Idiopathic Normal Pressure Hydrocephalus: An Overview of Pathophysiology, Clinical Features, Diagnosis and Treatment (original) (raw)
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DEMENTIA DUE TO NORMAL PRESSURE HYDROCEPHALUS – LITERATURE REVIEW (Atena Editora)
DEMENTIA DUE TO NORMAL PRESSURE HYDROCEPHALUS – LITERATURE REVIEW (Atena Editora), 2023
Introduction: Hydrocephalus is a neurological condition with several causes, where cerebrospinal fluid accumulates abnormally, resulting in serious health risks and high treatment costs. Objective: To review the relationship between normal pressure hydrocephalus and dementia. Result: This condition is classified as a form of dementia and its prevalence increases with age. This occurs mainly due to problems in the circulation of cerebrospinal fluid (CSF), without a significant increase in intracranial pressure. Typical clinical symptoms include walking difficulties, urinary incontinence and dementia, known as Hakim's Triad (PASSOS-NETO CEB, et al., 2022). Conclusion: the disease can be diagnosed prenatally or in neonates using USG, and in older children and adults using CT or MRI (PASSOS-NETO CEB, et al., 2022).
Movement Disorders Clinical Practice, 2016
Background: Idiopathic normal pressure hydrocephalus (INPH) is a treatable cause of gait disturbance, cognitive impairment, and urinary incontinence. This clinical triad of symptoms occurs in association with ventriculomegaly and normal cerebrospinal fluid (CSF) pressure. Although the treatment outcomes after CSF shunting for INPH have improved significantly since its first description in 1965, shortcomings in our understanding still remain. Not all INPH patients exhibit clinical improvement after shunting, and it is challenging to identify patients who are more likely to benefit from shunting. Methods: The Cochrane Library, Medline, Embase, and PubMed databases were searched for Englishlanguage publications between 1965 and October 2015. Reference lists of publications were also manually searched for additional publications. Results: The findings of this review indicate that, despite efforts to improve patient selection, the degree of clinical improvement after shunting continues to demonstrate significant variability both within and between studies. These discrepancies in treatment outcomes are the result of controversies in 3 distinct but interrelated domains: the underlying pathophysiology of INPH, the diagnosis of INPH, and the identification of likely shunt-responders. Conclusions: This review focuses on these 3 areas and their relation to surgical treatment outcomes. Despite the limitations of published outcome studies and limitations in our understanding of INPH pathophysiology, shunting is a safe and effective means of achieving meaningful clinical improvement in most patients with INPH. Normal pressure hydrocephalus (NPH) was first reported in 1965 as a triad of dementia, gait disturbance, and urinary incontinence, with associated ventricular dilatation and normal cerebrospinal fluid (CSF) pressure. 1 NPH can be classified as idiopathic NPH (INPH) or secondary NPH (SNPH), the latter of which occurs most commonly after subarachnoid hemorrhage, trauma, or meningitis. 2 Although CSF diversion via a shunt is the mainstay of NPH management, surgical outcomes in INPH have been consistently less successful than those in SNPH. 3-5 In addition, the reported rates of postoperative clinical improvement in INPH patients have varied from 24% to 96%. 6,7 These discrepancies in treatment outcomes are the result of controversies in 3 domains: the underlying pathophysiology of INPH, the diagnosis of INPH, and the identification of likely shunt-responders. This review focuses on these 3 areas of controversy and their relation to surgical outcomes. The Cochrane Library, Medline, Embase, and PubMed were searched for English-language publications between 1965 and October 2015 using a combination of medical subject headings and free text key words such as: "normal pressure hydrocephalus," "treatment," "outcome," and "cerebrospinal fluid shunt." Reference lists of publications
The Typical Triad of Idiopathic Normal Pressure Hydrocephalus in a 62-Year-Old Male
The Typical Triad of Idiopathic Normal Pressure Hydrocephalus in a 62-Year-Old Male, 2021
Normal pressure hydrocephalus (NPH) is a rare pathological condition of the brain in which the ventricles are enlarged due to cerebrospinal fluid accumulation and is associated with normal opening pressure on lumbar puncture with a large-volume cerebrospinal fluid (CSF) tap. This results in three classical symptoms: mental impairment, gait disturbance, and urinary or fecal incontinence. We present a case of idiopathic NPH in which a 64-year-old retired man with diabetes was brought to the emergency department after recurrent previous falls. The patient complained of an unsteady gait and presented with the typical triad of NPH which is mental impairment, gait disturbance, and incontinence. The patient was a known diabetic, and his gait was characterized by shuffling, bradykinesia, and mild drifting toward the right side. A head computed tomography scan revealed brain tissue volume loss, with disproportionate dilation of the lateral and third ventricles. A lumbar puncture with a large-volume CSF tapping produced normal opening pressure (18 mmHg); thus, the diagnosis of NPH was made. The patient underwent shunt surgery, and his balance and memory improved significantly after the procedure. Also, no event of fecal incontinence occurred. NPH resembles several neurodegenerative disorders. Due to this, it can be difficult to diagnose. Emergency physicians, as frontline healthcare providers, may encounter such cases.NPH should be considered in patients presenting with an unsteady gait, memory impairment, and urinary or fecal incontinence by taking a detailed history and conducting a physical examination to prevent future complications.
Profile of cognitive dysfunction and relation with gait disturbance in Normal Pressure Hydrocephalus
Clinical Neurology and Neurosurgery, 2014
Objectives: Although dementia is one of the most relevant symptoms of the idiopathic Normal-Pressure Hydrocephalus (iNPH) syndrome, some doubts remain about the nature of cognitive deficits in this disease. We aimed to determine the neuropsychological profile in iNPH and its relation with ventricular size, white matter vascular lesions (WML) and gait dysfunction. Methods: Seventeen iNPH patients and a control group (n = 14) were assessed with a battery of neuropsychological tests and a timed walk test. We calculated measures of frontal horn, occipital horn and third ventricle sizes and assessed white matter lesion (WML) load with a validated visual scale. Results: Patients differed significantly from controls in all cognitive tests, but did worse on the Rey Complex Figure test. We found no significant correlations between cognitive and imaging results in iNPH. Cognitive function was related to gait in controls, but not in iNPH patients. Conclusions: Patients presented widespread cognitive dysfunction with a predominance of visuo-spatial deficits. Dissociation between gait and cognitive dysfunction in iNPH patients suggests the existence of different pathophysiological mechanisms.
Pathogenesis of idiopathic Normal Pressure Hydrocephalus: A review of knowledge
Journal of Clinical Neuroscience, 2018
Idiopathic Normal Pressure Hydrocephalus (iNPH) is a frequent neuropsychiatric entity. Clinically it is characterised by Hakim's triad: Dementia, gait disturbance and urinary incontinence. While its symptomatology is typical, the etiology and thereby physiopathology of iNPH still remain enigmatic. This review summarizes and synthesizes different etiologic conceptions and physiopathologic aspects of iNPH. A research of literature via the PubMed/MEDLINE and the Cochrane database was conducted. Only English language articles clearly outlining a reasonable concept of physiopathology were included. Most authors advocate that iNPH is a result of chronically altered cerebrospinal fluid (CSF) dynamics, i.e. deranged CSF production, kinetics and reabsorption. In addition, there are vascular, metaboloneurodegenerative and hereditary factors. Neuroinflammation does not seem to play a significant role in the etiology of iNPH. All in all, iNPH seems to combine several pathogenetic factors leading to a self-reinforcing vicious circle. The majority of studies hint at CSF disturbances on grounds of altered hemodynamics.
The differential diagnosis and treatment of normal-pressure hydrocephalus
Deutsches Ärzteblatt international, 2012
Normal-pressure hydrocephalus (NPH) arises in adulthood and is characterized by a typical combination of clinical and radiological findings. The mean basal intracranial pressure is normal or mildly elevated. The typical signs of the disease are gait impairment, urinary incontinence, and dementia. The difficulty of distinguishing NPH from other neurodegenerative disorders is the likely reason why some 80% of cases remain unrecognized and untreated. According to current evidence, the spontaneous course of NPH ends, for the vast majority of patients, in dependence on nursing care. This review article is based on relevant publications retrieved by a selective search in Medline and on national and international guidelines for the management of NPH. Studies with a high evidence level are lacking; thus, the current state of knowledge about NPH is derived from studies of low or intermediate evidence levels, e.g., observational studies. Modern forms of treatment lead to clinical improvement ...
Reversible long-standing severe disability in idiopathic normal pressure hydrocephalus
I diopathic normal pressure hydrocephalus (iNPH) presents with the triad of impaired gait, impaired cognition, and urinary incontinence, with enlarged ventricles and normal CSF pressure. 1 Reports of CSF shunting and outcome after prolonged severe disability are few. We report a patient who responded dramatically to shunting despite severe motor and cognitive impairment and incontinence for more than a year.
Case report of idiopathic normal pressure hydrocephalus: a challenging diagnosis
Journal of Rehabilitation Medicine - Clinical Communications
Introduction: This report concerns the case of a 70-year-old man with idiopathic normal pressure hydrocephalus (iNPH). The diagnosis in the current case took more than 2 years. iNPH is characterised by ventriculomegaly with a known triad of symptoms: gait disturbance, cognitive impairments and urinary incontinence. Although this is a difficult diagnosis and other conditions must be ruled out, several points in the process could lead to a correct diagnosis. The aim of the report is to identify several reasons why the diagnosis was delayed for such a long time, as well as lessons for the future. Case: This patient developed several symptoms over time. First, he presented with depressive mood and altered behaviour. He later developed gait difficulties and, finally, urinary incontinence. Multiple consultations and examinations failed to provide an exact explanation for all his symptoms. After 2 years, a new doctor at the hospital started from scratch and recognised the iNPH triad, and t...
Idiopathic normal pressure hydrocephalus: Theoretical concept of a spinal etiology
Medical Hypotheses, 2006
Normal pressure hydrocephalus (NPH) is an adult syndrome characterised by a combination of gait disturbance, varying degrees of cognitive decline, urinary incontinence, ventricular enlargement and normal mean intracranial pressure. Since this syndrome was first described, its pathophysiology has been a matter of great debate, although it is now considered that NPH could be divided into two groups: cases with unknown etiology (idiopathic normal pressure hydrocephalus, or INPH) and those which develop from several known causes (such as trauma, meningitis or subarachnoid haemorrhage). The pathophysiology of INPH is still unclear and a matter of debate. In this manuscript, the current pathophysiological conditions of INPH are analysed and the authors put forward the theory that the disease is a dynamic syndrome which occurs in patients who have suffered a significant loss of spinal compliance over time. Consequently, intracranial pressure increases more during systole in INPH patients because it cannot be compensated for by the escape of CSF into the spinal canal as effectively, due to the reduced volume or lack of distension of the spinal canal. This leads to an increase in ventricular size and causes cumulative brain damage over a long period of time and accounts for the slow, progressive nature of NPH. The loss of spinal compliance with age is fundamental to the proposed theory which provides a theoretical justification for studying the spinal canal in INPH and investigating the relationship between the progressive narrowing of the spinal canal and the compensating ability of the craniospinal system.