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  IN THIS Article
 ::  Abstract
 :: Introduction
 :: Case Reports
 :: Discussion
 :: Conclusion
 ::  References
 ::  Article Figures

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  Table of Contents     
CASE REPORT
Year : 2013  |  Volume : 59  |  Issue : 2  |  Page : 131-134

Cerebral demyelination in children with collagenous colitis


1 Department of Pediatrics, Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA
2 Department of Pediatric Gastroenterology, Oklahoma University Health Sciences Center, Oklahoma City, Oklahoma, USA
3 Delta Pathology, Shreveport, Louisiana, USA
4 WK Pediatric Gastroenterology and Research, Willis-Knighton Hospital, Shreveport, Louisiana, USA

Date of Submission11-Jun-2012
Date of Decision08-Dec-2012
Date of Acceptance13-Feb-2013
Date of Web Publication21-Jun-2013

Correspondence Address:
S Z Hussain
WK Pediatric Gastroenterology and Research, Willis-Knighton Hospital, Shreveport, Louisiana
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0022-3859.113848

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 :: Abstract 

Collagenous colitis (CC) is a form of microscopic colitis characterized by the presence of inflammatory infiltrate and subepithelial deposition of collagen in the colon and it is a rare condition with a predominant prevalence in the adult population. Only few cases have been reported in children. We report two children with the CC with concomitant neurological manifestations. Both cases demonstrated variable neurological symptoms clinically and significant cerebral demyelination. In both patients, the gastrointestinal manifestations drastically improved with a short course of prednisolone. However, the neurological symptoms were persistent and progressive. To the best of our knowledge, similar association has not been reported in children.


Keywords: Children, collagenous colitis, demyelination, microscopic colitis


How to cite this article:
Sankararaman S, Osman M, Wellman G, Hussain S Z. Cerebral demyelination in children with collagenous colitis. J Postgrad Med 2013;59:131-4

How to cite this URL:
Sankararaman S, Osman M, Wellman G, Hussain S Z. Cerebral demyelination in children with collagenous colitis. J Postgrad Med [serial online] 2013 [cited 2019 Oct 16];59:131-4. Available from: http://www.jpgmonline.com/text.asp?2013/59/2/131/113848



 :: Introduction Top


Collagenous colitis (CC) is a form of microscopic colitis characterized by the presence of chronic inflammatory infiltrate and subepithelial deposition of collagen in the colon. CC is a rare disease seen in the adult population with a mean age of diagnosis at 64 years and with female predilection. [1] Only few cases have been reported in children. [2],[3],[4] The clinical symptoms are usually watery diarrhea without mucus or blood and rarely abdominal pain.


 :: Case Reports Top


Case 1

An 11-year-old Caucasian girl presented with 1 year history of recurrent lower abdominal pain. She also had intermittent attacks of watery diarrhea (four to five episodes/day) alternating with constipation. Her past medical history revealed asthma, dust mite allergy, attention deficit hyperactivity, and episodes of headache. Family history was not contributory. Growth and development were appropriate for the age. Physical examination was within normal limits including a normal abdominal examination and the absence of focal neurological signs. The laboratory work-up including complete blood count (CBC), comprehensive metabolic panel (CMP), erythrocyte sedimentation rate (ESR), and stool studies (culture, leucocytes and ova for parasites) were negative. Upper and lower gastrointestinal (GI) endoscopies did not reveal any gross abnormalities. Colonic biopsies demonstrated thick subepithelial collagenous bands and increased infiltration of lymphocytes and plasma cells in the lamina propria throughout the entire colon [Figure 1]a, which favored the diagnosis of CC. There was also evidence of epithelial damage and the presence of intraepithelial lymphocytes. Biopsies from stomach showed chronic gastritis with negative Helicobacter pylori test. There was no evidence of celiac disease (CD) or collagenous gastritis. The patient received a weaning 4 week course of prednisone (1.5 mg/kg/day). There was a dramatic improvement in the GI symptoms after the short course prednisone therapy and her GI symptoms did not recur. At 1 year follow-up, endoscopic biopsies showed complete resolution of the collagenous deposition and marked decrease in the inflammatory infiltrates [Figure 1]b. Meanwhile, she developed motor tics involving facial muscles and the upper extremities. She also developed nystagmus and her hyperactivity worsened. Ophthalmological screening was within normal limits. Neurometabolic work-up such as serum lactate, serum pyruvate, serum amino acids, and urine for organic acids were normal. Electroencephalograph did not reveal any abnormalities. Magnetic resonance imaging (MRI) of the brain showed diffuse significant demyelination of the brain [Figure 2] as well as an Arnold Chiari type I malformation [Figure 2]c. Patient was again seen at 18 months. Her GI symptoms did not recur, but her neurological/behavioral symptoms were persistent. More extensive work-up is planned and she is under follow-up.
Figure 1: Colonic biopsies of case 1: (a) Arrows denote a thickened subepithelial collage band. Arrow head denotes superficially predominate mononuclear cell infiltrate in the lamina propria and (b) At 1 year follow-up, please note the marked decrease in the cellular infiltrates and normal collagen thickness (H and E, ×200)

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Figure 2: Magnetic resonance imaging brain of case (1): (a-b) MR axial T2 weighted images showing hyperintense signals corresponding to the areas of demyelination (yellow arrows) and (c) MR coronal fluid attenuated inversion recovery sequence with hyperintense signals corresponding to the areas of demyelination (yellow arrows), as well as Arnold Chiari type I malformation (bottom horizontal orange arrow)

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Case 2

A 5-year-old Caucasian boy presented with a history of chronic watery diarrhea since birth. Mother reported five to six loose stools with undigested food almost every other day. Patient was diagnosed with toddler's diarrhea initially by the pediatrician and was reassured that the increased frequency of the loose stools would resolve. The patient had intermittent episodes of periumbilical pain with variable intensity. He also had mild developmental delay and behavioral problems. Past medical history was otherwise unremarkable including a normal newborn screening. Family history revealed that the patient's mother had a diagnosis of multiple sclerosis (MS). Patient's weight and height were at 10 th percentile for age. Physical examination revealed normal abdominal examination and he did not have any neurological deficits. A diagnostic work-up including CBC, CMP, ESR, tissue transglutaminase immunoglobulin A (IgA) antibody were within normal limits. Stools studies showed no evidence of bacterial infection or parasitic infestation. Upper and lower GI endoscopies showed normal appearing mucosa. Colonic biopsies demonstrated thick subepithelial collagenous bands and increased inflammatory cellular infiltration in the lamina propria throughout the entire colon which was consistent with CC [Figure 3]a. Surface epithelial damage and intraepithelial lymphocytes were also seen. There was no evidence of collagenous gastritis or villous atrophy. He received a 4 weeks course of prednisone (1.5 mg/kg/day), which was tapered and stopped. Follow-up visit after 1 year showed that the patient was free of GI symptoms. Follow-up endoscopic biopsies at 1 year showed histological improvement of the inflammatory cell infiltration with minimal residual collagen deposition [Figure 3]b. Six months through follow-up, the patient's neurological condition deteriorated and he developed motor clumsiness, lack of coordination, as well as worsening behavioral problems. An episode of focal seizure was also reported. MRI of the brain was performed which showed multiple areas of demyelination in the white matter of the brain [Figure 4]. The patient moved out of state and was lost to follow-up. More extensive work-up and long-term follow-up is planned through the patient's primary pediatrician.
Figure 3: Colonic biopsies of case 2: (a) Prior to the treatment with prednisone; arrows denote a thickened subepithelial collage band. Arrow head denotes superficially predominate mononuclear cell infiltrate in the lamina propria and (b) At 1 year follow-up please note the decrease in the cellular infiltrates and reduction of the collagen thickness (H and E, ×200)

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Figure 4: MR brain of case 2: (a-b) MR Sagittal FLAIR sequences showing small areas of hyperintense signals corresponding to the areas of demyelination (yellow arrows) and (c-d) MR axial FLAIR sequences showing small areas of hyperintense signals corresponding to the areas of demyelination (yellow arrows)

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 :: Discussion Top


CC is a form of microscopic colitis with a predominant prevalence in the adult population. [1] The initial CC case reports in children were documented in the year 1989. Since then, very few cases have been reported in children. [2],[3],[4] The clinical presentation in children is very similar to adults and consists mainly of chronic watery diarrhea, without mucus or blood, and occasional episodes of abdominal pain. Severe dehydration and constitutional symptoms are not common. [1] The incidence in adults ranges from 1.1-5/100,000 population [5],[6] while the exact incidence in children is unknown. The exact etiology of CC is still unknown and the treatment is mainly empirical. [4] Corticosteroids have been used with variable response. [4]

A variety of other GI, autoimmune and connective tissue disorders have been reported in patients with CC. [1] They include CD, inflammatory bowel disease, collagenous gastritis, eosinophilic gastroenteropathy, autoimmune thyroiditis, diabetes mellitus as well as scleroderma [4],[7],[8],[9] Unlike other GI diseases such as Whipple's disease or CD or inflammatory bowel disease, neurological manifestations are not commonly reported in patients with CC. [10] The association of CC and CD is well-known and in fact CC has been reported as the presenting feature of CD. [11] CD is being increasing recognized now and a variety of neurological manifestations such as ataxia, demyelination, seizures, myoclonus have been reported in CD. [12],[13] Even though our patients did not test positive for CD during an initial work-up, we plan to follow long-term as late onset CD is being increasingly diagnosed. Furthermore, the neurological symptoms preceding the onset of GI manifestations in CD have been well-reported. [14]

To the best of our knowledge, one case of CC with demyelination has been described in a 53 year old female with psoriatic arthritis, retrobulbar neuritis, and quadripyramidal syndrome. [15] The present report is of two pediatric cases with CC associated with demyelination of the central nervous system, both of whom demonstrated the classical clinical picture of CC, supported with the histological evidence. They also had good clinical response to the steroids. Interestingly in both cases, the neurological manifestations became evident only 6-12 months after the diagnosis of CC. In the second case, there was a family history of MS. Both patients did not have a history of preceding viral illness or recent vaccinations.

In our patients, the clinical spectrum of neurological symptoms included developmental delay, clumsiness, hyperactivity, tics, behavior problems, and seizure. The neurological symptoms were mild initially but continued to progress even after the resolution of GI symptoms.

Due to the short follow-up of our patients, at the present time it is unclear whether cerebral demyelination is a new systemic extra-intestinal manifestation of CC or it is merely a well-known extraintestinal manifestation of CD that will be presented in these patients in the future. Work up for other causes of demyelination needs to be done. In view of the strong association of CD with both CC and a variety of neurological symptoms, we believe that our patients require long-term follow-up to watch for the potential development of CD. As more and more cases are being reported, the exact pathogenesis of this demyelination in CC and the prevalence in children may be revealed in the future.


 :: Conclusion Top


CC is an extremely rare cause of diarrhea and abdominal pain in children. Children with CC may have neurological symptoms associated with cerebral demyelination. Currently, these neurological manifestations are not well-understood, have a wide clinical variability and may progress even after the resolution of the colonic disease.

 
 :: References Top

1.Olesen M, Eriksson S, Bohr J, Järnerot G, Tysk C. Microscopic colitis: A common diarrhoeal disease. An epidemiological study in Orebro, Sweden, 1993-1998. Gut 2004;53:346-50.  Back to cited text no. 1
    
2.Gremse DA, Boudreaux CW, Manci EA. Collagenous colitis in children. Gastroenterology 1993;104:906-9.  Back to cited text no. 2
    
3.Vanderhoof JA, Goble K, Young RJ. Collagenous colitis in a 4-year-old child: Response to budesonide. J Pediatr Gastroenterol Nutr 2010;50:688-90.  Back to cited text no. 3
    
4.Liu X, Xiao SY, Plesec TP, Jiang W, Goldblum JR, Lazenby AJ. Collagenous colitis in children and adolescents: Study of 7 cases and literature review. Mod Pathol 2013 [Epub ahead of print].  Back to cited text no. 4
    
5.Fernández-Bañares F, Salas A, Forné M, Esteve M, Espinós J, Viver JM. Incidence of collagenous and lymphocytic colitis: A 5-year population-based study. Am J Gastroenterol 1999;94:418-23.  Back to cited text no. 5
    
6.Pardi DS, Loftus EV Jr, Smyrk TC, Kammer PP, Tremaine WJ, Schleck CD, et al. The epidemiology of microscopic colitis: A population based study in Olmsted County, Minnesota. Gut 2007;56:504-8.  Back to cited text no. 6
    
7.Billiémaz K, Robles-Medranda C, Le Gall C, Gay C, Mory O, Clémenson A, et al. A first report of collagenous gastritis, sprue, and colitis in a 9-month-old infant: 14 years of clinical, endoscopic, and histologic follow-up. Endoscopy 2009;41:E233-4.  Back to cited text no. 7
    
8.Benchimol EI, Kirsch R, Viero S, Griffiths AM. Collagenous colitis and eosinophilic gastritis in a 4-year old girl: A case report and review of the literature. Acta Paediatr 2007;96:1365-7.  Back to cited text no. 8
    
9.Levy AM, Yamazaki K, Van Keulen VP, Burgart LJ, Sandborn WJ, Phillips SF, et al. Increased eosinophil infiltration and degranulation in colonic tissue from patients with collagenous colitis. Am J Gastroenterol 2001;96:1522-8.  Back to cited text no. 9
    
10.Dietrich W, Erbguth F. Neurological complications of inflammatory intestinal diseases. Fortschr Neurol Psychiatr 2003;71:406-14.  Back to cited text no. 10
    
11.Freeman HJ. Collagenous colitis as the presenting feature of biopsy-defined celiac disease. J Clin Gastroenterol 2004;38:664-8.  Back to cited text no. 11
    
12.Javed S, Safdar A, Forster A, Selvan A, Chadwick D, Nicholson A, et al. Refractory coeliac disease associated with late onset epilepsy, ataxia, tremor and progressive myoclonus with giant cortical evoked potentials - A case report and review of literature. Seizure 2012;21:482-5.  Back to cited text no. 12
    
13.Wills AJ. The neurology and neuropathology of coeliac disease. Neuropathol Appl Neurobiol 2000;26:493-6.  Back to cited text no. 13
    
14.Hanagasi HA, Gürol E, Sahin HA, Emre M. Atypical neurological involvement associated with celiac disease. Eur J Neurol 2001;8:67-9.  Back to cited text no. 14
    
15.Azzouz D, Gargouri A, Hamdi W, Kchir H, Hizem Y, Tekaya R, et al. Coexistence of psoriatic arthritis and collagenous colitis with inflammatory nervous system disease. Joint Bone Spine 2008;75:624-5.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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