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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.


Presentation

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]
  • Clinical Picture The typical Hakimʼs triad is predominant: gait disturbance, dementia, and urinary incontinence.[ncbi.nlm.nih.gov]
  • A vigilant physician should keep in mind that the presentation of gait disturbance, dementia, and urinary incontinence in a patient may indicate the likelihood of NPH.[ncbi.nlm.nih.gov]
  • Urinary incontinence is the loss of bladder control and inability to hold the urine. Patients may also experience the frequent urge to urinate. Urinary incontinence usually presents later in the progression of NPH.[neuro.memorialhermann.org]
  • Idiopathic normal pressure hydrocephalus (iNPH) is a ventriculomegaly syndrome characterized by dementia, urinary incontinence, and gait disturbance, which is potentially reversible after ventriculoperitoneal shunting (VPS).[ncbi.nlm.nih.gov]
Confusion
  • The symptoms are sometimes confused with those of Alzheimer’s or Parkinson’s disease. Treatment Normal Pressure Hydrocephalus is treated with a surgically-implanted shunt.[cna-ca.com]
  • In Alzheimer’s, memory loss and confusion tend to be early symptoms, whereas in NPH these appear later. Fortunately, once NPH is confirmed, chances are it can be treated.[lifenph.com]
  • Individuals with normal pressure hydrocephalus often have a combination of three symptoms that can help establish the diagnosis: mental confusion, gait disturbance and urinary incontinence.[neuro.northshorelij.com]
Apraxia
  • Normal pressure hydrocephalus (NPH), described by Hakim and Adams in 1965, is characterized by gait apraxia, urinary incontinence, and dementia.[ncbi.nlm.nih.gov]
  • Normal pressure hydrocephalus (NPH) is characterized by gait apraxia, urinary incontinence, and dementia. NPH in association with MD has been reported in only three cases.[ncbi.nlm.nih.gov]
  • The best predictors of outcome to include aqueductal flow void , thinning of the corpus callosum, effacement of the sulci with absence of cortical atrophy and gait apraxia as the dominant clinical symptom.[docneuro.com]
  • True ataxia and weakness are absent and the gait disturbance is referred to as gait apraxia. Sphincter disturbance - this is also due to involvement of the sacral nerve supply.[patient.info]
Apathy
  • Among 46 patients with suspicion of iNPH (77.6 6.7years; 34.8% women), 30 (65.2%) presented apathy (defined by a score 14 on the Starkstein apathy scale).[ncbi.nlm.nih.gov]
  • These findings require replication in a large sample of patients to determine whether methylphenidate may prove to be generally useful in enhancing cognition and reducing apathy in normal pressure hydrocephalus.[ncbi.nlm.nih.gov]
  • Connors, referred to the neurosurgeon, showed significant improvement in gait and apathy after her large volume CSF tap.[brightfocus.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]
  • 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, apathy[medlineplus.gov]
Gait Apraxia
  • Normal pressure hydrocephalus (NPH), described by Hakim and Adams in 1965, is characterized by gait apraxia, urinary incontinence, and dementia.[ncbi.nlm.nih.gov]
  • Normal pressure hydrocephalus (NPH) is characterized by gait apraxia, urinary incontinence, and dementia. NPH in association with MD has been reported in only three cases.[ncbi.nlm.nih.gov]
  • The best predictors of outcome to include aqueductal flow void , thinning of the corpus callosum, effacement of the sulci with absence of cortical atrophy and gait apraxia as the dominant clinical symptom.[docneuro.com]
  • 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,[medlineplus.gov]
Ataxia
  • An 80-year-old woman presented with progressive cognitive decline and with a 6-month history of gait ataxia. Brain MRI depicted enlarged ventricles and periventricular lesions.[ncbi.nlm.nih.gov]
  • Our patient's gait disturbance was unassociated with muscle weakness, spasticity, cerebellar ataxia, or Romberg's sign, and, therefore, was consistent with a frontal gait disorder.[ncbi.nlm.nih.gov]
  • Postoperatively, her symptoms improved except for the ataxia and impaired breathing. She was monitored cautiously for over 15 days. CT at discharge on the 18th postoperative day revealed decreased cyst size to 3.9 x 4.1 cm.[ncbi.nlm.nih.gov]
  • Though we performed a ventriculoperitoneal shunt operation, his trunk ataxia persisted. We finally diagnosed an arachnoid cyst in the fourth ventricle by direct ventricular infusion of enhanced material.[ncbi.nlm.nih.gov]
  • […] year history of headache and typical symptoms of normal-pressure hydrocephalus, including gait disturbance, urinary incontinence, and cognitive dysfunction, in addition to the more common symptoms of CPP, such as lower cranial nerve dysfunctions and ataxia[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].

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].

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. 

References

Article

  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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Last updated: 2018-06-21 19:26