Cerebellar Symptoms in Gluten Sensitivity: a Systematic Review of the Effect of a Gluten-Free Diet on Brain Imaging.

Cerebellar manifestations have been described in patients with gluten sensitivity (GS)-related disorders. A better understanding of the neurological manifestations of GS requires the use of neuroimaging techniques. We performed a systematic review on neuroimaging findings in GS patients with cerebellar symptoms. We also included a specific search on neuroimaging findings in GS patients with cerebellar manifestations on a gluten-free diet (GFD). PubMed, Embase, and Bireme were systematically searched to identify studies assessing neuroimaging features of adults with cerebellar manifestations and GS with or without enteropathy on a GFD. Ten studies with a total of 222 adult-GS patients were included. Magnetic resonance imaging was used in 100% of the studies. Cerebellar atrophy was evaluated in 7 studies and observed in 63% of the patients. White matter abnormalities were described in 2 studies. Single-photon emission computed tomography was used in 2 studies, and decreased cerebellar perfusion was detected in 92% of the included patients. No study employed nuclear medicine after the start of GFD. Magnetic resonance spectroscopy (MRS) was performed in 2 studies before and after GFD. An increase in the Naa/Cr ratio in cerebellar vermis was seen in 98% of the cases on a strict GFD. Cerebellar atrophy was found to be a prevalent condition in GS patients. MRS demonstrated to be useful in the follow-up of GS patients with cerebellar manifestations on a GFD. Prospective studies using nuclear medicine imaging are needed to study brain changes in GS patients on a GFD.

A serial SPECT-CT study in a celiac disease patient with cerebellar ataxia and psychiatric symptoms.

Celiac disease (CD) is an immune-mediated systemic disorder triggered by gluten and related prolamins in genetically predisposed individuals. Here, we described a case of a 31-year-old Caucasian woman who exhibited cerebellar and psychiatric dysfunctions. The patient underwent single-photon emission computed tomography (SPECT-CT) before and after a gluten-free diet (GFD). There was an improvement in cerebellar perfusion accompanied by a remission of cerebellar manifestations. The maintenance of the psychiatric manifestations was related to the persistence of the hypoperfusion in the frontal lobes. The patient's psychiatric symptoms did not change after 4 months under a GFD in the hospital. To our knowledge, this is the first case that shows the relationship between improvement in cerebellar perfusion and remission of cerebellar clinical manifestations in a CD patient under a GFD.

Calcified cavitating mesenteric lymph node syndrome: a rare complication of celiac disease.

A 47-year-old female presented with a lengthy history of dyspeptic symptoms, weight loss, and occasional diarrhea. A computed tomography (CT) scan showed several mesenteric nodular lesions, with peripheral calcifications, inversion of the fold pattern of the small intestine loops and an atrophic spleen.

Assessment of fractal dimension and panoramic radiomorphometric indices in women with celiac disease.

OBJECTIVES: To assess the panoramic radiomorphometric indices and fractal dimension in women with celiac disease. METHODS: The sample consisted of 20 women with celiac disease and 20 healthy women (control group). The mandibular cortical index classification, panoramic mandibular index, mental index, and fractal dimension were evaluated on panoramic radiographs. One-way ANOVA with post hoc Tukey test was used for comparison of the linear measurements and fractal dimension between the celiac and control groups, adopting a significance level of 5% RESULTS: There was no significant difference in panoramic radiomorphometric indices or fractal dimension between the celiac and control groups. CONCLUSIONS: Panoramic radiomorphometric indices and fractal dimension revealed no significant bone changes in women with celiac disease.

Magnetic resonance enterography in pediatric celiac disease / A enterografia por ressonância magnética na doença celíaca pediátrica

Abstract Objective: To assess if magnetic resonance enterography is capable of showing evidence/extent of disease in pediatric patients with biopsy-proven celiac disease by comparing with a control group, and to correlate the magnetic resonance enterography findings with anti-endomysial antibody level, which is an indicator of gluten-free dietary compliance. Methods: Thirty-one pediatric patients (mean age 11.7 ± 3.1 years) with biopsy-proven celiac disease and 40 pediatric patients as a control group were recruited in the study. The magnetic resonance enterography images of both patients with celiac disease and those of the control group were evaluated by two pediatric radiologists in a blinded manner for the mucosal pattern, presence of wall thickening, luminal distention of the small bowel, and extra-intestinal findings. Patient charts were reviewed to note clinical features and laboratory findings. The histopathologic review of the duodenal biopsies was re-conducted. Results: The mean duration of the disease was 5.6 ± 1.8 years (range: 3-7.2 years). In 24 (77%) of the patients, anti-endomysial antibody levels were elevated (mean 119.2 ± 66.6 RU/mL). Magnetic resonance enterography revealed normal fold pattern in all the patients. Ten (32%) patients had enlarged mesenteric lymph nodes. Conclusion: Although a majority of the patients had elevated anti-endomysial antibody levels indicating poor dietary compliance, magnetic resonance enterography did not show any mucosal abnormality associated with the inability of magnetic resonance enterography to detect mild/early changes of celiac disease in children. Therefore, it may not be useful for the follow-up of pediatric celiac disease.

Resumo Objetivo: Avaliar se a enterografia por ressonância magnética (ERM) consegue comprovar/mostrar a extensão da doença em pacientes pediátricos com doença celíaca (DC) comprovada por biópsia, comparar com um grupo de controle e correlacionar os achados da ERM com o nível de anticorpo antiendomísio (EMA) indicador de dieta sem glúten. Métodos: Foram recrutados 31 pacientes pediátricos (idade média entre 11,7 ± 3,1 anos) com DC comprovada por biópsia e 40 pacientes pediátricos em um grupo de controle. As imagens da ERM dos pacientes com DC e no grupo de controle foram avaliadas por dois radiologistas pediátricos às cegas para o padrão da mucosa, presença de espessamento da parede, dilatação luminal do intestino delgado e achados extraintestinais. Os prontuários dos pacientes foram revisados para anotação de características clínicas e achados laboratoriais. A avaliação histopatológica das biópsias duodenais foi feita novamente. Resultados: A duração média da doença foi 5,6 ± 1,8 anos (faixa de 3-7,2 anos). Em 24 (77%) dos pacientes, os níveis EMA estavam elevados (média 119,2 ± 66,6 RU/mL). A ERM revelou um padrão de pregas normal em todos os pacientes; 10 (32%) dos pacientes apresentaram gânglios linfáticos mesentéricos aumentados. Conclusão: Apesar de a maioria dos pacientes ter níveis elevados de EMA, o que indica uma dieta pobre, a ERM não mostrou anomalia na mucosa associada à incapacidade de a ERM detectar alterações leves/precoces de DC nas crianças. Portanto, ela pode não ser útil no acompanhamento da DC pediátrica.

Magnetic resonance enterography in pediatric celiac disease.

OBJECTIVE: To assess if magnetic resonance enterography is capable of showing evidence/extent of disease in pediatric patients with biopsy-proven celiac disease by comparing with a control group, and to correlate the magnetic resonance enterography findings with anti-endomysial antibody level, which is an indicator of gluten-free dietary compliance. METHODS: Thirty-one pediatric patients (mean age 11.7±3.1 years) with biopsy-proven celiac disease and 40 pediatric patients as a control group were recruited in the study. The magnetic resonance enterography images of both patients with celiac disease and those of the control group were evaluated by two pediatric radiologists in a blinded manner for the mucosal pattern, presence of wall thickening, luminal distention of the small bowel, and extra-intestinal findings. Patient charts were reviewed to note clinical features and laboratory findings. The histopathologic review of the duodenal biopsies was re-conducted. RESULTS: The mean duration of the disease was 5.6±1.8 years (range: 3-7.2 years). In 24 (77%) of the patients, anti-endomysial antibody levels were elevated (mean 119.2±66.6RU/mL). Magnetic resonance enterography revealed normal fold pattern in all the patients. Ten (32%) patients had enlarged mesenteric lymph nodes. CONCLUSION: Although a majority of the patients had elevated anti-endomysial antibody levels indicating poor dietary compliance, magnetic resonance enterography did not show any mucosal abnormality associated with the inability of magnetic resonance enterography to detect mild/early changes of celiac disease in children. Therefore, it may not be useful for the follow-up of pediatric celiac disease.

