Freezing of gait in idiopathic normal pressure hydrocephalus.

BACKGROUND: Reports of freezing of gait (FoG) in idiopathic normal pressure hydrocephalus (iNPH) are few and results are variable. This study's objective was to evaluate the frequency of FoG in a large cohort of iNPH patients, identify FoG-associated factors, and assess FoG's responsiveness to shunt surgery. METHODS: Videotaped standardized gait protocols with iNPH patients pre- and post-shunt surgery (n = 139; median age 75 (71-79) years; 48 women) were evaluated for FoG episodes by two observers (Cohens kappa = 0.9, p < 0.001). FoG episodes were categorized. Mini-mental state examination (MMSE) and MRI white matter hyperintensities (WMH) assessment using the Fazekas scale were performed. CSF was analyzed for Beta-amyloid, Tau, and Phospho-tau. Patients with and without FoG were compared. RESULTS: Twenty-two patients (16%) displayed FoG at baseline, decreasing to seven (8%) after CSF shunt surgery (p = 0.039). The symptom was most frequently exhibited during turning (n = 16, 73%). Patients displaying FoG were older (77.5 vs. 74.6 years; p = 0.029), had a slower walking speed (0.59 vs. 0.89 m/s; p < 0.001), a lower Tinetti POMA score (6.8 vs. 10.8; p < 0.001), lower MMSE score (21.3 vs. 24.0; p = 0.031), and longer disease duration (4.2 vs. 2.3 years; p < 0.001) compared to patients not displaying FoG. WMH or CSF biomarkers did not differ between the groups. CONCLUSIONS: FoG is occurring frequently in iNPH patients and may be considered a typical feature of iNPH. FoG in iNPH was associated with higher age, longer disease duration, worse cognitive function, and a more unstable gait. Shunt surgery seems to improve the symptom.

From disease to noninvasive intracranial monitoring / Da doença ao monitoramento intracraniano não invasivo

ABSTRACT Professor Sérgio Mascarenhas was a Brazilian researcher with a vast legacy. His work paved the way for new research possibilities by consolidating the use of innovation and transdisciplinary science. In Medicine, he proposed changes to what had previously been well-accepted concepts, and his contributions have influenced medical practices. Although many authors consider intracranial pressure (ICP) as an unrivaled variable for monitoring and diagnosis of many diseases, its clinical applicability is still the subject of debate in the literature because of the difficulty in standardizing protocols. Mascarenhas's research and the creation of a device for noninvasive monitoring of intracranial compliance are discussed and are shown to have led to the creation of Brain4care, a start-up, and a new perspective on the debate on ICP monitoring.

RESUMO O professor Sérgio Mascarenhas foi um pesquisador com vasto legado. Seu trabalho abriu caminho para novas possibilidades de pesquisa, consolidando o uso da inovação e da ciência transdisciplinar. Na Medicina, ele propôs mudanças no que antes eram conceitos bem-aceitos, e suas contribuições influenciaram a prática médica. Embora muitos autores considerem a pressão intracraniana (PIC) uma variável incomparável para o monitoramento e o diagnóstico de uma série de doenças, sua aplicabilidade clínica ainda é motivo de debate na literatura pela dificuldade de padronização de protocolos. A pesquisa do Professor Mascarenhas e a criação de um dispositivo para o monitoramento não invasivo da complacência intracraniana levaram à criação do Brain4care, uma start-up, e a uma nova perspectiva sobre o debate do monitoramento da PIC.

Normal pressure hydrocephalus: an update / Hidrocefalia de pressão normal: uma atualização

ABSTRACT Normal pressure hydrocephalus (NPH) has been a topic of debate since its introduction in publications. More frequent in the elderly population, it is characterized by gait disturbance, urinary urge incontinence and cognitive decline. Therefore, it is a clinical-radiological entity with relatively common findings for the age group, which together may have greater specificity. Therefore, its diagnosis must be careful for an adequate selection of patients for treatment with ventricular shunt, since the symptoms are potentially reversible. The tap test has a high positive predictive value as a predictor of therapeutic response, but a negative test does not exclude the possibility of treatment. Scientific efforts in recent years have been directed towards a better understanding of NPH and this narrative review aims to compile recent data from the literature in a didactic way for clinical practice.

RESUMO A hidrocefalia de pressão normal (HPN) é tema de debate desde sua introdução na literatura. Mais frequente na população idosa, caracteriza-se por distúrbio de marcha, urge-incontinência urinária e declínio cognitivo. Portanto, trata-se de uma entidade clínico-radiológica com achados relativamente comuns para a faixa etária, que em conjunto, podem ter maior especificidade. Sendo assim, seu diagnóstico deve ser criterioso para uma adequada seleção de pacientes para tratamento com a derivação ventricular, uma vez que os sintomas são potencialmente reversíveis. O tap test possui valor preditivo positivo alto preditor de resposta terapêutica, mas um teste negativo não exclui a possibilidade de tratamento. Esforços científicos nos últimos anos têm sido direcionados para melhor entendimento da HPN e essa revisão narrativa se propõe a compilar dados recentes da literatura de forma didática para a prática clínica.

Does Impaired Glymphatic Drainage Cause Glymphedema? A Review Tailored to Neurocritical Care and Neurosurgery.

Research into the glymphatic system reached an inflection point with steep trajectory in 2012 when it was formally recognized and named, but the historical roots for it are solid and deep, dating back to pioneers such as Cushing, Weed, and Dandy. We provide an overview of key discoveries of the glymphatic system, which promotes bulk flow of fluid and solutes throughout the brain parenchyma. We also discuss the lymphatic drainage of the central nervous system. Evidence is building that failure of the glymphatic system causes glymphedema in patients commonly managed by neurocritical care and neurosurgery specialists. We review research supporting this for decompressive craniectomy, subarachnoid hemorrhage, and normal-pressure hydrocephalus. We argue that it is time for a paradigm shift from the traditional model of cerebrospinal fluid circulation to a revised model that incorporates the glymphatic pathway and lymphatic clearance. These recent breakthroughs will inspire new therapeutic approaches to recognize, reverse, and restore glymphatic dysfunction and to leverage this pathway to deliver brain-wide therapeutics.

The splenial angle: a novel radiological index for idiopathic normal pressure hydrocephalus.

OBJECTIVES: To evaluate the utility of the splenial angle (SA), an axial angular index of lateral ventriculomegaly measured on diffusion tensor MRI color fractional anisotropy maps, in differentiating NPH from Alzheimer's disease (AD), Parkinson's disease (PD), and healthy controls (HC), and post-shunt changes in NPH, compared to Evans' index and callosal angle. METHODS: Evans' index, callosal angle, and SA were measured on brain MRI of 76 subjects comprising equal numbers of age- and sex-matched subjects from each cohort of NPH, AD, PD, and HC by two raters. Receiver operating characteristics (ROC) and multivariable analysis were used to assess the screening performance of each measure in differentiating and predicting NPH from non-NPH groups respectively. Temporal changes in the measures on 1-year follow-up MRI in 11 NPH patients (with or without ventriculoperitoneal shunting) were also assessed. RESULTS: Inter-rater and intra-rater reliability were excellent for all measurements (intraclass correlation coefficients > 0.9). Pairwise comparison showed that SA was statistically different between NPH and AD/PD/HC subjects (p < 0.0001). SA performed the best in predicting NPH, with an area under the ROC curve of > 0.98, and was the only measure left in the final model of the multivariable analysis. Significant (p < 0.01) change in SA was seen at follow-up MRI of NPH patients who were shunted compared to those who were not. CONCLUSIONS: The SA is readily measured on axial DTI color FA maps compared to the callosal angle and shows superior performance differentiating NPH from neurodegenerative disorders and sensitivity to ventricular changes in NPH after surgical intervention. KEY POINTS: • The splenial angle is a novel simple angular radiological index proposed for idiopathic normal pressure hydrocephalus, measured in the ubiquitous axial plane on DTI color fractional anisotropy maps. • The splenial angle quantitates the compression and stretching of the posterior callosal commissural fibers alongside the distended lateral ventricles in idiopathic normal pressure hydrocephalus (NPH) using tools readily accessible in clinical practice and shows excellent test-retest reliability. • Splenial angle outperforms Evans' index and callosal angle in predicting NPH from healthy, Parkinson's disease, and Alzheimer's disease subjects on ROC analysis with an area under the curve of > 0.98 and is sensitive to morphological ventricular changes in NPH patients after ventricular shunting.

Diagnostic performance and interobserver agreement of the callosal angle and Evans' index in idiopathic normal pressure hydrocephalus: a systematic review and meta-analysis.

OBJECTIVE: To evaluate the diagnostic performance and interobserver agreement of the callosal angle and Evans' index in idiopathic normal pressure hydrocephalus (iNPH). METHODS: A systematic search of MEDLINE and EMBASE was performed to find studies assessing the diagnostic performance or interobserver agreement of the callosal angle and Evans' index in iNPH. Pooled sensitivity and specificity of the two radiologic indices were calculated. The area under the curve (AUC) was obtained based on a hierarchical summary receiver operating characteristic curve. The diagnostic performances of both radiologic indices were compared in subgroup analysis. To evaluate interobserver agreement, the pooled correlation coefficient was calculated. RESULTS: Ten original articles (874 patients) were included. The pooled sensitivity and specificity of the callosal angle in the diagnosis of iNPH were 91% (95% CI, 86-94%) and 93% (95% CI, 89-96%), respectively. The pooled sensitivity and specificity of Evans' index were 96% (95% CI, 47-100%) and 83% (95% CI, 77-88%), respectively. Subgroup analysis demonstrated a significant higher specificity of the callosal angle than that of Evans' index (p < 0.01). The AUC of the callosal angle and Evans' index were 0.97 (95% CI, 0.95-0.98) and 0.87 (95% CI, 0.84-0.90), respectively. The pooled correlation coefficients for the callosal angle and Evans' index were 0.92 (95% CI, 0.82-0.96) and 0.92 (95% CI, 0.83-0.97), respectively. CONCLUSIONS: Our meta-analysis demonstrated a high performance of the callosal angle in the diagnosis of iNPH. Evans' index showed reasonable diagnostic performance with high sensitivity but low specificity. Interobserver agreements were excellent in both radiologic indices. KEY POINTS: • Callosal angle showed high diagnostic performance in idiopathic normal pressure hydrocephalus. • Evans' index showed reasonable diagnostic performance with high sensitivity but low specificity. • Interobserver agreements were excellent in both callosal angle and Evans' index.

Laparoscopic-Assisted Versus Mini-Open Laparotomy for Ventriculoperitoneal Shunt Placement in the Medicare Population.

BACKGROUND: Placement of the distal shunt catheter into the peritoneum during ventriculoperitoneal shunt (VPS) surgery can be done with either laparoscopic assistance or laparotomy. OBJECTIVE: To compare outcomes in laparoscopic-assisted vs laparotomy for placement of VPS in the Medicare population. METHODS: Patients undergoing VPS placement, between 2004 and 2014, were identified by International Classification of Disease, Ninth Revision and Current Procedural Terminology codes in the Medicare database. Demographic data including age, sex, comorbidities, and indications were collected. Six- and twelve-month complication rates were analyzed. RESULTS: A total of 1966 (3.2%) patients underwent laparoscopic-assisted VPS and 60 030 (96.8%) patients underwent nonlaparoscopic-assisted VPS placement. Compared with traditional open VPS placement, the laparoscopic approach was associated with decreased odds of distal revision at 6- and 12-mo postoperatively (6 mo: odds ratio [OR] = 0.41, 95% confidence interval [CI]: 0.21-0.74; 12 mo: OR = 0.60, 95% CI: 0.39-0.94). At 6- and 12-mo postoperatively, multivariable regression analysis demonstrated increased odds of distal revision in patients with a body mass index (BMI) > 30 Kg/M2, history of open abdominal surgery, and history of laparoscopic abdominal surgery. Additionally, history of prior abdominal surgery and BMI > 30 Kg/M2 were significantly associated with increase odds of shunt infection at 6 and 12-mo, respectively. CONCLUSION: In the largest retrospective analysis to date, patients with a history of abdominal surgery and obesity were found to be at increased risk of infection and distal revision after VPS placement. However, the laparoscopic approach for abdominal placement of the distal catheter was associated with reduced rates of distal revision in this population, suggesting an avenue for reducing complications in well-selected patients.

Clinical observation of Metazolamide in the treatment of normal pressure hydrocephalus / 中华老年医学杂志

Objective:To study the efficacy and safety of Methazolamide(MTZ)for the treatment of normal pressure hydrocephalus(NPH)patients.Methods:A randomized, double-blind, placebo-controlled, prospective clinical study was conducted in Aviation General Hospital.A total of 35 NPH patients including 29 idiopathic normal pressure hydrocephalus(iNPH)and 6 secondary normal pressure hydrocephalus(sNPH)received drug treatment in our hospital from September 2019 to March 2021.All patients were unsuitable for or refused surgical treatment for some reasons.The patients were divided into drug group(n=18)and control group(n=10), taking oral MTZ or placebo 25 mg twice daily, increasing to 50 mg twice daily after 1 week if there was no discomfort.The 10 m gait score, cognitive function score, brain MRI check were completed before and 1 month after oral administration.The assessment of idiopathic normal pressure hydrocephalus scale(iNPHGS)score were performed 1 month and 3 months after oral administration.The primary efficacy endpoint was iNPHGS score for 3 months treatment and the secondary efficacy endpoint was the assessment of above scales for 1 month treatment.Results:As compared with baseline, the effect of 1 month treatment showed that MOCA scores[(16.2±8.8)and(14.8±8.7)scores, t=-2.68, P=0.02], 10 m gait scores[(22.3±11.2)and(25.6±12.9), t=2.76, P=0.02], iNPHGS scores[(7.3±3.2)and(8.1±3.5), t=4.08, P<0.01]were improved.The effect of 3 month treatment showed that the iNPHGS score(6.1±2.4)was improved compared with baseline( t=5.07, P<0.01)and 1 month( t=4.11, P<0.01). But the above scores of the control group were not significantly improved compared with the baseline(all P>0.05). After 1 month treatment, the 10 m gait score and iNPHGS score in the drug group were improved compared with those in the control group(all P<0.05). After 3 months treatment, the iNPHGS score was improved compared with the baseline level in the control group( t=-4.41, P<0.05). The above 35 patients had no serious adverse reactions such as hypokalemia and acidosis.There was no significant difference in adverse events between the two groups( χ2=0.01, P=1.00). Conclusions:The treatment of MTZ could effectively improve the clinical symptoms of NPH patients with good safety.

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.

Relationships between intracranial compartment volumes and clinical symptoms in patients with idiopathic normal pressure hydrocephalus before and after cerebrospinal fluid shunt surgery / 中华老年医学杂志

Objective To investigate the relationships of intracranial compartment volumes with the severity of clinical symptoms before surgery,and the degree of symptom improvement one year after cerebrospinal fluid(CSF)shunt surgery in patients with idiopathic normal pressure hydrocephalus (iNPH).Methods Twenty-one patients meeting the diagnosis criteria of international guidelines of iNPH and undergoing CSF shunt surgery in Department of Neurosurgery in our hospital from 2016 to 2017 were included.All patients underwent brain MRI measurement before surgery,and were evaluated by using 3-meter timed up and go test(TUG),minimum mental state examination(MMSE),idiopathic normal pressure hydrocephalus grading scale (iNPHGS) and modified Rankin scale (mRS) before and one year after CSF shunt procedures.The ventricular volume,brain volume,pericerebral CSF volume,total intracranial volume and Evans' index were measured in the pre-operative imaging of the brain.The following four pre-operative intracranial compartment volumes were calculated:the relative ventricular volume,brain volume ratio,pericerebral CSF volume ratio and the ratio of ventricular volume to pericerebral CSF volume.Results The scores of gait,cognitive function and urinary function were improved after surgery in iNPH patients (all P ＜ 0.05).There were no significant difference in the correlation of intracranial compartment volumes(the relative ventricular volume,brain volume ratio,pericerebral CSF volume ratio and the ratio of ventricular volume to pericerebral CSF volume)and Evans' index with the severity of clinical symptoms including gait,cognitive function and urinary function before surgery,and with the degree of symptom improvement one year after surgery in iNPH patients (all P ＞ 0.05).There was no significant difference in intracranial compartment volumes between patients having improvement in mRS,TUG,MMSE and iNPHGS and patients having no improvement one year after surgery in iNPH patients(all P＞0.05).Conclusions Patients with iNPH can benefit from CSF shunt surgery and have improvements of clinical symptoms including gait,cognitive function and urinary function.Preoperative intracranial compartment volumes(the relative ventricular volume,brain volume ratio,pericerebral CSF volume ratio and the ratio of ventricular volume to pericerebral CSF volume)and Evans' index have no correlations with the severity of clinical symptoms before surgery,and have no correlations with the degree of symptom improvement one year after surgery in iNPH patients.For this reason,preoperative intracranial compartment volumes and Evans' index cannot be used to predict whether or not CSF shunt surgery can improve specific clinical symptoms.

Relationships between intracranial compartment volumes and clinical symptoms in patients with idiopathic normal pressure hydrocephalus before and after cerebrospinal fluid shunt surgery / 中华老年医学杂志

Objective@#To investigate the relationships of intracranial compartment volumes with the severity of clinical symptoms before surgery, and the degree of symptom improvement one year after cerebrospinal fluid(CSF)shunt surgery in patients with idiopathic normal pressure hydrocephalus(iNPH).@*Methods@#Twenty-one patients meeting the diagnosis criteria of international guidelines of iNPH and undergoing CSF shunt surgery in Department of Neurosurgery in our hospital from 2016 to 2017 were included.All patients underwent brain MRI measurement before surgery, and were evaluated by using 3-meter timed up and go test(TUG), minimum mental state examination(MMSE), idiopathic normal pressure hydrocephalus grading scale(iNPHGS)and modified Rankin scale(mRS)before and one year after CSF shunt procedures.The ventricular volume, brain volume, pericerebral CSF volume, total intracranial volume and Evans' index were measured in the pre-operative imaging of the brain.The following four pre-operative intracranial compartment volumes were calculated: the relative ventricular volume, brain volume ratio, pericerebral CSF volume ratio and the ratio of ventricular volume to pericerebral CSF volume.@*Results@#The scores of gait, cognitive function and urinary function were improved after surgery in iNPH patients(all P<0.05). There were no significant difference in the correlation of intracranial compartment volumes(the relative ventricular volume, brain volume ratio, pericerebral CSF volume ratio and the ratio of ventricular volume to pericerebral CSF volume)and Evans' index with the severity of clinical symptoms including gait, cognitive function and urinary function before surgery, and with the degree of symptom improvement one year after surgery in iNPH patients(all P>0.05). There was no significant difference in intracranial compartment volumes between patients having improvement in mRS, TUG, MMSE and iNPHGS and patients having no improvement one year after surgery in iNPH patients(all P>0.05).@*Conclusions@#Patients with iNPH can benefit from CSF shunt surgery and have improvements of clinical symptoms including gait, cognitive function and urinary function.Preoperative intracranial compartment volumes(the relative ventricular volume, brain volume ratio, pericerebral CSF volume ratio and the ratio of ventricular volume to pericerebral CSF volume)and Evans' index have no correlations with the severity of clinical symptoms before surgery, and have no correlations with the degree of symptom improvement one year after surgery in iNPH patients.For this reason, preoperative intracranial compartment volumes and Evans' index cannot be used to predict whether or not CSF shunt surgery can improve specific clinical symptoms.

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.

Standardized image evaluation in patients with idiopathic normal pressure hydrocephalus: consistency and reproducibility.

PURPOSE: Assess the agreement for two investigators between computed tomography (CT) and magnetic resonance imaging (MRI) for seven imaging features included in the iNPH Radscale, a radiological screening tool. METHODS: The study included 35 patients with idiopathic normal pressure hydrocephalus (iNPH) who were treated surgically from 2011 to 2015 at Uppsala University Hospital with preoperative CT and MRI performed with maximum 3 months between scans. Seven features were assessed: Evans' index, temporal horn size, callosal angle, periventricular white matter changes, narrow high convexity sulci, focally enlarged sulci, and enlarged Sylvian fissures. All scans were assessed by two investigators who were blinded to each other's results and to clinical data. RESULTS: The agreement between CT and MRI was almost perfect for Evans' index, temporal horns, narrow sulci, and Sylvian fissures (kappa and intraclass correlation, 0.84-0.91, p ≤ 0.001). There was substantial to almost perfect agreement for callosal angle and focally enlarged sulci. The concordance between modalities was fair for changes in periventricular white matter. CONCLUSION: CT and MRI are equally good for assessing radiological signs associated with iNPH except for periventricular white matter changes, as MRI has superior soft tissue contrast. The other imaging features can be evaluated consistently, and assessments are reproducible independent of modality. Therefore, the iNPH Radscale is applicable to both CT and MRI and may become an important tool for standardized evaluation in the workup in patients with suspected iNPH.

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.

Changes in sensorimotor-related thalamic diffusion properties and cerebrospinal fluid hydrodynamics predict gait responses to tap test in idiopathic normal-pressure hydrocephalus.

OBJECTIVES: To compare diffusion tensor (DT)-derived indices from the thalamic nuclei and cerebrospinal fluid (CSF) hydrodynamic parameters for the prediction of gait responsiveness to the CSF tap test in early iNPH patients. METHODS: In this study, 22 patients with iNPH and 16 normal controls were enrolled with the approval of an institutional review board. DT imaging and phase-contrast magnetic resonance imaging were performed in patients and controls to determine DT-related indices of the sensorimotor-related thalamic nuclei and CSF hydrodynamics. Gait performance was assessed in patients using gait scale before and after the tap test. The Mann-Whitney U test and receiver operating characteristic (ROC) curve analysis were applied to compare group differences between patients and controls and assess the predictive performance of gait responsiveness to the tap test in the patients. RESULTS: Fractional anisotropy (FA) and axial diffusivity showed significant increases in the ventrolateral (VL) and ventroposterolateral (VPL) nuclei of the iNPH group compared with those of the control group (p < 0.05). The predictions of gait responsiveness of ventral thalamic FA alone (area under the ROC curve [AUC] < 0.8) significantly outperformed those of CSF hydrodynamics alone (AUC < 0.6). The AUC curve was elevated to 0.812 when the CSF peak systolic velocity and FA value were combined for the VPL nucleus, yielding the highest sensitivity (0.769) and specificity (0.778) to predict gait responses. CONCLUSIONS: Combined measurements of sensorimotor-related thalamic FA and CSF hydrodynamics can provide potential biomarkers for gait response to the CSF tap test in patients with iNPH. KEY POINTS: • Ventrolateral and ventroposterolateral thalamic FA may predict gait responsiveness to tap test. • Thalamic neuroplasticity can be assessed through DTI in idiopathic normal-pressure hydrocephalus. • Changes in the CST associated with gait control could trigger thalamic neuroplasticity. • Activities of sensorimotor-related circuits could alter in patients with gait disturbance. • Management of patients with iNPH could be more appropriate.

[Neurodegenerative diseases]. / Neurodegenerative Erkrankungen.

Neurodegenerative diseases are sporadic and rare hereditary disorders of the central nervous system, which cause a slowly progressive loss of function of specific neuron populations and their connections. Severe impairments and care dependency can be the sequelae. Neurodegenerative disorders are diseases of older people; therefore, the demographic shift leads to an increase in the number of affected patients. Radiologists will also become more involved. For this reason important neurodegenerative diseases are presented in this article. In addition to Alzheimer's and Parkinson's diseases these also include frontotemporal lobar degeneration, Lewy body dementia, vascular dementia, Creutzfeldt-Jakob disease and Huntington's chorea. The clinical symptoms and diagnostics are described, whereby the focus lies on typical results of morphological imaging.