ISSN 0017-8748 doi: 10.1111/head.12429 Published by Wiley Periodicals, Inc.
Headache © 2014 American Headache Society
Brief Communications Cluster Belly: A Variant of Irritable Bowel Syndrome Matthew S. Robbins, MD
Cluster headache (CH) and irritable bowel syndrome (IBS) are pain disorders that possess relationships with circadian rhythms. However, they have not been compared to assess similarities that could yield pathophysiologic insights. A young male adult with periodic episodes of abdominal pain highly reminiscent of CH is described. Since childhood, he experienced severe attacks featuring excruciating, abdominal pain accompanied by prominent restlessness, lasting 30-120 minutes, occurring in the evening and in discrete 2- to 8-week periods, interspersed with remissions where typical triggers did not lead to attacks. Although all of the patient’s symptoms fell within the spectrum of IBS, the semiology was highly evocative of CH, based on the attack duration, restlessness, periodicity, and selective vulnerability to particular triggers only during attack periods. A subset of patients thought to have IBS may feature similar attack profiles and could suggest the importance of the hypothalamus in its pathophysiology, akin to CH. Key words: cluster headache, irritable bowel syndrome, hypothalamus, abdominal pain, abdominal migraine (Headache 2014;54:1644-1646)
Cluster headache (CH) distinguishes itself from all other headache disorders1 by its distinctive circadian and circannual periodicity, implicating the role of the hypothalamus in its underlying pathophysiology.2 CH and irritable bowel syndrome (IBS) are both disorders featuring recurrent attacks of pain that possess relationships with circadian rhythms and potentially hypothalamic derangements.The object of this study was to describe a patient with periodic attacks of abdominal pain who was diagnosed with IBS but had clinical features highly reminiscent of CH.
From the Montefiore Headache Center, Department of Neurology, Albert Einstein College of Medicine, Bronx, NY, USA. Address all correspondence to M.S. Robbins, 1575 Blondell Avenue, Suite 225, Bronx, NY 10461, USA. Accepted for publication June 21, 2014.
CASE REPORT A 20-year-old undergraduate student started experiencing episodes of abdominal pain at 6 years of age. Attacks consisted of moderate, crampy, diffuse abdominal pain episodes with flatulence, predictably triggered by greasy or creamy foods, particularly during times of stress, typically leading to diarrhea and relief after defecation. At 12 years of age, he began to experience different, more severe attacks, featuring excruciating, searing, crampy, usually unilateral and lower abdominal pain, accompanied by diaphoresis and mild nausea. He felt extraordinarily restless during the episodes, possessing the urge to repeatedly bend over or pace during the attacks, which lasted Conflict of Interest: Matthew S. Robbins, MD, has received honoraria from the American Headache Society, Prova Education, American College of Physicians, Medlink, North ShoreLIJ Hofstra School of Medicine, and SUNY Downstate College of Medicine, and receives book royalties from Wiley. Funding: None.
1644
Headache 30-120 minutes, predominantly occurring between 3 pm and 1 am. Attacks occurred in periods lasting 2-8 weeks, interspersed with remissions over months. Only during attack periods were triggers such as red wine and fried foods identified; these substances did not trigger pain outside of the attack periods. Defecation inconsistently provided relief. He had infrequent episodic tension-type headache whose attacks did not occur during periods of abdominal pain. He was otherwise healthy, a nonsmoker, used alcohol in moderation, and took no medications. There was no family history of any frequent or severe headache or pain disorder. His medical and neurological examinations were normal, and referral to a gastroenterologist leads to the diagnosis of IBS, with dietary counseling. Over the subsequent few years, he continued to have one to two annual clusters of attacks but did not seek specific medical treatment.
COMMENTS This patient fulfilled criteria for IBS, which is defined as a chronic, functional gastrointestinal disorder featuring recurrent abdominal pain and alterations in bowel habits, in the absence of any secondary cause.3 IBS is common, affecting 11% of the population, has a female predominance, aggregates in families, and is comorbid with many pain disorders, including headache, although not specifically CH.4 A recent population-based retrospective cohort study in Taiwan identified a nearly twofold increase in the incidence of IBS in persons with migraine after adjusting for age, gender, and medical comorbidities.5 In those with frequent migraine, adjusted incidence of IBS was 7.61 (95% confidence interval 6.33-9.13) in comparison with a migraine-free cohort.5 Although more local factors including gastrointestinal dysmotility, visceral hypersensitivity, intestinal inflammation, and infection have all been posited to play a role in the pathophysiology of IBS, comorbid pain and psychiatric conditions, as well as therapeutic responses to certain medications suggest an important role of the central nervous system.3 The most striking aspect of this case is the periodicity of attacks, which in IBS remains relatively unexplored. One clinical study of IBS in nurses
1645 revealed that those working rotating shifts had a significantly higher prevalence of IBS compared with day-shift nurses (48% vs 31%, P < 0.01). This discrepancy may imply the importance of a circadian rhythm disturbance in the pathogenesis of IBS, although this could relate to both the central and enteric nervous systems.6 Functional neuroimaging studies in IBS and visceral pain have yielded inconsistent results, without a clear signature, and the hypothalamus is not described to be a region of particular activation.7 In CH, the hypothalamus clearly plays an important role, based on the clinical features (attack periodicity, association with sleep, prominent autonomic features) as well as neuroendocrine abnormalities.2 Structural neuroimaging studies have detected increased grey matter in the inferior posterior hypothalamus and functional neuroimaging studies have revealed strong activation in the same region, which has been the target of deep brain stimulation in refractory chronic CH patients.2 Although all of the patient’s symptoms may fall within the spectrum of IBS,3 the semiology of the recurrent abdominal pain attacks are reminiscent of CH, based on the attack duration, restlessness, periodicity, and selective vulnerability to particular triggers only during attack periods. Abdominal migraine is currently an accepted diagnosis1 thought to be a precursor to migraine, which may have similar underlying neurobiology. Indirect evidence of the importance of the central nervous system in abdominal migraine, as well as shared pathophysiology with cephalgic migraine, is suggested by its response to varying nonanalgesic prophylactic medications.8 It is appealing to consider this case as a form of “abdominal” CH or “cluster belly” based on such an overlap of the clinical phenomenology. There are reports of utilizing medications that are effective in CH prophylaxis in the treatment of IBS, including single cases for verapamil9,10 and placebo-controlled studies for melatonin,11 although their mechanisms could instead be related to direct changes in gut motility and circadian rhythm regulation in the enteric nervous system. It is plausible that a subset of patients with IBS may feature similar attack profiles with periodicity, and ideally, this would be explored prospectively. In
1646 such patients thought to have IBS, the hypothalamus could be a central attack “generator” or an important epiphenomenon, akin to CH. As in episodic CH, such patients could be eligible for prophylactic therapies just during attack periods only.
STATEMENT OF AUTHORSHIP Category 1 (a) Conception and Design Matthew S. Robbins (b) Acquisition of Data Matthew S. Robbins (c) Analysis and Interpretation of Data Matthew S. Robbins Category 2 (a) Drafting the Manuscript Matthew S. Robbins (b) Revising It for Intellectual Content Matthew S. Robbins Category 3 (a) Final Approval of the Completed Manuscript Matthew S. Robbinss
November/December 2014
2.
3. 4.
5.
6.
7. 8.
9. 10.
REFERENCES 1. Headache Classification Committee of the International Headache Society. The International
11.
classification of headache disorders, 3rd edition (beta version). Cephalalgia. 2013;33:629-808. Holle D, Obermann M. Cluster headache and the hypothalamus: Causal relationship or epiphenomenon? Expert Rev Neurother. 2011;11:1255-1263. Mayer EA. Clinical practice. Irritable bowel syndrome. N Engl J Med. 2008;358:1692-1699. Canavan C, West J, Card T. The epidemiology of irritable bowel syndrome. Clin Epidemiol. 2014;6:7180. Lau CI, Lin CC, Chen WH, Wang HC, Kao CH. Association between migraine and irritable bowel syndrome: A population-based retrospective cohort study. Eur J Neurol. 2014 May 17. doi: 10.1111/ ene.12468. Nojkov B, Rubenstein JH, Chey WD, Hoogerwerf WA. The impact of rotating shift work on the prevalence of irritable bowel syndrome in nurses. Am J Gastroenterol. 2010;105:842-847. Derbyshire SW. Imaging visceral pain. Curr Pain Headache Rep. 2007;11:178-182. Worawattanakul M, Rhoads JM, Lichtman SN, Ulshen MH. Abdominal migraine: Prophylactic treatment and follow-up. J Pediatr Gastroenterol Nutr. 1999;28:37-40. McLeod J. Verapamil effective in irritable bowel syndrome? Med J Aust. 1983;2:119. Byrne S. Verapamil in the treatment of irritable bowel syndrome. J Clin Psychiatry. 1987;48:388. Siah KT, Wong RK, Ho KY. Melatonin for the treatment of irritable bowel syndrome. World J Gastroenterol. 2014;20:2492-2498.
Copyright of Headache: The Journal of Head & Face Pain is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Headache © 2014 American Headache Society
Brief Communications Cluster Belly: A Variant of Irritable Bowel Syndrome Matthew S. Robbins, MD
Cluster headache (CH) and irritable bowel syndrome (IBS) are pain disorders that possess relationships with circadian rhythms. However, they have not been compared to assess similarities that could yield pathophysiologic insights. A young male adult with periodic episodes of abdominal pain highly reminiscent of CH is described. Since childhood, he experienced severe attacks featuring excruciating, abdominal pain accompanied by prominent restlessness, lasting 30-120 minutes, occurring in the evening and in discrete 2- to 8-week periods, interspersed with remissions where typical triggers did not lead to attacks. Although all of the patient’s symptoms fell within the spectrum of IBS, the semiology was highly evocative of CH, based on the attack duration, restlessness, periodicity, and selective vulnerability to particular triggers only during attack periods. A subset of patients thought to have IBS may feature similar attack profiles and could suggest the importance of the hypothalamus in its pathophysiology, akin to CH. Key words: cluster headache, irritable bowel syndrome, hypothalamus, abdominal pain, abdominal migraine (Headache 2014;54:1644-1646)
Cluster headache (CH) distinguishes itself from all other headache disorders1 by its distinctive circadian and circannual periodicity, implicating the role of the hypothalamus in its underlying pathophysiology.2 CH and irritable bowel syndrome (IBS) are both disorders featuring recurrent attacks of pain that possess relationships with circadian rhythms and potentially hypothalamic derangements.The object of this study was to describe a patient with periodic attacks of abdominal pain who was diagnosed with IBS but had clinical features highly reminiscent of CH.
From the Montefiore Headache Center, Department of Neurology, Albert Einstein College of Medicine, Bronx, NY, USA. Address all correspondence to M.S. Robbins, 1575 Blondell Avenue, Suite 225, Bronx, NY 10461, USA. Accepted for publication June 21, 2014.
CASE REPORT A 20-year-old undergraduate student started experiencing episodes of abdominal pain at 6 years of age. Attacks consisted of moderate, crampy, diffuse abdominal pain episodes with flatulence, predictably triggered by greasy or creamy foods, particularly during times of stress, typically leading to diarrhea and relief after defecation. At 12 years of age, he began to experience different, more severe attacks, featuring excruciating, searing, crampy, usually unilateral and lower abdominal pain, accompanied by diaphoresis and mild nausea. He felt extraordinarily restless during the episodes, possessing the urge to repeatedly bend over or pace during the attacks, which lasted Conflict of Interest: Matthew S. Robbins, MD, has received honoraria from the American Headache Society, Prova Education, American College of Physicians, Medlink, North ShoreLIJ Hofstra School of Medicine, and SUNY Downstate College of Medicine, and receives book royalties from Wiley. Funding: None.
1644
Headache 30-120 minutes, predominantly occurring between 3 pm and 1 am. Attacks occurred in periods lasting 2-8 weeks, interspersed with remissions over months. Only during attack periods were triggers such as red wine and fried foods identified; these substances did not trigger pain outside of the attack periods. Defecation inconsistently provided relief. He had infrequent episodic tension-type headache whose attacks did not occur during periods of abdominal pain. He was otherwise healthy, a nonsmoker, used alcohol in moderation, and took no medications. There was no family history of any frequent or severe headache or pain disorder. His medical and neurological examinations were normal, and referral to a gastroenterologist leads to the diagnosis of IBS, with dietary counseling. Over the subsequent few years, he continued to have one to two annual clusters of attacks but did not seek specific medical treatment.
COMMENTS This patient fulfilled criteria for IBS, which is defined as a chronic, functional gastrointestinal disorder featuring recurrent abdominal pain and alterations in bowel habits, in the absence of any secondary cause.3 IBS is common, affecting 11% of the population, has a female predominance, aggregates in families, and is comorbid with many pain disorders, including headache, although not specifically CH.4 A recent population-based retrospective cohort study in Taiwan identified a nearly twofold increase in the incidence of IBS in persons with migraine after adjusting for age, gender, and medical comorbidities.5 In those with frequent migraine, adjusted incidence of IBS was 7.61 (95% confidence interval 6.33-9.13) in comparison with a migraine-free cohort.5 Although more local factors including gastrointestinal dysmotility, visceral hypersensitivity, intestinal inflammation, and infection have all been posited to play a role in the pathophysiology of IBS, comorbid pain and psychiatric conditions, as well as therapeutic responses to certain medications suggest an important role of the central nervous system.3 The most striking aspect of this case is the periodicity of attacks, which in IBS remains relatively unexplored. One clinical study of IBS in nurses
1645 revealed that those working rotating shifts had a significantly higher prevalence of IBS compared with day-shift nurses (48% vs 31%, P < 0.01). This discrepancy may imply the importance of a circadian rhythm disturbance in the pathogenesis of IBS, although this could relate to both the central and enteric nervous systems.6 Functional neuroimaging studies in IBS and visceral pain have yielded inconsistent results, without a clear signature, and the hypothalamus is not described to be a region of particular activation.7 In CH, the hypothalamus clearly plays an important role, based on the clinical features (attack periodicity, association with sleep, prominent autonomic features) as well as neuroendocrine abnormalities.2 Structural neuroimaging studies have detected increased grey matter in the inferior posterior hypothalamus and functional neuroimaging studies have revealed strong activation in the same region, which has been the target of deep brain stimulation in refractory chronic CH patients.2 Although all of the patient’s symptoms may fall within the spectrum of IBS,3 the semiology of the recurrent abdominal pain attacks are reminiscent of CH, based on the attack duration, restlessness, periodicity, and selective vulnerability to particular triggers only during attack periods. Abdominal migraine is currently an accepted diagnosis1 thought to be a precursor to migraine, which may have similar underlying neurobiology. Indirect evidence of the importance of the central nervous system in abdominal migraine, as well as shared pathophysiology with cephalgic migraine, is suggested by its response to varying nonanalgesic prophylactic medications.8 It is appealing to consider this case as a form of “abdominal” CH or “cluster belly” based on such an overlap of the clinical phenomenology. There are reports of utilizing medications that are effective in CH prophylaxis in the treatment of IBS, including single cases for verapamil9,10 and placebo-controlled studies for melatonin,11 although their mechanisms could instead be related to direct changes in gut motility and circadian rhythm regulation in the enteric nervous system. It is plausible that a subset of patients with IBS may feature similar attack profiles with periodicity, and ideally, this would be explored prospectively. In
1646 such patients thought to have IBS, the hypothalamus could be a central attack “generator” or an important epiphenomenon, akin to CH. As in episodic CH, such patients could be eligible for prophylactic therapies just during attack periods only.
STATEMENT OF AUTHORSHIP Category 1 (a) Conception and Design Matthew S. Robbins (b) Acquisition of Data Matthew S. Robbins (c) Analysis and Interpretation of Data Matthew S. Robbins Category 2 (a) Drafting the Manuscript Matthew S. Robbins (b) Revising It for Intellectual Content Matthew S. Robbins Category 3 (a) Final Approval of the Completed Manuscript Matthew S. Robbinss
November/December 2014
2.
3. 4.
5.
6.
7. 8.
9. 10.
REFERENCES 1. Headache Classification Committee of the International Headache Society. The International
11.
classification of headache disorders, 3rd edition (beta version). Cephalalgia. 2013;33:629-808. Holle D, Obermann M. Cluster headache and the hypothalamus: Causal relationship or epiphenomenon? Expert Rev Neurother. 2011;11:1255-1263. Mayer EA. Clinical practice. Irritable bowel syndrome. N Engl J Med. 2008;358:1692-1699. Canavan C, West J, Card T. The epidemiology of irritable bowel syndrome. Clin Epidemiol. 2014;6:7180. Lau CI, Lin CC, Chen WH, Wang HC, Kao CH. Association between migraine and irritable bowel syndrome: A population-based retrospective cohort study. Eur J Neurol. 2014 May 17. doi: 10.1111/ ene.12468. Nojkov B, Rubenstein JH, Chey WD, Hoogerwerf WA. The impact of rotating shift work on the prevalence of irritable bowel syndrome in nurses. Am J Gastroenterol. 2010;105:842-847. Derbyshire SW. Imaging visceral pain. Curr Pain Headache Rep. 2007;11:178-182. Worawattanakul M, Rhoads JM, Lichtman SN, Ulshen MH. Abdominal migraine: Prophylactic treatment and follow-up. J Pediatr Gastroenterol Nutr. 1999;28:37-40. McLeod J. Verapamil effective in irritable bowel syndrome? Med J Aust. 1983;2:119. Byrne S. Verapamil in the treatment of irritable bowel syndrome. J Clin Psychiatry. 1987;48:388. Siah KT, Wong RK, Ho KY. Melatonin for the treatment of irritable bowel syndrome. World J Gastroenterol. 2014;20:2492-2498.
Copyright of Headache: The Journal of Head & Face Pain is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
