MCID: SPN340
MIFTS: 27

Spontaneous Intracranial Hypotension

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Spontaneous Intracranial Hypotension

MalaCards integrated aliases for Spontaneous Intracranial Hypotension:

Name: Spontaneous Intracranial Hypotension 58
Cerebrospinal Fluid Leak, Spontaneous 70
Spontaneous Cerebrospinal Fluid Leak 58
Intracranial Hypotension, Essential 70

Characteristics:

Orphanet epidemiological data:

58
spontaneous intracranial hypotension
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

ICD10 via Orphanet 33 G96.0
UMLS via Orphanet 71 C0751731 C3544264 C3850067
Orphanet 58 ORPHA443180
UMLS 70 C0751731 C3850067

Summaries for Spontaneous Intracranial Hypotension

MalaCards based summary : Spontaneous Intracranial Hypotension, also known as cerebrospinal fluid leak, spontaneous, is related to intracranial hypotension and normal pressure hydrocephalus. An important gene associated with Spontaneous Intracranial Hypotension is PTGDS (Prostaglandin D2 Synthase). The drugs Hemostatics and Fibrin Tissue Adhesive have been mentioned in the context of this disorder. Affiliated tissues include spinal cord and pituitary.

Wikipedia : 73 A spontaneous cerebrospinal fluid leak (sCSF leak) is a cerebrospinal fluid leak - a leak of... more...

Related Diseases for Spontaneous Intracranial Hypotension

Diseases related to Spontaneous Intracranial Hypotension via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 151)
# Related Disease Score Top Affiliating Genes
1 intracranial hypotension 31.6 TF PTGDS
2 normal pressure hydrocephalus 29.5 TF PTGDS
3 kidney disease 29.1 TF PTGDS
4 headache 11.1
5 cerebrospinal fluid leak 10.9
6 ocular motor apraxia 10.7
7 thrombosis 10.7
8 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.4
9 ifap syndrome 2 10.4
10 frontotemporal dementia 10.4
11 exostosis 10.4
12 intracranial hypertension 10.4
13 thunderclap headache 10.4
14 siderosis 10.4
15 superficial siderosis 10.4
16 ataxia and polyneuropathy, adult-onset 10.3
17 marfan syndrome 10.3
18 migraine with or without aura 1 10.3
19 hydrocephalus 10.3
20 dementia 10.3
21 cranial nerve palsy 10.3
22 tremor 10.3
23 branchiootic syndrome 1 10.3
24 abducens nerve disease 10.3
25 rhinitis 10.2
26 encephalocele 10.2
27 systemic lupus erythematosus 10.2
28 patulous eustachian tube 10.2
29 optic nerve disease 10.2
30 radiculopathy 10.2
31 movement disease 10.2
32 connective tissue disease 10.2
33 neuropathy 10.2
34 lupus erythematosus 10.2
35 chiari malformation 10.2
36 tarlov cysts 10.2
37 back pain 10.2
38 abducens palsy 10.1
39 body mass index quantitative trait locus 1 10.1
40 b-lymphoblastic leukemia/lymphoma 10.1
41 parkinsonism 10.1
42 viral meningitis 10.1
43 respiratory failure 10.1
44 acoustic neuroma 10.1
45 ehlers-danlos syndrome 10.1
46 papilledema 10.1
47 neurilemmoma 10.1
48 sagittal sinus thrombosis 10.1
49 paraplegia 10.1
50 pathologic nystagmus 10.1

Graphical network of the top 20 diseases related to Spontaneous Intracranial Hypotension:



Diseases related to Spontaneous Intracranial Hypotension

Symptoms & Phenotypes for Spontaneous Intracranial Hypotension

Drugs & Therapeutics for Spontaneous Intracranial Hypotension

Drugs for Spontaneous Intracranial Hypotension (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Hemostatics Phase 2
2 Fibrin Tissue Adhesive Phase 2
3 Coagulants Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Randomized Trial of CT Fluoroscopy-guided Targeted Autologous Blood and Fibrin Glue Patching for Treatment of Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension. Completed NCT03276975 Phase 2 TISSEEL
2 Non-invasive Estimation of CSF Pressure Using MRI in Patients With Spontaneous Intracranial Hypotension Completed NCT03041441
3 Parallel Randomised Open Blind Evaluation Study of the Efficacy of 20° Trendelenburg Position During 24 Hours After Epidural Blood Patch in the Treatment of Spontaneous Intracranial Hypotension Recruiting NCT02261792

Search NIH Clinical Center for Spontaneous Intracranial Hypotension

Genetic Tests for Spontaneous Intracranial Hypotension

Anatomical Context for Spontaneous Intracranial Hypotension

MalaCards organs/tissues related to Spontaneous Intracranial Hypotension:

40
Spinal Cord, Pituitary

Publications for Spontaneous Intracranial Hypotension

Articles related to Spontaneous Intracranial Hypotension:

(show top 50) (show all 966)
# Title Authors PMID Year
1
Cerebrospinal Fluid Analysis. 61
33788511 2021
2
Spontaneous intracranial hypotension: features, diagnosis and management. 61
33686508 2021
3
Spinal subdural hygroma. 61
33393068 2021
4
Diagnostic Yield of Lateral Decubitus Digital Subtraction Myelogram Stratified by Brain MRI Findings. 61
33472917 2021
5
Sternberg's Canal and Defect: Is the Lateral Craniopharyngeal Canal a Source of Spontaneous Cerebrospinal Fluid Leak? Anatomic and Radiological Analysis in Pediatric and Adult Populations. 61
33475703 2021
6
Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome: A Systematic Review and Meta-analysis. 61
33393980 2021
7
Facial Nerve Paralysis Due to Spontaneous Intracranial Hypotension. 61
31547709 2021
8
Neuroimaging for Spontaneous Intracranial Hypotension Turned on Its Side. 61
33472920 2021
9
[Spontaneous intracranial hypotension complicated by atlantoaxial subluxation: a case report]. 61
33627579 2021
10
[Posture-dependent headache caused by spontaneous intracranial hypotension]. 61
33793124 2021
11
Spontaneous intracranial hypotension with brain sagging causing "prayer headache". 61
32736813 2021
12
Reversible parkinsonism and FTD due to spontaneous intracranial hypotension. 61
32981686 2021
13
Spontaneous intracranial hypotension and lumbosacral spondylolisthesis-case report of a rare association. 61
33493877 2021
14
CT-guided Fibrin Glue Occlusion of Cerebrospinal Fluid-Venous Fistulas. 61
33650903 2021
15
Arachnoiditis, a complication of epidural blood patch for the treatment of low-pressure headache: A case report and systematic review. 61
33583044 2021
16
Diffuse Calvarial Hyperostosis in Patients with Spontaneous Intracranial Hypotension. 61
33220476 2021
17
Cerebral venous thrombosis due to spontaneous intracranial hypotension: Reperfusion after epidural blood patch only. 61
33648781 2021
18
The importance of repeat digital subtraction myelography in the diagnosis of cryptogenic CSF-venous fistula causing spontaneous intracranial hypotension. 61
33525918 2021
19
Evaluation and diagnosis of spontaneous intracranial hypotension with magnetic resonance imaging 61
33582651 2021
20
Spinal Compliance Curves: Preliminary Experience with a New Tool for Evaluating Suspected CSF Venous Fistulas on CT Myelography in Patients with Spontaneous Intracranial Hypotension. 61
33602750 2021
21
A systematic review and meta-analysis of factors affecting the outcome of the epidural blood patching in spontaneous intracranial hypotension. 61
33611638 2021
22
Subarachnoid hemorrhage caused by spontaneous intracranial hypotension: two rare cases report. 61
33499724 2021
23
Predictors Associated with Outcomes of Epidural Blood Patch in Patients with Spontaneous Intracranial Hypotension. 61
33670838 2021
24
Letter to the editor regarding "Unilateral chronic subdural hematoma due to spontaneous intracranial hypotension: a report of four cases". 61
33538205 2021
25
A Novel Endovascular Therapy for CSF Hypotension Secondary to CSF-Venous Fistulas. 61
33541895 2021
26
Spontaneous spinal cerebrospinal fluid-venous fistulas in patients with orthostatic headaches and normal conventional brain and spine imaging. 61
33484155 2021
27
