MCID: NRM002
MIFTS: 56

Normal Pressure Hydrocephalus malady

Neuronal diseases, Rare diseases categories

Aliases & Classifications for Normal Pressure Hydrocephalus

About this section
Sources:
9Disease Ontology, 42NINDS, 11DISEASES, 43Novoseek, 47Orphanet, 60UMLS, 55SNOMED-CT, 33MeSH, 26ICD10 via Orphanet, 61UMLS via Orphanet, 25ICD10
See all sources

Normal Pressure Hydrocephalus, Aliases & Descriptions:

Name: Normal Pressure Hydrocephalus 9 42 11 47
Hydrocephalus - Normal Pressure 42
Hydrocephalus, Normal Pressure 60
Hydrocephalus Normal Pressure 43
 
Chronic Adult Hydrocephalus 47
Low Pressure Hydrocephalus 9
Nph 47


Classifications:

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


Characteristics (Orphanet epidemiological data):

47
normal pressure hydrocephalus:
Inheritance: Not applicable; Age of onset: Adult


External Ids:

Disease Ontology9 DOID:1572
MeSH33 D006850
Orphanet47 314928
SNOMED-CT55 30753002, 69663004
ICD10 via Orphanet26 G91.2
UMLS via Orphanet61 C0020258
ICD1025 G91.2

Summaries for Normal Pressure Hydrocephalus

About this section


NINDS:42 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 hydrocephalus - normal pressure, is related to hydrocephalus and diabetes mellitus, insulin-dependent, 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:63 Normal pressure hydrocephalus (NPH), also termed symptomatic hydrocephalus, is a type of brain... more...

Related Diseases for Normal Pressure Hydrocephalus

About this section

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

(show top 50)    (show all 135)
idRelated DiseaseScoreTop Affiliating Genes
1hydrocephalus33.0MAPT
2diabetes mellitus, insulin-dependent31.5NPY, GAL, ACE
3dementia31.1APOE, APP, MAPT
4hypoglycemia30.9GAL, NPY, BDNF
5binswanger's disease30.7MAPT, GFAP
6ischemia30.4GFAP, VIP, MAPT, GAL, BDNF
7vascular dementia30.4MAPT, APP, APOE
8hyperglycemia30.3TGFB1, ACE, GAL, NPY, BDNF
9huntington disease30.3BDNF, NPY, MAPT, APP
10vascular disease30.2APOE, ACE, NPY
11cerebrovascular disease30.0APOE, APP, ACE
12alzheimer disease29.6GFAP, PTGDS, APOE, APP, TGFBR2, MAPT
13cerebritis10.9
14diabetes mellitus, ketosis-prone10.8
15diabetic ketoacidosis10.8
16brittle diabetes10.8
17diabetes mellitus, noninsulin-dependent10.7
18cerebral atrophy10.5
19macular holes10.5PTGDS
20subependymal giant cell astrocytoma10.5GFAP
21semantic dementia10.5MAPT
22ganglioneuroblastoma10.4NPY, VIP
23arachnoiditis10.4
24intracranial hypertension10.4
25subcortical arteriosclerotic encephalopathy10.4
26status epilepticus10.4BDNF, NPY
27urinary tract obstruction10.4ACE
28bulimia nervosa10.3NPY, BDNF
29neuroblastoma10.3BDNF, VIP
30neurotic disorder10.3NPY, BDNF
31hyperprolactinemia10.3GAL, VIP
32neuroendocrine tumor10.3VIP, GAL, NPY
33hereditary cerebral amyloid angiopathy10.3APP
34systemic lupus erythematosus10.3
35syringomyelia10.3
36neuronitis10.3
37gait apraxia10.3
38apraxia10.3
39lupus erythematosus10.3
40peritonitis10.3
41polyneuropathy10.3
42arachnoid cysts10.3
43aneurysm10.3
44tremor10.3
45eating disorder10.3BDNF, NPY, GAL
46anxiety disorder10.3GAL, NPY, BDNF
47anorexia nervosa10.3GAL, NPY, BDNF
48inclusion body myositis10.3APP
49mood disorder10.3GAL, NPY, BDNF
50pituitary tumors10.3GAL, VIP, TGFBR2

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

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

About this section

Drug clinical trials:

Search ClinicalTrials for Normal Pressure Hydrocephalus

Search NIH Clinical Center for Normal Pressure Hydrocephalus

Genetic Tests for Normal Pressure Hydrocephalus

About this section

Anatomical Context for Normal Pressure Hydrocephalus

About this section

MalaCards organs/tissues related to Normal Pressure Hydrocephalus:

31
Brain, Testes, Spinal cord, Thalamus, Eye, Bone, Cortex, Olfactory bulb, Pituitary

Animal Models for Normal Pressure Hydrocephalus or affiliated genes

About this section

MGI Mouse Phenotypes related to Normal Pressure Hydrocephalus:

35 (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

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

About this section

Expression for genes affiliated with Normal Pressure Hydrocephalus

About this section
Search GEO for disease gene expression data for Normal Pressure Hydrocephalus.

Pathways for genes affiliated with Normal Pressure Hydrocephalus

About this section

Compounds for genes affiliated with Normal Pressure Hydrocephalus

About this section

Compounds related to Normal Pressure Hydrocephalus according to GeneCards Suite gene sharing:

(show top 50)    (show all 166)
idCompoundScoreTop Affiliating Genes
1helospectin4310.1GAL, NPY, VIP
2secretoneurin4310.0VIP, GAL, NPY
3trifluoroethanol43 1210.8VIP, NPY, MAPT, GAL
4thioflavine s439.8APP, MAPT, APOE
5phosphoramidon43 1210.6APP, VIP, ACE, NPY
6capsaicin43 2810.6GAL, VIP, NPY, BDNF
7formate439.5APOE, MAPT, GFAP, APP
8alpha msh439.5GAL, VIP, NPY
96-hydroxydopamine439.4BDNF, APP, VIP, GFAP, NPY
10atropine43 28 1211.4APP, VIP, GAL, NPY
11tacrine43 1210.4APOE, MAPT, APP
12donepezil43 49 24 1212.4APP, APOE, MAPT
135-hydroxytryptamine439.4GFAP, GAL, NPY, VIP, APP
14acth439.4BDNF, NPY, GAL, VIP, GFAP
15tetrodotoxin43 59 2811.3NPY, GFAP, APOE, BDNF, VIP, GAL
16streptozotocin439.3BDNF, NPY, TGFB1, GFAP, APOE
17isoproterenol43 1210.3GFAP, MAPT, VIP, NPY, APP
18nifedipine43 28 49 1212.2TGFB1, VIP, ACE, BDNF, NPY
19corticosterone43 59 2411.2GFAP, GAL, NPY, BDNF, VIP, APP
20gaba439.2BDNF, APP, GAL, VIP, NPY, GFAP
21gnrh439.2APP, BDNF, GAL, NPY, VIP, TGFBR2
22carbachol43 28 1211.2APP, VIP, GAL, MAPT, BDNF, NPY
23histamine43 28 2411.1TGFB1, PTGDS, BDNF, NPY, GAL, VIP
24verapamil43 28 49 24 1213.1VIP, GFAP, APP, ACE, TGFB1
25valine439.1APOE, BDNF, MAPT, VIP, APP
26norepinephrine43 24 1211.0ACE, BDNF, GAL, APP, VIP, NPY
27nmda43 2810.0APOE, GFAP, APP, MAPT, NPY, BDNF
28kainate43 2810.0TGFB1, GFAP, BDNF, APOE, NPY, MAPT
29nicotine43 28 49 1212.0APOE, NPY, APP, TGFB1, MAPT
30formaldehyde43 249.9NPY, GAL, MAPT, GFAP, APP, TGFB1
31adenylate438.9BDNF, GFAP, TGFBR2, GAL, NPY, VIP
32lactate438.8BDNF, NPY, MAPT, VIP, TGFB1, GFAP
33actinomycin d438.7BDNF, APOE, MAPT, TGFBR2, APP, TGFB1
34cyclosporin a43 28 5910.7MAPT, VIP, TGFBR2, APP, APOE, TGFB1
35arginine438.6MAPT, GFAP, APOE, APP, VIP, GAL
36cholesterol43 28 24 1211.6BDNF, TGFBR2, APOE, PTGDS, ACE, GFAP
37dopamine43 28 24 1211.6MAPT, GFAP, APOE, APP, VIP, GAL
38aspartate438.5GFAP, APOE, BDNF, APP, MAPT, VIP
39choline43 24 1210.5VIP, GAL, BDNF, NPY, MAPT, TGFB1
40vegf438.4GFAP, APP, TGFBR2, TGFB1, VIP, APOE
41testosterone43 59 24 1211.3MAPT, BDNF, NPY, GAL, VIP, APP
42glutamine438.3VIP, TGFB1, TGFBR2, NPY, APOE, MAPT
43glutamate438.2NPY, GAL, VIP, MAPT, APOE, TGFBR2
44acetylcholine43 49 28 24 1212.2NPY, MAPT, VIP, GAL, APP, ACE
45retinoic acid43 249.1TGFB1, PTGDS, GFAP, APP, TGFBR2, GAL
46alanine438.1TGFB1, GFAP, APOE, TGFBR2, VIP, APP
47estrogen438.0MAPT, GFAP, PTGDS, APOE, TGFB1, VIP
48creatinine438.0APOE, NPY, MAPT, ACE, TGFB1, APP
49serine438.0MAPT, GAL, VIP, BDNF, GFAP, APOE
50cycloheximide437.9GAL, PTGDS, GFAP, MAPT, CEBPZ, TGFB1

GO Terms for genes affiliated with Normal Pressure Hydrocephalus

About this section

Cellular components related to Normal Pressure Hydrocephalus according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1axonGO:00304249.3TGFB1, MAPT, APP
2neuronal cell bodyGO:00430258.3TGFB1, VIP, SNPH, APOE
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 Suite gene sharing:

(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 Suite gene sharing:

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

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies
  • Proteins
  • Kits and Assays

Sources for Normal Pressure Hydrocephalus

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet