Normal pressure hydrocephalus 1055119 212419150 2008-05-14T19:23:28Z 155.101.241.243 /* Treatment */ {{Infobox_Disease | Name = {{PAGENAME}} | Image = | Caption = | DiseasesDB = 9089 | ICD10 = {{ICD10|G|91|2|g|90}} | ICD9 = {{ICD9|331.9}} | ICDO = | OMIM = | MedlinePlus = 000752 | eMedicineSubj = neuro | eMedicineTopic = 277 | eMedicine_mult = {{eMedicine2|radio|479}} | MeshID = D006850 }} '''Normal pressure hydrocephalus''' (NPH) is a chronic type of communicating [[hydrocephalus]] whereby the increase in [[intracranial pressure]] (ICP) due to accumulation of cerebrospinal fluid (CSF) becomes stable and that the formation of CSF equilibrates with absorption. The ICP gradually falls but still maintains a slightly elevated level and the CSF pressure reaches a high normal level of 150 to 200 mmH<sub>2</sub>O. Measurements of CSF, therefore, are not usually elevated. Because of this equilibration, patients do not exhibit the classic signs of increased intracranial pressure such as headache, nausea, vomiting, or altered consciousness. (Though some studies have shown pressure elevations to occur only intermittently). However, patients do exhibit the classic triad of gait difficulties, urinary incontinence, and mental decline. It is often misdiagnosed as [[Parkinson's disease]], [[Alzheimer's disease]], and [[senility]] due to its chronic nature and its presenting symptoms [see below]. And, although the exact mechanism is unknown, normal-pressure hydrocephalus is thought to be a form of communicating hydrocephalus with impaired CSF reabsorption at the arachnoid villi. ==Clinical manifestations== NPH may exhibit the classic triad of [[urinary incontinence]], wide-based [[ataxia|ataxic]] gait, and [[dementia]](commonly referred to as "wet, wobbly and wacky"). *'''''[[Gait disturbance]] and [[Ataxia]]''''' is the first 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 lumbosacral motor fibers that run in this region. Often, this takes on the form of unsteadiness and impaired balance, especially on stairs and curbs. Weakness and tiredness may also be part of the complaint, although this is very vague. NPH gait disturbance is often characterized as a "magnetic gait," in which feet appear to be stuck to the walking surface until wrested upward and forward at each step. The gait may mimic a Parkinsonian gait, with short shuffling steps and stooped, forward-leaning posture, but there is no rigidity or tremor. A broad-based gait may be employed by the patient in order to compensate for the ataxia. *'''''[[Dementia]]''''' is predominantly frontal lobe in nature, with apathy, dullness in thinking, and slight inattention. Memory problems are usually the main problem, 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, consisting of increased frequency and urgency. Ultimately, the patient may exhibit "frontal lobe incontinence," where he becomes indifferent to his recurrent urinary symptoms. ==Diagnosis== Diagnosis of NPH is usually first led by a [[lumbar puncture]], followed by the evaluation of clinical response to removal of CSF. This can be followed by a [[CT scan|CT]], [[magnetic resonance imaging|MRI]], and continuous external lumbar CSF drainage during 3 or 4 days. *'''''[[Lumbar puncture]]''''' is usually the first step in diagnosis and the opening pressure measured carefully. In most cases, CSF pressure is usually above 155 mmH<sub>2</sub>O. Clinical improvement after removal of CSF (30 mL or more) has a high predictive value for subsequent success with [[cerebral shunt|shunting]]. This is called the "lumbar tap test" or "Fisher test". 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. *'''''[[CT scan]]''''' may show enlarged ventricles without convolutional atrophy. *'''''[[MRI]]''''' may show some degree of transependymal egress of water surrounding the ventricles. ==Treatment== NPH may be relieved by surgically implanting a ventriculoperitoneal [[shunt (medical)|shunt]] to drain excess cerebrospinal fluid to the abdomen where it is absorbed. Once the shunt is in place, the ventricles 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.<ref>Shunting normal-pressure hydrocephalus: do the benefits outweigh the risks? A multicenter study and literature review.[http://www.medscape.com/medline/abstract/1734324?src=emed_ckb_ref_0]</ref> 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.<ref>Poca MA, Mataró M, Del Mar Matarín M, Arikan F, Junqué C, Sahuquillo J. 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. J Neurosurg. 2004 May;100(5):855-66. PMID: 15137605 [PubMed - indexed for MEDLINE]</ref> == Shunts in Developing Countries == Since the cost of shunt systems from many developed countries is beyond the reach of common people in developing countries, most of them die without even getting a shunt. And the worse is the rate of revision in shunt systems that adds to the cost of shunting many times. Looking at this point, a study done by Dr. Benjamin C. Warf comparing different shunt systems and highliting the role of low cost shunt system in most of the developing countries. This study has been published in Journal of [[Neurosurgery]]: Pediatrics May 2005 issue. It is about comparing Chhabra shunt system from Surgiwear to those of the shunt systems from developed countries. The study was done in Uganda and the shunts were donated by International Federation for Spina Bifida and Hydrocephalus. == References == <references/> *[http://jnsonline.org/peds/issues/v102n4/pdf/p1020357.pdf Shunts in Africa] *[http://jnsonline.org/peds/issues/v102n4/pdf/p1020358.pdf Uganda: a prospective study in 195 children] *[http://jnsonline.org/ Journal Of Neurosurgery] Blumenfeld, Hal. Neuroanatomy through Clinical Cases, 2002. == External links == * [http://www.clevelandclinic.org/health/health-info/docs/3500/3525.asp?index=11569 Normal Pressure Hydrocephalus] at [[Cleveland Clinic]] * {{NINDS|normal_pressure_hydrocephalus}} {{Diseases of the nervous system}} [[Category:Neurology]] [[Category:Neurosurgery]] [[Category:Geriatrics]] [[de:Normaldruckhydrozephalus]] [[pl:Wodogłowie normotensyjne]]