Severe headaches are a frequent presentation to the Emergency Department, accounting for around 1% of presentations. Of these, 13-16% will have a sinister underlying pathology but accurate diagnosis of headaches on the medical take remain difficult for non-specialists. A retrospective study from the UK found that, of those presenting to Emergency Department with sudden onset severe headaches, only 60% were given a specific headache diagnosis, with one third of these given their diagnoses despite an inadequate history.
Locker TE, Thompson C, Rylance J, et al. The utility of clinical features in patients presenting with nontraumatic headache: an investigation of adult patients attending an emergency department. Headache. 2006; 46(6): 954–961. doi:10.1111/j.1526-4610.2006.00448.x.
Breen DP, Duncan CW, Pope AE, et al. Emergency department evaluation of sudden, severe headache. QJM. 2008; 101(6): 43– 443. doi:10.1093/qjmed/hcn036.
Schievink WI, Reimer R, Folger WN. Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. Case report. J Neurosurg. 1994; 80(4): 736–739. doi:10.3171/jns.1994.80.4.0736.
Mokri B, Maher CO, Sencakova D. Spontaneous CSF leaks: underlying disorder of connective tissue. Neurology. 2002; 58(5): 814–816. http://www.ncbi.nlm.nih.gov/pubmed/11889250. Accessed August 31, 2015.
V B, D R, E K, L L. Spontaneous Intracranial Hypotension Following a Yoga Class: A Case Report. Am J Med Case Reports. 2015; 3(10): 314–318. doi:10.12691/ajmcr-3-10-3.
Brady-McCreery KM, Speidel S, Hussein MAW, et al. Spontaneous intracranial hypotension with unique strabismus due to third and fourth cranial neuropathies. Binocul Vis Strabismus Q. 2002; 17(1): 43–48. http://www.ncbi.nlm.nih.gov/pubmed/11874382. Accessed January 30, 2016.
Kashmere JL, Jacka MJ, Emery D, et al. Reversible coma: a rare presentation of spontaneous intracranial hypotension. Can J Neurol Sci. 2004; 31(4): 565–568. http://www.ncbi.nlm.nih.gov/ pubmed/15595268. Accessed January 30, 2016.
Mokri B. Movement disorders associated with spontaneous CSF leaks: a case series. Cephalalgia. 2014; 34(14): 1134–1141. doi:10.1177/0333102414531154
Scott S, Davenport R. Low pressure headaches caused by spontaneous intracranial hypotension. BMJ. 2014; 349: g6219. http://www.ncbi. nlm.nih.gov/pubmed/25378383. Accessed January 30, 2016.
Headache Classification Committee, Society IH. The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013; 33(9): 629–808. doi:10.1177/0333102413485658.
Schievink WI. Novel neuroimaging modalities in the evaluation of spontaneous cerebrospinal fluid leaks. Curr Neurol Neurosci Rep. 2013; 13(7): 358. doi:10.1007/s11910-013-0358-z.
Wang Y-F, Lirng J-F, Fuh J-L, et al. Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology. 2009; 73(22): 1892–1898. doi:10.1212/ WNL.0b013e3181c3fd99.
Watanabe A, Horikoshi T, Uchida M, et al. Diagnostic value of spinal MR imaging in spontaneous intracranial hypotension syndrome. AJNR Am J Neuroradiol. 2009; 30(1): 147–151. doi:10.3174/ajnr.A1277.
Mokri B, Hunter SF, Atkinson JL, et al. Orthostatic headaches caused by CSF leak but with normal CSF pressures. Neurology. 1998; 51(3): 786–790. http://www.ncbi.nlm.nih.gov/pubmed/9748027. Accessed August 31, 2015.
Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia—evolution of a concept. Mayo Clin Proc. 1999; 74(11): 1113–1123. doi:10.4065/74.11.1113.
Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc. 1997; 72(5): 400–413. doi:10.1016/S0025- 6196(11)64858-1.
Graff-Radford SB, Schievink WI. High-pressure headaches, lowpressure syndromes, and CSF leaks: diagnosis and management. Headache. 2014; 54(2): 394–401. doi:10.1111/head.12283.
Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology. 2001; 57(10): 1921–1923. doi:10.1212/WNL.57.10.1921.
Ohtonari T, Ota S, Nishihara N, et al. A novel technique of multiple-site epidural blood patch administration for the treatment of cerebrospinal fluid hypovolemia. J Neurosurg. 2012; 116(5): 1049–1053. doi:10.3171/2012.1.JNS111568.
Hannerz J, Dahlgren G, Irestedt L, et al.Treatment of idiopathic intracranial hypotension: cervicothoracic and lumbar blood patch and peroral steroid treatment. Headache. 2006; 46(3): 508–511. doi:10.1111/j.1526-4610.2006.00383.x.
Binder DK, Dillon WP, Fishman RA, et al. Intrathecal saline infusion in the treatment of obtundation associated with spontaneous intracranial hypotension: technical case report. Neurosurgery. 2002; 51(3): 830–836; discussion 836-837. http://www.ncbi.nlm.nih.gov/ pubmed/12188967. Accessed January 30, 2016.
Severe headaches are a frequent presentation to the Emergency Department, accounting for around 1% of presentations. Of these, 13-16% will have a sinister underlying pathology but accurate diagnosis of headaches on the medical take remain difficult for non-specialists. A retrospective study from the UK found that, of those presenting to Emergency Department with sudden onset severe headaches, only 60% were given a specific headache diagnosis, with one third of these given their diagnoses despite an inadequate history.
Locker TE, Thompson C, Rylance J, et al. The utility of clinical features in patients presenting with nontraumatic headache: an investigation of adult patients attending an emergency department. Headache. 2006; 46(6): 954–961. doi:10.1111/j.1526-4610.2006.00448.x.
Breen DP, Duncan CW, Pope AE, et al. Emergency department evaluation of sudden, severe headache. QJM. 2008; 101(6): 43– 443. doi:10.1093/qjmed/hcn036.
Schievink WI, Reimer R, Folger WN. Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. Case report. J Neurosurg. 1994; 80(4): 736–739. doi:10.3171/jns.1994.80.4.0736.
Mokri B, Maher CO, Sencakova D. Spontaneous CSF leaks: underlying disorder of connective tissue. Neurology. 2002; 58(5): 814–816. http://www.ncbi.nlm.nih.gov/pubmed/11889250. Accessed August 31, 2015.
V B, D R, E K, L L. Spontaneous Intracranial Hypotension Following a Yoga Class: A Case Report. Am J Med Case Reports. 2015; 3(10): 314–318. doi:10.12691/ajmcr-3-10-3.
Brady-McCreery KM, Speidel S, Hussein MAW, et al. Spontaneous intracranial hypotension with unique strabismus due to third and fourth cranial neuropathies. Binocul Vis Strabismus Q. 2002; 17(1): 43–48. http://www.ncbi.nlm.nih.gov/pubmed/11874382. Accessed January 30, 2016.
Kashmere JL, Jacka MJ, Emery D, et al. Reversible coma: a rare presentation of spontaneous intracranial hypotension. Can J Neurol Sci. 2004; 31(4): 565–568. http://www.ncbi.nlm.nih.gov/ pubmed/15595268. Accessed January 30, 2016.
Mokri B. Movement disorders associated with spontaneous CSF leaks: a case series. Cephalalgia. 2014; 34(14): 1134–1141. doi:10.1177/0333102414531154
Scott S, Davenport R. Low pressure headaches caused by spontaneous intracranial hypotension. BMJ. 2014; 349: g6219. http://www.ncbi. nlm.nih.gov/pubmed/25378383. Accessed January 30, 2016.
Headache Classification Committee, Society IH. The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013; 33(9): 629–808. doi:10.1177/0333102413485658.
Schievink WI. Novel neuroimaging modalities in the evaluation of spontaneous cerebrospinal fluid leaks. Curr Neurol Neurosci Rep. 2013; 13(7): 358. doi:10.1007/s11910-013-0358-z.
Wang Y-F, Lirng J-F, Fuh J-L, et al. Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology. 2009; 73(22): 1892–1898. doi:10.1212/ WNL.0b013e3181c3fd99.
Watanabe A, Horikoshi T, Uchida M, et al. Diagnostic value of spinal MR imaging in spontaneous intracranial hypotension syndrome. AJNR Am J Neuroradiol. 2009; 30(1): 147–151. doi:10.3174/ajnr.A1277.
Mokri B, Hunter SF, Atkinson JL, et al. Orthostatic headaches caused by CSF leak but with normal CSF pressures. Neurology. 1998; 51(3): 786–790. http://www.ncbi.nlm.nih.gov/pubmed/9748027. Accessed August 31, 2015.
Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia—evolution of a concept. Mayo Clin Proc. 1999; 74(11): 1113–1123. doi:10.4065/74.11.1113.
Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc. 1997; 72(5): 400–413. doi:10.1016/S0025- 6196(11)64858-1.
Graff-Radford SB, Schievink WI. High-pressure headaches, lowpressure syndromes, and CSF leaks: diagnosis and management. Headache. 2014; 54(2): 394–401. doi:10.1111/head.12283.
Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology. 2001; 57(10): 1921–1923. doi:10.1212/WNL.57.10.1921.
Ohtonari T, Ota S, Nishihara N, et al. A novel technique of multiple-site epidural blood patch administration for the treatment of cerebrospinal fluid hypovolemia. J Neurosurg. 2012; 116(5): 1049–1053. doi:10.3171/2012.1.JNS111568.
Hannerz J, Dahlgren G, Irestedt L, et al.Treatment of idiopathic intracranial hypotension: cervicothoracic and lumbar blood patch and peroral steroid treatment. Headache. 2006; 46(3): 508–511. doi:10.1111/j.1526-4610.2006.00383.x.
Binder DK, Dillon WP, Fishman RA, et al. Intrathecal saline infusion in the treatment of obtundation associated with spontaneous intracranial hypotension: technical case report. Neurosurgery. 2002; 51(3): 830–836; discussion 836-837. http://www.ncbi.nlm.nih.gov/ pubmed/12188967. Accessed January 30, 2016.
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BSc MBBS FRCP,
FRCP(Ed),
Dip Med Ed (Wales), Acute Medical Unit, Royal
Bournemouth Hospital,
Bournemouth,
BH7 7DW
An Acute Severe Headache
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An Acute Severe Headache
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