MCID: NRM002
MIFTS: 64

Normal Pressure Hydrocephalus malady

Neuronal diseases, Rare diseases categories

Summaries for Normal Pressure Hydrocephalus

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NINDS:44 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 dementia, 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, testes and spinal cord, and related mouse phenotypes are taste/olfaction and adipose tissue.

Wikipedia:65 Normal pressure hydrocephalus (NPH), also termed symptomatic hydrocephalus, is a type of brain... more...

Aliases & Classifications for Normal Pressure Hydrocephalus

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Sources:
10Disease Ontology, 44NINDS, 12DISEASES, 49Orphanet, 62UMLS, 45Novoseek, 57SNOMED-CT, 35MeSH, 28ICD10 via Orphanet, 63UMLS via Orphanet, 27ICD10
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Normal Pressure Hydrocephalus, Aliases & Descriptions:

Name: Normal Pressure Hydrocephalus 10 44 12 49
Nph 49 62
Hydrocephalus - Normal Pressure 44
Hydrocephalus, Normal Pressure 62
 
Hydrocephalus Normal Pressure 45
Chronic Adult Hydrocephalus 49
Low Pressure Hydrocephalus 10


Classifications:

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


Characteristics (Orphanet epidemiological data):

49
normal pressure hydrocephalus:
Inheritance: Not applicable; Age of onset: Adult


External Ids:

Disease Ontology10 DOID:1572
MeSH35 D006850
Orphanet49 314928
SNOMED-CT57 30753002, 69663004
ICD10 via Orphanet28 G91.2
UMLS via Orphanet63 C0020258
ICD1027 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 137)
idRelated DiseaseScoreTop Affiliating Genes
1hydrocephalus32.4MAPT
2dementia31.1GFAP, PTGDS, APOE, APP, MAPT, GAL
3alzheimer disease30.6GFAP, PTGDS, APOE, APP, TGFBR2, MAPT
4hypoglycemia30.6GAL, NPY, BDNF
5hyperglycemia30.5TGFB1, ACE, GAL, NPY, BDNF
6ischemia30.4GFAP, VIP, MAPT, GAL, BDNF
7diabetes mellitus, insulin-dependent30.4NPY, GAL, ACE
8vascular dementia30.4MAPT, APP, APOE
9huntington disease30.4BDNF, NPY, MAPT, APP
10binswanger's disease30.3MAPT, GFAP
11vascular disease30.2APOE, ACE, NPY
12cerebrovascular disease30.2APOE, APP, ACE
13cerebritis10.9
14cerebral atrophy10.5
15brittle diabetes10.4
16arachnoiditis10.4
17intracranial hypertension10.4
18subcortical arteriosclerotic encephalopathy10.4
19systemic lupus erythematosus10.3
20syringomyelia10.3
21neuronitis10.3
22gait apraxia10.3
23apraxia10.3
24lupus erythematosus10.3
25peritonitis10.3
26polyneuropathy10.3
27arachnoid cysts10.3
28aneurysm10.3
29tremor10.3
30diabetes mellitus, noninsulin-dependent10.3
31diabetic ketoacidosis10.3
32maturity-onset diabetes of the young10.3
33maternally inherited diabetes and deafness10.3
34schizophrenia10.2
35rheumatoid arthritis10.2
36obsessive-compulsive disorder10.2
37essential tremor10.2
38cockayne syndrome, type a10.2
39cockayne syndrome, type b10.2
40arthritis10.2
41hypoglycemic coma10.2
42parametritis10.2
43herpes zoster10.2
44cerebellopontine angle meningioma10.2
45pseudotumor cerebri10.2
46prosopagnosia10.2
47paraganglioma10.2
48neuroleptic malignant syndrome10.2
49agnosia10.2
50cervicitis10.2

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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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:

Search ClinicalTrials for Normal Pressure Hydrocephalus

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:

33
Brain, Testes, Spinal cord, 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:

37 (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 530)
idTitleAuthorsYear
1
Optimized surgical treatment for normal pressure hydrocephalus: comparison between gravitational and differential pressure valves. (25666108)
2015
2
Dynamic state of water molecular displacement of the brain during the cardiac cycle in idiopathic normal pressure hydrocephalus. (25614094)
2015
3
DTI-MRI biomarkers in the search for normal pressure hydrocephalus aetiology: a review. (25345377)
2014
4
CSF dynamic analysis of a predictive pulsatility-based infusion test for normal pressure hydrocephalus. (24151060)
2014
5
Idiopathic normal pressure hydrocephalus: cerebral perfusion measured with pCASL before and repeatedly after CSF removal. (25138210)
2014
6
Journal club: role of endoscopic third ventriculostomy and ventriculoperitoneal shunt in idiopathic normal pressure hydrocephalus: preliminary results of a randomized clinical trial. (24141398)
2013
7
Normal pressure hydrocephalus in the spectrum of neurological complications of systemic lupus erythematosus. (22829132)
2013
8
Normal pressure hydrocephalus mimicking Alzheimer's disease: such an infrequent case? (23863346)
2013
9
Design and benchmark testing of a bicorporal pump for the treatment of normal-pressure hydrocephalus and idiopathic intracranial hypertension. (23215632)
2013
10
Poor cognitive outcome in shunt-responsive idiopathic normal pressure hydrocephalus. (23037817)
2013
11
EEG and Neuronal Activity Topography analysis can predict effectiveness of shunt operation in idiopathic normal pressure hydrocephalus patients. (24273735)
2013
12
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
13
The CSF tap test in normal pressure hydrocephalus: evaluation time, reliability and the influence of pain. (21801282)
2012
14
Lumboperitoneal shunts for the treatment of normal pressure hydrocephalus. (22705135)
2012
15
Secondary normal pressure hydrocephalus in a patient with isolated frontal dilatation--an insight into pathophysiology? (22083852)
2012
16
White matter involvement in idiopathic normal pressure hydrocephalus: a voxel-based diffusion tensor imaging study. (21512742)
2011
17
Normal pressure hydrocephalus: measure twice, shunt once. (21849649)
2011
18
Is the midbrain involved in the manifestation of gait disturbance in idiopathic normal-pressure hydrocephalus? (21132323)
2011
19
Potential placebo effect in assessing idiopathic normal pressure hydrocephalus. (21250799)
2011
20
Normal pressure hydrocephalus after gamma knife radiosurgery for vestibular schwannoma. (20739769)
2010
21
Normal-pressure hydrocephalus and cerebral blood flow. (20367076)
2010
22
CSF neurotransmitter metabolites and neuropsychiatric symptomatology in patients with normal pressure hydrocephalus. (19046799)
2009
23
Normal pressure hydrocephalus after radiosurgery for sphenoid ridge meningioma. (19013812)
2009
24
Morphologic features and flow void phenomenon in normal pressure hydrocephalus and other dementias: are they really significant? (19717316)
2009
25
Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. (19404152)
2009
26
Idiopathic normal pressure hydrocephalus: thoughts on etiology and pathophysiology. (19640656)
2009
27
Normal pressure hydrocephalus or neuroborreliosis? (19225737)
2009
28
Idiopathic normal pressure hydrocephalus: increased supplementary motor activity accounts for improvement after CSF drainage. (18931387)
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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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
1helospectin4510.1GAL, NPY, VIP
2secretoneurin4510.0VIP, GAL, NPY
3trifluoroethanol45 1310.8VIP, NPY, MAPT, GAL
4thioflavine s459.8APP, MAPT, APOE
5phosphoramidon45 1310.6APP, VIP, ACE, NPY
6capsaicin45 3010.6GAL, VIP, NPY, BDNF
7formate459.5APOE, MAPT, GFAP, APP
8alpha msh459.5GAL, VIP, NPY
96-hydroxydopamine459.4BDNF, APP, VIP, GFAP, NPY
10atropine45 30 1311.4APP, VIP, GAL, NPY
11tacrine45 1310.4APOE, MAPT, APP
12donepezil45 51 26 1312.4APP, APOE, MAPT
135-hydroxytryptamine459.4GFAP, GAL, NPY, VIP, APP
14acth459.4BDNF, NPY, GAL, VIP, GFAP
15tetrodotoxin45 61 3011.3NPY, GFAP, APOE, BDNF, VIP, GAL
16streptozotocin459.3BDNF, NPY, TGFB1, GFAP, APOE
17isoproterenol45 1310.3GFAP, MAPT, VIP, NPY, APP
18nifedipine45 30 51 1312.2TGFB1, VIP, ACE, BDNF, NPY
19corticosterone45 61 2611.2GFAP, GAL, NPY, BDNF, VIP, APP
20gaba459.2BDNF, APP, GAL, VIP, NPY, GFAP
21gnrh459.2APP, BDNF, GAL, NPY, VIP, TGFBR2
22carbachol45 30 1311.2APP, VIP, GAL, MAPT, BDNF, NPY
23histamine45 30 2611.1TGFB1, PTGDS, BDNF, NPY, GAL, VIP
24verapamil45 30 51 26 1313.1VIP, GFAP, APP, ACE, TGFB1
25valine459.1APOE, BDNF, MAPT, VIP, APP
26norepinephrine45 26 1311.0ACE, BDNF, GAL, APP, VIP, NPY
27nmda45 3010.0APOE, GFAP, APP, MAPT, NPY, BDNF
28kainate45 3010.0TGFB1, GFAP, BDNF, APOE, NPY, MAPT
29nicotine45 30 51 1312.0APOE, NPY, APP, TGFB1, MAPT
30formaldehyde45 269.9NPY, GAL, MAPT, GFAP, APP, TGFB1
31adenylate458.9BDNF, GFAP, TGFBR2, GAL, NPY, VIP
32lactate458.8BDNF, NPY, MAPT, VIP, TGFB1, GFAP
33actinomycin d458.7BDNF, APOE, MAPT, TGFBR2, APP, TGFB1
34cyclosporin a45 30 6110.7MAPT, VIP, TGFBR2, APP, APOE, TGFB1
35arginine458.6MAPT, GFAP, APOE, APP, VIP, GAL
36cholesterol45 30 26 1311.6BDNF, TGFBR2, APOE, PTGDS, ACE, GFAP
37dopamine45 30 26 1311.6MAPT, GFAP, APOE, APP, VIP, GAL
38aspartate458.5GFAP, APOE, BDNF, APP, MAPT, VIP
39choline45 26 1310.5VIP, GAL, BDNF, NPY, MAPT, TGFB1
40vegf458.4GFAP, APP, TGFBR2, TGFB1, VIP, APOE
41testosterone45 61 26 1311.3MAPT, BDNF, NPY, GAL, VIP, APP
42glutamine458.3VIP, TGFB1, TGFBR2, NPY, APOE, MAPT
43glutamate458.2NPY, GAL, VIP, MAPT, APOE, TGFBR2
44acetylcholine45 51 30 26 1312.2NPY, MAPT, VIP, GAL, APP, ACE
45retinoic acid45 269.1TGFB1, PTGDS, GFAP, APP, TGFBR2, GAL
46alanine458.1TGFB1, GFAP, APOE, TGFBR2, VIP, APP
47estrogen458.0MAPT, GFAP, PTGDS, APOE, TGFB1, VIP
48creatinine458.0APOE, NPY, MAPT, ACE, TGFB1, APP
49serine458.0MAPT, GAL, VIP, BDNF, GFAP, APOE
50cycloheximide457.9GAL, 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:00304249.3MAPT, TGFB1, APP
2neuronal cell bodyGO:00430258.3APOE, TGFB1, VIP, SNPH
3extracellular spaceGO:00056158.2LRG1, PTGDS, APOE, TGFB1, ACE, NPY
4extracellular regionGO:00055767.1BDNF, 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:000763110.0BDNF, NPY, GAL
2common-partner SMAD protein phosphorylationGO:00071829.9TGFB1, TGFBR2
3response to cholesterolGO:00707239.9TGFBR2, TGFB1
4negative regulation of neuroblast proliferationGO:00074069.9TGFB1, BDNF
5axon cargo transportGO:00080889.9MAPT, APP
6mononuclear cell proliferationGO:00329439.9ACE, TGFB1
7negative regulation of blood vessel endothelial cell migrationGO:00435379.7TGFB1, APOE
8pathway-restricted SMAD protein phosphorylationGO:00603899.7TGFB1, TGFBR2
9digestive tract developmentGO:00485659.7TGFB1, TGFBR2
10myeloid dendritic cell differentiationGO:00430119.6TGFBR2, TGFB1
11positive regulation of axon extensionGO:00457739.5MAPT, APOE
12agingGO:00075689.5APOE, TGFBR2, TGFB1

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

idNameGO IDScoreTop Affiliating Genes
1lipoprotein particle bindingGO:00718139.8MAPT, APOE
2neuropeptide hormone activityGO:00051849.5NPY, GAL, VIP

Products for genes affiliated with Normal Pressure Hydrocephalus

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Sources for Normal Pressure Hydrocephalus

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4CDC
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet