Neurosarcoidosis as a rapidly progressive dementia associated with normal pressure hydrocephalus / Neurosarcoidosis como demencia rápidamente progresiva asociada a hidrocefalia normotensiva

Abstract Neurosarcoidosis (NS) is a rare subtype of sarcoidosis with a poor prognosis and diverse clinical presentations that often poses a diagnostic and therapeutic challenge. We describe the case of a 53-year-old male with an initial diagnosis of lingual sarcoidosis, who subsequently developed ataxia and rapidly progressive cognitive im pairment. A lumbar puncture revealed hypoglycorrha chia, hyperproteinorrachia, lymphocytic pleocytosis, and elevated IL-6 levels (600 pg/ml). Cerebrospinal fluid flow cytometry showed an elevated CD4 lymphocyte con centration and a CD4+/CD8+ ratio of 3.91, indicative of NS. Brain MRI showed hyperintense periventricular and subcortical lesions on FLAIR/T2 resembling progressive multifocal leukoencephalopathy (PML), although nega tive PCR for JC virus ruled out the differential diagnosis. Following a favorable evolutionary course with cortico steroid pulses, the patient relapsed with normotensive hydrocephalus, treated with immunosuppressants and ventriculoperitoneal shunting with a good response to date. This case underscores the importance of maintain ing a high index of suspicion for NS in individuals with sarcoidosis and neurologic symptoms. In these cases, ce rebrospinal fluid biomarkers such as IL-6 and CD4+/CD8+ ratio are essential to guide the diagnosis. Furthermore, it highlights that hydrocephalus is a rare complication and requires a multidisciplinary approach, including medical and neurosurgical treatment.

Resumen La neurosarcoidosis es un subtipo raro de sarcoidosis con mal pronóstico y diversas presentaciones clínicas que a menudo plantea un reto diagnóstico y terapéutico. Describimos el caso de un varón de 53 años con diagnóstico inicial de sarcoidosis lingual, que posterior mente desarrolló ataxia y deterioro cognitivo de rápida evolución. Una punción lumbar reveló hipoglucorraquia, hiperproteinorraquia, pleocitosis linfocítica y niveles elevados de IL-6 (600 pg/ml). La citometría de flujo del líquido cefalorraquídeo mostró una concentración ele vada de linfocitos CD4 y un cociente CD4+/CD8+ de 3.91, indicativo de neurosarcoidosis. La RM cerebral evidenció lesiones hiperintensas periventriculares y subcorticales en FLAIR/T2 que se asemejaban a una leucoencefalopa tía multifocal progresiva (LMP), aunque la PCR negativa para el virus JC descartó el diagnóstico diferencial. Tras un curso evolutivo favorable con pulsos de corticoides, el paciente recayó con hidrocefalia normotensiva, tratada con inmunosupresores y derivación ventriculoperitoneal con buena respuesta hasta la fecha. Este caso subraya la importancia de mantener un alto índice de sospecha de neurosarcoidosis en individuos con sarcoidosis y síntomas neurológicos. En estos casos, los biomarcadores del líquido cefalorraquídeo tales como la IL-6 y el cociente CD4+/CD8+ son esenciales para orientar el diagnóstico. Además, destaca que la hidro cefalia es una complicación poco frecuente y requiere un abordaje multidisciplinario, que incluya tratamiento médico y neuroquirúrgico.

Ten Steps for NPH Management: Advancements in Diagnosis and Treatment of Adult Hydrocephalus / Dez etapas para o manejo da NPH: avanços no diagnóstico e tratamento da hidrocefalia em adultos

