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    Normal Pressure Hydrocephalus

    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.

    The disorder is caused by this process: endocrine.

    Presentation

    urogenital
    Urinary Incontinence
    • Urinary incontinence usually presents later in the progression of NPH.[neuro.memorialhermann.org]
    • Its typical symptoms are urinary incontinence , dementia , and gait disturbance.[en.wikipedia.org]
    • Overview Normal-pressure hydrocephalus is characterized by gait disorder, cognitive decline, and urinary incontinence, and shunt surgery is the only established effective treatment.[medlink.com]
    • The urinary incontinence is often described as urgency in its milder form; later true urinary incontinency may develop.[my.clevelandclinic.org]
    • It is characterized by an abnormal gait (walk), urinary incontinence, and short-term memory problems.[nhfonline.org]
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  • psychiatrical
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  • musculoskeletal
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  • gastrointestinal
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  • neurologic
    Confusion
    • Dementia should not be confused with delirium although cognition is disordered in both.[merckmanuals.com]
    • In Alzheimer’s, memory loss and confusion tend to be early symptoms, whereas in NPH these appear later.[lifenph.com]
    • These are possible symptoms of NPH: Trouble walking (feels like the feet are stuck to the ground) Poor balance Falling Changes in the way you walk Forgetfulness and confusion Mood changes Depression Difficulty responding to questions Loss of bladder control[hopkinsmedicine.org]
    • NPH can be difficult to diagnose because its symptoms may be confused with other disorders such as Alzheimer’s disease and Parkinson’s disease.[froedtert.com]
    Apraxia
    • Apraxia and related syndromes.[patient.info]
    • CLINICAL FEATURES  CLASSIC TRIAD  GAIT DISTURBANCE -is typically the earliest feature noted and considered to be the most responsive to treatment  Apraxia of gait – no weakness or ataxia  bradykinetic, broad based, magnetic, and shuffling.  URINARY[slideshare.net]
    • There are 3 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, apathy[mountsinai.org]
    • It is classically characterised by the triad of gait apraxia, urinary incontinence, and dementia, although not all patients with NPH have all three.[radiopaedia.org]
    • Gait apraxia in communicating hydrocephalus.[ajnr.org]
    Apathy
    • 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]
    • Dementia can be described as poor memory, difficulty with attention, slowness in processing information, loss of interest in daily activities, apathy and dullness in thinking and actions.[froedtert.com]
    • There are 3 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, apathy[mountsinai.org]
    • Decline in thinking skills that includes overall slowing of thought processes, apathy, impaired planning and decision-making, reduced concentration and changes in personality and behavior.[alz.org]
    • […] falls Trouble taking a first step, the sensation that feet are stuck to the floor Freezing while walking Urinary symptoms include: Frequent urination Urgency to urinate Urinary incontinence Mild dementia symptoms include: Memory loss Speech difficulties Apathy[ucirvinehealth.org]
    Gait Apraxia
    • There are 3 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, apathy[mountsinai.org]
    • It is classically characterised by the triad of gait apraxia, urinary incontinence, and dementia, although not all patients with NPH have all three.[radiopaedia.org]
    • True ataxia and weakness are absent and the gait disturbance is referred to as gait apraxia.[patient.info]
    • Gait apraxia in communicating hydrocephalus.[ajnr.org]
    • The aberrant pattern of ambulation often is composed of a slow gait; short, shuffling steps; and a wide-based stance. 4 , 15 , 21 The term “gait apraxia” is sometimes used to describe the problem with locomotion experienced in patients with NPH.[aafp.org]
    Ataxia
    • NPH is characterized by gradual loss of memory loss ( dementia ), balance disorder (ataxia), incontinence of urine and a general slowing of activity.[medicinenet.com]
    • True ataxia and weakness are absent and the gait disturbance is referred to as gait apraxia.[patient.info]
    • CLINICAL FEATURES  CLASSIC TRIAD  GAIT DISTURBANCE -is typically the earliest feature noted and considered to be the most responsive to treatment  Apraxia of gait – no weakness or ataxia  bradykinetic, broad based, magnetic, and shuffling.  URINARY[slideshare.net]
    • […] deterioration, and convulsions.hydrocephal ic communicating hydrocephalus that in which there is free access of fluid between the ventricles of the brain and the spinal canal. normal-pressure hydrocephalus , normal-pressure occult hydrocephalus dementia, ataxia[medical-dictionary.thefreedictionary.com]
    Shuffling Gait
    • Known as NPH, it is believed to plague more than 300,000 Americans, many of whom don’t realize that the disorder’s hallmark symptoms — a distinct, wide-stance shuffling gait, dementia, and incontinence — can be treated and often reversed.[bostonglobe.com]
    • Her neurological examination was normal except for a broad-based shuffling gait.[aansneurosurgeon.org]
    • The classic triad consists of the following: Abnormal gait: Earliest feature and most responsive to treatment; bradykinetic, broad-based, magnetic, and shuffling gait Urinary incontinence: Urinary frequency, urgency, or frank incontinence Dementia: Prominent[emedicine.medscape.com]
    Pyramidal Tract Signs
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  • Entire body system
    Apathy
    • 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]
    • Dementia can be described as poor memory, difficulty with attention, slowness in processing information, loss of interest in daily activities, apathy and dullness in thinking and actions.[froedtert.com]
    • There are 3 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, apathy[mountsinai.org]
    • Decline in thinking skills that includes overall slowing of thought processes, apathy, impaired planning and decision-making, reduced concentration and changes in personality and behavior.[alz.org]
    • […] falls Trouble taking a first step, the sensation that feet are stuck to the floor Freezing while walking Urinary symptoms include: Frequent urination Urgency to urinate Urinary incontinence Mild dementia symptoms include: Memory loss Speech difficulties Apathy[ucirvinehealth.org]
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  • 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].

    Laboratory

    Serum
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  • Imaging

    MR
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  • CT
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  • Test Results

    Lumbar Puncture
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  • 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].

    Complications

    Dementia
    Alzheimer Disease
    • Treatments While many NPH symptoms are similar to Alzheimer's disease symptoms, the good news is that unlike Alzheimer's disease, NPH is treatable and reversible to a certain point.[dementia.org]
    • The dementia symptoms of NPH can be similar to those of Alzheimer disease.[michiganspineandbrainsurgeons.com]
    • Because the symptoms of NPH are similar to those of Alzheimer’s disease , Parkinson’s disease and other disorders, NPH may be misdiagnosed.[froedtert.com]
    • The dementia progresses less rapidly than that seen with Alzheimer's disease.[patient.info]
    • Image of a patient with Alzheimer’s disease reveals enlarged ventricles and significant volume loss of brain parenchyma.[aafp.org]
    Hydrocephalus
    • The Normal Pressure Hydrocephalus Center at Johns Hopkins Bayview Medical Center Normal Pressure Hydrocephalus at Cleveland Clinic normal_pressure_hydrocephalus at NINDS When it really is NPH at Likvor[en.wikipedia.org]
    • Hydrocephalus , NPH - Normal press hydrocephal , Normal-pressure hydrocephalus , HYDROCEPHALUS, NORMAL-PRESSURE , NORMAL PRESSURE HYDROCEPHALY , normal pressure hydrocephalus , NPH (normal pressure hydrocephalus) , normal pressure hydrocephalus (diagnosis[fpnotebook.com]
    • Hydrocephalus in older people (normal pressure hydrocephalus, NPH) Hydrocephalus that develops in older people (normal pressure hydrocephalus, or NPH) can occur after a brain injury, bleeding in the brain or an infection.[nhs.uk]
    • Congenital Hydrocephalus Congenital hydrocephalus develops around the time of birth, but it can persist into adulthood.[brighamandwomens.org]
    • Normal Pressure Hydrocephalus Normal pressure hydrocephalus is an adult onset form of hydrocephalus.[neurosurgicalassociatespc.com]

    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].

    Sex distribution
    Age distribution

    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. 

    Other symptoms

    Impaired Tandem Gait
    • The gait disturbance was mild (cautious gait or impaired tandem gait) in 12 of 65 patients (18%) and severe/marked (unaided gait not possible or considerable unstable gait) in 53 of 65 (82%).[stroke.ahajournals.org]
    Dilation of Lateral Ventricles
    • The mean Evans' index was 35.6 4.0% ranging 0.30 to 0.48, which indicated that the dilation of lateral ventricle in this group was mild to moderate, and marked dilation was rare.[fluidsbarrierscns.biomedcentral.com]

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    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)

    • Comparative analysis of the gait disorder of normal pressure hydrocephalus and Parkinson's disease - H Stolze, JP Kuhtz-Buschbeck, H Drücke - Journal of Neurology, , 2001 - jnnp.bmj.com
    • 'EXPANDING CEREBRAL LACUNAE'IN A HYPERTENSIVE PATIENT WITH NORMAL PRESSURE HYDROCEPHALUS - C Derouesne, F Gray - and applied neurobiology, 1987 - Wiley Online Library
    • Evaluation of patients with progressive intellectual deterioration - FR Freemon - Archives of neurology, 1976 - Am Med Assoc
    • Diagnosis and management of normal-pressure hydrocephalus - JAL Vanneste - Journal of neurology, 2000 - Springer
    • Normal Pressure Hydrocephalus Guidelines From the Guidelines Committee of Idiopathic Normal Pressure Hydrocephalus, the Japanese Society of Normal Pressure - M ISHIKAWA, M HASHIMOTO, N KUWANA - Neurologia medico- , 2008 - J-STAGE
    • Alzheimer's disease comorbidity in normal pressure hydrocephalus: prevalence and shunt response - J Golomb, J Wisoff, DC Miller, I Boksay - Journal of Neurology, , 2000 - jnnp.bmj.com
    • Association of deep white matter infarction with chronic communicating hydrocephalus: implications regarding the possible origin of normal-pressure hydrocephalus. - WG Bradley, AR Whittemore - American journal , 1991 - Am Soc Neuroradiology
    • Binswanger's disease and normal-pressure hydrocephalus: Clinical and neuropsychological comparison - R Gallassi, A Morreale, P Montagna - Archives of , 1991 - Am Med Assoc
    • Leptomeningeal biopsy specimens correlated with cerebrospinal fluid outflow resistance and B-wave activity in patients suspected of normal-pressure hydrocephalus - RA Bech, M Juhler, G Waldemar, L Klinken - Neurosurgery, 1997 - journals.lww.com
    • A treatable form of dementia due to normal-pressure, communicating hydrocephalus. - ME Hill, WM Lougheed, HJ Barnett - Medical Association Journal, 1967 - ncbi.nlm.nih.gov
    • Cerebrospinal fluid flow and production in patients with normal pressure hydrocephalus studied by MRI - P Gideon, F Ståhlberg, C Thomsen, F Gjerris - Neuroradiology, 1994 - Springer
    • Computed tomography of the brain in the diagnosis of and prognosis in normal pressure hydrocephalus - C Wikkelsö, H Andersson, C Blomstrand, M Matousek - Neuroradiology, 1989 - Springer
    • Cognitive recovery in idiopathic normal pressure hydrocephalus: a prospective study - C Raftopoulos, J Deleval, C Chaskis, A Leonard - , 1994 - journals.lww.com
    • An investigation of dementia among elderly outpatients - UGV Cunha - Acta Psychiatrica Scandinavica, 1990 - Wiley Online Library
    • Cognitive recovery in idiopathic normal pressure hydrocephalus: a prospective study - C Raftopoulos, J Deleval, C Chaskis, A Leonard - , 1994 - journals.lww.com
    • Comparative analysis of the gait disorder of normal pressure hydrocephalus and Parkinson's disease - H Stolze, JP Kuhtz-Buschbeck, H Drücke - Journal of Neurology, , 2001 - jnnp.bmj.com
    • Comparison between the lumbar infusion and CSF tap tests to predict outcome after shunt surgery in suspected normal pressure hydrocephalus - B Kahlon, G Sundbärg, S Rehncrona - Journal of Neurology, , 2002 - jnnp.bmj.com

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