Normal pressure hydrocephalus

Normal pressure hydrocephalus
Classification and external resources
Specialty	neurology
ICD-10	G91.2
ICD-9-CM	331.9, 331.5
DiseasesDB	9089
MedlinePlus	000752
eMedicine	neuro/277 radio/479
Patient UK	Normal pressure hydrocephalus
MeSH	D006850

Normal pressure hydrocephalus (NPH), also termed symptomatic hydrocephalus, is a type of brain malfunction caused by decreased absorption of cerebrospinal fluid (CSF). Its typical symptoms are gait disturbance, urinary incontinence, and dementia or mental decline. It is difficult to diagnose because the symptoms are common to several other diseases. The usual treatment is installation of a shunt to drain excess CSF into another part of the body. This treatment can reverse the symptoms and restore normal functioning, or it may do so partially, or it may not succeed.

Types

There are two types of normal pressure hydrocephalus: idiopathic and secondary. The secondary type of NPH can be due to a subarachnoid haemorrhage, head trauma, tumour, infection in the central nervous system, or a complication of cranial surgery.^[1]

Mechanism

NPH is caused by an increase in intracranial pressure (ICP) due to an abnormal accumulation of CSF in the ventricles of the brain, which can cause the ventricles to enlarge (ventriculomegaly). The intracranial pressure gradually falls but still remains slightly elevated, and the CSF pressure reaches a high normal level of 150 to 200 mm H₂O. Measurements of ICP, therefore, are not usually elevated. 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. However, the enlarged ventricles put increased pressure on the adjacent cortical tissue and cause myriad effects in the patient. The classic symptom triad (gait disturbance, urinary incontinence, and dementia) was first described by Hakim and Adams in 1965.^[2] NPH is often misdiagnosed as Parkinson's disease, Alzheimer's disease, or dementia, due to its chronic nature and nonspecific presenting symptoms.

Signs and symptoms

NPH may exhibit a classic triad of clinical findings (known as the Adams triad or Hakim's triad) of urinary incontinence, gait disturbance, and dementia (commonly referred to as "wet, wobbly and wacky" or "weird walking water").

• Gait disturbance is typically the initial and most prominent symptom of the triad and may be progressive, due to expansion of the ventricular system, particularly at the level of the lateral ventricles, leading to traction on the corticospinal motor fibers descending to the lumbosacral spinal cord. The gait disturbance can be classified as mild (cautious gait or difficulty with tandem gait), marked (evident difficulty walking or considerable unstable gait) or severe (unaided gait not possible)^[3] In the early stages, most often this gait disturbance occurs in the form of unsteadiness and impaired balance, especially when encountering stairs and curbs. Weakness and tiredness of the legs may also be part of the complaint, although examination discloses no paresis or ataxia.^[4] Often a mobility aid is used for added stability, once the patient has reached the mild to marked stage. Such aids may include a quad cane or wheeled walker. The patient's gait at the marked stage will often show a decrease in step height and foot-floor clearance, as well as a decrease in walking speed. This style is often referred to as a magnetic gait, in which the feet appear to be stuck to the walking surface, and is considered the characteristic gait disturbance of NPH. The gait may begin to mimic a Parkinsonian gait, with short shuffling steps and stooped, forward-leaning posture, but there is no rigidity or tremor. An increased tendency to fall backwards is also seen, and a broad-based gait may be employed by the patient in order to increase their base of support and thereby their stability. In the very late stages, the patient can progress from an inability to walk, to an inability to stand, sit, rise from a chair or turn over in bed; this advanced stage is referred to as "hydrocephalic astasia-abasia".^[4]
• Dementia is predominantly frontal lobe in nature and of the subcortical type of dementia. It presents in the form of apathy, forgetfulness, inertia, inattention, decreased speed of complex information procession (also described as a dullness in thinking and actions), and disturbed manipulation of acquired knowledge, which is reflective of the loss of integrity of the frontal lobes.^[5] Memory problems are usually a component of the overall problem and have been predominant in some cases, which can lead to the misdiagnosis of Alzheimer's disease. However, in NPH there may be an obvious discrepancy between (often severely) impaired recall and intact or much less impaired recognition. The dementia is thought to result from traction on frontal and limbic fibers that also run in the periventricular region.
• Urinary incontinence appears late in the illness, and is found to be of the spastic hyperreflexic, increased-urgency type associated with decreased inhibition of bladder contractions and detrusor instability.^[5] In the most severe cases, bladder hyperreflexia is associated with a lack of concern for micturition due to the severe frontal lobe cognitive impairment.^[5] This is also known as "frontal lobe incontinence", where the patient becomes indifferent to their recurrent urinary symptoms.

Diagnosis

Diagnosis of NPH is usually first led by brain imaging, either CT or MRI, to rule out any mass lesions in the brain. This is then followed by lumbar puncture and evaluation of clinical response to removal of CSF. This can be followed by continuous external lumbar CSF drainage during 3 or 4 days.

• CT scan may show enlarged ventricles without convolutional atrophy.
• MRI may show some degree of transependymal migration of CSF surrounding the ventricles on T2/FLAIR sequence. Imaging however cannot differentiate between pathologies with similar clinical picture like Alzheimer's dementia, vascular dementia or Parkinson's disease.^[6]
• Following imaging, lumbar puncture is usually the first step in diagnosis and the CSF opening pressure is measured carefully. In most cases, CSF pressure is usually above 155 mmH₂O. Clinical improvement after removal of CSF (30 mL or more) has a high predictive value for subsequent success with shunting. This is called the "lumbar tap test" or Miller Fisher test. On the contrary, a "negative" test has a very low predictive accuracy, as many patients may improve after a shunt in spite of lack of improvement after CSF removal.
• Infusion test is a test that may have higher sensitivity and specificity than a lumbar puncture, but is not performed in most centers. The outflow conductance (Cout) of the cerebrospinal fluid (CSF) system is a parameter considered by some centers to be predictive in selection for hydrocephalus surgery. Cout can be determined through an infusion test. This is not a test that is normally performed prior to shunting, but may become more accepted.
• In some centers, External lumbar drainage has been shown to have the highest sensitivity and specificity with regards to predicting a successful outcome following surgery.^[7]

Treatment

Patients with dementia who are confined to a nursing home and may have undiagnosed NPH can possibly become independent again once treated. So far only one study was able to evaluate the prevalence of NPH, both diagnosed and undiagnosed, among residents of assisted-living facilities, showing a prevalence in 9 to 14% of the residents.^[8]

NPH may be relieved by surgically implanting a ventriculoperitoneal shunt to drain excess cerebrospinal fluid to the abdomen where it is absorbed. Once the shunt is in place, the ventricles usually diminish in size in 3 to 4 days, regardless of the duration of the hydrocephalus. Even though the ventricular swelling diminishes, only 21% of patients show a marked improvement in symptoms. The most likely patients to show improvement are those that show only gait disturbance, mild or no incontinence, and mild dementia.^[9] A more recent study (2004) found better outcomes, concluding that if patients with idiopathic normal pressure hydrocephalus are correctly identified, shunt insertion yielded beneficial outcomes in 86% of patients, in either gait disturbance (81%), improved continence (70%), or both. They also observed that measurements in the diagnostic clinical triad, the cortical sulci size, and periventricular lucencies were related to outcome. However, other factors such as age of the patient, symptom duration, dilation of ventricles, and the degree of presurgical dementia were unrelated to outcome.^[10]

Epidemiology

Recent population-based studies have estimated the prevalence of NPH to be about 0.5% in those over 65 years old, with an incidence of about 5.5 patients per 100,000 of people per year.^[11][12] This is in accordance with comparable findings stating that although normal pressure hydrocephalus can occur in both men and women of any age, it is found more often in the elderly population, with a peak onset generally in the sixth to seventh decades.^[5]

See also

Low pressure hydrocephalus

References

1. National Institute of Neurological Disorders and Stroke. (2011, April 29). NINDS Normal Pressure Hydrocephalus Information Page. Retrieved from http://www.ninds.nih.gov/disorders/normal_pressure_hydrocephalus/normal_pressure_hydrocephalus.htm
2. Adams, R. D.; Fisher, C. M.; Hakim, S.; Ojemann, R. G.; Sweet, W. H. (15 July 1965). "Symptomatic Occult Hydrocephalus with Normal Cerebrospinal-Fluid Pressure". New England Journal of Medicine 273 (3): 117–126. doi:10.1056/NEJM196507152730301. PMID 14303656.
3. Krauss, J.K., Faist, M., Schubert, M., Borremans, J.J., Lucking, C.H., & Berger, W. (2001). Evaluation of Gait in Normal Pressure Hydrocephalus Before and After Shunting. In R’uzicka, E., Hallett, M., & Jankovic, J. (Eds.), Gait Disorders (pp.301-309). Philadelphia, PA: Lippincott Williams & Wilkins.
4. Ropper, A.H. & Samuels, M.A. (2009). Adams and Victor’s Principles of Neurology (9th edition). New York, NY: McGraw-Hill Medical.
5. Younger, D.S. (2005). Adult Normal Pressure Hydrocephalus. In Younger, D.S. (Ed.), Motor Disorders (2nd edition) (pp. 581-584). Philadelphia, PA: Lippincott Williams & Wilkins.
6. Tarnaris, Andrew; Toma, Ahmed K; Kitchen, Neil D; Watkins, Laurence D (1 December 2009). "Ongoing search for diagnostic biomarkers in idiopathic normal pressure hydrocephalus". Biomarkers in Medicine 3 (6): 787–805. doi:10.2217/bmm.09.37. PMID 20477715.
7. Marmarou, A; Bergsneider, M; Klinge, P; Relkin, N; Black, PM (September 2005). "The value of supplemental prognostic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus.". Neurosurgery 57 (3 Suppl): S17–28; discussion ii–v. doi:10.1227/01.neu.0000168184.01002.60. PMID 16160426.
8. Marmarou, Anthony; Young, Harold F.; Aygok, Gunes A. (1 April 2007). "Estimated incidence of normal-pressure hydrocephalus and shunt outcome in patients residing in assisted-living and extended-care facilities". Neurosurgical FOCUS 22 (4): 1–8. doi:10.3171/foc.2007.22.4.2. PMID 17613187.
9. Vanneste, J; Augustijn, P; Dirven, C; Tan, WF; Goedhart, ZD (January 1992). "Shunting normal-pressure hydrocephalus: do the benefits outweigh the risks? A multicenter study and literature review.". Neurology 42 (1): 54–9. doi:10.1212/wnl.42.1.54. ISSN 0028-3878. PMID 1734324.
10. Poca, Maria A.; Mataró, Maria; Matarín, Maria Del Mar; Arikan, Fuat; Junqué, Carmen; Sahuquillo, Juan (1 May 2004). "Is the placement of shunts in patients with idiopathic normal pressure hydrocephalus worth the risk? Results of a study based on continuous monitoring of intracranial pressure". Journal of Neurosurgery 100 (5): 855–866. doi:10.3171/jns.2004.100.5.0855. PMID 15137605.
11. Brean, A.; Eide, P. K. (1 July 2008). "Prevalence of probable idiopathic normal pressure hydrocephalus in a Norwegian population". Acta Neurologica Scandinavica 118 (1): 48–53. doi:10.1111/j.1600-0404.2007.00982.x. PMID 18205881.
12. Tanaka, Naofumi; Yamaguchi, Satoshi; Ishikawa, Hiroyasu; Ishii, Hiroshi; Meguro, Kenichi (1 January 2009). "Prevalence of Possible Idiopathic Normal-Pressure Hydrocephalus in Japan: The Osaki-Tajiri Project". Neuroepidemiology 32 (3): 171–175. doi:10.1159/000186501. PMID 19096225.

External links

• 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