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Normal Pressure Hydrocephalus
Hydrocephalus Normal Pressure
Normal pressure hydrocephalus is a type of hydrocephalus caused by the build-up of cerebrospinal fluid, characterized clinically by dementia, abnormal gait and urinary incontinence. Normal pressure hydrocephalus may occur due to secondary causes or may be idiopathic.

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WIKIDATA, CC BY 3.0
WIKIDATA, CC BY 4.0

Presentation

  • Normal pressure hydrocephalus is a slow, progressive disorder with symptoms such as abnormal gait, dementia and urinary incontinence. Ataxia is rarely observed. Gait is bradykinetic, shuffling and broad based.
  • Urinary symptoms are often presented as frank incontinence. 
  • Dementia: In patient with NPH, dementia is characterized by memory loss (prominent) and bradyphrenia. Other deficits are decreased attention, inertia, and forgetfulness. 
  • Comorbidity is common in patients with NPH with diseases such as cerebrovascular disease.

Entire Body System

  • Falling

    All four had presented with pure akinesia with gait freezing, accompanied by unsteadiness and falls. An awareness that PSP or PD can mimic the clinical symptoms of iNPH may help to avoid invasive and futile cerebrospinal fluid shunting procedures. [ncbi.nlm.nih.gov]

    It also increases the risk of falling. Dementia. This often involves confusion, short-term memory loss/forgetfulness, trouble paying attention, changes in mood, and a lack of interest in daily activities. Problems with bladder control. [my.clevelandclinic.org]

    Possible Complications Problems that may result from NPH or its treatment include: Complications of surgery (infection, bleeding, shunt that does not work well) Loss of brain function ( dementia ) that becomes worse over time Injury from falls Shortened [mountsinai.org]

  • Difficulty Walking

    Loss of bladder control, which tends to appear somewhat later in the disease than difficulty walking and cognitive decline. [alz.org]

    Normal-pressure hydrocephalus consists of difficulty walking, urinary incontinence, and dementia due to an increase in the fluid that normally surrounds the brain. [msdmanuals.com]

    These signs are difficulty walking, difficulty holding in urine, and difficulty thinking. The difficulty walking usually presents as a slowed, shuffling walk, in which the feet appear stuck to the ground. [medfriendly.com]

    He had depression, poor cognitive function, choreiform movements, difficulty pronouncing words, and difficulty walking. His symptoms had worsened markedly over several months. [ncbi.nlm.nih.gov]

  • Swelling

    Most patients experience a reduction in swelling within a few days after surgery, and an immediate improvement in their ability to walk and function. [neuro.memorialhermann.org]

    Treatment may include: Breathing support Draining of cerebrospinal fluid to lower pressure in the brain Medicines to decrease swelling Removal of part of the skull, especially in the first 2 days of a stroke that involves brain swelling If a tumor, hemorrhage [nlm.nih.gov]

    Bleeding from a blood vessel or aneurysm in the brain ( subarachnoid hemorrhage ) Certain head injuries Meningitis or similar infections Surgery on the brain ( craniotomy ) As CSF builds up in the brain, the fluid-filled chambers (ventricles) of the brain swell [mountsinai.org]

    This atrophied brain can allow the ventricles to swell with CSF fluid, which distorts the brain’s shape without causing an elevation in normal brain pressure. [cna-ca.com]

Gastrointestinal

  • Nausea

    Nausea, vomiting, and signs of increased intracranial pressure do not occur. A 71-year-old male patient was scheduled for total knee replacement due to osteoarthritis of right knee joint. [ncbi.nlm.nih.gov]

    Headaches, nausea and difficulty focusing may also accompany the symptoms of NPH, due to the swelling of the brain. Normal pressure hydrocephalus is a progressive illness, in which symptoms may worsen over time. [neuro.memorialhermann.org]

    Because of this, patients do not exhibit the classic signs that accompany increased intracranial pressure such as headache, nausea, vomiting, or altered consciousness, although some studies have shown pressure elevations to occur intermittently. [en.wikipedia.org]

