NPH
MCID: NRM002
MIFTS: 65

Normal Pressure Hydrocephalus (NPH) malady

Neuronal diseases, Rare diseases categories

Summaries for Normal Pressure Hydrocephalus

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NINDS:43 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 based summary: Normal Pressure Hydrocephalus, also known as NPH, is related to hydrocephalus and binswanger's disease, and has symptoms including autosomal recessive inheritance, urinary incontinence 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:64 Normal pressure hydrocephalus (NPH), also termed symptomatic hydrocephalus, is a type of brain... more...

Description from OMIM:46 236690

Aliases & Classifications for Normal Pressure Hydrocephalus

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Sources:
9Disease Ontology, 43NINDS, 11DISEASES, 48Orphanet, 61UMLS, 44Novoseek, 34MeSH, 56SNOMED-CT, 46OMIM, 27ICD10 via Orphanet, 62UMLS via Orphanet, 26ICD10
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Normal Pressure Hydrocephalus, Aliases & Descriptions:

Name: Normal Pressure Hydrocephalus 9 43 11 48
Nph 48 61
Hydrocephalus - Normal Pressure 43
Hydrocephalus, Normal Pressure 61
 
Hydrocephalus Normal Pressure 44
Chronic Adult Hydrocephalus 48
Low Pressure Hydrocephalus 9


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
normal pressure hydrocephalus:
Inheritance: Sporadic; Prevalence: 1-5/10000; Age of onset: Adulthood


External Ids:

Disease Ontology9 DOID:1572
MeSH34 D006850
OMIM46 236690
SNOMED-CT56 30753002, 69663004
ICD10 via Orphanet27 G91.2
UMLS via Orphanet62 C0020258
ICD1026 G91.2

Related Diseases for Normal Pressure Hydrocephalus

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

(show top 50)    (show all 146)
idRelated DiseaseScoreTop Affiliating Genes
1hydrocephalus33.0MAPT
2binswanger's disease31.0MAPT, GFAP
3type 1 diabetes mellitus31.0NPY, GAL, ACE
4hypoglycemia30.9GAL, NPY, BDNF
5progressive supranuclear palsy30.8MAPT, APOE
6parkinson's disease30.6BDNF, MAPT, APOE
7ischemia30.5GFAP, VIP, MAPT, GAL, BDNF
8hypertension30.4APOE, TGFB1, ACE, NPY
9dementia30.4GFAP, PTGDS, APOE, APP, MAPT, GAL
10diabetes mellitus30.3APOE, TGFB1, ACE, NPY
11hyperglycemia30.2TGFB1, ACE, GAL, NPY, BDNF
12vascular disease30.2APOE, ACE, NPY
13vascular dementia30.2MAPT, APP, APOE
14pick's disease30.2MAPT, APP, APOE
15frontotemporal dementia30.1APOE, APP, MAPT
16cerebrovascular disease30.1MAPT, ACE, VIP, APOE
17huntington's disease30.1BDNF, NPY, MAPT, APP
18multiple sclerosis29.9GFAP, PTGDS, APOE, TGFB1, NPY, BDNF
19alzheimer's disease29.9GFAP, PTGDS, APOE, APP, TGFBR2, MAPT
20cerebritis10.9
21type 2 diabetes mellitus10.6
22cerebral atrophy10.6
23macular holes10.5PTGDS
24subependymal giant cell astrocytoma10.5GFAP
25semantic dementia10.5MAPT
26brittle diabetes10.4
27ganglioneuroblastoma10.4NPY, VIP
28arachnoiditis10.4
29intracranial hypertension10.4
30subcortical arteriosclerotic encephalopathy10.4
31status epilepticus10.4BDNF, NPY
32urinary tract obstruction10.4ACE
33bulimia nervosa10.4NPY, BDNF
34neuroblastoma10.3BDNF, VIP
35bipolar disorder10.3NPY, BDNF
36hyperprolactinemia10.3GAL, VIP
37neuroendocrine tumor10.3VIP, GAL, NPY
38systemic lupus erythematosus10.3
39syringomyelia10.3
40neuronitis10.3
41gait apraxia10.3
42apraxia10.3
43lupus erythematosus10.3
44meningioma10.3
45peritonitis10.3
46polyneuropathy10.3
47arachnoid cysts10.3
48aneurysm10.3
49cerebral amyloid angiopathy10.3APP
50eating disorder10.3BDNF, NPY, GAL

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



Diseases related to normal pressure hydrocephalus

Symptoms for Normal Pressure Hydrocephalus

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Clinical features from OMIM:

