MCID: HYD006
MIFTS: 70

Hydrocephalus malady

Neuronal diseases, Fetal diseases categories

Summaries for Hydrocephalus

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Sources:
42NIH Rare Diseases, 33MedlinePlus, 43NINDS, 63Wikipedia, 46OMIM, 32MalaCards
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NINDS:43 Hydrocephalus is a condition in which the primary characteristic is excessive accumulation of cerebrospinal fluid (CSF) -- the clear fluid that surrounds the brain and spinal cord. This excessive accumulation results in an abnormal dilation of the spaces in the brain called ventricles. This dilation causes potentially harmful pressure on the tissues of the brain. Hydrocephalus may be congenital or acquired. Congenital hydrocephalus is present at birth and may be caused by genetic abnormalities or Acquired hydrocephalus develops at the time of birth or at some point afterward and can affect individuals of all ages. For example, h Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to CSF. In infancy, the most obvious indication of hydrocephalus is often the rapid increase in head circumference or an unusually large head size. In older children and adults, symptoms may include headache followed by vomiting, nausea, papilledema (swelling of the optic disk, which is part of the optic nerve), downward deviation of the eyes (called "sunsetting"), problems with balance, poor coordination, gait disturbance, urinary incontinence, slowing or loss of development (in children), lethargy, drowsiness, irritability, or other changes in personality or cognition, including memory loss. Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as ultrasonography, computer tomography (CT), magnetic resonance imaging (MRI), or pressure-monitoring techniques.

MalaCards: Hydrocephalus, also known as hydrocephalus, nonsyndromic, autosomal recessive, is related to normal pressure hydrocephalus and obstructive hydrocephalus, and has symptoms including hydrocephalyand autosomal recessive inheritance. An important gene associated with Hydrocephalus is CCDC88C (coiled-coil domain containing 88C), and among its related pathways is Transcriptional Regulatory Network in Embryonic Stem Cell. The compounds paraffin and histamine have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and testes, and related mouse phenotypes are behavior/neurological and nervous system.

NIH Rare Diseases:42 Hydrocephalus is a condition characterized by excessive accumulation of fluid in the brain. although it was once known as "water on the brain," the "water" is actually cerebrospinal fluid (csf) - clear fluid that surrounds the brain and spinal cord. excess csf builds up when it cannot drain from the brain due to a blockage in a passage through which the fluid normally flows.  this excess fluid causes an abnormal widening of spaces in the brain called ventricles; this can create harmful pressure on brain tissue. symptoms vary with age, disease progression, and individual differences in tolerance to the condition. hydrocephalus may be congenital (present at birth) or acquired. the causes are not fully understood; it may result from inherited genetic abnormalities or developmental disorders; complications of premature birth; diseases such as meningitis; tumors; traumatic head injury; or other causes. it is most often treated by surgically inserting a shunt system. last updated: 8/22/2011

MedlinePlus:33 Hydrocephalus is the buildup of too much cerebrospinal fluid in the brain. normally, this fluid cushions your brain. when you have too much, though, it puts harmful pressure on your brain. hydrocephalus can be congenital, or present at birth. causes include genetic problems and problems with how the fetus develops. an unusually large head is the main sign of congenital hydrocephalus. hydrocephalus can also happen after birth. this is called acquired hydrocephalus. it can occur at any age. causes can include head injuries, strokes, infections, tumors, and bleeding in the brain. symptoms include headache vomiting and nausea blurry vision balance problems bladder control problems thinking and memory problems hydrocephalus can permanently damage the brain, causing problems with physical and mental development. if untreated, it is usually fatal. with treatment, many people lead normal lives with few limitations. treatment usually involves surgery to insert a shunt. a shunt is a flexible but sturdy plastic tube. the shunt moves the cerebrospinal fluid to another area of the body where it can be absorbed. medicine and rehabilitation therapy can also help. nih: national institute of neurological disorders and stroke

Wikipedia:63 Hydrocephalus[a]/ˌhaɪdrɵˈsɛfələs/, also known as \"water on the brain\", is a medical condition... more...

Description from OMIM:46 236600,615219

Aliases & Classifications for Hydrocephalus

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 22GTR, 43NINDS, 10DISEASES, 44Novoseek, 33MedlinePlus, 60UMLS, 9diseasecard, 46OMIM, 48Orphanet, 34MeSH, 39NCIt, 56SNOMED-CT, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 25ICD10
See all sources

Classifications:

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


Aliases & Descriptions:

hydrocephalus 8 42 22 43 10 44 33 60
hydrocephalus, nonsyndromic, autosomal recessive 8 9 46
congenital hydrocephalus 48
ventriculomegaly 42
hydrocephaly 42


External Ids:

Disease Ontology8 DOID:10908
NCIt39 C3111
ICD10 via Orphanet26 Q03.0, Q03.1, Q03.8 Q03.9, more
SNOMED-CT via Orphanet57 47032000
ICD1025 Q03

Related Diseases for Hydrocephalus

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Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Hydrocephalus Due to Aqueductal Stenosis family:

hydrocephalus Hydrocephalus Due to Congenital Stenosis of Aqueduct of Sylvius
Nonsyndromic Hydrocephalus, Ccdc88c-Related

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

(show top 50)    (show all 706)
idRelated DiseaseScoreTop Affiliating Genes
1normal pressure hydrocephalus31.6NPY, MAPT, PTGDS
2obstructive hydrocephalus31.4TSC1
3dementia30.7MAPT, PTGDS
4hypertension30.6NPY, TGFB1
5intracranial hypertension30.4TSC1
6craniosynostosis30.1TWIST1, RECQL4
7meningioma30.0TSC1, PTGDS
8glaucoma30.0AQP4, FOXC1, NPY
9multiple sclerosis29.8NPY, PTGDS, AQP4, TGFB1
10baller-gerold syndrome29.8TWIST1, RECQL4
11respiratory failure29.6NPY, TSC1
12adenocarcinoma29.6MAPT, TSC1, AQP4, TGFB1
13communicating hydrocephalus11.0
14cerebritis10.7
15meningitis10.7
16choroiditis10.5
17arachnoiditis10.5
18spina bifida10.5
19syringomyelia10.4
20alzheimer's disease10.3
21choroid plexus papilloma10.3
22hydrocephalus due to aqueductal stenosis10.3
23meningoencephalitis10.3
24polymicrogyria10.3
25dandy-walker syndrome10.2
26polydactyly10.2
27papilloma10.2
28myelomeningocele10.2
29peritonitis10.2
30l1 syndrome10.2
31neural tube defects10.2
32megalencephaly, polymicrogyria, and hydrocephalus syndrome10.2
33spina bifida cystica10.2
34myelocystocele10.2
35spina bifida aperta10.2
36vacterl hydrocephaly10.2
37arteriovenous malformation10.2
38bacterial meningitis10.2
39achondroplasia10.1
40hydranencephaly10.1
41cervicitis10.1
42bor-duane hydrocephalus contiguous gene syndrome10.1
43posterior meningocele10.1
44vacterl association with hydrocephaly, x-linked10.1
45proliferative vasculopathy and hydraencephaly-hydrocephaly syndrome10.1
46vacterl association10.1
47hypertensive encephalopathy10.1
48primary ciliary dyskinesia10.1
49neuronitis10.1
50chronic meningitis10.1

Graphical network of the top 20 diseases related to Hydrocephalus:



Diseases related to hydrocephalus

Clinical Features for Hydrocephalus

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Sources:
46OMIM, 48Orphanet
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Clinical features from OMIM:

236600,615219

Clinical synopsis from OMIM:

236600

Symptoms:

48
  • hydrocephaly
  • autosomal recessive inheritance

Drugs & Therapeutics for Hydrocephalus

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Hydrocephalus

Drug clinical trials:

Search ClinicalTrials for Hydrocephalus

Search NIH Clinical Center for Hydrocephalus

Search CenterWatch for Hydrocephalus

Genetic Tests for Hydrocephalus

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Sources:
22GTR
See all sources

Genetic tests related to Hydrocephalus:

id Genetic test Affiliating Genes
1 Hydrocephalus22

Anatomical Context for Hydrocephalus

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Sources:
30LifeMap Discovery™, 32MalaCards
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MalaCards organs/tissues related to Hydrocephalus:

32
Brain, Spinal cord, Testes, Eye, Pituitary, Pineal, Bone, Thalamus, Liver, Heart, Cortex, Endothelial, Hypothalamus, Occipital lobe, Placenta, B cells, Olfactory bulb, Spleen, Lung, Kidney

LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine

Cells/anatomical compartments in embryo or adult related to Hydrocephalus:
id TissueAnatomical CompartmentCell Relevance
1 BrainChoroid PlexusChoroid Plexus Progenitor Cells Affected by disease
2 BrainChoroid PlexusMature Choroid Plexus Cells Affected by disease

Animal Models for Hydrocephalus or affiliated genes

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Sources:
36MGI
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Publications for Hydrocephalus

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Sources:
50PubMed
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Articles related to Hydrocephalus:

(show top 50)    (show all 1890)
idTitleAuthorsYear
1
Journal club: role of endoscopic third ventriculostomy and ventriculoperitoneal shunt in idiopathic normal pressure hydrocephalus: preliminary results of a randomized clinical trial. (24141398)
2013
2
Endoscopic third ventriculostomy for obstructive hydrocephalus. (23673173)
2013
3
Repeated hydrocephalus in recurrent intraventricular neurocysticercosis: An uncommon presentation. (23546368)
2013
4
Outcome of ventriculoperitoneal shunt implantation for treatment of congenital internal hydrocephalus in dogs and cats: 36 cases (2001-2009). (23517207)
2013
5
The assessment of bulging fontanel and splitting of sutures in premature infants: an interrater reliability study by the Hydrocephalus Clinical Research Network. (23121114)
2013
6
The European iNPH Multicentre Study on the predictive values of resistance to CSF outflow and the CSF Tap Test in patients with idiopathic normal pressure hydrocephalus. (23250963)
2013
7
Hydrocephalus in 389 patients with aneurysm-associated subarachnoid hemorrhage. (23562295)
2013
8
Imaging of the entire cerebrospinal fluid volume with a multistation 3D SPACE MR sequence: feasibility study in patients with hydrocephalus. (23239062)
2013
9
Acute hydrocephalus following heroin induced leukoencephalopathy. (22983307)
2013
10
Comparative study of lumboperitoneal shunt versus ventriculoperitoneal shunt in post meningitis communicating hydrocephalus in children. (24262455)
2013
11
Shunting for normal pressure hydrocephalus in patients with neurodegenerative disorders. (23673998)
2013
12
Post-traumatic hydrocephalus after ventricular shunt placement in a Singaporean neurosurgical unit. (23415060)
2013
13
Hearing loss following ventriculoperitoneal shunt in communicating hydrocephalus patients: a pilot study. (24318317)
2013
14
Abulia and post-tap agitation in normal-pressure hydrocephalus. (23487236)
2013
15
What is the appropriate shunt system for normal pressure hydrocephalus? (22116436)
2012
16
Predictors and outcomes of shunt-dependent hydrocephalus in patients with aneurysmal sub-arachnoid hemorrhage. (22765765)
2012
17
Indications for endoscopic third ventriculostomy in normal pressure hydrocephalus. (22116437)
2012
18
Acute signs of elevated intracranial pressure caused by hydrocephalus occlusus following an intraventricular hemorrhage after falling from a highchair. (22562606)
2012
19
Hydrodynamics of the Certasa8c programmable valve for the treatment of hydrocephalus. (22643114)
2012
20
Paget's disease of bone presented as normal pressure hydrocephalus: A case report and review of literature. (24250876)
2012
21
Post-mortem findings in 10 patients with presumed normal-pressure hydrocephalus and review of the literature. (21696417)
2012
22
Frontal and temporal horn ratio: a valid and reliable index to determine ventricular size in paediatric hydrocephalus patients? (22327698)
2012
23
Pediatric hydrocephalus: Does the shunt device pressure selection affect the outcome? (22529548)
2012
24
Prognosis and treatment of acute hydrocephalus following aneurysmal subarachnoid haemorrhage. (22361252)
2012
25
Potential placebo effect in assessing idiopathic normal pressure hydrocephalus. (21250799)
2011
26
Giant syringobulbia associated with cerebellopontine angle arachnoid cyst and hydrocephalus. (21721885)
2011
27
Longitudinal evaluation of an N-ethyl-N-nitrosourea-created murine model with normal pressure hydrocephalus. (19924295)
2009
28
Idiopathic normal pressure hydrocephalus: the benefits and problems of shunting. (19194387)
2009
29
Bilateral posterior periventricular nodular heterotopia with cerebellar hypoplasia, communicating hydrocephalus and bilateral hippocampal sclerosis. A case report. (24206947)
2009
30
Normal pressure hydrocephalus and lumbar tap test. (19146632)
2009
31
Features of gait most responsive to tap test in normal pressure hydrocephalus. (18359152)
2008
32
Mechanism of bladder dysfunction in idiopathic normal pressure hydrocephalus. (18092331)
2008
33
Development of involuntary movements after ventriculoperitoneal shunting for normal pressure hydrocephalus in a patient with chronic-phase thalamic haemorrhage. (17952717)
2007
34
Idiopathic normal-pressure hydrocephalus: clinical comorbidity correlated with cerebral biopsy findings and outcome of cerebrospinal fluid shunting. (17012342)
2007
35
Megalencephaly and perisylvian polymicrogyria with postaxial polydactyly and hydrocephalus: a rare brain malformation syndrome associated with mental retardation and seizures. (15627943)
2004
36
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
37
Severe strongyloidiasis complicated by meningitis and hydrocephalus in an HTLV-1 carrier with increased proviral load. (14691660)
2003
38
Secondary amenorrhea caused by hydrocephalus due to aqueductal stenosis : report of two cases. (11511805)
2001
39
Use of a ventriculosubgaleal shunt in the management of hydrocephalus in children with posterior fossa tumors. (8869172)
1996
40
Subcortical arteriosclerotic encephalopathy (Binswanger's disease). Report of a case simulating psychiatric disease and normal pressure hydrocephalus. (3473291)
1987
41
CSF dynamics and pressure-volume relationships in communicating hydrocephalus. (3941350)
1986
42
Hydrocephalus and transient cortical blindness. (6507566)
1984
43
Concerning biological shunting-methods for the treatment of communicating hydrocephalus. (425861)
1979
44
Metrizamide and radionuclide cisternography in communicating hydrocephalus. (424538)
1979
45
Chronic communicating hydrocephalus with normal CSE pressures: a cisternographic-pathologic correlation. (4462439)
1974
46
Experimental hydrocephalus. 3. Light microscopic findings in acute and subacute obstructive hydrocephalus in the monkey. (4984824)
1970
47
Mesencephalic cyst in fourth ventricle causing obstructive hydrocephalus. (14467022)
1962
48
Benign subtentorial supracollicular cyst as a cause of obstructive hydrocephalus; report of a case. (13053260)
1953
49
Successful medical treatment of obstructive hydrocephalus in tuberculous meningitis. (15429963)
1950
50
THE OPERATIVE TREATMENT OF COMMUNICATING HYDROCEPHALUS. (17857226)
1938

Genetic Variations for Hydrocephalus

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Expression for genes affiliated with Hydrocephalus

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Hydrocephalus

Search GEO for disease gene expression data for Hydrocephalus.

Pathways for genes affiliated with Hydrocephalus

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51QIAGEN
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Pathways related to Hydrocephalus according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.5FOXC1, L1CAM, ZIC3

Compounds for genes affiliated with Hydrocephalus

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Sources:
44Novoseek, 28IUPHAR, 24HMDB
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Compounds related to Hydrocephalus according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1paraffin4410.5TGFB1, TSC1, MAPT, NPY
2histamine44 28 2412.4TGFB1, AQP4, PTGDS, NPY
3kainate44 2811.2NPY, MAPT, TGFB1

GO Terms for genes affiliated with Hydrocephalus

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16Gene Ontology
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Biological processes related to Hydrocephalus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of mitotic cell cycleGO:04593010.5TGFB1, FOXC1
2negative regulation of skeletal muscle tissue developmentGO:04864210.5TGFB1, TWIST1
3positive regulation of transcription regulatory region DNA bindingGO:200067910.4TGFB1, TWIST1
4germ cell migrationGO:00835410.2TGFB1, FOXC1

Products for genes affiliated with Hydrocephalus

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Hydrocephalus

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet