and recurrent urinary infections in the elderly. The clinical and imaging features of NPH may be incomplete or nonspecific, posing a diagnostic challenge for medical doctors and often requiring expert assessment to minimize unsuccessful surgical treatments. Recent advances resulting from the use of non-invasive MRI methods for quantifying cerebral blood flow, in particular arterial spin-labeling (ASL), and the frequent association of NPH and obstructive sleep apnea (OSA), offer new avenues to understand and treat NPH.

RESUMO. A hidrocefalia de pressão normal (HPN) é uma síndrome potencialmente reversível marcada por ventrículos cerebrais alargados (ventriculomegalia), declínio cognitivo, apraxia da marcha e incontinência urinária. Revisar criticamente o conceito, a fisiopatologia, o diagnóstico e o tratamento da HPN idiopática e secundária. Os autores acessaram as bases de dados Medline e Pubmed entre janeiro de 2012 e dezembro de 2018, utilizando as palavras-chave "normal-pressure hydrocephalus" / "idiopathic normal-pressure hydrocephalus" / "secondary normal-pressure hydrocephalus" / "NPH" / "ventriculoperitoneal shunt". A busca inicial resultou em 341 artigos. Após cuidadosa seleção, 54 estudos foram escolhidos e pesquisas adicionais foram incluídas durante o processo de elaboração do manuscrito. A HPN é uma importante causa de demência potencialmente reversível, quedas frequentes e infecção urinária recorrente em idosos. As características clínicas e de imagem da HPN podem ser incompletas ou inespecíficas, de modo que este se torna um diagnóstico difícil para médicos. Não raro uma avaliação por especialista é necessária, visando minimizar tratamentos cirúrgicos ineficazes. Avanços recentes advindos do uso não invasivo de ressonância magnética para quantificação do fluxo sanguíneo cerebral, em particular arterial spin-labeling (ASL), assim como a usual associação entre HPN e apneia obstrutiva do sono representam novos meios de entender e de tratar a HPN.

Development of the Brazilian Portuguese version of the "Grading scale for idiopathic normal pressure hydrocephalus": cross-cultural adaptation, reliability and validity / Validação da escala de hidrocefalia de pressão normal idiopática para o português brasileiro: aplicação, confiabilidade e validade

ABSTRACT Objective: The current study translated to Portuguese and validated the normal pressure hydrocephalus (NPH) scale originally developed in English as the Grading Scale for Idiopathic Normal Pressure Hydrocephalus. Methods: Following Guillemin's validation protocol, the last version of the Portuguese NPH scale was applied to 121 consecutive patients with a diagnosis of normal pressure hydrocephalus (73 men and 48 women) from the Group of Cerebral Hydrodynamics from July 2010 to March 2012. Results: The mean age was 71.09 years old, ranging from 35 to 92 years. The rate of agreement and reproducibility was high, as confirmed by Cohen's Kappa coefficient, with excellent intraobserver correlation for the NPH scale items individually evaluated: gait (0.80), dementia (0.90) and incontinence (0.87). Conclusions: The Portuguese version of the Grading Scale for Idiopathic Normal Pressure Hydrocephalus was successfully translated and validated for use in Brazilian patients.

RESUMO Objetivos: o presente estudo valida para a língua portuguesa a escala de hidrocefalia de pressão normal (HPN) desenvolvida em língua inglesa como "Escala de classificação para hidrocefalia de pressão normal idiopática". Métodos: Usando o método de Guillemin, uma versão traduzida da escala foi aplicada em 121 pacientes consecutivos com diagnóstico de hidrocefalia de pressão normal (73 homens e 48 mulheres) no Grupo de Hidrodinâmica Cerebral do Hospital das Clínicas da FMUSP de julho de 2010 a março de 2012. Resultados: a média de idade foi de 71,09 anos, variando de 35 a 92 anos. A taxa de concordância e reprodutibilidade foi alta, conforme confirmado pelo coeficiente Kappa, com excelente correlação intraobservador para itens de escala HPN que avaliou a marcha (0,80), demência (0,90) e incontinência (0,87). Conclusões: a versão em português da escala de graduação para pacientes com HPN foi traduzida e validada com sucesso para uso em pacientes brasileiros de ambos os sexos.

Gait in normal pressure hydrocephalus: characteristics and effects of the CSF tap test / Marcha na hidrocefalia de pressão normal: características e efeitos do tap-test

ABSTRACT Normal pressure hydrocephalus (NPH), described by Hakim and Adams in 1965, is characterized by gait apraxia, urinary incontinence, and dementia. It is associated with normal cerebrospinal fluid (CSF) pressure and ventricular dilation that cannot be attributed to cerebral atrophy. Objectives: To evaluate gait characteristics in patients with idiopathic NPH and investigate the effect of the CSF tap test (CSF-TT) on gait. Methods: Twenty-five patients diagnosed with probable idiopathic NPH were submitted to the CSF-TT. The procedure aimed to achieve changes in gait parameters. Results: Fifteen gait parameters were assessed before and after the CSF-TT. Five showed a statistically significant improvement (p < 0.05): walking speed (p < 0.001), cadence (p < 0.001), step length (p < 0.001), en bloc turning (p = 0.001), and step height (p = 0.004). Conclusion: This study demonstrated that gait speed was the most responsive parameter to the CSF-TT, followed by cadence, step length, en bloc turning, and step height.

RESUMO A hidrocefalia de pressão normal (HPN), descrita por Hakim-Adams em 1965, caracteriza-se por apraxia de marcha, incontinência urinária e demência e está associada com pressão normal do líquido cefalorraquidiano e dilatação ventricular não atribuída a atrofia cerebral. Objetivos: Avaliar as características da marcha em pacientes com HPN idiopática e o efeito do "tap-test" (TT) na marcha. Métodos: Vinte e cinco pacientes com o diagnóstico HPN idiopática provável, foram avaliados com o TT. O procedimento tem como objetivo causar mudanças nas características da marcha. Resultados: Quinze parâmetros da marcha foram avaliados com o TT. Cinco mostraram melhora estatisticamente significativa (p < 0,05): velocidade da marcha (p < 0,001), cadência (p < 0,001), comprimento do passo (p < 0,001), giro em "bloco" (p = 0,001) e altura do passo (p = 0,004). Conclusão: Este estudo demonstrou que a velocidade da marcha foi o parâmetro que mais respondeu ao efeito do TT, seguido da cadência, comprimento do passo, giro em "bloco" e altura do passo.

Four-and-a-half-year Follow-up of Idiopathic Normal Pressure Hydrocephalus Patient Treated with a Ventriculoperitoneal Shunt

A 68-year-old man presented with progressive impairment of gait, balance, and memory. He exhibited a slow and unstable gait. Formal neuropsychological evaluation showed a deficit in visuospatial function and memory with frontal dysfunctions. He was diagnosed as idiopathic normal pressure hydrocephalus with an Evans' ratio of 0.35. Four-and-a-half years after ventriculoperitoneal shunt placement, he showed a marked improvement in gait, as well as in visuospatial and frontal lobe functions. He benefited from shunt surgery for at least four-and-a-half years.

Hydrocephalus in a Patient with Alzheimer's Disease

BACKGROUND: Normal pressure hydrocephalus (NPH) is an etiology of dementia that is reversible following cerebrospinal fluid shunt placement, however, surgical intervention not always clinically effective and the respons to shunt therapy is poorly understood. Furthermore, NPH is a source of comorbidity in diseases with neurodegenerative pathology, such as Alzheimer's disease (AD). CASE REPORT: A 61-year-old woman presented to the neurology clinic with progressive gait difficulties and cognitive impairment over five years. Nine years after ventriculoperitoneal (VP) shunt treatment, the patient began to experience frequent falls. There was no improvement in clinical symptoms after the alteration of valve pressure on the VP shunt. An 18F-florbetaben amyloid positron emission tomography scan showed increased diffusion uptake over the bilateral cortices, precuneus, and posterior cingulate cortex. CONCLUSIONS: The patient of NPH was unresponsive to shunt therapy due to the development of AD.

The Glymphatic System: A Review

The brain represents 2% of the adult body mass; conversely, it is responsible for 20% to 25% of the glucose and 20% of the oxygen consumption, receiving 15% of the cardiac output. This substantial metabolic rate is associated with a significant production of biological debris, which is potentially toxic. Therefore, a complex and efficient clearance system is required to prevent the accumulation of byproducts and ensure optimal function. However, until today, there is little knowledge about this topic. The glymphatic system, also known as perivascular pathway, is a recently described glialdependent network that is responsible for the clearance of metabolites from the central nervous system (CNS), playing a role equivalent to the one played by the lymphatic vessels present in other organs. Studies have demonstrated that the glymphatic pathway has a paramount role in protein homeostasis, and that the malfunction of this system may be related to the development of neurodegenerative disorders such as Alzheimer disease and normal pressure hydrocephalus. They also showed that body posture, exercise and the state of consciousness influence the glymphatic transport. In this context, the understanding of this clearance system could not only clarify the pathophysiology of several diseases, but also contribute to future therapeutic interventions. In the present article, we will evaluate the glymphatic pathway, focusing on the factors that regulate its flow, as well as on its role in CNS physiology and in disease initiation and progression, including dementia, hydrocephalus, glaucoma and traumatic brain injury. Ultimately, this review also aims to encourage further research on novel therapeutic targets.

Meningiomas petroclivales: estrategia quirúrgica endoscópica a propósito de dos casos / Petroclival meningioma: endoscopic approach and case report

A pesar del desarrollo de las técnicas quirúrgicas de base de cráneo, los meningiomas petroclivales constituyen un reto para el neurocirujano debido a su localización y relación con estructuras neurológicas y vasculares críticas. Se reportan 2 pacientes con diagnóstico de meningioma petroclival que recibieron tratamiento por etapas incluyendo derivación ventrículo peritoneal asistida por endoscopia para la hidrocefalia, abordaje endonasal endoscópico (AEE) extendido al ápex petroso, keyhole subtemporal y retromastoideo con remoción de la lesión. La evolución fue satisfactoria. Se concluyó que los abordajes endoscópicos y por etapas constituyen una excelente opción en el tratamiento de los meningiomas petroclivales.

In spite of the development of the skull base surgery techniques, petroclival meningiomas are a challenge for neurosurgeon due to their localization and relationship with neurovascular structures. Those are two patient with diagnostic of petroclival meningioma whom received treatment step by step included ventricle peritoneal shunt with endoscopic guide for hydrocephalus, extended endonasal approach to petrous apex, subtemporal and retrosigmoid keyhole. The endoscopic approach is an excellent option in the treatment of petroclival meningioma.

Cisternografía radioisotópica con 99Tc-DTPA para confirmación diagnóstica de la hidrocefalia oculta normotensa / 99 Tc-DTPA radionuclide cisternography to confirm the diagnosis of normal pressure hydrocephalus

Se presenta el caso clínico de una paciente que ingresó en el Hospital Clinicoquirúrgico Hermanos Ameijeiras con antecedentes de etilismo crónico y cuadro clínico progresivo de deterioro cognitivo-conductual, trastornos de la marcha y esfinterianos, a quien se le realizó una cisternografía radioisotópica con el objetivo de comprobar su eficacia en el estudio de la dinámica del líquido cefalorraquídeo para diagnosticar la hidrocefalia oculta normotensa. La resonancia magnética de cráneo evolutiva evidenció hidrocefalia y la cisternografía radioisotópica confirmó el diagnóstico de hidrocefalia oculta normotensa. A la paciente se le realizó una derivación ventrículo peritoneal con la que se obtuvo una respuesta clínica evolutiva favorable. Se concluye que la cisternografía radioisotópica continúa siendo una herramienta útil para confirmar este diagnóstico y predecir la respuesta al tratamiento derivativo(AU)

A female patient who was admitted to Hermanos Ameijeiras Clinical and Surgical Hospital is presented here. She had a history of chronic alcohol abuse and progressive clinical deterioration of cognitive behavioral, abnormal gait and sphincter disorders. This patient had a radioisotope cisternography in order to test its effectiveness in the study of the dynamics of cerebrospinal fluid, and to diagnose normotensive hidden hydrocephalus. This MRI revealed hydrocephalus and skull evolutionary radioisotope cisternography confirmed the diagnosis of normal pressure hidden hydrocephalus. This patient received a shunt with a favorable evolutionary clinical response. It is concluded that cisternography radioisotope remains a useful tool to confirm this diagnosis and predict response to derivative treatment(AU)

Síndrome de hidrocefalia normotensa del adulto / Adult Normal-Pressure Hydrocephalus Syndrome

La hidrocefalia normotensa es un síndrome clínico, que se caracteriza por el desarrollo progresivo de anomalías de la marcha, deterioro intelectual e incontinencia urinaria, la misma se diagnostica sobre todo, en pacientes mayores de 60 años. En las imágenes cerebrales se ve dilatación ventricular sin una importante atrofia cerebral. Ocurre como complicación tardía de infección intracerebral, hemorragia subaracnoidea y trauma craneal. Se presenta una paciente femenina de 69 años de edad, de tez blanca, con historia de hipertensión arterial desde hace 6 años con trastornos del equilibrio que han resultado en caídas con fracturas de cadera, clavícula, húmero y contusiones faciales. En los últimos años el trastorno del equilibrio empeoró e imposibilitó las tareas de la vida diaria, con lentitud en la marcha, asociado a un trastorno del lenguaje que, en su evolución, dificultó la comunicación verbal y, posteriormente, presentó dificultad para recordar hechos recientes además de incontinencia urinaria de urgencia(AU)

Normotensive hydrocephalus is a clinical syndrome, characterized by the progressive development of walking abnormalities, intellectual deterioration and urinary incontinence. It is mostly diagnosed in patients over 60 years of age. Ventricular dilation with no major brain atrophy is seen in cerebral images. It occurs as a late complication of intra-cerebral infection, subarachnoid hemorrhage and cranial trauma. A 69-year-old white female patient with a history of arterial hypertension for the past 6 years presented balance disorders that have led to falls resulting in fractures of hip, clavicle, humerus and facial contusions. In last years the disorder of the balance worsened and made her impossible to perform the daily life tasks, her march got slow, associated to a disorder of speech, that made difficult the verbal communication at her examination, later, she presented difficulty to remember recent facts, as well as urge urinary incontinence(AU)

Performance of the fixed pressure valve with antisiphon device SPHERA® in the treatment of normal pressure hydrocephalus and prevention of overdrainage / Efeito da válvula de pressão fixa com antisifão SPHERA®no tratamento da hidrocefalia de pressão normal e prevenção de hiperdrenagem

ABSTRACT Normal pressure hydrocephalus (NPH) is characterized by the triad of gait apraxia, dementia and urinary incontinence associated with ventriculomegaly and normal pressure of cerebrospinal fluid. Treatment is accomplished through the implantation of a ventricular shunt (VPS), however some complications are still frequent, like overdrainage due to siphon effect. This study analyses the performance of a valve with anti-siphon device (SPHERA®) in the treatment of patients with NPH and compares it with another group of patients with NPH who underwent the same procedure without anti-siphon mechanism (PS Medical® valve). 30 patients were consecutively enrolled in two groups with 15 patients each and followed clinically and radiologically for 1 year. Patients submitted to VPS with SPHERA® valve had the same clinical improvement as patients submitted to VPS with PS Medical®. However, complications and symptomatology due to overdrainage were significantly lower in SPHERA® group, suggesting it as a safe tool to treat NPH.

RESUMO A hidrocefalia de pressão normal (HPN) é caracterizada pela tríade de sintomas de apraxia de marcha, demência e incontinência urinária. O tratamento padrão é realizado através de implantação de derivação ventricular, porém várias complicações são frequentes, como a hiperdrenagem secundária ao efeito sifão. Este estudo avaliou o resultado da válvula SPHERA® no tratamento desses pacientes em comparação com um grupo controle (PS Medical®). 30 pacientes foram consecutivamente alocados em dois grupos de 15 e seguidos por 1 ano. Pacientes com a válvula SPHERA® tiveram o mesmo grau de melhora clínica em comparação ao grupo controle, no entanto as complicações diagnósticadas e sintomatologia secundária à hiperdrenagem foi significativamente inferior no grupo da válvula SPHERA® group, sugerindo-a como uma ferramenta segura e aplicável.