Objectives The authors of the present study intend to describe a straightforward protocol for normal pressure hydrocephalus diagnosis and management, with the employment of a multidisciplinary team approach effort. Methods Using a strict methodological approach for initial diagnosis, taking into consideration occupational therapy and physical therapy assessment, the authors have set out to elaborate a simple protocol for suspicion and, once diagnosed, treatment of normal pressure hydrocephalus. We have used the MoCA (Montreal Cognitive Assessment) and walking assessment that included speed, independence, and distance (SID), 10 m walk test, TUG (timed up and go) evaluation, 6-minute Walk Test, MiniBESTest, as the main factors for pre and post lumbar drainage assessment, after which, the alternatives were deliberated and followed, or not, by ventriculoperitoneal shunt insertion. Results The authors have described a protocol, consisting of ten easy steps, which involves a multidisciplinary team, including occupational therapy and physical therapy professionals, as well as neurologists and neurosurgeons for improved and objective assessment prior to insertion of lumbar drain and, thereafter, detecting the population at most benefit for ventriculoperitoneal shunt insertion. We have described the Ten Step Approach for Normal Pressure Hydrocephalus management, including from initial clinical presentation and imaging, to pre and post lumbar drainage, for lastly deciding upon necessity for ventriculoperitoneal shunt insertion. Conclusions A straightforward protocol for normal pressure hydrocephalus seems not only feasible, but simple to implement in most neurosurgical departments, with good accuracy of prediction of lumbar drainage assessment to shunting outcomes.

PRE- AND POST- SHUNT CEREBRAL PERFUSION MEASURED BY DYNAMIC SUSCEPTIBILITY CONTRAST MRI IN PATIENTS WITH NORMAL PRESSURE HYDROCEPHALUS.

Background: NPH-related dementia is the only surgically manageable dementia. Response to ventricular shunting in NPH is variable. This study aims to establish a possible correlation between the changes in cerebral perfusion with clinical response to shunting. The study group consisted of 20 patients with NPH Methods: diagnoses who underwent ventricular shunting. Pre- and 3-month post- shunt DSC-MRI was done to assess the change in relative cerebral blood ?ow (rCBF). Mean ± SD values of rCBF in frontal grey matter, frontal periventricular white Results: matter, parietal periventricular white matter and hippocampus were 0.91 ± 0.11, 0.61 ± 0.09, 0.47 ± 0.11 and 0.93 ± 0.06 before shunt surgery and 0.98 ± 0.06, 0.70 ± 0.07, 0.54 ± 0.07 and 0.98 ± 0.06 after shunt surgery in NPH patients who responded to shunt surgery; and 0.78 ± 0.17, 0.37 ± 0.14, 0.41 ± 0.11 and 0.89 ± 0.10 before shunt surgery and 0.79 ± 0.14, 0.37 ± 0.19, 0.38 ± 0.09 and 0.84 ± 0.07 after shunt surgery in NPH patients who did not respond to shunt surgery. Therefore, regional rCBF in these regions increased in responders after shunt surgery, with a signi?cant statistical difference (p-value < 0.05) while no signi?cant change was seen in shunt non-responders (p-value > 0.05). There is a correlation between a signi?cant increase in Conclusion: regional rCBF and clinical improvement after shunt surgery

Balance and Stability for Patients with Idiopathic Normal Pressure Hydrocephalus / 中国康复理论与实践

@#Objective:To observe the features of posture control in patients with idiopathic normal pressure hydrocephalus (iNPH). Methods:From May, 2017 to May, 2018, patients with iNPH in our hospital (<italic>n</italic> = 13) and healthy controls (<italic>n</italic> = 15) were measured with sensory organization test on Balance Manager. The balance scores and strategy scores under six conditions were recorded and the comprehensive balance scores were calculated. Results:The balance scores decreased in the patients in the conditions of eyes-open/stable-support, eyes-close/stable-support, eyes-close/unstable-support and eyes-disturb/unstable-support compared with those of the controls (|<italic>Z</italic>| > 2.042, <italic>P</italic> < 0.05), as well as the comprehensive balance scores (<italic>Z</italic> = -3.617, <italic>P</italic> < 0.001); while the strategy scores increased in the conditions of eyes-close/stable-support, eyes-open/unstable-support, eyes-close/unstable-support and eyes-disturb/unstable-support (|<italic>Z</italic>| > 2.333, <italic>P</italic> < 0.05). Conclusion:There are balance disorders in the patients with iNPH, mainly associating with the disorders of proprioceptive and vestibular senses, and application of hip strategy.

Assessment of Relationship between In-hospital Rehabilitation Period after Cerebrospinal Shunt Surgery and Improvement of Gait Disturbance in Idiopathic Normal Pressure Hydrocephalus Patients with Severe Gait Disturbance / The Japanese Journal of Rehabilitation Medicine

Objective：Currently, there is no reliable evidence on the usefulness of rehabilitation for idiopathic, normal-pressure hydrocephalus (iNPH）patients after cerebrospinal shunt surgery. Therefore, in this study we investigated the association between the duration of rehabilitation and outcomes.Methods：We evaluated the changes before and after cerebrospinal shunt surgery and rehabilitation by applying the 3-m timed up-and-go test (TUG), 10-m straight walk test, 180-degree turn, 30-seconds chair-stand test (CS-30), mini-mental state examination (MMSE), and frontal assessment battery (FAB) in 81 patients with iNPH. Clinical outcomes were classified as excellent, good, or unsatisfactory.Results：At discharge, TUG, 180-degree turn, and CS-30 were significantly improved in 38 patients who had been in rehabilitation for ≥2 weeks, compared to those with ＜2 weeks-rehabilitation after shunt surgery. However, there was no significant difference between MMSE and FAB tests in the two groups. Rehabilitation for ≥2 weeks significantly improved the patient outcome, especially for those patients with severe gait disturbance before treatment. In 53 patients whose initial TUG time was ≥13.5 s, rehabilitation for ≥2 weeks was effective, and resulted in an excellent outcome (odds ratio：4.52, 95％ confidence interval：1.22-18.7, P value：0.012).Conclusion：In-hospital rehabilitation after cerebrospinal shunt surgery was useful for patients with iNPH and severe disability in daily activities. Therefore, we suggest that these patients require in-hospital rehabilitation after shunt surgery until sufficient improvement of gait disturbance is achieved, in order to maximize activities of daily living.

Assessment of Relationship between In-hospital Rehabilitation Period after Cerebrospinal Shunt Surgery and Improvement of Gait Disturbance in Idiopathic Normal Pressure Hydrocephalus Patients with Severe Gait Disturbance / The Japanese Journal of Rehabilitation Medicine

Objective：Currently, there is no reliable evidence on the usefulness of rehabilitation for idiopathic, normal-pressure hydrocephalus (iNPH）patients after cerebrospinal shunt surgery. Therefore, in this study we investigated the association between the duration of rehabilitation and outcomes.Methods：We evaluated the changes before and after cerebrospinal shunt surgery and rehabilitation by applying the 3-m timed up-and-go test (TUG), 10-m straight walk test, 180-degree turn, 30-seconds chair-stand test (CS-30), mini-mental state examination (MMSE), and frontal assessment battery (FAB) in 81 patients with iNPH. Clinical outcomes were classified as excellent, good, or unsatisfactory.Results：At discharge, TUG, 180-degree turn, and CS-30 were significantly improved in 38 patients who had been in rehabilitation for ≥2 weeks, compared to those with ＜2 weeks-rehabilitation after shunt surgery. However, there was no significant difference between MMSE and FAB tests in the two groups. Rehabilitation for ≥2 weeks significantly improved the patient outcome, especially for those patients with severe gait disturbance before treatment. In 53 patients whose initial TUG time was ≥13.5 s, rehabilitation for ≥2 weeks was effective, and resulted in an excellent outcome (odds ratio：4.52, 95％ confidence interval：1.22-18.7, P value：0.012).Conclusion：In-hospital rehabilitation after cerebrospinal shunt surgery was useful for patients with iNPH and severe disability in daily activities. Therefore, we suggest that these patients require in-hospital rehabilitation after shunt surgery until sufficient improvement of gait disturbance is achieved, in order to maximize activities of daily living.

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.

Neuropsychiatric manifestations in patient with normal pressure hydrocephalus improved with therapeutic lumbar tapping

Normal pressure hydrocephalus is a communicating hydrocephalus without evident obstruction of the normal pathway of CSF flow. Normal-pressure hydrocephalus is a common cause of reversible dementia and it can also present with various psychiatric symptoms. A 76-year old man was brought to psychiatry OPD with history suggestive of decreased need for sleep for 8 days, disinhibited behaviour, increased libido, increased activity and increased talk for 4 days. On examination patient was noticed to be having increased psychomotor activity with increased talk, mood reported to be happy with elated affect, no content or perceptual disturbances were elicited. Cognitive functions were within normal limits. Personal and social judgement was impaired with grade 0 insight. General physical and systematic examinations were within normal limits. MRI reports showed ventricular enlargement suggestive of normal pressure hydrocephalus. Patient underwent diagnostic and therapeutic lumbar tapping. There was significant improvement in patient’s behavioural symptoms following therapeutic lumbar tapping. Therapeutic lumbar tapping in this case of normal pressure hydrocephalus was effective in the management of manic symptoms. Early identification of organic cause in late onset psychiatric disorders is necessary. Prompt intervention of the organic cause was effective in the management of manic symptoms.

Different Outcomes in Two Cases of Idiopathic Normal Pressure Hydrocephalus in the Elderly Living Alone Diagnosed by Internists during Hospitalization / 日本プライマリ・ケア連合学会誌

Idiopathic normal pressure hydrocephalus (iNPH) is a disorder observed in elderly patients causing gait disturbance, urinary incontinence and cognitive impairment. Due to the rapidly aging society, the number of patients with iNPH continues to increase yearly. However, iNPH is often overlooked because of its insidious and variable presentation. In the primary care setting, iNPH can be coincidentally discovered by neuroimaging findings, including disproportionately enlarged subarachnoid space hydrocephalus (DESH). We report two cases of iNPH in the elderly living alone that had different outcomes depending upon the availability of neurosurgery specialists. It is essential for primary care physicians to consult neurosurgeons without delay because prompt diagnosis and treatment can improve the patient's quality of life and prolong their duration of living at home.

Cerebrospinal Fluid Levels of β-Amyloid 40 and β-Amyloid 42 are Proportionately Decreased in Amyloid Positron-Emission Tomography Negative Idiopathic Normal-Pressure Hydrocephalus Patients

BACKGROUND AND PURPOSE: Cerebrospinal fluid (CSF) biomarkers of Alzheimer's disease (AD) could be misleading in idiopathic normal-pressure hydrocephalus (iNPH). We therefore investigated the CSF biomarkers in 18F-florbetaben amyloid-negative positron-emission tomography (PET) [amyloid PET(−)] iNPH, amyloid-positive PET [amyloid PET(+)] AD, and cognitively normal (CN) subjects. METHODS: Ten amyloid PET(+) AD patients (56.7±5.6 years old, mean±standard deviation), 10 amyloid PET(−) iNPH patients (72.8±4.5 years old), and 8 CN subjects (61.2±6.5 years old) were included. We measured the levels of β-amyloid (Aβ)40, Aβ42, total tau (t-tau) protein, and phosphorylated tau (p-tau) protein in the CSF using enzyme-linked immunosorbent assays. RESULTS: The level of Aβ42 and the Aβ42/Aβ40 ratio in the CSF were significantly lower in AD than in iNPH or CN subjects. The Aβ40 level did not differ significantly between AD and iNPH (p=1.000), but it did between AD and CN subjects (p=0.032). The levels of both t-tau and p-tau were higher in AD than in iNPH or CN subjects. The levels of Aβ42, Aβ40, t-tau, and p-tau were lower in iNPH than in CN subjects, but there was no significant difference after controlling for age. CONCLUSIONS: Our results suggest that the mechanism underlying low CSF Aβ levels differs between amyloid PET(−) iNPH and amyloid PET(+) AD subjects. The lower levels of all CSF biomarkers in iNPH patients might be due to reduced clearances from extracellular fluid and decreased brain metabolism of the periventricular zone in iNPH resulting from glymphatic dysfunction.

Síndrome Neuropsicológico Frontal como Consecuencia de Hidrocefalia de Tensión Normal: estudio de caso / Frontal Neuropsychological Syndrome as a Consequence of Normal Tension Hydrocephalus: a case study

Resumen La manifestación clínica de la Hidrocefalia de Tensión Normal (NPH) implica pérdida del control de esfínter vesical, trastorno de la marcha y deterioro cognitivo. Este estudio buscó describir el estado neuropsicológico funcional de una paciente con NPH a través del análisis sindrómico, aplicando pruebas neuropsicológicas cualitativas y la evaluación de la actividad intelectual. Se evidenciaron dificultades y errores característicos del síndrome frontal severo con predominio de dificultades severas en la regulación y control de la actividad. Se observaron errores sistemáticos derivados de una falta de planeación, de selectividad y de autocrítica, en tareas perceptivas, verbales e intelectuales complejas. En el caso observado, el análisis neuropsicológico cualitativo apoya la afirmación de atrofia cortical con predominio frontal en esta enfermedad.

Abstract The clinical manifestation of Normal Pressure Hydrocephalus (NPH) implies loss of bladder sphincter control, gait disorder and cognitive impairment. The aim of this study is to describe the NPH patient's functional neuropsychological state through a syndromic analysis, applying qualitative neuropsychological tests and assessment of intellectual activity. It is evidenced difficulties and errors, which characterize severe syndrome with self-regulation and control of the activity. In the case of NPH, such syndrome is a consequence of organic frontal damage. Systematic errors derived from a lack of planning, selectivity and self-criticism were observed in complex perceptual, verbal and intellectual tasks. In this case, the qualitative neuropsychological analysis supports the affirmation of a cortical atrophy with frontal predominance in NPH.

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.

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.

Hydrocephalus in Patient with Multiple System Atrophy: Innocent Bystander or Guilty Party?

BACKGROUND: Normal pressure hydrocephalus (NPH) is a poorly understood condition, which typically presents with the triad of gait disturbance, urinary incontinence and cognitive decline. Diagnosis of NPH is often challenging due to its varied presentation and overlap with other neurodegenerative diseases including multiple system atrophy (MSA). CASE REPORT: A 68-year-old male developed rapidly progressive gait difficulty, urinary incontinence and memory impairment. Neurologic examination showed parkinsonism affecting the right side and impaired postural reflexes. Brain MRI showed enlargement of the ventricles and narrowing of the high convexity cerebrospinal fluid (CSF) spaces with relative dilated Sylvian fissure, the supporting features of NPH. 18F-fluorinated-N-3-fluoropropyl-2-b-carboxymethoxy-3-b-(4-iodophenyl) nortropane (¹⁸F-FP-CIT) PET showed decreased FP-CIT binding in the left posterior putamen and ¹⁸F-fluorodeoxyglucose PET showed decreased metabolism in the left basal ganglia, consistent with findings of MSA. CSF removal was performed and the symptoms were improved. The patient underwent ventriculo-peritoneal shunt and his gait and cognition improved. CONCLUSIONS: NPH is a potentially treatable neurological disorder. Therefore, it is necessary to consider the possibility of accompanying NPH when hydrocephalus is present in other neurodegenerative diseases.

Asymmetric and Upper Body Parkinsonism in Patients with Idiopathic Normal-Pressure Hydrocephalus

BACKGROUND AND PURPOSE: Our aims were to analyze the characteristics of parkinsonian features and to characterize changes in parkinsonian motor symptoms before and after the cerebrospinal fluid tap test (CSFTT) in idiopathic normal-pressure hydrocephalus (INPH) patients. METHODS: INPH subjects were selected in consecutive order from a prospectively enrolled INPH registry. Fifty-five INPH patients (37 males) having a positive response to the CSFTT constituted the final sample for analysis. The mean age was 73.7±4.7 years. The pre-tap mean Unified Parkinson's Disease Rating Scale motor (UPDRS-III) score was 24.5±10.2. RESULTS: There was no significant difference between the upper and lower body UPDRS-III scores (p=0.174). The parkinsonian signs were asymmetrical in 32 of 55 patients (58.2%). At baseline, the Timed Up and Go Test and 10-meter walking test scores were positively correlated with the total motor score, global bradykinesia score, global rigidity score, upper body score, lower body score, and postural instability/gait difficulties score of UPDRS-III. After the CSFTT, the total motor score, global bradykinesia score, upper body score, and lower body score of UPDRS-III significantly improved (p<0.01). There was a significant decrease in the number of patients with asymmetric parkinsonism (p<0.05). CONCLUSIONS: In the differential diagnosis of elderly patients presenting with asymmetric and upper body parkinsonism, we need to consider a diagnosis of INPH. The association between gait function and parkinsonism severity suggests the involvement of similar circuits producing gait and parkinsonian symptoms in INPH.

Neuroimaging in normal pressure hydrocephalus / Neuroimagem na hidrocefalia de pressão normal

ABSTRACT Normal pressure hydrocephalus (NPH) is a syndrome characterized by the triad of gait disturbance, mental deterioration and urinary incontinence, associated with ventriculomegaly and normal cerebrospinal fluid (CSF) pressure. The clinical presentation (triad) may be atypical or incomplete, or mimicked by other diseases, hence the need for supplementary tests, particularly to predict postsurgical outcome, such as CSF tap-tests and computed tomography (CT) or magnetic resonance imaging (MRI). The CSF tap-test, especially the 3 to 5 days continuous external lumbar drainage of at least 150 ml/day, is the only procedure that simulates the effect of definitive shunt surgery, with high sensitivity (50-100%) and high positive predictive value (80-100%). According to international guidelines, the following are CT or MRI signs decisive for NPH diagnosis and selection of shunt-responsive patients: ventricular enlargement disproportionate to cerebral atrophy (Evans index >0.3), and associated ballooning of frontal horns; periventricular hyperintensities; corpus callosum thinning and elevation, with callosal angle between 40º and 90º; widening of temporal horns not fully explained by hippocampal atrophy; and aqueductal or fourth ventricular flow void; enlarged Sylvian fissures and basal cistern, and narrowing of sulci and subarachnoid spaces over the high convexity and midline surface of the brain. On the other hand, other imaging methods such as radionuclide cisternography, SPECT, PET, and also DTI or resting-state functional MRI, although suitable for NPH diagnosis, do not yet provide improved accuracy for identifying shunt-responsive cases.

RESUMO. A hidrocefalia de pressão normal (HPN) é uma síndrome caracterizada por alteração da marcha, transtorno mental-cognitivo e incontinência urinária, associados a ventriculomegalia e pressão liquórica normal. A apresentação clínica (tríade) pode ser atípica ou incompleta, ou pode ser mimetizada por outras doenças, daí a necessidade de testes suplementares, principalmente para predição do resultado cirúrgico, tais como teste da punção lombar e tomografia computadorizada (TC) ou ressonância magnética (MR) de crânio. O teste da punção liquórica lombar, especialmente a drenagem externa contínua (≥150 ml/dia, por 3 a 5 dias), é o único método que simula o efeito da cirurgia, com alta sensibilidade (50-100%) e alto valor preditivo positivo (80-100%). Consensos internacionais consideram os seguintes achados da TC ou RM como decisivos para o diagnóstico de HPN e a seleção de pacientes bons respondedores à cirurgia: dilatação ventricular desproporcional em relação ao grau de atrofia cerebral (índice de Evans >0.3), associada a arredondamento dos cornos frontais; hipersinal difuso periventricular; adelgaçamento e elevação do corpo caloso, com ângulo do corpo caloso entre 40º e 90º; dilatação dos cornos temporais não explicada por atrofia hipocampal; sinal do fluxo vazio no aqueduto e quarto ventrículo; dilatação das fissuras Sylvianas e cisterna basal, e estreitamento ou apagamento dos sulcos e espaços subaracnoides nas superfícies cerebrais da convexidade alta e linha média. Por outro lado, a cisternografia isotópica, SPECT, PET, e mesmo técnicas mais modernas de RM funcional e tensor de difusão, embora compatíveis com o diagnóstico de HPN, não melhoram a acurácia na identificação de casos responsivos à cirurgia.

Evidences in the treatment of idiopathic normal pressure hydrocephalus / Evidências no tratamento da hidrocefalia de pressão normal idiopática.

Summary Introduction: idiopathic normal pressure hydrocephalus (INPH) is characterized by gait apraxia, cognitive dysfunction and urinary incontinence. There are two main treatment options: ventriculoperitoneal shunt (VPS) and endoscopic third ventriculostomy (ETV). However, there are doubts about which modality is superior and what type of valve should be applied. We are summarizing the current evidence in INPH treatment. Methods: an electronic search of the literature was conducted on the Medline, Embase, Scielo and Lilacs databases from 1966 to the present to obtain data published about INPH treatment. Results: the treatment is based on three pillars: conservative, ETV and VPS. The conservative option has fallen into disuse after various studies showing good results after surgical intervention. ETV is an acceptable mode of treatment, but the superiority of VPS has made the latter the gold standard. Conclusion: well-designed studies with a high level of appropriate evidence are still scarce, but the current gold standard for treatment of INPH is conducted using VPS. .

Resumo Introdução: a hidrocefalia de pressão normal idiopática (HPNI) é caracterizada por apraxia da marcha, disfunção cognitiva e incontinência urinária. Existem duas principais opções terapêuticas: derivação ventriculoperitoneal (DVP) e terceiro ventriculostomia endoscópica (TVE). No entanto, há dúvidas sobre qual modalidade é superior e que tipo de válvula deve ser aplicada. Este artigo resume as evidências atuais no tratamento de HPNI. Métodos: uma busca eletrônica da literatura foi realizada nas bases de dados Medline, Embase, SciELO e Lilacs, de 1966 até o momento presente para revelar os dados publicados sobre o tratamento da HPNI. Resultados: o tratamento é baseado em três pilares: conservador isolado, TVE e DVP. A opção conservadora caiu em desuso depois de vários estudos revelarem bons resultados após a intervenção cirúrgica. A TVE é uma modalidade de tratamento aceitável, mas a superioridade da DVP torna-a o padrão-ouro. Conclusão: estudos com evidência de alto nível, adequados e bem desenhados, ainda são escassos. O tratamento padrão-ouro atual de HPNI é realizado com DVP. .

Hiponatremia severa e hidrocefalia normotensiva del adulto: Reporte de un caso / Severe hyponatremia and normotensive hydrocephalus in the adult: Report of a case

Presentamos una paciente de 62 años de edad que ingresa por cefalea, alteraciones conductuales, síndrome tónico-frontal simétrico y síndrome cerebeloso de dos meses de evolución asociado a hiponatremia crónica severa. Se plantea el diagnóstico de síndrome de secreción inadecuada de hormona antidiurética (ADH) secundario a hidrocefalia normotensiva del adulto, presentando clara mejoría con la derivación ventrículo-peritoneal. Este caso pretende aportar información sobre una asociación descrita pero poco frecuente.

We present a 62-year-old patient presenting with a two-month episode of headaches, disrupted behavior, symmetric tonic frontal syndrome and cerebellar syndrome associated with severe chronic hyponaetremia. The diagnosis upon admission was an inadequate anti-diuretic hormone (ADH) secretion syndrome secondary to normotensive hydrocephalus of the adult, clearly improving after the ventricular-peritoneal shunt. This case provides information on a rarely reported association.

Analysis of Lumbar Puncture Test in 5 Patients with Good Prognosis of Shunt Operation for Normal Pressure Hydrocephalus

Five patients with favorable outcomes after a shunt operation in normal pressure hydrocephalus were analyzed with the aim of identifying consistent findings in a lumbar puncture (LP) test. The cases commonly showed improvement in at least one cognition and two gait LP parameters. We suggest that when judging the effects of LP on a shunt operation, the gait parameters need to be tailored to the gait status and the analyzed LP parameters should be evaluated at least twice at different times.

Idiopathic Normal Pressure Hydrocephalus Accompanied by Seizure: A Case Report.

The clinical features of idiopathic normal pressure hydrocephalus (iNPH) are characterized by cognitive impairments, gait disturbances, and/or urinary incontinence. These symptoms can also include seizures following a shunt placement but there is a lack of data regarding seizures as a presenting symptom in patients with iNPH. Thus, the present report describes a case of iNPH accompanied by seizures which resolved after the placement of a shunt.