NPH
MCID: NRM002
MIFTS: 61

Normal Pressure Hydrocephalus (NPH) malady

Neuronal diseases, Rare diseases categories

Summaries for Normal Pressure Hydrocephalus

About this section
Sources:
45NINDS, 66Wikipedia, 48OMIM, 34MalaCards
See all sources

Fully expand this MalaCard

Download this MalaCard
NINDS:45 Normal pressure hydrocephalus (NPH) is an abnormal buildup of cerebrospinal fluid (CSF) in the brain's ventricles, or cavities. It occurs if the normal flow of CSF throughout the brain and spinal cord is blocked in some way. This causes the ventricles to enlarge, putting pressure on the brain. Normal pressure hydrocephalus can occur in people of any age, but it is most common in the elderly. It may result from a subarachnoid hemorrhage, head trauma, infection, tumor, or complications of surgery. However, many people develop NPH even when none of these factors are present. In these cases the cause of the disorder is unknown.

MalaCards: Normal Pressure Hydrocephalus, also known as hydrocephalus - normal pressure, is related to hydrocephalus and dementia. An important gene associated with Normal Pressure Hydrocephalus is PTGDS (prostaglandin D2 synthase 21kDa (brain)), and among its related pathways are ALK1 signaling events and IL-4 Signaling and its Primary Biological Effects in Different Immune Cell Types. The compounds helospectin and secretoneurin have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and testes, and related mouse phenotypes are taste/olfaction and adipose tissue.

Wikipedia:66 Normal pressure hydrocephalus (NPH), also termed symptomatic hydrocephalus, is a type of brain... more...

Description from OMIM:48 236690

Aliases & Classifications for Normal Pressure Hydrocephalus

About this section
Sources:
9Disease Ontology, 45NINDS, 11DISEASES, 46Novoseek, 50Orphanet, 63UMLS, 59SNOMED-CT, 48OMIM, 36MeSH, 64UMLS via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 26ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Neuronal diseases


Characteristics (Orphanet epidemiological data):

50
normal pressure hydrocephalus:
Inheritance: Sporadic; Prevalence: 1-5/10000; Age of onset: Adulthood


Aliases & Descriptions:

normal pressure hydrocephalus 9 45 11 50
hydrocephalus - normal pressure 45
hydrocephalus, normal pressure 63
hydrocephalus normal pressure 46
chronic adult hydrocephalus 50
low pressure hydrocephalus 9
nph 50


External Ids:

Disease Ontology9 DOID:1572
OMIM48 236690
MeSH36 D006850
UMLS via Orphanet64 C0020258
SNOMED-CT59 69663004, 30753002
ICD10 via Orphanet27 G91.2
SNOMED-CT via Orphanet60 30753002
ICD1026 G91.2

Related Diseases for Normal Pressure Hydrocephalus

About this section
Sources:
18GeneCards, 19GeneDecks
See all sources

Diseases related to Normal Pressure Hydrocephalus via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 151)
idRelated DiseaseScoreTop Affiliating Genes
1hydrocephalus32.4MAPT
2dementia31.0GFAP, PTGDS, APOE, APP, MAPT, GAL
3type 1 diabetes mellitus30.8NPY, GAL, ACE
4alzheimer's disease30.8GFAP, PTGDS, APOE, APP, TGFBR2, MAPT
5binswanger's disease30.6MAPT, GFAP
6hypoglycemia30.5GAL, NPY, BDNF
7hyperglycemia30.5TGFB1, ACE, GAL, NPY, BDNF
8hypertension30.5APOE, TGFB1, ACE, NPY
9progressive supranuclear palsy30.5MAPT, APOE
10parkinson's disease30.5BDNF, MAPT, APOE
11diabetes mellitus30.4APOE, TGFB1, ACE, NPY
12ischemia30.4GFAP, VIP, MAPT, GAL, BDNF
13vascular dementia30.4MAPT, APP, APOE
14huntington's disease30.4BDNF, NPY, MAPT, APP
15vascular disease30.2APOE, ACE, NPY
16pick's disease30.2MAPT, APP, APOE
17frontotemporal dementia30.2APOE, APP, MAPT
18cerebrovascular disease30.2MAPT, ACE, VIP, APOE
19multiple sclerosis30.1GFAP, PTGDS, APOE, TGFB1, NPY, BDNF
20cerebritis10.9
21cerebral atrophy10.5
22type 2 diabetes mellitus10.5
23maturity-onset diabetes of the young, type 110.5
24arachnoiditis10.4
25intracranial hypertension10.4
26subcortical arteriosclerotic encephalopathy10.4
27diabetic ketoacidosis10.3
28maturity-onset diabetes of the young10.3
29brittle diabetes10.3
30epiphyseal dysplasia multiple with early-onset diabetes mellitus10.3
31immunodysregulation, polyendocrinopathy and enteropathy x-linked10.3
32maturity-onset diabetes of the young, type 210.3
33maturity-onset diabetes of the young, type 610.3
34diabetes, type 210.3
35rh-mod syndrome10.3
36syringomyelia10.3
37neuronitis10.3
38gait apraxia10.3
39systemic lupus erythematosus10.3
40apraxia10.3
41lupus erythematosus10.3
42meningioma10.3
43peritonitis10.3
44polyneuropathy10.3
45arachnoid cysts10.3
46hypoglycemic coma10.2
47parametritis10.2
48obsessive-compulsive disorder10.2
49paget's disease of bone10.2
50herpes zoster10.2

Graphical network of the top 20 diseases related to Normal Pressure Hydrocephalus:



Diseases related to normal pressure hydrocephalus

Symptoms for Normal Pressure Hydrocephalus

About this section
Sources:
48OMIM
See all sources


Clinical features from OMIM:

236690

Drugs & Therapeutics for Normal Pressure Hydrocephalus

About this section
Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Normal Pressure Hydrocephalus

Drug clinical trials:

Search ClinicalTrials for Normal Pressure Hydrocephalus

Search NIH Clinical Center for Normal Pressure Hydrocephalus

Search CenterWatch for Normal Pressure Hydrocephalus

Genetic Tests for Normal Pressure Hydrocephalus

About this section

Anatomical Context for Normal Pressure Hydrocephalus

About this section
Sources:
34MalaCards
See all sources

MalaCards organs/tissues related to Normal Pressure Hydrocephalus:

34
Brain, Spinal cord, Testes, Thalamus, Bone, Eye, Pituitary, Cortex, Olfactory bulb

Animal Models for Normal Pressure Hydrocephalus or affiliated genes

About this section
Sources:
38MGI
See all sources

MGI Mouse Phenotypes related to Normal Pressure Hydrocephalus:

38 (show all 21)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053949.6BDNF, MAPT, APOE
2MP:00053759.0APOE, TGFBR2, TGFB1, ACE, NPY
3MP:00020068.9PTGDS, TGFBR2, TGFB1, ACE, MAPT
4MP:00053888.9BDNF, VIP, TGFB1, TGFBR2, APOE
5MP:00053818.8GFAP, APOE, TGFBR2, TGFB1, NPY, BDNF
6MP:00053708.7APOE, TGFBR2, TGFB1, ACE, NPY
7MP:00053918.7GFAP, APOE, TGFBR2, TGFB1, MAPT, BDNF
8MP:00028738.7GFAP, APP, TGFBR2, VIP, MAPT, NPY
9MP:00053798.7APOE, TGFBR2, TGFB1, VIP, ACE, GAL
10MP:00053678.6NPY, MAPT, ACE, TGFB1, APOE
11MP:00030128.6PTGDS, APOE, APP, MAPT, BDNF
12MP:00053698.6GFAP, APOE, APP, TGFBR2, TGFB1, MAPT
13MP:00107718.5PTGDS, APOE, APP, TGFB1, MAPT, GAL
14MP:00053898.4APOE, APP, TGFB1, VIP, ACE, GAL
15MP:00053877.9GFAP, PTGDS, APOE, APP, TGFBR2, TGFB1
16MP:00107687.6GFAP, PTGDS, APOE, APP, TGFBR2, TGFB1
17MP:00053787.5TGFBR2, APP, APOE, GFAP, TGFB1, ACE
18MP:00053767.4GFAP, PTGDS, APOE, APP, TGFBR2, TGFB1
19MP:00036317.1TGFBR2, APP, APOE, GFAP, TGFB1, SNPH
20MP:00053847.0TGFBR2, APP, APOE, GFAP, TGFB1, ACE
21MP:00053866.3BDNF, GFAP, PTGDS, APOE, APP, TGFBR2

Publications for Normal Pressure Hydrocephalus

About this section
Sources:
53PubMed
See all sources

Articles related to Normal Pressure Hydrocephalus:

(show top 50)    (show all 506)
idTitleAuthorsYear
1
CSF dynamic analysis of a predictive pulsatility-based infusion test for normal pressure hydrocephalus. (24151060)
2014
2
Journal club: role of endoscopic third ventriculostomy and ventriculoperitoneal shunt in idiopathic normal pressure hydrocephalus: preliminary results of a randomized clinical trial. (24141398)
2013
3
Normal pressure hydrocephalus in the spectrum of neurological complications of systemic lupus erythematosus. (22829132)
2013
4
Normal pressure hydrocephalus mimicking Alzheimer's disease: such an infrequent case? (23863346)
2013
5
Design and benchmark testing of a bicorporal pump for the treatment of normal-pressure hydrocephalus and idiopathic intracranial hypertension. (23215632)
2013
6
Poor cognitive outcome in shunt-responsive idiopathic normal pressure hydrocephalus. (23037817)
2013
7
EEG and Neuronal Activity Topography analysis can predict effectiveness of shunt operation in idiopathic normal pressure hydrocephalus patients. (24273735)
2013
8
Assessment of a quick reference table algorithm for determining initial postoperative pressure settings of programmable pressure valves in patients with idiopathic normal pressure hydrocephalus: SINPHONI subanalysis. (22653392)
2012
9
What is the appropriate shunt system for normal pressure hydrocephalus? (22116436)
2012
10
The CSF tap test in normal pressure hydrocephalus: evaluation time, reliability and the influence of pain. (21801282)
2012
11
Lumboperitoneal shunts for the treatment of normal pressure hydrocephalus. (22705135)
2012
12
Secondary normal pressure hydrocephalus in a patient with isolated frontal dilatation--an insight into pathophysiology? (22083852)
2012
13
The value of the cerebrospinal fluid tap test for predicting shunt effectiveness in idiopathic normal pressure hydrocephalus. (22239832)
2012
14
White matter involvement in idiopathic normal pressure hydrocephalus: a voxel-based diffusion tensor imaging study. (21512742)
2011
15
Normal pressure hydrocephalus: measure twice, shunt once. (21849649)
2011
16
Is the midbrain involved in the manifestation of gait disturbance in idiopathic normal-pressure hydrocephalus? (21132323)
2011
17
Potential placebo effect in assessing idiopathic normal pressure hydrocephalus. (21250799)
2011
18
Normal pressure hydrocephalus after gamma knife radiosurgery for vestibular schwannoma. (20739769)
2010
19
Normal-pressure hydrocephalus and cerebral blood flow. (20367076)
2010
20
CSF neurotransmitter metabolites and neuropsychiatric symptomatology in patients with normal pressure hydrocephalus. (19046799)
2009
21
Normal pressure hydrocephalus after radiosurgery for sphenoid ridge meningioma. (19013812)
2009
22
Morphologic features and flow void phenomenon in normal pressure hydrocephalus and other dementias: are they really significant? (19717316)
2009
23
Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. (19404152)
2009
24
Idiopathic normal pressure hydrocephalus: thoughts on etiology and pathophysiology. (19640656)
2009
25
Normal pressure hydrocephalus or neuroborreliosis? (19225737)
2009
26
Neuroradiological diagnosis of idiopathic normal-pressure hydrocephalus: the search for the Holy Grail. (19847909)
2009
27
Idiopathic normal pressure hydrocephalus: increased supplementary motor activity accounts for improvement after CSF drainage. (18931387)
2008
28
Idiopathic normal pressure hydrocephalus: new findings and thoughts on etiology. (18192342)
2008
29
Sensitivity and predictive value of occupational and physical therapy assessments in the functional evaluation of patients with suspected normal pressure hydrocephalus. (18843422)
2008
30
Changes in intracranial pulse pressure amplitudes after shunt implantation and adjustment of shunt valve opening pressure in normal pressure hydrocephalus. (18936877)
2008
31
Gait disturbance in normal pressure hydrocephalus: a clinical study. (17052947)
2007
32
Cerebrospinal fluid tau, phospho-tau181 and beta-amyloid1-42 in idiopathic normal pressure hydrocephalus: a discrimination from Alzheimer's disease. (17250725)
2007
33
Noninvasive MRI assessment of intracranial compliance in idiopathic normal pressure hydrocephalus. (17610284)
2007
34
Intracranial pressure parameters in idiopathic normal pressure hydrocephalus patients with or without improvement of cognitive function after shunt treatment. (17077633)
2007
35
Proton magnetic resonance spectroscopy in patients with normal pressure hydrocephalus. (24351260)
2006
36
Normal pressure hydrocephalus. (16913194)
2006
37
Normal pressure hydrocephalus: dementia's hidden cause. (16862055)
2006
38
Is it possible to optimize treatment of patients with idiopathic normal pressure hydrocephalus by implanting an adjustable Medos Hakim valve in combination with a Miethke shunt assistant? (16671490)
2006
39
Polyneuropathy complicating the diagnosis of normal pressure hydrocephalus: case report and review of literature. (17100034)
2006
40
Clinical outcome of patients with idiopathic normal pressure hydrocephalus three years after shunt implantation. (16671489)
2006
41
Broadening a classic clinical triad: The hypokinetic motor disorder of normal pressure hydrocephalus also affects the hand. (16375891)
2006
42
Baseline neuropsychological profile and cognitive response to cerebrospinal fluid shunting for idiopathic normal pressure hydrocephalus. (16020945)
2005
43
The value of supplemental prognostic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus. (16160426)
2005
44
Angiotensin I converting enzyme polymorphism effects in patients with normal pressure hydrocephalus syndrome before and after surgery. (15729525)
2005
45
Pulse wave encephalopathy: a spectrum hypothesis incorporating Alzheimer's disease, vascular dementia and normal pressure hydrocephalus. (14962623)
2004
46
Changes in cerebral blood flow during cerebrospinal fluid pressure manipulation in patients with normal pressure hydrocephalus: a methodological study. (15129190)
2004
47
Is a combination of Tc-SPECT or perfusion weighted magnetic resonance imaging with spinal tap test helpful in the diagnosis of normal pressure hydrocephalus? (12640067)
2003
48
Clinical experiences with different valve systems in patients with normal-pressure hydrocephalus: evaluation of the Miethke dual-switch valve. (12172931)
2002
49
Shunt related changes in somatostatin, neuropeptide Y, and corticotropin releasing factor concentrations in patients with normal pressure hydrocephalus. (11181849)
2001
50
Persistence of intraventricular air after pneumoencephalography: its failure to distinguish between cerebral atrophy and normal pressure hydrocephalus. (1226627)
1975

