What is surfers myopathy

In , Thompson et al. They were commonly taking their first lesson and otherwise young and healthy without prior spinal or vascular problems. Thompson et al. Though the reported outcomes usually turned out to be debilitating patients needed wheelchairs to ambulate or required catheterization , some experienced good or full recovery within 24—72 hours of presentation [ 13 ].

The patient in our report who was treated with induced hypertension also showed early recovery; therefore, this treatment appears to be crucial for good long-term neurologic outcomes. Nakamoto et al. Including the cases of this report, other reported cases and series to date have demonstrated no significant differences from the results in our study.

Diffusion-weighted images were obtained in several cases, and most but not all cases demonstrated restricted diffusion, thus it could be a good method for differentiating ischemic insult from other types of myelopathy, determining the approximate time of ongoing ischemia, and predicting disease prognosis [ 20 , 30 ] but it does not enhance diagnostic workup [ 13 ]. The continuous prone hyperextended posture while paddling can possibly cause avulsion of perforating vessels, vasospasm of the artery of Adamkiewicz, or transient ischemia in areas of borderline perfusion as a result of spinal cord tension due to hyperextension.

Prolonged spinal hyperextension has been previously reported as a possible mechanism for spinal cord injury [ 23 ]. Other possible mechanisms have been proposed, including venous infarction from obstruction of the inferior vena cava obstruction by the liver while lying prone over the surfboard, or fibrocartilaginous embolism, which is potentially triggered by the Valsalva maneuver while standing up on the surfboard [ 2 , 8 , 9 , 26 ].

Spinal angiography can find underlying vascular anomalies or anatomical variations that are vulnerable to ischemic insult or confirm intravascular mechanisms disrupting spinal cord perfusion.

Moreover, it allows direct intra-arterial intervention i. Nevertheless, spinal angiography has been used infrequently in previous reports, which is possibly due to the inherent risk and limited availability of spinal angiography as well as the decision of a physician who is not familiar with this disease.

In their case report, Karabegovic et al. Freedman et al. There are no standardized treatment options for this rare disease as there is no strong evidence or controlled studies due to its rarity.

They recommended that, when available, emergent spinal angiography should be considered with administration of intra-arterial tPA in the presence of confirmed embolic occlusion or nicardipine in the presence of vasospasm. They recommended considering intravenous tPA in case there is no identified occlusion or emergent spinal angiography is not available.

Lumbar drain placement can also be considered as it allows CSF sampling which can help rule out other etiologies of acute atraumatic spinal cord injury and reduces CSF pressures to 10—15 mmHg which showed a neuroprotective effect from previous reports of thoracic surgery and acute spinal cord injury [ 3 , 10 , 17 , 31 ].

Elevating mean arterial pressure MAP over 85 mmHg for at least 24 hours was also recommended [ 13 ]. Hawryluk et al. In our case series, the patient showing early recovery and good long-term neurologic outcome was treated with induced hypertension, which can be achieved initially with intravenous fluids, careful use of narcotics for pain, and vasopressors [ 13 ].

It has been reported that reducing CSF pressure and increasing MAP can synergistically improve spinal cord perfusion [ 4 , 17 , 21 , 31 ]. High-dose steroid therapy was frequently used in previous reports in the context of acute spinal cord injury in consideration of the NASCIS III methylprednisolone protocol [ 5 , 6 ]. But until now, steroid therapy has not been studied for nontraumatic ischemic spinal cord injuries.

Though this condition shows rapid progression of neurologic deterioration and severe deficits, some patients experience good or full recovery within 24—72 hours of presentation. Therefore, surfers and instructors should be educated on detecting the early signs and symptoms of this condition, as these interventions are necessary to prevent further deterioration. Hydration, induced hypertension, early spinal angiography with intra-arterial intervention, intravenous tPA, and high-dose steroid therapy are considered acute treatments, but there is still a lack of data for establishing standardized treatments.

As seen in this and previous reports, early recovery is important for good long-term neurological outcome. No potential conflict of interest relevant to this article was reported. Informed consent was obtained from all individual participants included in this study.

National Center for Biotechnology Information , U. J Korean Neurosurg Soc. Published online Oct June Ho Choi , M. Author information Article notes Copyright and License information Disclaimer.

Address for reprints : Jin Hoon Park, M. This article has been cited by other articles in PMC. Keywords: Water sports, Spinal cord injuries. Open in a separate window. Case 3 A year-old Korean male with no medical history had a first time surfing lesson during which he felt a sudden cramping pain in his lower back.

Footnotes No potential conflict of interest relevant to this article was reported. References 1. Rehabilitation and long-term course of nontraumatic myelopathy associated with surfing. Am J Phys Med Rehabil. Nontraumatic myelopathy associated with surfing. T2 weighted MRI Figure 1 demonstrated expansion and abnormal hyperintensity involving the conus medullaris of the spinal cord level with T12 and L1.

Diffusion weighted and gadolinium contrast MRI Figure 1 revealed diffusion restriction signals corresponding to the previous hyperintense T2 signal detected.

This was consistent with a recent infarct at the level. No abnormal contrast enhancement was seen and there was no suggestion of disk herniation. CT angiography of the abdomen and pelvis showed normal vessels with normal flow.

No aneurysms or thrombi were seen. Catheter angiography was not performed due to concern of further spinal cord insult. The patient was commenced on intravenous dexamethasone with almost immediate improvement in sensation, supplemented with intravenous fluid therapy to regulate systolic blood pressure and urinary catheterisation due to retention.

He was discharged six days later mobilising independently with repeat MRI Figure 2 demonstrating no proximal infarct extension. At discharge he was actively weaning off the dexamethasone and reported ongoing saddle and perineal anaesthesia. Although the overwhelming majority of literature surrounding SM is on young surfing patients, our report is not the first to demonstrate consistent features with a non-surfing etiology 7.

Other risk factors initially proposed by Thompson et al. Dehydration itself may contribute to a hyper-coagulable state however its role in the aetiology has not been previously assessed in any available studies. Regardless, in our present case there was no history of either set of risk factors or an apparent history of a hyperextension motion.

This raises the issue that while the presence of surfing and acute hyperextension may have previously been predictors of SM, the diagnosis should be considered in all cases involving strenuous activity to the spine. This however is not consistent with the etiological hypothesis surrounding SM made by Freedman et al. The most recent proposed mechanism by Freedman et al. In the setting of surfing this relates to the prone position on the board. This factor was not formerly assessed in our case but has been supported by other studies 6 , Naturally, this explains why the lower spinal cord and conus medullaris may be vulnerable to arterial insufficiency as evident in the majority of published literature.

Lastly, due to this mechanism and the acute nature of myelopathy, the presence of disc herniation should be considered as a relevant differential. Other suggested pathophysiological mechanisms include vascular avulsion of perforating vessels, vasospasm of the artery of Adamkiewicz, and fibrocartilaginous disc embolisation.

Thus, publicizing this rare but serious condition within and outside the medical literature may be an effective intervention. The term surfer's myelopathy was first introduced by Thompson et al 1 in the report of 9 patients with this condition. All were young, healthy, novice surfers who acutely developed back pain and, within an hour, progressive neurological deficits. Fortunately, all but 1 of these patients had good or complete recovery of strength. However, since then, there have been 55 additional cases including the case here reported in 12 articles, for which prognosis was less optimistic Table 1.

Authors on this subject have concluded, on the basis of mounting circumstantial evidence, that the most likely cause of this condition is ischemic compromise to the spinal cord. Second, magnetic resonance imaging MRI findings in surfer's myelopathy are most closely related to those seen in spinal cord infarct.

Third, those afflicted are young individuals in good states of health who have no underlying spinal pathology. Lastly, reported workups have excluded other rare potential causes such as transverse myelitis, infection, neoplasm, vascular malformation, and inflammatory disorder. To date, no case report has included angiographic evidence of the compromised flow.

The only reported prior angiograms have demonstrated normal findings. The purpose of this work is to report this illustrative case, to systematically review the existent literature, and to provide considerations for prevention and interventions to mitigate the adverse effects of surfer's myelopathy. This study is a case report and systematic review of the literature.

On August 25, , we queried PubMed for the following search terms: surfer's myelopathy, surfers myelopathy, surfer myelopathy, surfing and myelopathy, and surfing and spinal cord injury.

The 15 individual articles found were all reviewed. The references for each were also reviewed, which identified a single additional article; however, reading of this article demonstrated that it was likely the same patient as reported by Dhaliwal et al.

Clinical information was categorized into examination, imaging, treatment, and outcome findings. Each full article was reviewed, and the specified clinical information was abstracted. The primary findings were summarized in tabular form Table 1. In addition to this systematic review, we provide an additional illustrative case that embodies the prototypical elements of this condition. The subject in this case provided consent for use of his health information.

A healthy year-old male, elite high-school athlete with opportunities to play football at the collegiate level was on vacation with his family in Hawaii in taking his first surfing lesson. About halfway through the lesson, without a traumatic event, he developed acute, relatively severe pain and spasms in his back.

Within a few minutes, he developed tingling in his bilateral lower extremities, followed by rapidly progressive weakness. He was taken to the local emergency room and evaluated. His sensory level was located halfway between the umbilicus and the inguinal crease T Similar to most reported cases, he did not have a dissociated sensory loss ie, absent anterolateral and preserved dorsal column sense , which would indicate a true anterior spinal cord syndrome; rather, he had a complete loss of both spinothalamic pain, temperature, and light touch and dorsal column sensation 2-point discrimination, vibration and proprioception.

MRIs were obtained early and late in follow-up , and they demonstrated characteristic evidence of spinal cord infarction from the T9 level to the conus medullaris Figure 1. These findings included longitudinal T2 hyperintensity and spinal cord swelling acutely Figure 1A and 1B , followed by atrophy at the final follow-up Figure 1C and 1D from the midthoracic spine to the conus medullaris.

The patient was admitted to the intensive care unit for close evaluation. He was afebrile, and his laboratory findings were unremarkable, including inflammatory markers and at a later date hypercoagulable studies ie, protein C and S levels.

He demonstrated no immediate neurological recovery and was transferred to a SCI rehabilitation center closer to his home. The patient had no history of spinal, vascular, or venothrombotic disorders, nor were any vascular malformations identified on advanced imaging. During his evaluation, the patient was referred to a tertiary center for a second opinion. Spinal angiography was performed 4. The angiogram failed to demonstrate the right T12 radicular artery Figure 2. In addition, no artery of Adamkiewicz was visualized.

An alternative explanation is that in the absence of a dominant radicular artery, the mechanics of the surfing lesson induced a period of hypoperfusion through multiple thoracic radicular arteries and collaterals, precipitating the ischemia. T2-sequence axial A and sagittal B images from the and C and D magnetic resonance imaging scans.

The earlier images show hyperintense signal within the central portion of the spinal cord, extending several levels, without intrinsic spinal column disease. Superselective angiogram images showing a coned down, digitally subtracted A and native image B of the right T11 contrast injection.

This image shows the normal radicular anatomy, including a caudal collateral branch, which typically anastamoses with the T12 radicular vessel, but in this case, there is no evidence of flow at T Likewise, when supraselective catheterization of the right T12 was performed, there was no distal flow, indicating that the vessel was completely occluded. Additionally, an artery of Adamkiewicz was not detected on this examination.

The 3 largest series were composed by different groups of authors who reported on cases occurring in 1 or more of the same 3 community hospitals in the state of Hawaii over a similar window of time; thus, it is possible that some but not all, owing to incomplete overlap of exact time periods, patient ages, and characteristics, and study sites, of the 51 cases reported in these 3 series are redundant.

Given the atraumatic nature of this disease and the lack of a clear predisposing factor for the observed geographic proclivity, it has been proposed that recent long-haul flights are a predisposing factor.

Whether this is due to dehydration altered rheology or stasis has not been addressed, but at least 1 case involved a local Hawaiian resident without recent air travel. Complete sensory loss anterolateral and dorsal column is most common. Thirty-three of 64 patients In all but 1 case in which urinary function was reported, it was abnormal ie, retention at presentation 43 of 44, In 25 of 60 Thus, with the exclusion of ASIA A complete patients, steroids had a beneficial effect on all but 3 patients with an incomplete deficit.

Acute spinal cord infarction is one of the rarest mechanisms of SCI, and it is traditionally associated with the poorest prognosis. The anterior spinal artery sends 2 branches into the matter of the spinal cord, called the sulcal arteries. The arborization of these vessels provides circulation to the anterior two-thirds of the spinal cord. In , Robertson et al 20 reviewed the experience at Mayo Clinic over a year period and identified the largest cohort of spinal cord infarctions reported to date.

This epidemiological article details the clinical characteristics of spinal cord infarction and prognosis. As with all SCIs, the most prognostic finding was maximal motor deficit.

The ability to recover from ischemic insult to the spinal cord suggests that there is some room for improving outcomes for surfer's myelopathy. In , Thompson et al 1 identified a new type of anterior spinal cord syndrome, which they called surfer's myelopathy. Nine cases were reported, 2 sporadic cases in 3 years, that sparked the author to create an internally maintained registry that detected 7 subsequent cases over the next 18 months. This study defined the prototypical characteristics of surfer's myelopathy.

Patients are novice surfers, commonly on their first lesson, who are otherwise young and healthy without prior spinal or vascular problems. Thompson et al and others have stated that patients are typically thin with underdeveloped back musculature; however, this physical feature has been least consistent across other reported cases and was not a factor in the case reported here.

The back pain can be mild or intense. By the time they reached the hospital, their neurological deficit had reached its nadir. Bladder control, which was abnormal in all patients, returned to normal in 6 of 9 patients in this series. In addition to being the first to characterize this condition, the authors were the first to propose that this was an ischemic injury to the spinal cord.

Thus, they speculated whether measures used in hemorrhagic cerebral infarct ie, triple H therapy [hypertension, hypervolemia, and hemodilution] could be used to improve outcomes in spinal infarct. These authors also provided high-dose steroids in some cases.

In response to the Thompson et al original work, other groups have reported similar cases of surfer's myelopathy, but unlike the series reported by Thompson et al, the outcomes have been worse Table 1. Thus, one can infer that for every surfer's myelopathy with permanent severe or complete motor deficits, there are several less severe cases and that this condition presents sporadically in areas of the world where surfing is prevalent.

In regard to diagnosing surfer's myelopathy acutely, Nakamoto et al 11 have provided the most comprehensive review of the MRI characteristics of surfer's myelopathy in their report of 23 cases occurring in Hawaii from to In fact, in all but 2 cases reported to date, standard T2 MRI sequences obtained within the first 24 hours of symptom onset have shown characteristic ischemic changes. Eleven of 23 cases had gadolinium contrast added.

This provided no diagnostic benefit in all cases. Although gadolinium does not contribute to the diagnosis of surfer's myelopathy, it can be helpful for ruling out other pathologies.

Nakamoto et al 11 did not have diffusion-weighted MRI to review, but others have. Diffusion-weighted imaging, which is an MRI technique that is highly sensitive for detecting regions with restricted diffusion of water related to acute ischemic cellular injury, has demonstrated areas of hyperintensity and restricted diffusion in most 10 of 15, with the other 5 studies being negative or equivocal but not all cases.

Angiography has been rarely used in prior cases. Some of the reason for the limited use of angiography likely represents lack of resources or capabilities at the initial treating facility, but some also represents a decision to exclude this confirmatory imaging modality. Because the leading hypothesized cause of surfer's myelopathy is acute ischemic insult from occlusion embolic or intimal flap , spasm, external compression possibly from psoas spasm , or avulsion, spinal angiography represents the best and possibly only option to confirm the exact mechanism of the ischemic insult.

We believe that the angiographic findings in this case the absent right T12 radicular artery and lack of an artery of Adamkiewicz implicate vascular occlusion as the cause of this severe case of surfer's myelopathy. Chung et al noted that the limitations of computed tomographic angiogram prevented them from evaluating the patency of this vessel to the level of the spinal cord.

The angiogram in the case reported here was performed late; therefore, there was no opportunity to provide intra-arterial treatments to affect outcome. Given these facts, we recommend that spinal angiography be considered in the acute evaluation and management of surfer's myelopathy Figure 1. Lastly, in agreement with most authors, we also postulate that the prolonged prone hyperextension that is typical of novice surfers is a mechanical event that is capable of instigating the vascular insult.

Hyperextension can distract the radicular vessels, which can compromise flow. Alternatively or in concert, the altered venous return that occurs from lying prone for prolonged periods of time on a surfboard can contribute to vascular insufficiency. We believe that the intravascular mechanism that leads to the disrupted perfusion of the spinal cord in these novice surfers is occlusion by embolus or vasospasm induced by prolonged hyperextension.

We base this belief on the facts that none of the cases reported to date had MRI evidence of vessel avulsion ie, hemorrhage and that there have been no reported cases of a similar acute myelopathy in novice or elite butterfly stroke swimmers, who repetitively and violently hyperextend their flexible trunks for brief periods of time, often while performing a Valsalva maneuver.

Therefore, it is more likely that prolonged hyperextension plays the putative role, which has been a previously reported mechanism for SCI. Things like altered rheology dehydration from long-haul flight and baseline coagulability probably also contribute to this event. Unless urgent angiography becomes a standardized approach to surfer's myelopathy, we will likely never know the anatomic mechanisms that alter spinal cord blood flow.