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Transverse Myelitis As Manifestation of Celiac Disease in a Toddler

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Transverse Myelitis As Manifestation of Celiac Disease in a Toddler Transverse Myelitis as Manifestation of Celiac Disease in a Toddler Hilde Krom, MD, a Fleur Sprangers, MD, PhD,b René van den Berg, MD, PhD, c Marc Alexander Benninga, MD, PhD, a Angelika Kindermann, MD, PhDa We present a 17-month-old girl with rapidly progressive unwillingness abstract to sit, stand, play, and walk. Furthermore, she lacked appetite, vomited, lost weight, and had an iron deficiency. Physical examination revealed a cachectic, irritable girl with a distended abdomen, dystrophic legs with paraparesis, disturbed sensibility, and areflexia. An MRI scan revealed abnormal high signal intensity on T2-weighted images in the cord on the thoracic level, without cerebral abnormalities, indicating transverse myelitis (TM). Laboratory investigations revealed elevated immunoglobulin A antibodies against gliadin (1980.0 kU/L; normal, 0–10.1 kU/L) and tissue transglutaminase (110.0 kU/L; normal, 0–10.1 kU/L). Gastroscopy a Department of Pediatric Gastroenterology and Nutrition, revealed villous atrophy in the duodenal biopsies and lymphocytic gastritis Emma Children’s Hospital, Amsterdam, Netherlands; bFlevoziekenhuis, Almere, Netherlands; and cDepartment according to Marsh IIIb, compatible with celiac disease (CD). After the start of Radiology, Academic Medical Centre, Amsterdam, of a gluten free diet and methylprednisolone, she recovered completely. To Netherlands our knowledge, this is the first pediatric case of TM as manifestation of CD. Dr Krom drafted the initial manuscript; Dr We suggest that all children with TM or other neurologic manifestations of Sprangers treated the patient and reviewed and unknown origin should be screened for CD. revised the manuscript; Drs van den Berg and Benninga reviewed and revised the manuscript; Dr Kindermann treated the patient and followed her and reviewed and revised the manuscript; Celiac disease (CD) is an inflammatory the first manifestation of CD, also in and all authors approved the fi nal manuscript as condition of the small intestine, the absence of intestinal pathology.6 submitted. triggered by the ingestion of gluten. 1 DOI: 10.1542/peds.2016-1381 It is the most common genetically Accepted for publication Oct 24, 2016 food-based intolerance, 2 and 1 of the CASE REPORT Address correspondence to Hilde Krom, MD, most common chronic diseases in Department of Pediatric Gastroenterology and childhood1 (0.4%–1.3% 3). The typical A 17-month-old girl was admitted to Nutrition, Emma Children’s Hospital, Box 22660, 1100 DD, Amsterdam, Netherlands. E-mail: h.krom@ presentation involves gastrointestinal our hospital because of unwillingness to sit, stand, play, and walk. These amc.uva.nl symptoms such as abdominal pain, complaints started at the age of 15 PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, chronic and/or intermittent diarrhea, months during a period of illness, 1098-4275). or constipation. However, atypical or but were now rapidly progressive. Copyright © 2017 by the American Academy of extraintestinal manifestations, such Before presentation, she was a healthy Pediatrics as fatigue, iron deficiency anemia, toddler, with a normal growth and FINANCIAL DISCLOSURE: The authors have dental enamel hypoplasia, low bone indicated they have no fi nancial relationships development. Pregnancy and birth relevant to this article to disclose. mineral density, isolated elevation were uncomplicated (at term, weight of aminotransferase levels, and 3500 g). She was vaccinated according FUNDING: No external funding. short stature, seem to become more to the National Dutch program and POTENTIAL CONFLICT OF INTEREST: The authors common and are more prevalent when received her last vaccination at the age have indicated they have no potential confl icts of interest to disclose. age increases in children.2 Neurologic of 14 months. Gluten was introduced complications are reported in 8% to at the age of 7 months. Since the age 26% of adults with CD. 3 – 5 In children of 15 months she lacked appetite, To cite: Krom H, Sprangers F, van den Berg R, with CD, the risk of developing vomited frequently, and lost 1 kg of et al. Transverse Myelitis as Manifestation of Celiac neurologic complications is lower. 3 weight, which started after an upper Disease in a Toddler. Pediatrics. 2017;139(3): e20161381 Neurologic complaints may even be airway tract infection accompanied Downloaded from www.aappublications.org/news by guest on September 27, 2021 PEDIATRICS Volume 139 , number 3 , March 2017 :e 20161381 CASE REPORT by a gastroenteritis. There was no fever or abdominal pain. Defecation was variable in frequency (once in 5 days up to once a day), with a normal to hard consistency without blood in the stools. In addition to her complaints, an iron deficiency (iron 4.3 μmol/L) was diagnosed, for which iron supplementation was started. Family history revealed hay fever and rubber allergy in mother, and asthmatic disease in father. At physical examination, a cachectic, irritable, crying girl was seen (height –1.9 SD; weight –3.0 SD; weight for height –2.1 SD). Her abdomen was FIGURE 1 distended with loose abdominal MRI spinal cord: axial T2-weighted image. FIGURE 2 skin. Peristalsis was normal and no T2-weighted axial image at the Th9 level reveals MRI spinal cord: sagittal T2-weighted image. high signal intensity in the central part (white On the sagittal T2-weighted images, swelling of defense or hepatosplenomegaly was matter) of the spinal cord. found. Legs and buttocks revealed the lower spinal cord up to the conus level was noticed with increased signal intensity. muscle wasting. At neurologic acid were normal, which excluded examination, a paraparesis, disturbed neuroblastoma. Immunoglobulin adeno- and rotavirus, and in addition sensibility, and areflexia of both legs (Ig)A (1.63 g/L; normal 0.12–1.6 culture revealed no growth. were found, without Babinski sign. g/L) and IgM (1.0 g/L; normal There was no paraparesis of the 0.09–0.74 g/L) were elevated. Due to Although CSF was normal, due to arms, with normal reflexes. supplementation, the iron status was the rapidly progressive neurologic An MRI scan of cerebrum and spinal improved but still decreased. Levels symptoms in combination with cord revealed abnormal high signal of creatine kinase (416 U/L; normal, the MRI findings, she was given intensity at the thoracic level, most 0–250 U/L) and creatine kinase-MB a diagnosis of TM. Considering prominent on the axial T2-weighted (13.8 μg/L; normal 0–5.2 μg/L) TM can be seen in the context images at Th9 ( Fig 1). On the sagittal were elevated. Albumin was below of neuromyelitis optica, the T2-weighted images, swelling of the normal range (32 g/L; normal 37–55 ophthalmologist was consulted, lower spinal cord up to the conus g/L). Antinuclear antibodies and but no signs of neuritis optica level was noticed with increased extractable nuclear antigens were not were found. Intravenous signal intensity ( Fig 2). Cerebral found. methylprednisolone therapy was findings were normal. started for TM with a high dose of Further studies (complete blood cell 30 mg/kg for 3 days, followed by a The complaints of constipation and count, electrolytes, ureum, glucose, 6-week reduction phase. paraparesis with areflexia of the elastase, aspartate transaminase, legs in combination with imaging alanine transaminase, alkaline Further investigation revealed highly findings were suspected for a low- phosphatase, amylase, lactate elevated IgA antibodies against tissue grade process or a (post)infectious dehydrogenase, thiamine, free transglutaminase (1980.0 kU/L; or inflammatory origin, such as thyroxine, thyrotropin, thyroxine- normal 0–10.1 kU/L) and gliadin transverse myelitis (TM). An acute binding globulin, reverse T3, IgG, (110.0 kU/L; normal 0–10.1 kU/L). disseminated encephalomyelitis cytomegalovirus-, Epstein-Barr Additional gastroscopy revealed was less probable because of normal virus-, and Mycoplasma serology and subtotal villous atrophy in the cerebral imaging. urine analysis) were normal. duodenal biopsies and lymphocytic Chest radiograph and repeated Cerebrospinal fluid (CSF) was clear gastritis according to Marsh IIIb. ultrasounds revealed neither masses, and had neither erythrocytes nor paraaortal lymphadenopathy, nor leukocytes, a normal level of protein The diagnosis of CD was made, and metastases. The tumor marker and glucose, normal IgG and IgM a gluten free diet (GFD) was started. β-human chorionic gonadotropin indexes, and no oligoclonal bands. It Additional nocturnal tube feeding was absent. Catecholamines was negative for Parecho-, Entero-, was started due to her failure to vanilglycolic acid and vanilacetic varicella zoster, herpes zoster, and thrive and low oral intake. Downloaded from www.aappublications.org/news by guest on September 27, 2021 e2 KROM et al After starting methylprednisolone vaccination and idiopathic forms are acute encephalopathy, 11 status treatment and GFD, her symptoms usually monophasic, whereas TM in epilepticus/encephalopathy, 15 started to improve. She gained relation with multiple sclerosis and peripheral neuropathy, 17 recurrent weight, was able to sit, became neuromyelitis optica are relapsing transient hemiplegia with infarction less irritable, and at the age of 17.5 diseases associated with high risk of the brain, 18 and 2 children with months (2 weeks after admittance) of future attacks of TM and other cerebral venous sinus thrombosis. 16 she was discharged. neurologic events. 8 A GFD alone 12, 17 or combined with other treatments 11, 13, 15, 16, 18 led to At the age of 18 months she was able TM as manifestation of CD has not improvement 13, 18 or even complete to stand and walk, and neurologic been described earlier, and to date resolution 11, 12, 15 – 17 of the various examination was completely only 1 adult patient with CD has neurologic symptoms in these normal. A control MRI scan revealed been reported to have TM. 10 pediatric CD cases. normalization of earlier diagnosed Interestingly, both TM and CD are spinal cord abnormalities. CSF again related with other autoimmune Although more cases of neurologic was normal.
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