Variations for Normal Pressure Hydrocephalus

About this section

Expression for genes affiliated with Normal Pressure Hydrocephalus

About this section
Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Normal Pressure Hydrocephalus

Search GEO for disease gene expression data for Normal Pressure Hydrocephalus.

Pathways for genes affiliated with Normal Pressure Hydrocephalus

About this section
Sources:
51PathCards, 39NCBI BioSystems Database, 55R&D Systems, 54QIAGEN, 52PharmGKB, 31KEGG, 5Cell Signaling Technology, 56Reactome
See all sources

Compounds for genes affiliated with Normal Pressure Hydrocephalus

About this section
Sources:
46Novoseek, 12DrugBank, 30IUPHAR, 52PharmGKB, 25HMDB, 62Tocris Bioscience
See all sources

Compounds related to Normal Pressure Hydrocephalus according to GeneCards/GeneDecks:

(show top 50)    (show all 166)
idCompoundScoreTop Affiliating Genes
1helospectin4610.1GAL, NPY, VIP
2secretoneurin4610.0VIP, GAL, NPY
3trifluoroethanol46 1210.8VIP, NPY, MAPT, GAL
4thioflavine s469.8APP, MAPT, APOE
5phosphoramidon46 1210.6APP, VIP, ACE, NPY
6capsaicin46 3010.6GAL, VIP, NPY, BDNF
7formate469.5APOE, MAPT, GFAP, APP
8alpha msh469.5GAL, VIP, NPY
96-hydroxydopamine469.4BDNF, APP, VIP, GFAP, NPY
10atropine46 30 1211.4APP, VIP, GAL, NPY
11tacrine46 1210.4APOE, MAPT, APP
12donepezil46 52 25 1212.4APP, APOE, MAPT
135-hydroxytryptamine469.4GFAP, GAL, NPY, VIP, APP
14acth469.4BDNF, NPY, GAL, VIP, GFAP
15tetrodotoxin46 62 3011.3NPY, GFAP, APOE, BDNF, VIP, GAL
16streptozotocin469.3BDNF, NPY, TGFB1, GFAP, APOE
17isoproterenol46 1210.3GFAP, MAPT, VIP, NPY, APP
18nifedipine46 30 52 1212.2TGFB1, VIP, ACE, BDNF, NPY
19corticosterone46 62 2511.2GFAP, GAL, NPY, BDNF, VIP, APP
20gaba469.2BDNF, APP, GAL, VIP, NPY, GFAP
21gnrh469.2APP, BDNF, GAL, NPY, VIP, TGFBR2
22carbachol46 30 1211.2APP, VIP, GAL, MAPT, BDNF, NPY
23histamine46 30 2511.1TGFB1, PTGDS, BDNF, NPY, GAL, VIP
24verapamil46 30 52 25 1213.1VIP, GFAP, APP, ACE, TGFB1
25valine469.1APOE, BDNF, MAPT, VIP, APP
26norepinephrine46 25 1211.0ACE, BDNF, GAL, APP, VIP, NPY
27nmda46 3010.0APOE, GFAP, APP, MAPT, NPY, BDNF
28kainate46 3010.0TGFB1, GFAP, BDNF, APOE, NPY, MAPT
29nicotine46 30 52 1212.0APOE, NPY, APP, TGFB1, MAPT
30formaldehyde46 259.9NPY, GAL, MAPT, GFAP, APP, TGFB1
31adenylate468.9BDNF, GFAP, TGFBR2, GAL, NPY, VIP
32lactate468.8BDNF, NPY, MAPT, VIP, TGFB1, GFAP
33actinomycin d468.7BDNF, APOE, MAPT, TGFBR2, APP, TGFB1
34cyclosporin a46 30 6210.7MAPT, VIP, TGFBR2, APP, APOE, TGFB1
35arginine468.6MAPT, GFAP, APOE, APP, VIP, GAL
36cholesterol46 30 25 1211.6BDNF, TGFBR2, APOE, PTGDS, ACE, GFAP
37dopamine46 30 25 1211.6MAPT, GFAP, APOE, APP, VIP, GAL
38aspartate468.5GFAP, APOE, BDNF, APP, MAPT, VIP
39choline46 25 1210.5VIP, GAL, BDNF, NPY, MAPT, TGFB1
40vegf468.4GFAP, APP, TGFBR2, TGFB1, VIP, APOE
41testosterone46 62 25 1211.3MAPT, BDNF, NPY, GAL, VIP, APP
42glutamine468.3VIP, TGFB1, TGFBR2, NPY, APOE, MAPT
43glutamate468.2NPY, GAL, VIP, MAPT, APOE, TGFBR2
44acetylcholine46 52 30 25 1212.2NPY, MAPT, VIP, GAL, APP, ACE
45retinoic acid46 259.1TGFB1, PTGDS, GFAP, APP, TGFBR2, GAL
46alanine468.1TGFB1, GFAP, APOE, TGFBR2, VIP, APP
47estrogen468.0MAPT, GFAP, PTGDS, APOE, TGFB1, VIP
48creatinine468.0APOE, NPY, MAPT, ACE, TGFB1, APP
49serine468.0MAPT, GAL, VIP, BDNF, GFAP, APOE
50cycloheximide467.9GAL, PTGDS, GFAP, MAPT, CEBPZ, TGFB1

GO Terms for genes affiliated with Normal Pressure Hydrocephalus

About this section
Sources:
17Gene Ontology
See all sources

Cellular components related to Normal Pressure Hydrocephalus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1axonGO:0304249.3MAPT, TGFB1, APP
2neuronal cell bodyGO:0430258.3APOE, TGFB1, VIP, SNPH
3extracellular spaceGO:0056158.2LRG1, PTGDS, APOE, TGFB1, ACE, NPY
4extracellular regionGO:0055767.1BDNF, LRG1, PTGDS, APOE, APP, TGFB1

Biological processes related to Normal Pressure Hydrocephalus according to GeneCards/GeneDecks:

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1feeding behaviorGO:00763110.0BDNF, NPY, GAL
2common-partner SMAD protein phosphorylationGO:0071829.9TGFB1, TGFBR2
3response to cholesterolGO:0707239.9TGFBR2, TGFB1
4negative regulation of neuroblast proliferationGO:0074069.9TGFB1, BDNF
5axon cargo transportGO:0080889.9MAPT, APP
6mononuclear cell proliferationGO:0329439.9ACE, TGFB1
7negative regulation of blood vessel endothelial cell migrationGO:0435379.7TGFB1, APOE
8pathway-restricted SMAD protein phosphorylationGO:0603899.7TGFB1, TGFBR2
9digestive tract developmentGO:0485659.7TGFB1, TGFBR2
10myeloid dendritic cell differentiationGO:0430119.6TGFBR2, TGFB1
11positive regulation of axon extensionGO:0457739.5MAPT, APOE
12agingGO:0075689.5APOE, TGFBR2, TGFB1

Molecular functions related to Normal Pressure Hydrocephalus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1lipoprotein particle bindingGO:0718139.8MAPT, APOE
2neuropeptide hormone activityGO:0051849.5NPY, GAL, VIP

Products for genes affiliated with Normal Pressure Hydrocephalus

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Normal Pressure Hydrocephalus

About this section
4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet