Unusual association of diseases/symptoms Unexplained paraparesis following subarachnoid haemorrhage Morgan J Bressington,1 Gulam Zilani,1 Dmitri Shastin,1,2 Chirag Patel1 1 Department of Neurosurgery, University Hospital of Wales, Cardiff, UK 2 School of Medicine, Cardiff University, Cardiff, UK Correspondence to Dr Morgan J Bressington, ​MJBressington@​doctors.​org.​uk Accepted 18 December 2018 Summary Spinal arachnoid cysts (SAC) are rare in isolation and the exact aetiology is still debated. Primary (congenital) cysts are caused by structural abnormalities in the arachnoid layer and largely affect the thoracic region. Secondary cysts are induced by a multitude of factors, infection, trauma or iatrogenic response, and can affect any level of the spinal cord. While subarachnoid haemorrhage (SAH) is a relatively common condition with significant repercussions, it is extremely uncommonly associated with SAC. When present, it may develop in the months and years after the original bleed, giving rise to new neurological symptoms. Prompt treatment is needed to halt or reverse the worsening of symptoms and questions are still being asked about how best to approach this condition. A 42-year-old man presented with chronic back pain, severe worsening ataxia and numbness below the umbilicus, 7 months after treatment for a World Federation of Neurosurgical Societies grade five (WFNS V) SAH. Imaging revealed a SAC extending from T12 to L4 and causing thecal compression. This was treated with a L3 laminectomy andmarsupialisation. An improvement in neurological function was observed at 6 months. Aetiology of the SAC and its association with SAH are discussed and a review of the relevant literature is provided. Background © BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ. To cite: Bressington MJ, Zilani G, Shastin D, et al. BMJ Case Rep 2019;12:e227666. doi:10.1136/bcr-2018227666 Aneurysmal subarachnoid haemorrhage (aSAH) is one of the most common neurosurgical emergencies affecting at least 9 per 100 000 people per year in Western populations.1Patients who develop aSAH are at risk of serious complications including rebleeding, delayed cerebral ischaemia, hydrocephalus, epilepsy and certain other medical problems;1 2 however, spinal complications are extremely rare. Spinal arachnoid cysts (SAC) are described as expanding diverticuli of the subarachnoid space lined only by arachnoid and usually communicating through a relatively narrow neck.3 While most SAC are primary, a proportion have a history of a preceding spinal insult, for example, inflammation or trauma.4 SAC in association with a previous subarachnoid haemorrhage (SAH) has only been described in a handful of cases.4–20 We present a patient who was admitted with a slowly evolving paraparesis featuring upper motor neuron signs on the background of a previous aSAH; the association and management are discussed in the context of the relevant literature. Case presentation A 42-year-old man presented with 8 months of progressively worsening back pain and 2 months of increasing ataxia, bilateral lower limb weakness and paraesthesia from umbilicus down. This was accompanied by erectile dysfunction and urinary hesitancy but no incontinence. Past history of Grade V SAH was that it had occurred 10 months previously, for which the patient was in hospital for 3 months, requiring left posterior communicating artery (PCOM) aneurysm embolisation. He received external ventricular drainage as an inpatient but no permanent cerebrospinal fluid (CSF) diversion. He was discharged to his normal place of residence without lasting neurological deficits. His past medical history included Barrett’s oesophagus and a hiatus hernia. There was no family history of note. He was a lorry driver by trade but was yet unable to return to work following his SAH. There was no history of smoking or drinking. Medications on admission were omeprazole 40 mg OD, tramadol 50 mg PRN and diazepam 5 mg PRN. Admission examination findings consisted of reduced lower (4/5 bilaterally) but not upper limb power. Reflexes were mildly reduced globally, plantar reflexes downgoing. Sensation to light touch, pin prick and temperature was reduced bilaterally below the umbilicus. Proprioception was normal. Digital rectal examination found normal rectal tone and no saddle anaesthesia. Investigations CT head was unchanged since previous discharge and magnetic resonance angiography head showed no new bleed, no change in previously coiled aneurysm and no hydrocephalus. MRI of the whole spine (figure 1) demonstrated an intradural extramedullary cyst extending from T12 to L4 that followed CSF signal in all MRI sequences with associated compression of the theca. There was an associated cord signal change opposite T10-12 vertebral bodies but no evidence of syringomyelia or other lesions further cranially. Differential diagnosis Differential diagnosis focused on the cystic lesions of the intradural extramedullary compartment. This includes congenital pathology such as a primary arachnoid cyst or dysraphic lesions (eg, neurenteric cyst, neuroepithelial cyst, dermoid and epidermoid cyst). Possible acquired pathology includes Bressington MJ, et al. BMJ Case Rep 2019;12:e227666. doi:10.1136/bcr-2018-227666 1 BMJ Case Rep: first published as 10.1136/bcr-2018-227666 on 27 February 2019. Downloaded from http://casereports.bmj.com/ on 3 March 2019 by guest. Protected by copyright. Case report Unusual association of diseases/symptoms Outcome and follow-up During his 5-day inpatient stay including 48 hours flat bed rest followed by physiotherapy, the patient reported an improvement in symptoms and quickly returned to baseline mobility. A 6-month follow-up in neurosurgical outpatient clinic demonstrated further improvement in function and successful halting of progressive neurological worsening. MRI showed resolution of the cyst with relaxed theca (figure 2). Discussion Figure 1 A sagittal lumbosacral T2-weighted MRI showing intradural, extramedullary lesion extending from T12 to L4 (yellow arrowheads) with associated thecal compression (yellow arrow). The lesion follows CSF signal in all MRI sequences (others not shown) and is strongly suggestive of a spinal cyst. neoplastic malformation (cystic schwannoma), infectious disease (cysts of parasitic origin) or secondary arachnoid cyst such as that seen with adhesive arachnoiditis. Based on history and radiological findings the most likely diagnosis here was that of a SAC secondary to SAH. Treatment The patient underwent L3 laminectomy and marsupialisation of the anterior intradural arachnoid cyst. In short, the patient was 2 In a large retrospective series of 130 patients, Klekamp classified spinal intradural arachnoid cysts as either primary or secondary.4 Of the 21 secondary SAC (16%), six were associated with SAH as in this case; the other common associations were spinal meningitis (seven patients) and spinal cord injury (four patients). While a direct causal relationship between a preceding SAH and the development of SAC remains to be demonstrated, it has long been known that ectopic blood, such as from a ruptured aneurysm, induces inflammation and fibrosis of the meninges, including those in the spine.21 SAC associated with aSAH exist almost exclusively in the thoracic region,4 7–9 11–13 15 18 possibly because the kyphotic curvature makes this area dependent leading to blood pooling during prolonged bed rest.4 22 Indeed, histological specimens have been shown to contain lysed erythrocytes and leucocytic infiltrate6 or non-specific fibrosis with scattered lymphocytes and hemosiderin.15 In a number of cases, increased CSF protein15 and collagen synthesis markers8 have been noted on lumbar puncture although this is not consistent throughout the literature.8 12 Further, certain features of secondary arachnoid cysts distinguish them from their primary counterparts: as observed by Klekamp, the former tend to show a more generalised pathology, with widespread arachnoid thickening; as a result, the walls are better defined on preoperative imaging.4 Some SAC can be multiloculated, presenting additional management challenges.10 12 15 Cardiac-gated cine MR studies show limited CSF flow.4 Clinically, patients often present with a prolonged, evolving picture of lower limb weakness, hypoaesthesia, gait and sphincter disturbance,4 12 while pain is less common.4 The interval between the ictus of SAH and the clinical presentation of SAC can range from 1 month8 17 to 6 years.9 There is a frequent association between secondary arachnoid cysts and arachnoid scarring (AS),10–12 14 15 the latter sometimes causing symptoms on its own without the cyst developing8 23 and is likely a result of the same pathological process of reactive arachnoid proliferation. In a proportion of patients, a syrinx can be observed4 12 and together this forms a distinct pathological triad; however, as the syrinx is usually distal to AS/SAC it may represent a secondary phenomenon resulting from obstructed CSF pathways rather than an Bressington MJ, et al. BMJ Case Rep 2019;12:e227666. doi:10.1136/bcr-2018-227666 BMJ Case Rep: first published as 10.1136/bcr-2018-227666 on 27 February 2019. Downloaded from http://casereports.bmj.com/ on 3 March 2019 by guest. Protected by copyright. placed prone under general anaesthesia and an L3 laminectomy was performed with intraoperative X-ray confirmation. The dura was opened under microscopic vision, preserving the arachnoid layer. The arachnoid layer was then opened to find the cyst anteriorly within the thecal sac and nerve roots stretched over it. The roots were dissected off the cyst wall with sharp dissection to sufficiently expose its wall to enable marsupialisation. The cyst fluid was under pressure and consistent with CSF appearance. The cyst was opened and the pressure released via marsupialisation. No further exploration/excision was warranted as the roots were adequately decompressed and the appearances confirmed an arachnoid cyst. The dura was closed under the microscope with Nylon 4–0 sutures. Unusual association of diseases/symptoms Learning points ►► Lack of positive findings in head scans on patients with objective neurological symptoms should raise suspicion of cord pathology. ►► Delayed diagnosis can lead to worsening neurology which may be irreversible. ►► Spinal arachnoid cyst is a rare but documented side effect of subarchanoid haemorrhage and should be considered in patients presenting with new neurology. ►► Identification of risk factors in such a rare presentation may be useful to highlight patients at risk of arachnoid cyst as a late side effect. Figure 2 A follow-up postoperative scan at 6 months. Appearances show decreased compression of thecal sac (yellow arrow) after cyst decompression. Contributors MB - patient interaction and consent, first draft, literature review, final revision. GZ - concept, patient interaction, clinical section. DS - concept, literature review, final revision. CP - concept, patient interaction, overall supervision. independent entity. The inconsistent association between the three pathologies may explain the wide variability in time to presentation and severity. Symptoms are thought to result from disturbances in spinal cord blood flow due to AS and/or external compression from the cyst/syrinx.4 8 It is unclear why only a small proportion of SAH patients go on to develop SAC. Kok et al proposed that rupture of a posterior circulation aneurysm leads to a larger contamination with blood of spinal CSF, thus representing a possible risk factor for SAH8; indeed, most reports of SAH associated with SAC and/or SAH had the aneurysm in posterior circulation (where identified) Competing interests None declared. Bressington MJ, et al. BMJ Case Rep 2019;12:e227666. doi:10.1136/bcr-2018-227666 Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors. Patient consent for publication Obtained. Provenance and peer review Not commissioned; externally peer reviewed. References 1 Connolly ES, Rabinstein AA, Carhuapoma JR, et al. Guidelines for the management of aneurysmal subarachnoid hemorrhage: a guideline for healthcare professionals from the American Heart Association/american Stroke Association. Stroke 2012;43:1711–37. 2 D’Souza S. Aneurysmal Subarachnoid Hemorrhage. J Neurosurg Anesthesiol 2015;27:222–40. 3 BMJ Case Rep: first published as 10.1136/bcr-2018-227666 on 27 February 2019. Downloaded from http://casereports.bmj.com/ on 3 March 2019 by guest. Protected by copyright. although some patients had paramesencephalic haemorrhage instead16 24 and in one, AComA aneurysm was the cause.11 Choice of CSF drainage/diversion (repeat lumbar punctures/ lumbar drain), approach to aneurysm treatment (clipping6–8 10 18 vs endovascular occlusion11 13) have not shown consistent relationship. None of the published reports suggested a history of infectious meningitis. A report by Taguchi et al described a piece of fibrin glue mixed with bone dust adherent to the SAC in a patient in whom fibrin was previously used during craniotomy and clipping of their aneurysm, suggesting distal migration and a potential role in cyst genesis7; nevertheless, this unusual association has not been reported by others. Treatment is usually offered where there is progressive neurological deficit with a radiologically demonstrated cord compression.4 12 The goal is prevention of further deterioration, consistent with the outcomes in Klekamp’s series showing stabilisation but no significant improvement in symptoms postoperatively.4 Of management options, laminectomy and extirpation of the cyst is the preferred surgical strategy,4 7 12 15 although insertion of cystoperitoneal6 11 or cystosubarachnoid shunt10 with or without cyst fenestration has been described with good results and is usually undertaken when complete extirpation is not possible. Steroid administration (16 mg betamethasone IM for 2 months) was tried in one case without benefit.14 Despite the initial positive results with surgery, relapse is common and can occur in up to 85% at 10 years,4 necessitating careful patient selection and preoperative counselling. In summary, similar cases identified in literature demonstrate that the pathogenesis of SAC in association with SAH remains uncertain. Exact risk factors leading to secondary SAC formation need to be explored to optimise prevention and better define treatment strategy. Unusual association of diseases/symptoms 14 Ginanneschi F, Palma L, Rossi A. Arachnoid cyst and arachnoiditis following idiopathic spinal subarachnoid haemorrhage. Br J Neurosurg 2008;22:578–9. 15 Marshman LA, David KM, King A, et al. Delayed fibrotic obliteration of the spinal subarachnoid space after cerebral aneurysmal subarachnoid hemorrhage: case report. Neurosurgery 2007;61:E659–60. 16 Thines L, Khalil C, Fichten A, et al. Spinal arachnoid cyst related to a nonaneurysmal perimesencephalic subarachnoid hemorrhage: case report. Neurosurgery 2005;57:E817. 17 Jourdan C, Artru F, Convert J, et al. [A rare and severe complication of meningeal hemorrhage: spinal arachnoiditis with paraplegia]. Agressologie 1990;31:413–4. 18 Nakata Y, Yagishita A, Taniguchi M. [The presyrinx state due to adhesive arachnoiditis: a case report]. No To Shinkei 2006;58:500–4. 19 Weiss RM, Sweeney L, Dreyfuss M. Circumscribed adhesive spinal arachnoiditis. J Neurosurg 1962;19:435–8. 20 Nelson J. Intramedullary cavitation resulting from adhesive spinal arachnoiditis. Arch Neurol Psychiatry 1943;50:1. 21 Hammes EM. Reaction of the meninges to blood. Arch Neurol Psychiatry 1944;52:505–14. 22 Tjandra JJ, Varma TR, Weeks RD. Spinal arachnoiditis following subarachnoid haemorrhage. Aust N Z J Surg 1989;59:84–7. 23 van Heerden J, McAuliffe W. Spinal arachnoiditis as a consequence of aneurysmrelated subarachnoid haemorrhage. J Med Imaging Radiat Oncol 2013;57:61–4. 24 Basaran R, Kaksi M, Efendioglu M, et al. Spinal arachnoid cyst associated with arachnoiditis following subarachnoid haemorrhage in adult patients: a case report and literature review. Br J Neurosurg 2015;29:285–9. Copyright 2019 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit https://www.bmj.com/company/products-services/rights-and-licensing/permissions/ BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ►► Submit as many cases as you like ►► Enjoy fast sympathetic peer review and rapid publication of accepted articles ►► Access all the published articles ►► Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact consortiasales@bmjgroup.com Visit casereports.bmj.com for more articles like this and to become a Fellow 4 Bressington MJ, et al. BMJ Case Rep 2019;12:e227666. doi:10.1136/bcr-2018-227666 BMJ Case Rep: first published as 10.1136/bcr-2018-227666 on 27 February 2019. Downloaded from http://casereports.bmj.com/ on 3 March 2019 by guest. Protected by copyright. 3 Kendall BE, Valentine AR, Keis B. Spinal arachnoid cysts: clinical and radiological correlation with prognosis. Neuroradiology 1982;22:225–34. 4 Klekamp J. A new classification for pathologies of spinal meninges-part 2: primary and secondary intradural arachnoid cysts. Neurosurgery 2017;81:217–29. 5 Hardman J, Neil-Dwyer G. Spinal cord compression secondary to a thoracic meningeal cyst after subarachnoid haemorrhage: a case report. J Neurol Neurosurg Psychiatry 1994;57:1145–6. 6 Lorenzana-Honrado L, Cabezudo-Artero JM, Gozez-Perals L. Arachnoid cyst. J Neurosurg 1996;85:734–5. 7 Taguchi Y, Suzuki R, Okada M, et al. Spinal arachnoid cyst developing after surgical treatment of a ruptured vertebral artery aneurysm: a possible complication of topical use of fibrin glue. Case report. J Neurosurg 1996;84:526–9. 8 Kok AJ, Verhagen WI, Bartels RH, et al. Spinal arachnoiditis following subarachnoid haemorrhage: report of two cases and review of the literature. Acta Neurochir 2000;142:795–9. 9 Brodbelt AR, Stoodley MA. Syringomyelia and the arachnoid web. Acta Neurochir 2003;145:707–11. 10 Tumialán LM, Cawley CM, Barrow DL. Arachnoid cyst with associated arachnoiditis developing after subarachnoid hemorrhage. Case report. J Neurosurg 2005;103:1088–91. 11 Eneling J, Boström S, Rossitti S. Subarachnoid hemorrhage-associated arachnoiditis and syringomyelia. Clin Neuroradiol 2012;22:169–73. 12 Ishizaka S, Hayashi K, Otsuka M, et al. Syringomyelia and arachnoid cysts associated with spinal arachnoiditis following subarachnoid hemorrhage. Neurol Med Chir 2012;52:686–90. 13 Abhinav K, Bradley M, Aquilina K, et al. Spinal arachnoiditis and cyst formation with subarachnoid haemorrhage. Br J Neurosurg 2012;26:574–5.