LOW BONE MINERAL DENSITY IN BRAZILIAN PATIENTS AT DIAGNOSIS OF CELIAC DISEASE.

BACKGROUND: Low bone mineral density is considered an extra-intestinal manifestation of celiac disease with reduced bone mass, increased bone fragility, and risk of fractures. Celiac disease is considered a condition at high risk for secondary osteoporosis and the evaluation of bone density is very important in the clinical management of these patients. OBJECTIVE: The present study aimed to investigate bone alterations in celiac patients from Curitiba, South Region of Brazil at diagnosis, correlating the findings with age and gender. METHODS: Patients who were included in the study were attended to in a private office of the same physician from January 2009 to December 2013. The diagnosis of celiac disease was done through clinical, serological and histological findings. All data were collected from the medical charts of the patients. After the diagnosis of celiac disease, evaluation for low bone mineral density was requested by dual-energy X-ray absorptiometry (DEXA). DEXA bone densitometer was used to estimate low bone mineral density at the lumbar spine and femur. RESULTS: A total of 101 patients, 82 (81.2%) female and 19 (18.8%) male subjects, with mean age of 39.0±3.03 years were included. At celiac disease diagnosis, 36 (35.6%) were younger than 30 years, 41 (40.6%) were between 31 and 50 years, and 24 (23.8%) were older than 50 years. Among the evaluated patients, 69 (68.3%) presented low bone mineral density, being 47% with osteopenia and 32% with osteoporosis. Patients who were older than 51 years and diagnosed with celiac disease presented low bone mineral density in 83.3% (20/24) of the cases. As expected, age influenced significantly the low bone mineral density findings. Among women, low bone mineral density was present with high frequency (60%) from 30 to 50 years. In patients diagnosed older than 60 years (n=8), all the women (n=5) and two of the three men had osteoporosis. CONCLUSION: This study demonstrated that 69% of Brazilian patients with celiac disease at diagnosis had low bone mineral density, being more frequent in women older than 50 years.

Abnormal Skeletal Strength and Microarchitecture in Women With Celiac Disease.

CONTEXT: Osteoporosis is often a presenting sign of celiac disease (CD). Whether skeletal fragility in CD is associated with microarchitectural abnormalities is not known. OBJECTIVE: The objective of the study was to evaluate microarchitecture and biomechanical properties of bone in CD. DESIGN: This was a case-control study. SETTING: The study was conducted at a university hospital outpatient facility. PATIENTS: Patients included premenopausal women with newly diagnosed CD (n = 33) and healthy controls (n = 33). MAIN OUTCOME MEASURES: Areal bone mineral density by dual-energy x-ray absorptiometry was measured as was trabecular and cortical volumetric bone mineral density (vBMD) and microarchitecture by high-resolution peripheral computed tomography of the distal radius and tibia. Whole-bone stiffness estimated by finite element analysis. PTH, 25-hydroxyvitamin D, and bone turnover markers were also measured. RESULTS: Groups had similar age, race, and body mass index. Both groups had sufficient 25-hydroxyvitamin D and normal calcium and PTH. Areal bone mineral density was lower in CD. By high-resolution peripheral computed tomography, CD had lower trabecular vBMD, fewer, more widely, and irregularly spaced trabeculae at both the radius and tibia (8%-33%). At the tibia, they also had lower total density (8%) and thinner cortices (10%). Whole-bone stiffness and failure load were lower (11%-21%) in CD at both sites. Biomechanical deficits were associated with trabecular abnormalities. CONCLUSIONS: Women with CD had abnormal vBMD and microarchitecture at both the radius and tibia. Trabecular bone was preferentially affected. These deficits were associated with lower estimates of skeletal strength. These findings suggest a potential structural mechanism for skeletal fragility in CD and support further research into the pathogenesis of fracture in this population.

Cápsula endoscópica: quando indicar seu uso? / Endoscopic capsule: when to indicate its use?

Muito do diagnóstico e tratamento das patologias do intestino delgado tem sido discutido e estudado na última década, desde a introdução da cápsula endoscópica na prática médica. Esta importante inovação tecnológica possibilitou o rompimento da última fronteira endoscópica do trato digestivo, permitindo o acesso endoscópico a toda a extensão do intestino delgado, o qual, devido a suas peculiaridades anatômicas e extensão, permanecia acessível somente à enteroscopia intraoperatória.

Both diagnosis and treatment of small intestine pathology has been discussed and studied in last decade since the introduction of endoscocopy capsule in medical practice. This important technological innovation allowed to break the last endoscopy borderline of digestive tract permitting endoscopy access for all extent of small intestine which due its anatomical peculiarities and extent remained accessible only to intraoperative enteroscopy.

Enfermedad celíaca. Enteropatía inducida por gluten / Celiac disease: gluten-sensitive enteropathy. Current status

la enfermedad celíaca se ha constituido en una entidad con una alta prevalencia en la población mundial, gracias al mejor conocimiento de las formas clínicas atípicas, latentes, monosintomáticas, etc.;a las enfermedades asociadas, al desarrollo de determinaciones de laboratorio con alta confiabilidad que detectan los casos clínicos enmascarados, los familiares de celíacos y que constituyen un excelente método de creening en la población asintomática

A prospective, longitudinal study of the long-term effect of treatment on bone density in children with celiac disease.

OBJECTIVE: Because osteopenia is a frequent complication of celiac disease, we evaluated the impact of a long-term gluten-free diet (GFD), initiated during childhood, on bone density. study design: Patients with celiac disease (n = 19; mean age, 14.2 +/- 2.6 years) were studied after 4.3 +/- 0.6 years of GFD. Bone density had been measured at diagnosis and after 1 year of GFD. We also studied 211 healthy children as a control group. Bone mineral density was measured by dual-energy x-ray absorptiometry. Intact parathyroid hormone (PTH) and bone-specific alkaline phosphatase (BALP) levels were measured in serum, and N-terminal telopeptide of type I collagen (NTx) was measured in urine. RESULTS: Although at diagnosis bone mineral content, bone area, and bone mineral density were significantly lower than in control subjects, the 3 measurements were normal after GFD. None of the patients on a GFD showed elevated values of PTH. Patients on a GFD had BALP (110.2 +/- 67.2 U/L) and NTx levels (261.9 +/- 187.8 nmol bone collagen equivalents/mmol creatinine) that were significantly higher than those of control subjects. The levels of BALP and NTx were significantly higher in patients with good compliance with the GFD, compared with patients with poorer compliance. CONCLUSIONS: This study shows that bone mineral content, bone area, and bone mineral density improve significantly with a GFD.

Risk of fractures in celiac disease patients: a cross-sectional, case-control study.

OBJECTIVES: Although osteopenia and osteoporosis are well-recognized complications of celiac disease, no controlled studies have been done to assess the prevalence of fractures in a large cohort of patients. The objectives of this study were to determine the prevalence of bone fractures and vertebral deformities in celiacs and to analyze the relationship between fractures and clinical data of patients. METHODS: We studied 165 patients with a well-established diagnosis of celiac disease. A similar number of age- and gender-matched control subjects with functional GI disorders were evaluated. The design of the study was cross-sectional, with a retrospective historical review through a personal interview of all subjects. All patients underwent bone mineral density measurement by dual-energy, x-ray absorptiometry and spinal x-ray. Vertebral deformities were determined by visual inspection of spinal x-rays and by morphometric analysis. RESULTS: Among celiacs, 41 patients (25%) referred have had from one to five fractures in the peripheral skeleton. On the contrary, only 14 (8%) control subjects experienced fractures. This difference was highly significant (odds ratio, 3.5; 95% confidence interval [CI], 1.8-7.2; p<0.0001). Although inspection of spinal x-rays showed evidence of vertebral deformities in the lumbar spine in only two patients, a more detailed examination of lateral x-rays using morphometric criteria detected lumbar spine vertebral deformities in nine (five also had fractures in the peripheral skeleton) and in four controls (odds ratio, 2.8; 95% CI, 0.7-11.5; p = NS). Eighty percent of fractures were detected before the diagnosis of celiac disease or in patients who were noncompliant with the gluten-free diet; only 7% of patients experienced fractures after starting treatment. Regression analysis adjusted for multiple comparisons showed that patients with fractures were diagnosed with celiac disease later (p<0.06) and remained undiagnosed for more prolonged periods (p<0.05). There was a trend, which did not reach statistical significance, for a lower bone mineral density in the lumbar spine and total skeleton among patients with fractures. CONCLUSIONS: This study has demonstrated that patients with celiac disease had a high prevalence of bone fractures in the peripheral skeleton. Most of these events occurred before diagnosis or while patients were noncompliant with gluten-containing diet. Our results suggest that early diagnosis and effective treatment of celiac disease were the most relevant measures to protect patients from the risk of fractures.

Bone mineral density in patients with pancreatic insufficiency and steatorrhea.

OBJECTIVES: Low bone mineral density (BMD) has been demonstrated in some patients with chronic intestinal disorders accompanied by diarrhea and malabsorption. However, very few studies have evaluated BMD in patients with pancreatic insufficiency due to cystic fibrosis. Our aim was to assess the prevalence and severity of bone loss in a cohort of patients with pancreatic insufficiency as a consequence of chronic pancreatitis. METHODS: Fourteen patients with chronic pancreatitis were studied. All of them presented with severe pancreatic insufficiency (secretin test: bicarbonate < or = 40 mEq/L) and steatorrhea (fecal fat > 7 g/day) and had been abstinent from alcohol for a median of 2.5 yr (range 1-15 yr). BMD was measured with a total-body scanner for dual-energy x-ray absorptiometry. Results were expressed as T-score (number of SD by which a patient density differs from the mean of sex-matched 30-yr-old healthy controls) in lumbar spine (L2-L4) and femoral neck. Total serum calcium, 25-(OH)D3, alkaline phosphatase, and midmolecular parathyroid hormone were determined. RESULTS: Ten patients demonstrated osteopenia (T-score -1 to -2.5) in the lumbar spine and in the femoral neck. Three patients displayed osteoporosis (T-score < -2.5) in the lumbar spine and two in the femoral neck. Mean T-scores (+/- SEM) were -1.44 +/- 0.37 in the lumbar spine and -1.79 +/- 0.27 in the femoral neck. Total and ionic serum calcium, serum parathyroid hormone, and alkaline phosphatase were in the normal range in all patients. Serum 25-(OH)D3 was below normal range in 7 of 12 patients. T-scores of patients with pancreatitis of alcoholic etiology (n = 10) were similar to those of nonalcoholic patients (n = 4). BMD did not correlate with age, bicarbonate secretion, fecal fat excretion, stool volume, parameters of mineral metabolism, duration of alcoholism, or mean alcohol intake. CONCLUSIONS: Most patients with pancreatic insufficiency as a consequence of chronic pancreatitis exhibit osteopenia, and some show evidence of osteoporosis. Identifying the intimate mechanisms for low BMD are beyond the limitations of the present study. More in-depth metabolic studies are necessary to define the pathogenic mechanism of osteopenia associated with chronic pancreatic disorders.

Superior mesenteric artery blood flow in celiac disease.

Measurements of the hemodynamic parameters of the superior mesenteric artery were performed in 18 patients with celiac disease. Ten were studied at the time of diagnosis, when a small bowel biopsy showed a flat mucosa. The remaining eight patients were studied after complete clinical and histological recovery induced by a gluten-free diet. Doppler ultrasound flowmetry was used to measure blood flow in physiological and fasting conditions and after a mixed liquid test meal (Ensure-Plus). The results were compared with those of healthy subjects (N = 7). Mean basal flow was 50% higher in untreated celiac disease patients than in healthy controls and patients with chronic pancreatitis (P = NS). Postprandial mesenteric blood flow was significantly increased (P < 0.002) and delayed in time (P < 0.005) in celiac disease as compared to controls. Successful treatment reduced the mesenteric blood flow in celiac disease to normal values. Our study demonstrates that pathophysiological changes in the small bowel mucosa during the active clinical phase of celiac disease induce an abnormal splanchnic circulation.