CSF-Venous Fistula. 61
33475890 2021
28
Cerebrospinal Fluid-Venous Fistulas: A Systematic Review and Examination of Individual Patient Data. 61
33438744 2021
29
Decubitus CT Myelography for CSF-Venous Fistulas: A Procedural Approach. 61
33122215 2021
30
Brain volume changes in spontaneous intracranial hypotension: Revisiting the Monro-Kellie doctrine. 61
32847387 2021
31
Spinal CSF-Venous Fistulas in Morbidly and Super Obese Patients with Spontaneous Intracranial Hypotension. 61
33334852 2021
32
The domino effect of acephalgic spontaneous intracranial hypotension. 61
32989589 2021
33
Pseudosubarachnoid hemorrhage: A systematic review of causes, diagnostic modalities, and outcomes in patients who present with pseudosubarachnoid hemorrhage. 61
33598345 2021
34
Delayed recurrence of spontaneous intracranial hypotension syndrome mimicking a Chiari I malformation: Case report with a review of the literature. 61
33276003 2020
35
Unilateral chronic subdural hematoma due to spontaneous intracranial hypotension: a report of four cases. 61
31535558 2020
36
Clinical and radiological prognostic factors in spontaneous intracranial hypotension: a case series. 61
32845492 2020
37
Headache in spontaneous intracranial hypotension: an overview with indications for differential diagnosis in the clinical practice. 61
33034803 2020
38
Syrinx Secondary to Chiari-like Tonsillar Herniation in Spontaneous Intracranial Hypotension. 61
32711143 2020
39
Monro-Kellie Hypothesis: Increase of Ventricular CSF Volume after Surgical Closure of a Spinal Dural Leak in Patients with Spontaneous Intracranial Hypotension. 61
33177057 2020
40
An Adult Case of Hemifacial Spasm as the Predominant Manifestation of Spontaneous Intracranial Hypotension. 61
32979212 2020
41
An unusual postural headache: a case report. 61
33183329 2020
42
Postpartum Headache-Unmasking the Zebra Among the Horses: A Case Report. 61
33185404 2020
43
Traumatic vs Spontaneous Cerebrospinal Fluid Hypotension Headache: Our experience in a series of 137 cases. 61
32791437 2020
44
The relationship between spontaneous cerebrospinal fluid leak and idiopathic intracranial hypertension. 61
33257267 2020
45
Time to Resolution of Inadvertent Subdural Contrast Injection during a Myelogram: When Can the Study Be Reattempted? 61
32855185 2020
46
Spontaneous Intracranial Hypotension Complicated by Subdural Effusions Treated by Surgical Relief of Cranial Venous Outflow Obstruction. 61
33403194 2020
47
Spontaneous Intracranial Hypotension with a Reversible Splenial Lesion after Swimming. 61
32581170 2020
48
Safety of Consecutive Bilateral Decubitus Digital Subtraction Myelography in Patients with Spontaneous Intracranial Hypotension and Occult CSF Leak. 61
32883671 2020
49
Intracranial hypotension secondary to spontaneous spinal cerebrospinal fluid fistula: Three case reports. 61
33082102 2020
50
Misdiagnosis of Spontaneous Intracranial Hypotension Presenting as Acute Mental Deterioration Caused by Unilateral Acute Subdural Hematoma: Case Report. 61
33163434 2020

Variations for Spontaneous Intracranial Hypotension

Expression for Spontaneous Intracranial Hypotension

Search GEO for disease gene expression data for Spontaneous Intracranial Hypotension.

Pathways for Spontaneous Intracranial Hypotension

GO Terms for Spontaneous Intracranial Hypotension

Cellular components related to Spontaneous Intracranial Hypotension according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 perinuclear region of cytoplasm GO:0048471 8.62 TF PTGDS

Sources for Spontaneous Intracranial Hypotension

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
Content
Loading form....