To assess automated volumetric analysis as a potential presurgical diagnostic tool or as a method to potentially shed light on normal pressure hydrocephalus (NPH) pathophysiology. MRI imaging according to our protocol was performed in 29 NPH patients, 45 non-NPH (but suspected) patients and 15 controls. Twenty patients underwent a second MRI 3 months after ventriculoperitoneal (VP) shunt surgery. All structures relevant to NPH diagnosis were automatically segmented using commercial software. The results were subsequently tested using ANOVA analysis. Significant differences in the volumes of the corpus callosum, left hippocampus, internal globus pallidus, grey and white matter and ventricular volumes were observed between NPH group and healthy controls. However, the differences between NPH and non-NPH groups were non-significant. Three months after, VP shunt insertion decreased ventricular volume was the only clearly significant result (p value 0.0001). Even though a detailed volumetric study shows several significant differences, volumetric analysis as a standalone method does not provide a simple diagnostic biomarker, nor does it shed a light on an unknown NPH aetiology.
- MeSH
- Cohort Studies MeSH
- Middle Aged MeSH
- Humans MeSH
- Magnetic Resonance Imaging MeSH
- Hydrocephalus, Normal Pressure diagnosis physiopathology surgery MeSH
- Aged MeSH
- Organ Size MeSH
- Ventriculoperitoneal Shunt MeSH
- Treatment Outcome MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
Normotenzní hydrocefalus (NPH) je chorobou osob typicky vyššího věku, která se projevuje rozšířením komorového systému mozku, tlakem likvoru ve fyziologických mezích a postupným rozvojem poruchy chůze, demence a inkontinence. Léčbu známe – provedení zkratové operace – ale existují nejasnosti v patofyziologii této nemoci a tím i v optimálním diagnostickém algoritmu. Jedním z hlavních problémů je nedostatečné zachycení nemocných NPH a většina pacientů tak zůstává bez diagnózy a bez adekvátní léčby. Cílem této přehledové práce je na NPH upozornit a shrnout aktuální poznatky v patofyziologii, klasifikaci a diagnostických funkčních testech.
Normal pressure hydrocephalus (NPH) is a disease of an elderly people. It is characterized by ventricular enlargement, normal pressure of the cerebrospinal fluid and progressive onset of gait disturbance, dementia and incontinency. The treatment is known (shunt surgery), but there are still controversies in the pathophysiology, thus in the optimal diagnostic algorithm. One of the crucial problems of NPH is underdiagnosing. The majority of NPH patients is not diagnosed and not treated properly. The aim of this review article is to draw attention to this disease and to summarize current knowledge about the pathophysiology, classification and supplementary tests.
- Keywords
- tap test, lumbální drenáž, lumbální infuzní test,
- MeSH
- Dementia etiology physiopathology MeSH
- Diagnostic Techniques, Neurological * MeSH
- Drainage MeSH
- Urinary Incontinence etiology physiopathology MeSH
- Intracranial Pressure physiology MeSH
- Cognition Disorders etiology physiopathology MeSH
- Humans MeSH
- Cerebrospinal Fluid physiology MeSH
- Gait Disorders, Neurologic etiology physiopathology MeSH
- Hydrocephalus, Normal Pressure * diagnosis physiopathology MeSH
- Spinal Puncture MeSH
- Cerebrospinal Fluid Pressure physiology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
Normal pressure hydrocephalus (NPH) is an important differential diagnosis of neurodegenerative diseases. The prevalence of dementia is increasing in line with the worldwide increase in life expectancy. NPH can be divided into idiopathic (iNPH) and secondary (sNPH) which is important in terms of clinical symptoms, future progress, and the outcome of possible treatment. The full clinical triad is not prevalent in all of the cases and the pathophysiology of iNPH remains unclear. Diagnosis is based on the evaluation of clinical symptoms (Hakim's triad) combined with an MRI assessment, evaluation of CSF dynamic parameters by different methods such as a tap test, lumbar infusion test (LIT), and external lumbar drainage (ELD). Despite the development of diagnostic techniques and strategies in management, NPH remains to be a challenge for the specialists despite more than 50 years of research. However, results of this research have brought new opportunities in the diagnosis, therapy, and quality of life as well as survival time of NPH patients with improved symptoms. The aim of this article is to present the pathophysiological hypotheses of NPH and an overview of the diagnostic techniques used for the evaluation of NPH patients.
- MeSH
- Humans MeSH
- Hydrocephalus, Normal Pressure diagnosis epidemiology physiopathology therapy MeSH
- Prevalence MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
- Review MeSH
Minimonografie se zabývá komplexním pohledem na normotenzní hydrocefalus (normal pressure hydrocephalus; NPH). NPH je klinickou jednotkou vznikající na podkladě alterace chování mozkomíšního moku uvnitř intrakraniálního prostoru. Klinická manifestace NPH zahrnuje typickou Adams-Hakimovu trias (porucha chůze, kognitivní deficit, močová inkontinence), přičemž zhruba polovina pacientů nemá tuto trias kompletní. Na MR je zobrazena ventrikulomegalie často s disproporcí velikosti subarachnoidálních prostor (zvětšené incisurae Sylvii, úzké prostory mezi sulky na konvexitách) bez známek obstrukce toku mozkomíšního moku. Z pohledu terapie jsou do jisté míry akceptovány i lehká kortikální atrofie či výskyt chronických ischemických změn. Základní součástí NPH je přítomnost normální velikosti tlaku mozkomíšního moku při lumbální punkci. S pomocí speciálních invazivních testů však lze alteraci chování moku uvnitř intrakraniálního prostoru s významnou přesností odhadovat (lumbální infuzní test, monitorování intrakraniálního tlaku). Výběr chirurgických kandidátů je ve většině případů prováděn na základě prediktivních testů (tap test, prodloužená lumbální drenáž). Patofyziologie nejčastější idiopatické formy NPH není po více než 50 letech od prvních poznatků dostatečně objasněna. Idiopatická forma NPH vzniká nejčastěji v pokročilém věku, a je tak významnou diferenciální diagnózou neurodegenerativních onemocnění. Tato diagnóza nabývá na důležitosti faktem, že je do značné míry ovlivnitelná chirurgickou léčbou v podobě implantace zkratu, který zajišťuje odtok mozkomíšního moku (ve většině případů ventrikulo-peritoneální zkrat).
The mini-monograph presents a comprehensive view on normal pressure hydrocephalus (NPH). NPH is a clinical diagnosis developed due to altered behavior of CSF within the intracranial space. The clinical manifestation of NPH includes the typical Adams-Hakim triad – gait disorder, cognitive deficit, urinary incontinence – with about half of the patients not having the complete triad. MRI shows ventriculomegaly, often with disproportion in the size of subarachnoid spaces (dilated Sylvian fissures, high convexity tightness) without an obstruction in CSF flow. From the point of therapy, mild cortical atrophy or the occurrence of chronic ischemic changes is to some extent accepted. The basic attribute of NPH is the presence of normal CSF pressure during lumbar puncture, but with the help of special invasive tests the alteration of CSF behavior within the intracranial space can be measured with a significant estimated accuracy (lumbar infusion test, intracranial pressure monitoring). Selection of surgical candidates is in most cases performed on the basis of predictive CSF diversion tests (tap test, extended lumbar drainage). The pathophysiology of the most common idiopathic form of NPH is only partially explained despite more than 50 years from the first findings. Idiopathic form of NPH typically arises at an advanced age, and thus it is an important differential diagnosis of neurodegenerative diseases. The importance of the diagnosis increases with the fact that the natural course of the disease could be to a large extent modified by surgical treatment in the form of implantation of a CSF shunt system a ventriculo-peritoneal shunt (in most cases).
Cytokine production and immune activation are associated with various pathological conditions including neurodegenerative disorders. One of them is multiple sclerosis (MS), known autoimmune disease. Inflammatory changes were also reported in normal pressure hydrocephalus (NPH), neurodegenerative disorder, which pathophysiology remains still unclear. The aim of this research was to compare the group of MS subjects with NPH patients and controls and to evaluate the potential inflammatory substance of NPH in comparison with autoimmune inflamed MS. METHODS: The levels of IL-1β, IL-4, IL-6, IL-10, IL-17A, IL-17F, IL-21, IL-22, IL-23, IL-25, IL-31, IL-33, INF-γ, sCD40L and TNF-α were measured in cerebrospinal fluid (CSF) and plasma in subjects with MS (n=15), NPH (n=18) and controls (n=11) by multiplex assay. RESULTS: The increased levels of IL-1β, IL-6, IL-10, IL-21 and TNF-α in cerebrospinal fluid of NPH subjects in comparison with MS patients and controls were found. Regarding the MS patients, we have confirmed increased IL-33 levels in cerebrospinal fluid and periphery as well as the increase of IL-1β and IL-10 in cerebrospinal fluid and IL-4 and sCD40L in plasma. CONCLUSION: The enlarged brain ventricles in NPH may repress and activate brain structures to the production of IL-1β, IL-6, IL-10, IL-21 and TNF-α, reflecting the inflammatory basis in NPH affected brain. The elevation of the above mentioned cytokines in MS was confirmed.
