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Avellis syndrome as presenting manifestation of ulcerative colitis
Journal of the Neurological Sciences 325 (2013) 160–161
Contents lists available at SciVerse ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Short communication
Avellis syndrome as presenting manifestation of ulcerative colitis Bo-Lin Ho a, b, d, Fang-Jung Yu c, Chiou-Lian Lai b, d, Hsin-Hsin Lin b, d, e,⁎ a
Department of Neurology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, Taiwan Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan c Department of Gastroenterology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan d Department of Master's Program in Neurology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan e Department of Neurology, Kaohsiung Municipal Hsiaokang Hospital, Kaohsiung, Taiwan b
a r t i c l e
i n f o
Article history: Received 17 June 2012 Received in revised form 12 November 2012 Accepted 27 November 2012 Available online 17 January 2013 Keywords: Ulcerative colitis Avellis syndrome Inflammatory bowel disease Vasculitis White-matter lesion
a b s t r a c t We described a 41-year-old woman presenting with subacute onset of left hemihypesthesia, left facial palsy, dysphagia and dysgeusia. A cranial T2-weighted magnetic resonance imaging revealed bilateral inhomogeneous medullary hyperintensities. The clinical manifestations conformed to Avellis syndrome, and were linked to the diagnosis of ulcerative colitis which was proved by serological findings and pathological evidence on rectosigmoid mucosa. She recovered favorably under conservative medical treatment with complete remission over one month of follow-up. Brainstem syndromes are rarely associated with neurological complications of ulcerative colitis and can be the presenting manifestation beyond gastrointestinal symptoms. © 2012 Elsevier B.V. All rights reserved.
1. Introduction Avellis syndrome was originally described as the combination of ipsilateral palatolaryngeal paresis with contralateral hemiparesis and/ or hemihypesthesia. In addition to brainstem ischemia, few specific mechanisms have been reported to produce Avellis syndrome, including cranial trauma, infection-related arteritis or rheumatoid vasculitis [1–3]. Being vascular in origin, this classical but rare brainstem syndrome can represent a part of emerging manifestations of systemic diseases, and thus lead to a comprehensive survey for the underlying pathogenesis. Herein we reported the first case of atypical Avellis syndrome as the notable presentation of ulcerative colitis (UC). 2. Case report A 41-year-old woman developed progressive numbness of the leftside extremities and trunk for 2 weeks. She had been previously healthy without known systemic disease. On admission, the patient was afebrile and fully alert. Neurological examination revealed dysphagia, dysgeusia, mild left central facial palsy, a paralyzed soft palate and absent gag reflex on the left side. Hypesthesia to light touch and pain senses was detected in the left-side face, trunk and extremities. Limb coordination was intact.
⁎ Corresponding author at: Department of Neurology, Kaohsiung Medical University Hospital, No.100, Tz-You 1st Road, Kaohsiung 80756, Taiwan. Tel.: +886 73121101x6833; fax: +886 73162158. E-mail address: [email protected] (H.-H. Lin). 0022-510X/$ – see front matter © 2012 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.jns.2012.11.015
Hematological examinations showed mild normocytic anemia (hemoglobin 11.6 g/dL, hematocrit 34.7%) without evidence of coagulopathy. Serum titers of anti-neutrophil cytoplasmic autoantibodies directed to proteinase-3 (PR3-ANCA) were positive. Rheumatoid factor, anti-nuclear and anti-DNA antibodies were negative. Serum protein electrophoresis revealed polyclonal gammopathy. Cerebrospinal fluid analysis was unremarkable. Auditory brainstem response, visual and somatosensory evoked potentials, and nerve conduction studies were normal. A cranial T2-weighted magnetic resonance imaging (MRI) disclosed inhomogeneous hyperintense lesions in the dorsal portion of the upper medulla oblongata affecting both sides (Fig. 1). Colonoscopy was performed because of the occult blood in the routine stool examination, and revealed diffuse ulceration with hyperemic mucosal change extending from the sigmoid colon to the rectum. The pathological findings of a colonic biopsy demonstrated dense lymphocytic infiltration limited to the intestinal mucosa, distorted glands and crypt abscesses, compatible with UC. Under conservative treatment of short-term steroid, her neurological symptoms had resolved gradually by the one-month follow-up. 3. Discussion Avellis syndrome is a rare condition of alternating hemiparesis which mostly resulted from medullary infarction secondary to vertebrobasilar artery thrombosis. Typical presentations of Avellis syndrome are ipsilateral paralysis of the soft palate and vocal cord, with contralateral loss of pain and temperature sensation. The major topographical localization is rostral midlateral medulla with involvement of nucleus ambiguous [4]. In
B.-L. Ho et al. / Journal of the Neurological Sciences 325 (2013) 160–161
161
Fig. 1. T2-weighted MRI (A) showed bilateral hyperintense lesions in the upper medulla oblongata. Schematic representation (B) of the medullary lesions (shadowed areas) demonstrated asymmetrical involvement of the left spinotrigeminal nucleus and tract, left nucleus ambiguous, and right lateral spinothalamic tract.
our patient, the corresponding upper medullary lesions asymmetrically affected the left spinotrigeminal nucleus and tract, left nucleus ambiguous, and also the right lateral spinothalamic tract, which resulted in left-sided pharyngeal palsy and contralateral hemihypesthesia. However, left hemihypesthesia was contrasted by more severe involvement of right lateral spinothalamic tract than the other side caused by left spinotrigeminal lesion. An aberrant supranuclear pathway looping around the nucleus ambiguous to the facial nucleus was presumed to be responsible for the ipsilateral central facial palsy [5]. Without detectable vertebrobasilar stenosis in MR angiography, small-vessel vasculitides of perforating branches of vertebral arteries were the major pathologies to cause such atypical Avellis syndrome. The presence of PR3-ANCA and fecal occult blood indicated to the further investigations of UC. The extraintestinal symptoms are observed in 20%–40% of patients with inflammatory bowel diseases (IBD), and are more prevalent in those with Crohn's disease than in UC. However, only 3% of IBD patients had neurologic involvement in a large retrospective study [6]. Neurological manifestations of UC are uncommon and may variably develop in clinical pattern and in temporal association to the intestinal disorders. UC can manifest both in the peripheral and central nervous systems (CNS), comprising peripheral neuropathies, thromboembolic events, systemic and cerebral vasculitis, white-matter lesions (WMLs), demyelinating diseases, and epileptic seizures [7,8]. In previous studies, less than half of UC patients who were all neurologically symptom-free had focal WMLs on brain MRI, and its clinical relevance didn't infer to active CNS diseases [9,10]. Multiple sclerosis and acute disseminated encephalomyelitis are noteworthy considerations of such lesions, while their distinctive neuroradiological patterns are helpful to distinguish each entity [7]. The underlying mechanisms of CNS WMLs in UC patients remain indefinite. An immune-mediated or inflammatory response as active demyelination, cerebral vasculitis may play a major role in the pathogenesis. As the differentiation between major pathogenic entities of focal WMLs is usually unrecognizable on brain MRI, we investigated vasculitic markers and cardiovascular risk factors to distinguish vasculitis from atherosclerotic origin [9]. The presence of ANCA is strongly associated with small-vessel vasculitis secondary to autoimmune etiology, and UC with positive PR3-ANCA may belong to a specific subtype [11]. Similar to our observation, those IBD patients with
multifocal CNS WMLs usually appeared to resolve with favorable outcomes clinically and radiologically after receiving corticosteroids or immunomodulating treatments [12]. In conclusion, UC may well induce brainstem vasculitides as a rare occasion of Avellis syndrome via extraintestinal immune-mediated responses. Among the neurological aspects of IBD, cranial nerve syndromes are particularly infrequent and can be the presenting manifestation of UC. Conflict of interest None. References [1] Kitanaka C, Sugaya M, Yamada H. Avellis syndrome after minor head trauma: report of two cases. Surg Neurol 1992;37:236–9. [2] Habek M, Mubrin Z, Brinar VV. Avellis syndrome due to borreliosis. Eur J Neurol 2007;14:112–4. [3] Kashihara K, Ishizu H, Shomori T, Iwane H, Ota H. Avellis syndrome in systemic rheumatoid vasculitis. Rinsho Shinkeigaku 1995;35:1155–9. [4] Kataoka S, Hori A, Hirose G, Nakanishi M, Yamakawa J. Avellis' syndrome: the neurological-topographical correlation. Eur Neurol 2001;45:292–3. [5] Takahashi K, Kitani M, Fukuda H. A case of Avellis' syndrome with ipsilateral central facial palsy due to a small medullary infarction. Rinsho Shinkeigaku 2000;40: 409–11. [6] Lossos A, River Y, Eliakim A, Steiner I. Neurologic aspects of inflammatory bowel disease. Neurology 1995;45:416–21. [7] Scheid R, Teich N. Neurologic manifestations of ulcerative colitis. Eur J Neurol 2007;14:483–93. [8] Benavente L, Morís G. Neurologic disorders associated with inflammatory bowel disease. Eur J Neurol 2011;18:138–43. [9] Geissler A, Andus T, Roth M, Kullmann F, Caesar I, Held P, et al. Focal white-matter lesions in brain of patients with inflammatory bowel disease. Lancet 1995;345: 897–8. [10] Hart PE, Gould SR, MacSweeney JE, Clifton A, Schon F. Brain white-matter lesions in inflammatory bowel disease. Lancet 1998;351:1558. [11] Xu J, Yang CH, Chen XY, Li XH, Dai M, Xiao SD. A subset of ulcerative colitis with positive proteinase-3 antineutrophil cytoplasmic antibody. World J Gastroenterol 2008;14:7012–5. [12] de Lau LM, de Vries JM, van der Woude CJ, Kuipers EJ, Siepman DA, Sillevis Smitt PA, et al. Acute CNS white matter lesions in patients with inflammatory bowel disease. Inflamm Bowel Dis 2009;15:576–80.
Contents lists available at SciVerse ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Short communication
Avellis syndrome as presenting manifestation of ulcerative colitis Bo-Lin Ho a, b, d, Fang-Jung Yu c, Chiou-Lian Lai b, d, Hsin-Hsin Lin b, d, e,⁎ a
Department of Neurology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, Taiwan Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan c Department of Gastroenterology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan d Department of Master's Program in Neurology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan e Department of Neurology, Kaohsiung Municipal Hsiaokang Hospital, Kaohsiung, Taiwan b
a r t i c l e
i n f o
Article history: Received 17 June 2012 Received in revised form 12 November 2012 Accepted 27 November 2012 Available online 17 January 2013 Keywords: Ulcerative colitis Avellis syndrome Inflammatory bowel disease Vasculitis White-matter lesion
a b s t r a c t We described a 41-year-old woman presenting with subacute onset of left hemihypesthesia, left facial palsy, dysphagia and dysgeusia. A cranial T2-weighted magnetic resonance imaging revealed bilateral inhomogeneous medullary hyperintensities. The clinical manifestations conformed to Avellis syndrome, and were linked to the diagnosis of ulcerative colitis which was proved by serological findings and pathological evidence on rectosigmoid mucosa. She recovered favorably under conservative medical treatment with complete remission over one month of follow-up. Brainstem syndromes are rarely associated with neurological complications of ulcerative colitis and can be the presenting manifestation beyond gastrointestinal symptoms. © 2012 Elsevier B.V. All rights reserved.
1. Introduction Avellis syndrome was originally described as the combination of ipsilateral palatolaryngeal paresis with contralateral hemiparesis and/ or hemihypesthesia. In addition to brainstem ischemia, few specific mechanisms have been reported to produce Avellis syndrome, including cranial trauma, infection-related arteritis or rheumatoid vasculitis [1–3]. Being vascular in origin, this classical but rare brainstem syndrome can represent a part of emerging manifestations of systemic diseases, and thus lead to a comprehensive survey for the underlying pathogenesis. Herein we reported the first case of atypical Avellis syndrome as the notable presentation of ulcerative colitis (UC). 2. Case report A 41-year-old woman developed progressive numbness of the leftside extremities and trunk for 2 weeks. She had been previously healthy without known systemic disease. On admission, the patient was afebrile and fully alert. Neurological examination revealed dysphagia, dysgeusia, mild left central facial palsy, a paralyzed soft palate and absent gag reflex on the left side. Hypesthesia to light touch and pain senses was detected in the left-side face, trunk and extremities. Limb coordination was intact.
⁎ Corresponding author at: Department of Neurology, Kaohsiung Medical University Hospital, No.100, Tz-You 1st Road, Kaohsiung 80756, Taiwan. Tel.: +886 73121101x6833; fax: +886 73162158. E-mail address: [email protected] (H.-H. Lin). 0022-510X/$ – see front matter © 2012 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.jns.2012.11.015
Hematological examinations showed mild normocytic anemia (hemoglobin 11.6 g/dL, hematocrit 34.7%) without evidence of coagulopathy. Serum titers of anti-neutrophil cytoplasmic autoantibodies directed to proteinase-3 (PR3-ANCA) were positive. Rheumatoid factor, anti-nuclear and anti-DNA antibodies were negative. Serum protein electrophoresis revealed polyclonal gammopathy. Cerebrospinal fluid analysis was unremarkable. Auditory brainstem response, visual and somatosensory evoked potentials, and nerve conduction studies were normal. A cranial T2-weighted magnetic resonance imaging (MRI) disclosed inhomogeneous hyperintense lesions in the dorsal portion of the upper medulla oblongata affecting both sides (Fig. 1). Colonoscopy was performed because of the occult blood in the routine stool examination, and revealed diffuse ulceration with hyperemic mucosal change extending from the sigmoid colon to the rectum. The pathological findings of a colonic biopsy demonstrated dense lymphocytic infiltration limited to the intestinal mucosa, distorted glands and crypt abscesses, compatible with UC. Under conservative treatment of short-term steroid, her neurological symptoms had resolved gradually by the one-month follow-up. 3. Discussion Avellis syndrome is a rare condition of alternating hemiparesis which mostly resulted from medullary infarction secondary to vertebrobasilar artery thrombosis. Typical presentations of Avellis syndrome are ipsilateral paralysis of the soft palate and vocal cord, with contralateral loss of pain and temperature sensation. The major topographical localization is rostral midlateral medulla with involvement of nucleus ambiguous [4]. In
B.-L. Ho et al. / Journal of the Neurological Sciences 325 (2013) 160–161
161
Fig. 1. T2-weighted MRI (A) showed bilateral hyperintense lesions in the upper medulla oblongata. Schematic representation (B) of the medullary lesions (shadowed areas) demonstrated asymmetrical involvement of the left spinotrigeminal nucleus and tract, left nucleus ambiguous, and right lateral spinothalamic tract.
our patient, the corresponding upper medullary lesions asymmetrically affected the left spinotrigeminal nucleus and tract, left nucleus ambiguous, and also the right lateral spinothalamic tract, which resulted in left-sided pharyngeal palsy and contralateral hemihypesthesia. However, left hemihypesthesia was contrasted by more severe involvement of right lateral spinothalamic tract than the other side caused by left spinotrigeminal lesion. An aberrant supranuclear pathway looping around the nucleus ambiguous to the facial nucleus was presumed to be responsible for the ipsilateral central facial palsy [5]. Without detectable vertebrobasilar stenosis in MR angiography, small-vessel vasculitides of perforating branches of vertebral arteries were the major pathologies to cause such atypical Avellis syndrome. The presence of PR3-ANCA and fecal occult blood indicated to the further investigations of UC. The extraintestinal symptoms are observed in 20%–40% of patients with inflammatory bowel diseases (IBD), and are more prevalent in those with Crohn's disease than in UC. However, only 3% of IBD patients had neurologic involvement in a large retrospective study [6]. Neurological manifestations of UC are uncommon and may variably develop in clinical pattern and in temporal association to the intestinal disorders. UC can manifest both in the peripheral and central nervous systems (CNS), comprising peripheral neuropathies, thromboembolic events, systemic and cerebral vasculitis, white-matter lesions (WMLs), demyelinating diseases, and epileptic seizures [7,8]. In previous studies, less than half of UC patients who were all neurologically symptom-free had focal WMLs on brain MRI, and its clinical relevance didn't infer to active CNS diseases [9,10]. Multiple sclerosis and acute disseminated encephalomyelitis are noteworthy considerations of such lesions, while their distinctive neuroradiological patterns are helpful to distinguish each entity [7]. The underlying mechanisms of CNS WMLs in UC patients remain indefinite. An immune-mediated or inflammatory response as active demyelination, cerebral vasculitis may play a major role in the pathogenesis. As the differentiation between major pathogenic entities of focal WMLs is usually unrecognizable on brain MRI, we investigated vasculitic markers and cardiovascular risk factors to distinguish vasculitis from atherosclerotic origin [9]. The presence of ANCA is strongly associated with small-vessel vasculitis secondary to autoimmune etiology, and UC with positive PR3-ANCA may belong to a specific subtype [11]. Similar to our observation, those IBD patients with
multifocal CNS WMLs usually appeared to resolve with favorable outcomes clinically and radiologically after receiving corticosteroids or immunomodulating treatments [12]. In conclusion, UC may well induce brainstem vasculitides as a rare occasion of Avellis syndrome via extraintestinal immune-mediated responses. Among the neurological aspects of IBD, cranial nerve syndromes are particularly infrequent and can be the presenting manifestation of UC. Conflict of interest None. References [1] Kitanaka C, Sugaya M, Yamada H. Avellis syndrome after minor head trauma: report of two cases. Surg Neurol 1992;37:236–9. [2] Habek M, Mubrin Z, Brinar VV. Avellis syndrome due to borreliosis. Eur J Neurol 2007;14:112–4. [3] Kashihara K, Ishizu H, Shomori T, Iwane H, Ota H. Avellis syndrome in systemic rheumatoid vasculitis. Rinsho Shinkeigaku 1995;35:1155–9. [4] Kataoka S, Hori A, Hirose G, Nakanishi M, Yamakawa J. Avellis' syndrome: the neurological-topographical correlation. Eur Neurol 2001;45:292–3. [5] Takahashi K, Kitani M, Fukuda H. A case of Avellis' syndrome with ipsilateral central facial palsy due to a small medullary infarction. Rinsho Shinkeigaku 2000;40: 409–11. [6] Lossos A, River Y, Eliakim A, Steiner I. Neurologic aspects of inflammatory bowel disease. Neurology 1995;45:416–21. [7] Scheid R, Teich N. Neurologic manifestations of ulcerative colitis. Eur J Neurol 2007;14:483–93. [8] Benavente L, Morís G. Neurologic disorders associated with inflammatory bowel disease. Eur J Neurol 2011;18:138–43. [9] Geissler A, Andus T, Roth M, Kullmann F, Caesar I, Held P, et al. Focal white-matter lesions in brain of patients with inflammatory bowel disease. Lancet 1995;345: 897–8. [10] Hart PE, Gould SR, MacSweeney JE, Clifton A, Schon F. Brain white-matter lesions in inflammatory bowel disease. Lancet 1998;351:1558. [11] Xu J, Yang CH, Chen XY, Li XH, Dai M, Xiao SD. A subset of ulcerative colitis with positive proteinase-3 antineutrophil cytoplasmic antibody. World J Gastroenterol 2008;14:7012–5. [12] de Lau LM, de Vries JM, van der Woude CJ, Kuipers EJ, Siepman DA, Sillevis Smitt PA, et al. Acute CNS white matter lesions in patients with inflammatory bowel disease. Inflamm Bowel Dis 2009;15:576–80.