236690

HPO human phenotypes related to Normal Pressure Hydrocephalus:

(show all 7)
id Description Frequency HPO Source Accession
1 autosomal recessive inheritance HP:0000007
2 urinary incontinence HP:0000020
3 dementia HP:0000726
4 global developmental delay HP:0001263
5 gait disturbance HP:0001288
6 normal pressure hydrocephalus HP:0002343
7 bowel incontinence HP:0002607

Drugs & Therapeutics for Normal Pressure Hydrocephalus

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Drug clinical trials:

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Search NIH Clinical Center for Normal Pressure Hydrocephalus

Genetic Tests for Normal Pressure Hydrocephalus

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Anatomical Context for Normal Pressure Hydrocephalus

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MalaCards organs/tissues related to Normal Pressure Hydrocephalus:

32
Brain, Spinal cord, Testes, Thalamus, Eye, Bone, Cortex, Olfactory bulb, Pituitary

Animal Models for Normal Pressure Hydrocephalus or affiliated genes

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MGI Mouse Phenotypes related to Normal Pressure Hydrocephalus:

36 (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

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Articles related to Normal Pressure Hydrocephalus:

(show top 50)    (show all 522)
idTitleAuthorsYear
1
DTI-MRI biomarkers in the search for normal pressure hydrocephalus aetiology: a review. (25345377)
2014
2
CSF dynamic analysis of a predictive pulsatility-based infusion test for normal pressure hydrocephalus. (24151060)
2014
3
Idiopathic normal pressure hydrocephalus: cerebral perfusion measured with pCASL before and repeatedly after CSF removal. (25138210)
2014
4
Journal club: role of endoscopic third ventriculostomy and ventriculoperitoneal shunt in idiopathic normal pressure hydrocephalus: preliminary results of a randomized clinical trial. (24141398)
2013
5
Normal pressure hydrocephalus in the spectrum of neurological complications of systemic lupus erythematosus. (22829132)
2013
6
Normal pressure hydrocephalus mimicking Alzheimer's disease: such an infrequent case? (23863346)
2013
7
Design and benchmark testing of a bicorporal pump for the treatment of normal-pressure hydrocephalus and idiopathic intracranial hypertension. (23215632)
2013
8
Poor cognitive outcome in shunt-responsive idiopathic normal pressure hydrocephalus. (23037817)
2013
9
EEG and Neuronal Activity Topography analysis can predict effectiveness of shunt operation in idiopathic normal pressure hydrocephalus patients. (24273735)
2013
10
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
11
What is the appropriate shunt system for normal pressure hydrocephalus? (22116436)
2012
12
The CSF tap test in normal pressure hydrocephalus: evaluation time, reliability and the influence of pain. (21801282)
2012
13
Lumboperitoneal shunts for the treatment of normal pressure hydrocephalus. (22705135)
2012
14
Secondary normal pressure hydrocephalus in a patient with isolated frontal dilatation--an insight into pathophysiology? (22083852)
2012
15
White matter involvement in idiopathic normal pressure hydrocephalus: a voxel-based diffusion tensor imaging study. (21512742)
2011
16
Normal pressure hydrocephalus: measure twice, shunt once. (21849649)
2011
17
Is the midbrain involved in the manifestation of gait disturbance in idiopathic normal-pressure hydrocephalus? (21132323)
2011
18
Potential placebo effect in assessing idiopathic normal pressure hydrocephalus. (21250799)
2011
19
Normal pressure hydrocephalus after gamma knife radiosurgery for vestibular schwannoma. (20739769)
2010
20
Normal-pressure hydrocephalus and cerebral blood flow. (20367076)
2010
21
CSF neurotransmitter metabolites and neuropsychiatric symptomatology in patients with normal pressure hydrocephalus. (19046799)
2009
22
Normal pressure hydrocephalus after radiosurgery for sphenoid ridge meningioma. (19013812)
2009
23
Morphologic features and flow void phenomenon in normal pressure hydrocephalus and other dementias: are they really significant? (19717316)
2009
24
Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. (19404152)
2009
25
Idiopathic normal pressure hydrocephalus: thoughts on etiology and pathophysiology. (19640656)
2009
26
Normal pressure hydrocephalus or neuroborreliosis? (19225737)
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

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Expression for genes affiliated with Normal Pressure Hydrocephalus

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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

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Compounds for genes affiliated with Normal Pressure Hydrocephalus

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

(show top 50)    (show all 166)
idCompoundScoreTop Affiliating Genes
1helospectin4410.1GAL, NPY, VIP
2secretoneurin4410.0VIP, GAL, NPY
3trifluoroethanol44 1210.8VIP, NPY, MAPT, GAL
4thioflavine s449.8APP, MAPT, APOE
5phosphoramidon44 1210.6APP, VIP, ACE, NPY
6capsaicin44 2910.6GAL, VIP, NPY, BDNF
7formate449.5APOE, MAPT, GFAP, APP
8alpha msh449.5GAL, VIP, NPY
96-hydroxydopamine449.4BDNF, APP, VIP, GFAP, NPY
10atropine44 29 1211.4APP, VIP, GAL, NPY
11tacrine44 1210.4APOE, MAPT, APP
12donepezil44 50 25 1212.4APP, APOE, MAPT
135-hydroxytryptamine449.4GFAP, GAL, NPY, VIP, APP
14acth449.4BDNF, NPY, GAL, VIP, GFAP
15tetrodotoxin44 60 2911.3NPY, GFAP, APOE, BDNF, VIP, GAL
16streptozotocin449.3BDNF, NPY, TGFB1, GFAP, APOE
17isoproterenol44 1210.3GFAP, MAPT, VIP, NPY, APP
18nifedipine44 29 50 1212.2TGFB1, VIP, ACE, BDNF, NPY
19corticosterone44 60 2511.2GFAP, GAL, NPY, BDNF, VIP, APP
20gaba449.2BDNF, APP, GAL, VIP, NPY, GFAP
21gnrh449.2APP, BDNF, GAL, NPY, VIP, TGFBR2
22carbachol44 29 1211.2APP, VIP, GAL, MAPT, BDNF, NPY
23histamine44 29 2511.1TGFB1, PTGDS, BDNF, NPY, GAL, VIP
24verapamil44 29 50 25 1213.1VIP, GFAP, APP, ACE, TGFB1
25valine449.1APOE, BDNF, MAPT, VIP, APP
26norepinephrine44 25 1211.0ACE, BDNF, GAL, APP, VIP, NPY
27nmda44 2910.0APOE, GFAP, APP, MAPT, NPY, BDNF
28kainate44 2910.0TGFB1, GFAP, BDNF, APOE, NPY, MAPT
29nicotine44 29 50 1212.0APOE, NPY, APP, TGFB1, MAPT
30formaldehyde44 259.9NPY, GAL, MAPT, GFAP, APP, TGFB1
31adenylate448.9BDNF, GFAP, TGFBR2, GAL, NPY, VIP
32lactate448.8BDNF, NPY, MAPT, VIP, TGFB1, GFAP
33actinomycin d448.7BDNF, APOE, MAPT, TGFBR2, APP, TGFB1
34cyclosporin a44 29 6010.7MAPT, VIP, TGFBR2, APP, APOE, TGFB1
35arginine448.6MAPT, GFAP, APOE, APP, VIP, GAL
36cholesterol44 29 25 1211.6BDNF, TGFBR2, APOE, PTGDS, ACE, GFAP
37dopamine44 29 25 1211.6MAPT, GFAP, APOE, APP, VIP, GAL
38aspartate448.5GFAP, APOE, BDNF, APP, MAPT, VIP
39choline44 25 1210.5VIP, GAL, BDNF, NPY, MAPT, TGFB1
40vegf448.4GFAP, APP, TGFBR2, TGFB1, VIP, APOE
41testosterone44 60 25 1211.3MAPT, BDNF, NPY, GAL, VIP, APP
42glutamine448.3VIP, TGFB1, TGFBR2, NPY, APOE, MAPT
43glutamate448.2NPY, GAL, VIP, MAPT, APOE, TGFBR2
44acetylcholine44 50 29 25 1212.2NPY, MAPT, VIP, GAL, APP, ACE
45retinoic acid44 259.1TGFB1, PTGDS, GFAP, APP, TGFBR2, GAL
46alanine448.1TGFB1, GFAP, APOE, TGFBR2, VIP, APP
47estrogen448.0MAPT, GFAP, PTGDS, APOE, TGFB1, VIP
48creatinine448.0APOE, NPY, MAPT, ACE, TGFB1, APP
49serine448.0MAPT, GAL, VIP, BDNF, GFAP, APOE
50cycloheximide447.9GAL, PTGDS, GFAP, MAPT, CEBPZ, TGFB1

GO Terms for genes affiliated with Normal Pressure Hydrocephalus

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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

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Sources for Normal Pressure Hydrocephalus

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
29IUPHAR
30KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet