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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
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Normal pressure hydrocephalus (NPH), also termed Hakim's syndrome and symptomatic hydrocephalus, is a type of brain malfunction caused by expansion of the lateral cerebral ventricles and distortion of the fibers in the corona radiata. Its typical symptoms are urinary incontinence, dementia, and gait disturbance. CSF pressure is usually normal. Ventricles are chronically dilated.

The usual treatment is surgical installation of a ventriculoperitoneal shunt to drain excess CSF into the lining of the abdomen where the CSF will eventually be absorbed.

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, wacky and wobbly" or "weird walking water").

NPH is caused by an increase in intracranial pressure (ICP) due to an abnormal accumulation of CSF in the ventricles of the brain, leading to 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, 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. NPH is often misdiagnosed as Parkinson's disease, Alzheimer's disease, or dementia, due to its chronic nature and nonspecific presenting symptoms.


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