KEGG   DISEASE: Normal pressure hydrocephalus
Entry
H01676                      Disease                                
Name
Normal pressure hydrocephalus
Description
Normal pressure hydrocephalus (NPH) is a progressive brain disease characterized by gait impairment, urinary incontinence, and dementia, and by ventricular enlargement in the absence of elevated intracranial pressure. NPH can be secondary to disease processes that cause inflammation of the arachnoid, such as subarachnoid hemorrhage, traumatic brain injury, or meningitis. As many as half of patients with NPH have no identifiable risk factor, and it is called idiopathic normal pressure hydrocephalus (INPH). Although INPH is usually considered an acquired form of hydrocephalus because the onset of symptoms is not until late in life, it has been reported that a subset of patients with INPH may actually have congenital hydrocephalus. Symptoms can be alleviated with a cerebrospinal fluid (CSF) shunt.
Category
Nervous system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 08 Diseases of the nervous system
  Disorders of cerebrospinal fluid pressure or flow
   8D64  Hydrocephalus
    H01676  Normal pressure hydrocephalus
Other DBs
ICD-11: 8D64.04
MeSH: D006850
OMIM: 236690 611808
Reference
  Authors
Golz L, Ruppert FH, Meier U, Lemcke J
  Title
Outcome of modern shunt therapy in patients with idiopathic normal pressure hydrocephalus 6 years postoperatively.
  Journal
J Neurosurg 121:771-5 (2014)
DOI:10.3171/2014.6.JNS131211
Reference
  Authors
Wilson RK, Williams MA
  Title
Evidence that congenital hydrocephalus is a precursor to idiopathic normal pressure hydrocephalus in only a subset of patients.
  Journal
J Neurol Neurosurg Psychiatry 78:508-11 (2007)
DOI:10.1136/jnnp.2006.108761
Reference
PMID:16239882 (treatment)
  Authors
McGirt MJ, Woodworth G, Coon AL, Thomas G, Williams MA, Rigamonti D
  Title
Diagnosis, treatment, and analysis of long-term outcomes in idiopathic normal-pressure hydrocephalus.
  Journal
Neurosurgery 57:699-705; discussion 699-705 (2005)
DOI:10.1227/01.NEU.0000175724.00147.10
Reference
  Authors
Zhang J, Williams MA, Rigamonti D
  Title
Heritable essential tremor-idiopathic normal pressure hydrocephalus (ETINPH).
  Journal
Am J Med Genet A 146A:433-9 (2008)
DOI:10.1002/ajmg.a.31958
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