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Subarachnoid hemorrhage

MedGen UID:
11625
Concept ID:
C0038525
Disease or Syndrome
Synonyms: Hemorrhage, Subarachnoid; Hemorrhages, Subarachnoid; SAH (Subarachnoid Hemorrhage); SAHs (Subarachnoid Hemorrhage); Subarachnoid Hemorrhage; Subarachnoid Hemorrhages
SNOMED CT: SAH - Subarachnoid hemorrhage (21454007); Subarachnoid intracranial hemorrhage (21454007); Subarachnoid hemorrhage (21454007)
 
HPO: HP:0002138
Monarch Initiative: MONDO:0005099

Definition

Hemorrhage occurring between the arachnoid mater and the pia mater. [from HPO]

Conditions with this feature

Glycogen storage disease, type II
MedGen UID:
5340
Concept ID:
C0017921
Disease or Syndrome
Pompe disease is classified by age of onset, organ involvement, severity, and rate of progression. Infantile-onset Pompe disease (IOPD; individuals with onset before age 12 months with cardiomyopathy) may be apparent in utero but more typically onset is at the median age of four months with hypotonia, generalized muscle weakness, feeding difficulties, failure to thrive, respiratory distress, and hypertrophic cardiomyopathy. Without treatment by enzyme replacement therapy (ERT), IOPD commonly results in death by age two years from progressive left ventricular outflow obstruction and respiratory insufficiency. Late-onset Pompe disease (LOPD; including: (a) individuals with onset before age 12 months without cardiomyopathy; and (b) all individuals with onset after age 12 months) is characterized by proximal muscle weakness and respiratory insufficiency; clinically significant cardiac involvement is uncommon.
Aneurysm, intracranial berry, 2
MedGen UID:
325285
Concept ID:
C1837894
Disease or Syndrome
Intracranial berry aneurysms are saccular outpouchings of the intracranial arteries, most commonly at arterial bifurcations, characterized by arterial wall remodeling. Most cases of ruptured intracranial berry aneurysms result in a subarachnoid hemorrhage, associated with high morbidity and mortality (summary by van der Voet et al., 2004). For a discussion of genetic heterogeneity of intracranial berry aneurysm, see ANIB1 (105800).
Telangiectasia, hereditary hemorrhagic, type 2
MedGen UID:
324960
Concept ID:
C1838163
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Vascular hyalinosis
MedGen UID:
376398
Concept ID:
C1848590
Disease or Syndrome
A rare systemic disease characterized by progressive hyalinosis involving capillaries, arterioles and small veins of the digestive tract, kidneys, and retina, associated with idiopathic cerebral calcifications, manifesting with severe diarrhea (with rectal bleeding and malabsorption), nephropathy (with renal failure and systemic hypertension), chorioretinal scarring, and subarachnoid hemorrhage. Poikiloderma and premature greying of the hair may be additionally observed.
Hereditary hemorrhagic telangiectasia type 4
MedGen UID:
341824
Concept ID:
C1857688
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Stormorken syndrome
MedGen UID:
350028
Concept ID:
C1861451
Disease or Syndrome
Stormorken syndrome is an autosomal dominant disorder characterized by mild bleeding tendency due to platelet dysfunction, thrombocytopenia, anemia, asplenia, tubular aggregate myopathy, congenital miosis, and ichthyosis. Additional features may include headache or recurrent stroke-like episodes (summary by Misceo et al., 2014).
Cerebral amyloid angiopathy, APP-related
MedGen UID:
414044
Concept ID:
C2751536
Disease or Syndrome
Cerebral amyloid angiopathy, or cerebroarterial amyloidosis, refers to a pathologic process in which amyloid protein progressively deposits in cerebral blood vessel walls with subsequent degenerative vascular changes that usually result in spontaneous cerebral hemorrhage, ischemic lesions, and progressive dementia. APP-related CAA is the most common form of CAA (Revesz et al., 2003, 2009).
Aneurysm-osteoarthritis syndrome
MedGen UID:
462437
Concept ID:
C3151087
Disease or Syndrome
Loeys-Dietz syndrome (LDS) is characterized by vascular findings (cerebral, thoracic, and abdominal arterial aneurysms and/or dissections), skeletal manifestations (pectus excavatum or pectus carinatum, scoliosis, joint laxity, arachnodactyly, talipes equinovarus, cervical spine malformation and/or instability), craniofacial features (widely spaced eyes, strabismus, bifid uvula / cleft palate, and craniosynostosis that can involve any sutures), and cutaneous findings (velvety and translucent skin, easy bruising, and dystrophic scars). Individuals with LDS are predisposed to widespread and aggressive arterial aneurysms and pregnancy-related complications including uterine rupture and death. Individuals with LDS can show a strong predisposition for allergic/inflammatory disease including asthma, eczema, and reactions to food or environmental allergens. There is also an increased incidence of gastrointestinal inflammation including eosinophilic esophagitis and gastritis or inflammatory bowel disease. Wide variation in the distribution and severity of clinical features can be seen in individuals with LDS, even among affected individuals within a family who have the same pathogenic variant.
Telangiectasia, hereditary hemorrhagic, type 1
MedGen UID:
1643786
Concept ID:
C4551861
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Combined immunodeficiency due to DOCK8 deficiency
MedGen UID:
1648410
Concept ID:
C4722305
Disease or Syndrome
Hyper-IgE syndrome-2 with recurrent infections (HIES2) is an autosomal recessive immunologic disorder characterized by recurrent staphylococcal infections of the skin and respiratory tract, eczema, elevated serum immunoglobulin E, and hypereosinophilia. It is distinguished from autosomal dominant HIES1 (147060) by the lack of connective tissue and skeletal involvement (Renner et al., 2004). For a discussion of genetic heterogeneity of hyper-IgE syndrome, see 147060. See also TYK2 deficiency (611521), a clinically distinct disease entity that includes characteristic features of both autosomal recessive HIES2 and mendelian susceptibility to mycobacterial disease (MSMD; 209950) (Minegishi et al., 2006).
Aneurysm, intracranial berry, 12
MedGen UID:
1684660
Concept ID:
C5231484
Disease or Syndrome
Rupture of an intracranial aneurysm (IA), an outpouching or sac-like widening of a cerebral artery, leads to a subarachnoid hemorrhage (SAH), a sudden-onset disease that can lead to severe disability and death. Several risk factors such as smoking, hypertension, and excessive alcohol intake are associated with subarachnoid hemorrhage (summary by Krischek and Inoue, 2006).

Professional guidelines

PubMed

Osgood ML
Curr Neurol Neurosci Rep 2021 Jul 26;21(9):50. doi: 10.1007/s11910-021-01136-9. PMID: 34308493
Chung DY, Olson DM, John S, Mohamed W, Kumar MA, Thompson BB, Rordorf GA
Curr Neurol Neurosci Rep 2019 Nov 26;19(12):94. doi: 10.1007/s11910-019-1009-9. PMID: 31773310Free PMC Article
Filler L, Akhter M, Nimlos P
Semin Neurol 2019 Feb;39(1):20-26. Epub 2019 Feb 11 doi: 10.1055/s-0038-1677023. PMID: 30743289

Recent clinical studies

Etiology

Hoh BL, Ko NU, Amin-Hanjani S, Chou SH-Y, Cruz-Flores S, Dangayach NS, Derdeyn CP, Du R, Hänggi D, Hetts SW, Ifejika NL, Johnson R, Keigher KM, Leslie-Mazwi TM, Lucke-Wold B, Rabinstein AA, Robicsek SA, Stapleton CJ, Suarez JI, Tjoumakaris SI, Welch BG
Stroke 2023 Jul;54(7):e314-e370. Epub 2023 May 22 doi: 10.1161/STR.0000000000000436. PMID: 37212182
Minhas JS, Moullaali TJ, Rinkel GJE, Anderson CS
Stroke 2022 Apr;53(4):1065-1073. Epub 2022 Mar 8 doi: 10.1161/STROKEAHA.121.036139. PMID: 35255708
Rinkel GJ, Ruigrok YM
Int J Stroke 2022 Jan;17(1):30-36. Epub 2021 Jun 17 doi: 10.1177/17474930211024584. PMID: 34042530Free PMC Article
Griswold DP, Fernandez L, Rubiano AM
J Neurotrauma 2022 Jan;39(1-2):35-48. Epub 2021 Apr 22 doi: 10.1089/neu.2021.0007. PMID: 33637023Free PMC Article
Neifert SN, Chapman EK, Martini ML, Shuman WH, Schupper AJ, Oermann EK, Mocco J, Macdonald RL
Transl Stroke Res 2021 Jun;12(3):428-446. Epub 2020 Oct 19 doi: 10.1007/s12975-020-00867-0. PMID: 33078345

Diagnosis

Hoh BL, Ko NU, Amin-Hanjani S, Chou SH-Y, Cruz-Flores S, Dangayach NS, Derdeyn CP, Du R, Hänggi D, Hetts SW, Ifejika NL, Johnson R, Keigher KM, Leslie-Mazwi TM, Lucke-Wold B, Rabinstein AA, Robicsek SA, Stapleton CJ, Suarez JI, Tjoumakaris SI, Welch BG
Stroke 2023 Jul;54(7):e314-e370. Epub 2023 May 22 doi: 10.1161/STR.0000000000000436. PMID: 37212182
Rinkel GJ, Ruigrok YM
Int J Stroke 2022 Jan;17(1):30-36. Epub 2021 Jun 17 doi: 10.1177/17474930211024584. PMID: 34042530Free PMC Article
Griswold DP, Fernandez L, Rubiano AM
J Neurotrauma 2022 Jan;39(1-2):35-48. Epub 2021 Apr 22 doi: 10.1089/neu.2021.0007. PMID: 33637023Free PMC Article
Li K, Barras CD, Chandra RV, Kok HK, Maingard JT, Carter NS, Russell JH, Lai L, Brooks M, Asadi H
World Neurosurg 2019 Jun;126:513-527. Epub 2019 Mar 18 doi: 10.1016/j.wneu.2019.03.083. PMID: 30898740
Long B, Koyfman A, Runyon MS
Emerg Med Clin North Am 2017 Nov;35(4):803-824. Epub 2017 Aug 24 doi: 10.1016/j.emc.2017.07.001. PMID: 28987430

Therapy

Treggiari MM, Rabinstein AA, Busl KM, Caylor MM, Citerio G, Deem S, Diringer M, Fox E, Livesay S, Sheth KN, Suarez JI, Tjoumakaris S
Neurocrit Care 2023 Aug;39(1):1-28. Epub 2023 May 18 doi: 10.1007/s12028-023-01713-5. PMID: 37202712
Geraldini F, De Cassai A, Diana P, Correale C, Boscolo A, Zampirollo S, Disarò L, Carere A, Cacco N, Navalesi P, Munari M
Neurocrit Care 2022 Jun;36(3):1071-1079. Epub 2022 Apr 13 doi: 10.1007/s12028-022-01493-4. PMID: 35419702
Pontes FGB, da Silva EM, Baptista-Silva JC, Vasconcelos V
Cochrane Database Syst Rev 2021 May 10;5(5):CD013312. doi: 10.1002/14651858.CD013312.pub2. PMID: 33971026Free PMC Article
Balami JS, White PM, McMeekin PJ, Ford GA, Buchan AM
Int J Stroke 2018 Jun;13(4):348-361. Epub 2017 Nov 24 doi: 10.1177/1747493017743051. PMID: 29171362
Dorhout Mees SM, Rinkel GJ, Feigin VL, Algra A, van den Bergh WM, Vermeulen M, van Gijn J
Cochrane Database Syst Rev 2007 Jul 18;2007(3):CD000277. doi: 10.1002/14651858.CD000277.pub3. PMID: 17636626Free PMC Article

Prognosis

Lauzier DC, Jayaraman K, Yuan JY, Diwan D, Vellimana AK, Osbun JW, Chatterjee AR, Athiraman U, Dhar R, Zipfel GJ
Stroke 2023 May;54(5):1426-1440. Epub 2023 Mar 3 doi: 10.1161/STROKEAHA.122.040072. PMID: 36866673Free PMC Article
Rufus P, Moorthy RK, Joseph M, Rajshekhar V
Neurol India 2021 Nov-Dec;69(Supplement):S420-S428. doi: 10.4103/0028-3886.332264. PMID: 35102998
Long B, Koyfman A, Runyon MS
Emerg Med Clin North Am 2017 Nov;35(4):803-824. Epub 2017 Aug 24 doi: 10.1016/j.emc.2017.07.001. PMID: 28987430
Portegies ML, Koudstaal PJ, Ikram MA
Handb Clin Neurol 2016;138:239-61. doi: 10.1016/B978-0-12-802973-2.00014-8. PMID: 27637962
Molyneux AJ, Kerr RS, Yu LM, Clarke M, Sneade M, Yarnold JA, Sandercock P; International Subarachnoid Aneurysm Trial (ISAT) Collaborative Group
Lancet 2005 Sep 3-9;366(9488):809-17. doi: 10.1016/S0140-6736(05)67214-5. PMID: 16139655

Clinical prediction guides

Sengupta J, Alzbutas R
Biomed Res Int 2022;2022:5416726. Epub 2022 Jan 27 doi: 10.1155/2022/5416726. PMID: 35111845Free PMC Article
Mahta A, Murray K, Reznik ME, Thompson BB, Wendell LC, Furie KL
J Stroke Cerebrovasc Dis 2021 Sep;30(9):105939. Epub 2021 Jun 23 doi: 10.1016/j.jstrokecerebrovasdis.2021.105939. PMID: 34171650
de Rooij NK, Rinkel GJ, Dankbaar JW, Frijns CJ
Stroke 2013 Jan;44(1):43-54. Epub 2012 Dec 18 doi: 10.1161/STROKEAHA.112.674291. PMID: 23250997
Boswell S, Thorell W, Gogela S, Lyden E, Surdell D
J Stroke Cerebrovasc Dis 2013 Aug;22(6):750-7. Epub 2012 Mar 30 doi: 10.1016/j.jstrokecerebrovasdis.2012.02.001. PMID: 22465208
Frontera JA, Claassen J, Schmidt JM, Wartenberg KE, Temes R, Connolly ES Jr, MacDonald RL, Mayer SA
Neurosurgery 2006 Jul;59(1):21-7; discussion 21-7. doi: 10.1227/01.neu.0000243277.86222.6c. PMID: 16823296

Recent systematic reviews

Mead GE, Sposato LA, Sampaio Silva G, Yperzeele L, Wu S, Kutlubaev M, Cheyne J, Wahab K, Urrutia VC, Sharma VK, Sylaja PN, Hill K, Steiner T, Liebeskind DS, Rabinstein AA
Int J Stroke 2023 Jun;18(5):499-531. Epub 2023 Mar 1 doi: 10.1177/17474930231156753. PMID: 36725717Free PMC Article
Geraldini F, De Cassai A, Diana P, Correale C, Boscolo A, Zampirollo S, Disarò L, Carere A, Cacco N, Navalesi P, Munari M
Neurocrit Care 2022 Jun;36(3):1071-1079. Epub 2022 Apr 13 doi: 10.1007/s12028-022-01493-4. PMID: 35419702
Sharifian-Dorche M, Bahmanyar M, Sharifian-Dorche A, Mohammadi P, Nomovi M, Mowla A
J Neurol Sci 2021 Sep 15;428:117607. Epub 2021 Aug 3 doi: 10.1016/j.jns.2021.117607. PMID: 34365148Free PMC Article
Pontes FGB, da Silva EM, Baptista-Silva JC, Vasconcelos V
Cochrane Database Syst Rev 2021 May 10;5(5):CD013312. doi: 10.1002/14651858.CD013312.pub2. PMID: 33971026Free PMC Article
Negro A, Delaruelle Z, Ivanova TA, Khan S, Ornello R, Raffaelli B, Terrin A, Reuter U, Mitsikostas DD; European Headache Federation School of Advanced Studies (EHF-SAS)
J Headache Pain 2017 Oct 19;18(1):106. doi: 10.1186/s10194-017-0816-0. PMID: 29052046Free PMC Article

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