    These further symptoms include: Nausea Headache Blurred vision, difficulty focusing eyes Memory loss Personality and behavior changes Apathy and withdrawal Speech problems Mood changes Causes While there is no known singular cause for normal pressure [dementia.org]

    […] incontinence Mild dementia symptoms include: Memory loss Speech difficulties Apathy and withdrawal Changes in mood or behavior Problems with attention, reasoning or judgment Difficulty concentrating and completing tasks Other symptoms may include: Headaches Nausea [ucirvinehealth.org]

  • Vomiting

    Nausea, vomiting, and signs of increased intracranial pressure do not occur. A 71-year-old male patient was scheduled for total knee replacement due to osteoarthritis of right knee joint. [ncbi.nlm.nih.gov]

    Because of this, patients do not exhibit the classic signs that accompany increased intracranial pressure such as headache, nausea, vomiting, or altered consciousness, although some studies have shown pressure elevations to occur intermittently. [en.wikipedia.org]

    Methods: This is a case report of a 40 year old widow, who presented with eight weeks history of recurrent vomiting, fearfulness and withdrawal to self following a traumatic experience. [omicsonline.org]

    Older children and adults: Behavior changes Decreased alertness Headache Lethargy Nervous system symptoms, including weakness, numbness, eye movement problems, and double vision Seizures Vomiting A health care provider will usually make the diagnosis [nlm.nih.gov]

Neurologic

  • Meningism

    Chronic C. gattii meningitis should be aware in a patient presenting with normal pressure hydrocephalus. [ncbi.nlm.nih.gov]

    It is usually idiopathic, but may also be secondary to various conditions including subarachnoid hemorrhage, cranial trauma, and meningitis. [omicsonline.org]

    Other secondary causes are head injury, meningitis, tumors, previously compensated congenital hydrocephalus and subarachnoid hemorrhage. [symptoma.com]

    About 50% are idiopathic and the other 50% are related to a secondary cause (meningitis, hemorrhage etc.). Unlike other forms of communicating hydrocephalus intraventricular pressure is not increased. [docneuro.com]

  • Cognitive Impairment

    Cognitive impairments were the most common symptoms (n = 6) followed by gait disturbances (n = 3); however, urinary incontinence was not observed. No full clinical triad was present in the patients and only 3 participants had 2 of the triad. [ncbi.nlm.nih.gov]

  • Communicating Hydrocephalus

    Symptoms related to communicating hydrocephalus resolved after tumor resection. [ncbi.nlm.nih.gov]

    Normal pressure hydrocephalus is a form of communicating hydrocephalus in which the intracranial pressure, as measured by lumbar puncture, is normal or intermittently raised. Failure to reabsorb CSF is compensated by reduced production. [gpnotebook.co.uk]

    For a discussion of secondary causes of communicating hydrocephalus, please refer to the article hydrocephalus. [radiopaedia.org]

  • Forgetful

    No neurological symptoms and signs except mild forgetfulness were detected during physical examination following admission. [ncbi.nlm.nih.gov]

    His family say he is increasingly forgetful, and he has had urinary incontinence recently. [bmj.com]

    It typically affects adults in their 60s and 70s, who may forget a friend’s name or no longer enjoy a walk around the block. Implanting one of our shunts may help relieve symptoms. [medtronic.com]

    This often involves confusion, short-term memory loss/forgetfulness, trouble paying attention, changes in mood, and a lack of interest in daily activities. Problems with bladder control. [my.clevelandclinic.org]

    There are three main symptoms of NPH: Changes in the way a person walks: difficulty when beginning to walk (gait apraxia), feel as if they are stuck to the ground (magnetic gait) Slowing of mental function: forgetfulness, difficulty paying attention, [mountsinai.org]

  • Headache

    It is somewhat unexpected to have headaches in patients with idiopathic normal pressure hydrocephalus (INPH) for which the treatment is drainage of cerebrospinal fluid (CSF) using shunt. [ncbi.nlm.nih.gov]

    Although the invasive tests carry theoretical risks (infection, post-procedure headache, bleeding, localised pain and nerve root damage), evidence suggests that these are actually very safe procedures. [ 6 ] In these patients, it is also useful to check [patient.info]

Urogenital

  • Urinary Incontinence

    Normal pressure hydrocephalus is a type of hydrocephalus caused by the build-up of cerebrospinal fluid, characterized clinically by dementia, abnormal gait and urinary incontinence. [symptoma.com]

    His family say he is increasingly forgetful, and he has had urinary incontinence recently. [bmj.com]

    Urinary incontinence appears late in the illness, and is present in 50% of patients at time of treatment. Urinary dysfunction begins as increased frequency often at night, and progresses to urge incontinence and permanent incontinence. [en.wikipedia.org]

    In typical cases gait and balance disturbances appear either before or concurrently with urinary incontinence or the onset of dementia. [clinicaltrials.gov]

    INTRODUCTION: Normal pressure hydrocephalus (NPH), described by Hakim and Adams in 1965, is characterized by gait apraxia, urinary incontinence, and dementia. [ncbi.nlm.nih.gov]

Workup

Though some symptoms of normal pressure hydrocephalus mimic Alzheimer’s disease and Parkinson’s disease, the combination of dementia, urinary incontinence and abnormal gait in conjunction is not common in either disease. Thus, the diagnosis of NPH must be specific.

  • Personal history: Medical professional inquire the patient about the medical and mental problems, family history, life-style, and medications. Physical examination of the patient forms the first step to manage NPH. Some neuropsychological tests are also performed to assess the symptoms of dementia. 
  • Laboratory tests: There is no specific test available to diagnose NPH. They primarily focus on assessing the symptoms of the disease. 
  • Imaging: CT scan and MRI of the head cannot confirm NPH, though the ventricular enlargement can be observed in these images. Citernography highlights the amount of absorption of the CSF. In order to establish the diagnosis of NPH, MRI and CT scan must show Evan’s index of 0.3. One or more of the symptoms such as temporal horn enlargement, periventricular edema or signal changes and fourth ventricular flow void can help confirm NPH [6].

MR

  • Mass Lesion of the Brain

    Conditions that may cause this type of NPH include: impaired absorption of cerebral spinal fluid hemorrhage mass lesions meningitis traumatic brain injury The three symptoms that characterize NPH are walking abnormalities, dementia, and impaired bladder [healthline.com]

    As an intracranial mass lesion or oedematous brain expands, some compensation is possible as cerebrospinal fluid (CSF) and blood move into the spinal canal and extracranial vasculature respectively. [trauma.org]

Treatment

  • Medical care: Patient with normal pressure hydrocephalus does not respond significantly to levodopa or dopamine agonist challenge.
  • Surgical care: Surgical CSF shunting is performed to manage NPH. Detailed testing must be done before and after the drainage of CSF. After the shunt surgery, there is significant improvement in the gait and mental status. The improvement in the gait of the patient can be assessed by videotaping the movement before and after the shunting. Another method to manage NPH is external lumbar drainage (ELD). In ELD, the clinicians use indwelling CSF catheter. The drainage catheter is placed for 3 days, which allows the body, sufficient time to return the neuronal function [9] [10].

Prognosis

Prognosis of normal pressure hydrocephalus is variable. Permanent neurological deficit/death is reported in 6% of all cases, with surgery requiring in 22% of the cases. There is a good long-term survival rate of 39% [7].

Complications

Shunt surgery can cause complications such as catheter breakage, cerebral infarct, infection, seizures, catheter breakage and in some cases even death in up to 10 % of the patients [8].

Etiology

About 50% of the patients with NPH have idiopathic cause. Other secondary causes are head injury, meningitis, tumors, previously compensated congenital hydrocephalus and subarachnoid hemorrhage [2].

Epidemiology

Normal pressure hydrocephalus affects the elderly with prevalence ranging from 3.3 per 100,000 (age: 50 to 59 years), 49.3 per 100,000 (age: 60 to 69 years) and 181.7 per 100,000 (age: 70 to 79 years). In a study in Japan, NPH is seen in 2.9% of the elderly subjects, while data from a Norwegian study showed the incidence of 5.5 in every 100,000 [3] [4] [8].

Pathophysiology

The clinical symptoms of NPH result from the distortion of the central portion of the corona radiata. This alteration is believed to occur due to the distended ventricles.

Nuclear imaging studies indicate the interstitial edema of the white matter in the brain and compression of the brainstem structures leading to impaired bloodflow, which manifests as dysfunction of gait and urinary incontinence. There is also a distortion of the periventricular limbic system, causing dementia in such patients [5].

Prevention

  1. Reducing the risk of head injury. Wearing the safety helmet while biking, skiing or rollerblading.
  2. Quitting smoking. 
  3. Having a healthy life-style with exercise and a healthy diet [10].

Summary

Hydrocephalus is a condition when there is accumulation of surplus cerebrospinal fluid (CSF) in the ventricles of the brain, and the drainage and absorption of extra CSF is compromised. In order to compensate this excess fluid, the ventricles enlarge causing several symptoms of normal pressure hydrocephalus (NPH).

This phenomenon is mostly seen in older patients, with the average age of patients being above 60 years. NPH develops slowly, and affects the parts of brain that control the bladder, the legs, and the cognitive functions such as memory, problem solving, speaking and reasoning. This decline of the cognitive process can cause dementia. Other symptoms of NPH are urinary incontinence, and abnormal gait. Since the symptoms of NPH mimic those of Alzheimer’s disease and Parkinson’s disease, the diagnosis of this disease remains a challenge [1].

Patient Information

Definition

Normal pressure hydrocephalus (NPH) is generally seen in patients with ages 60 and above. This disease develops slowly and affects the brain, bladder and legs. Loss of cognitive functions such as memory, problem solving, speaking and reasoning are affected by NPH. 

Cause

Though half of the patient with NPH have idiopathic cause, the secondary causes are head injury, meningitis, subarachnoid hemorrhage and tumors.

Symptoms

The common symptoms of the NPH are dementia, abnormal gait and urinary incontinence. 

Diagnosis

Family history and personal history detail the symptoms of the disease, allowing for the diagnosis of NPH. Physical examination of the patient forms the first step to manage the disorder. Though there is no specific lab test available to diagnose NPH, some imaging tests can be useful in correct diagnosis.

Treatment

Surgery is the most effective treatment modality to manage NPH. 

References

  1. Hakim S, Adams RD. The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci. Jul-Aug 1965;2(4):307-27.
  2. Graff-Radford NR, Godersky JC. Symptomatic congenital hydrocephalus in the elderly simulating normal pressure hydrocephalus. Neurology. Dec 1989;39(12):1596-60
  3. Brean A, Eide PK. Prevalence of probable idiopathic normal pressure hydrocephalus in a Norwegian population. Acta Neurol Scand. Jul 2008;118(1):48-53. 
  4. Hiraoka K, Meguro K, Mori E. Prevalence of idiopathic normal-pressure hydrocephalus in the elderly population of a Japanese rural community. Neurol Med Chir (Tokyo). May 2008;48(5):197-99; discussion 199-200. 
  5. Hamlat A, Adn M, Sid-ahmed S, et al. Theoretical considerations on the pathophysiology of normal pressure hydrocephalus (NPH) and NPH-related dementia. Med Hypotheses. 2006;67(1):115-23. Epub 2006 Mar 13.
  6. Gyldensted C. Measurements of the normal ventricular system and hemispheric sulci of 100 adults with computed tomography. Neuroradiology. Dec 31 1977;14(4):183-92.
  7. Pujari S, Kharkar S, Metellus P, et al. Normal pressure hydrocephalus: long-term outcome after shunt surgery. J Neurol Neurosurg Psychiatry. 2008 Nov;79(11):1282-6. Epub 2008 Mar 20.
  8. Shprecher D, Schwalb J, Kurlan R. Normal pressure hydrocephalus: diagnosis and treatment. Curr Neurol Neurosci Rep. 2008 Sep;8(5):371-6.
  9. Williams MA, Razumovsky AY, Hanley DF. Comparison of Pcsf monitoring and controlled CSF drainage diagnose normal pressure hydrocephalus. Acta Neurochir Suppl. 1998;71:328-30.
  10. Lumbar infusion test for the investigation of normal pressure hydrocephalus, NICE Interventional Procedure Guideline (June 2008)
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