- Email: [email protected]
Limbic encephalitis and coeliac disease. Just a casual association?
S82
Abstracts
Material and methods: Serum samples from 330 patients with PN (192 M/138 F; age range 16-85 yrs) attending our Neurology Unit between 1993 and 2004 and screened for anti-ganglioside reactivity, participated in this retrospective study. The study group comprised 90 subjects with acute inflammatory polyradiculoneupathy [AlP; 48.2 ± 18 (mean ± SD) yrs], 56 with chronic inflammatory polyradiculoneuropathy (CIP; 58 ± 13 yrs); 74 with distal symmetric polyneuropathy (DSP; 59.8 ± 13 yrs), and 110 with acquired motor neuron disease (MND; 54 ± 13 yrs). Sera were analyzed forthe presence of tTGA and, in positive cases, for anti-endomysium autibodies (EMA) as coufirmation test. Also, tTGA positive patients were retested for after-therapy anti-tTG reactivity. EMA was performed by indirect immunofluorescence and h-tTGA by ELISA, using commercially available Kits (Eurospital, Trieste). Anti-glycolipid (GMl, GDla, GDlb, GM3 and sulfatides) antibodies (IgG and IgM) were measured as described (J Peripher Nerv Syst 2004;9: 138-43). Correlations were analyzed using the Spearman rank test. Results: Eight (4 with AIP, 3 with DSP and 1 with MND) out of 330 patients (2.4%) were positive for tTGA, but none of them was subsequently found to be EMA positive. Seven tTGA positive patients had other serological markers of antoimmnnity (2 antinuclear antibodies, 3 anti-phospholipid antibodies, and 2 anti-smooth mnscle cell antibodies). All patients reexamined after therapy were found tTGA negative. tTGA values correlated with impairment of neurophysiological findings, but not with the presence of anti-glycolipid antibodies. Conclusions: Based on these results, we conclude that CD should not be considered in the diagnostic work np of patients with PN; other stndies, however, are needed to investigate basic mechanisms nnderlying tTGA formation in peripheral nerve disorders.
PA.78 LIMBIC ENCEPHALITIS AND COELIAC DISEASE. JUST A CASUAL ASSOCIATION? S. Mati 1, D. Renzi 2, M.G. Giovannini 3 , F. Cerbai 3, C. Melani 3, M. Cincotta 4 , F. Pinto 1, A. Calabro *,2
lNeurology Unit University ofFlorence, Florence 2Gastroenterology Unit University ofFlorence, Florence 3 Pharmacology, University ofFlorence, Florence 4Neurology Unit Ospedale Santa Maria Nuova, Florence Background and aim: Limbic encephalitis (LE) is a rare neurological syndrome characterized by memory impairment, psychiatric disturbances and temporal seizures. We describe the clinical and laboratory features of a patient with a history of fatal LE initially misdiagnosed as paraneoplastic, probably affected by coeliac disease (CD). Material and methods: Anti-tissne tranglutaminase (tTGA) and antiendomysium antibodies (EMA) were determined by commerciallyavailable Kits (Eurospital, Trieste). HLA typing was performed using the LIPA-DRB and -DQB kits (Innogenetics, Gent, Belgium). Immunohistochemical and immnnofluorescence analyses on rat and mouse CNS sections were performed by standard procednres. Results: Case report A 33-year-old man was admitted to the Hospital in Dec 1994 with progressive spatial and verbal memory disturbances and motor neuropathy. MRI showed bilateral increased 12-wighted signal in hippocampal regions. Oligoclonal bands were present on CSF. Serum and CSF anti-Hn antibodies were disclosed, and he was diagnosed with a paraneoplastic LE. However, repeated total body CT were negative for the presence of tumoral mass. The neurological condition gradually deteriorated despite immunomodulatory therapy. The patient developed epilepsy, cerebellar ataxia and dementia, and died in 2002 for cardiac complications of an epileptic generalized status. In 2004, following areevaluation of clinical and anamnestic data, we were informed that one of the two patient's daughters had been diagnosed with CD in 1999. We therefore evaluated EMA and tTGA on serum samples collected at the disease onset.
Both tTGA and EMA were positive. HLA typing of family members showed that the patient's daughters were both DR5-DQ7 and DR7DQ2 heterozygotes, whereas the wife was DR5-DQ7 homozygote. This indirectly demonstrated that onr patient had the CD-associated DQ2 haplotype. Immunohistochemistry on rat and mouse CNS slices showed autoantibodies reacting with the cytoplasm of many CNS neurons but not of liver, kidney or skeletal muscle cells, both in serum (> 1:50,000) and CSF (> 1:25). Western-blot on rat brain homogenate showed the presence of a immunoreactive band at 45 kDa. Conclusions: The case of onr patient demonstrates a new possible link between CD and nonparaneoplastic LE. We suggest that isolated LE cases of unknown origin can be the consequence of an undiagnosed CD, and propose a CD screening for patients with idiopathic LE.
PA.79 EFFICACY OF BUDESONIDE IN THE EARLY PHASE OF TREATMENT OF CELIAC DISEASE WITH GASTROINTESTINAL SYMPTOMS C. Ciacci *, L. Maiuri, C. Bucci, A. Merchionda, N. Vicidomini, S. Forestiero, F. Sabbatini, P. Iovino
Dipartimento di Medicina Clinica e Sperimentale, Napoli Background and aim: Budesonide is a glucocorticosteroid with high local anti-inflammatory effect. The budesonide controlled-release formulation (Entocir) target sites are the ileum and colon. Gluten ingestion canses an immunomediated gastrointestinal disease in intolerant patients. Aim was to investigate efficacy and safety of budesonide associated to gluten free diet in the early phase of treatment of celiac disease patients with gastrointestinal symptoms. Material and methods: Prospective, randomized study. 18 adnlt patients with newly diagnosed celiac disease were enrolled into the stndy if they had a clear malabsorption syndrome. Before starting gluten free diet (GFD) all patients underwent clinical evaluation, laboratory tests, upper and lower endoscopy with jejunal and rectal biopsy respectively, and a visual analogue scale for well-being. They were randomly assigned to two treatments: gluten-free diet or gluten-free diet plus budesonide 3 mg bis in die, for 4 weeks. In order to anticipate the activation of budesonide in the npper intestinal segments, patients were advised to open the capsnles and suspend the pellets in a small qnantity of water and to take omeprazole 20 mg/die at least two hour prior administration of budesonide. Results: In our series, budesonide treatment induced a greater increase of body weight, a decrease of number of bowel movements per day and of stool weight than GFD alone. Well being analogue scale scores were higher in patients treated with both GFD and bndesonide when compared to those treated with GFD alone. Conclusions: Budesonide shows efficacy in the treatment of symptomatic adult celiac patients. The efficacy might be increased by a pharmaceutical formnla active in first segments of ilenm.
PA.80 PRIMARY DUODENAL MALT LYMPHOMA AND HELICOBACTER PYLORI ERADICATION A. Savio*, D. Giacomin, G. Viviani, B. Kildani, A. Zappella, F. Pirali
Ospedale S. Orsola Fatebenefratelli, Brescia Background and aim: A few cases of regression of extra-gastric low grade MALT lymphoma after eradication of Helicobacter pylori have been reported so far. Six ont of the 9 duodenal cases reported showed regression after antibacterial therapy while the other 3 showed persistence with dissemination after 10 months in one. Material and methods: We report a case of primary duodenal low grade
Abstracts
Material and methods: Serum samples from 330 patients with PN (192 M/138 F; age range 16-85 yrs) attending our Neurology Unit between 1993 and 2004 and screened for anti-ganglioside reactivity, participated in this retrospective study. The study group comprised 90 subjects with acute inflammatory polyradiculoneupathy [AlP; 48.2 ± 18 (mean ± SD) yrs], 56 with chronic inflammatory polyradiculoneuropathy (CIP; 58 ± 13 yrs); 74 with distal symmetric polyneuropathy (DSP; 59.8 ± 13 yrs), and 110 with acquired motor neuron disease (MND; 54 ± 13 yrs). Sera were analyzed forthe presence of tTGA and, in positive cases, for anti-endomysium autibodies (EMA) as coufirmation test. Also, tTGA positive patients were retested for after-therapy anti-tTG reactivity. EMA was performed by indirect immunofluorescence and h-tTGA by ELISA, using commercially available Kits (Eurospital, Trieste). Anti-glycolipid (GMl, GDla, GDlb, GM3 and sulfatides) antibodies (IgG and IgM) were measured as described (J Peripher Nerv Syst 2004;9: 138-43). Correlations were analyzed using the Spearman rank test. Results: Eight (4 with AIP, 3 with DSP and 1 with MND) out of 330 patients (2.4%) were positive for tTGA, but none of them was subsequently found to be EMA positive. Seven tTGA positive patients had other serological markers of antoimmnnity (2 antinuclear antibodies, 3 anti-phospholipid antibodies, and 2 anti-smooth mnscle cell antibodies). All patients reexamined after therapy were found tTGA negative. tTGA values correlated with impairment of neurophysiological findings, but not with the presence of anti-glycolipid antibodies. Conclusions: Based on these results, we conclude that CD should not be considered in the diagnostic work np of patients with PN; other stndies, however, are needed to investigate basic mechanisms nnderlying tTGA formation in peripheral nerve disorders.
PA.78 LIMBIC ENCEPHALITIS AND COELIAC DISEASE. JUST A CASUAL ASSOCIATION? S. Mati 1, D. Renzi 2, M.G. Giovannini 3 , F. Cerbai 3, C. Melani 3, M. Cincotta 4 , F. Pinto 1, A. Calabro *,2
lNeurology Unit University ofFlorence, Florence 2Gastroenterology Unit University ofFlorence, Florence 3 Pharmacology, University ofFlorence, Florence 4Neurology Unit Ospedale Santa Maria Nuova, Florence Background and aim: Limbic encephalitis (LE) is a rare neurological syndrome characterized by memory impairment, psychiatric disturbances and temporal seizures. We describe the clinical and laboratory features of a patient with a history of fatal LE initially misdiagnosed as paraneoplastic, probably affected by coeliac disease (CD). Material and methods: Anti-tissne tranglutaminase (tTGA) and antiendomysium antibodies (EMA) were determined by commerciallyavailable Kits (Eurospital, Trieste). HLA typing was performed using the LIPA-DRB and -DQB kits (Innogenetics, Gent, Belgium). Immunohistochemical and immnnofluorescence analyses on rat and mouse CNS sections were performed by standard procednres. Results: Case report A 33-year-old man was admitted to the Hospital in Dec 1994 with progressive spatial and verbal memory disturbances and motor neuropathy. MRI showed bilateral increased 12-wighted signal in hippocampal regions. Oligoclonal bands were present on CSF. Serum and CSF anti-Hn antibodies were disclosed, and he was diagnosed with a paraneoplastic LE. However, repeated total body CT were negative for the presence of tumoral mass. The neurological condition gradually deteriorated despite immunomodulatory therapy. The patient developed epilepsy, cerebellar ataxia and dementia, and died in 2002 for cardiac complications of an epileptic generalized status. In 2004, following areevaluation of clinical and anamnestic data, we were informed that one of the two patient's daughters had been diagnosed with CD in 1999. We therefore evaluated EMA and tTGA on serum samples collected at the disease onset.
Both tTGA and EMA were positive. HLA typing of family members showed that the patient's daughters were both DR5-DQ7 and DR7DQ2 heterozygotes, whereas the wife was DR5-DQ7 homozygote. This indirectly demonstrated that onr patient had the CD-associated DQ2 haplotype. Immunohistochemistry on rat and mouse CNS slices showed autoantibodies reacting with the cytoplasm of many CNS neurons but not of liver, kidney or skeletal muscle cells, both in serum (> 1:50,000) and CSF (> 1:25). Western-blot on rat brain homogenate showed the presence of a immunoreactive band at 45 kDa. Conclusions: The case of onr patient demonstrates a new possible link between CD and nonparaneoplastic LE. We suggest that isolated LE cases of unknown origin can be the consequence of an undiagnosed CD, and propose a CD screening for patients with idiopathic LE.
PA.79 EFFICACY OF BUDESONIDE IN THE EARLY PHASE OF TREATMENT OF CELIAC DISEASE WITH GASTROINTESTINAL SYMPTOMS C. Ciacci *, L. Maiuri, C. Bucci, A. Merchionda, N. Vicidomini, S. Forestiero, F. Sabbatini, P. Iovino
Dipartimento di Medicina Clinica e Sperimentale, Napoli Background and aim: Budesonide is a glucocorticosteroid with high local anti-inflammatory effect. The budesonide controlled-release formulation (Entocir) target sites are the ileum and colon. Gluten ingestion canses an immunomediated gastrointestinal disease in intolerant patients. Aim was to investigate efficacy and safety of budesonide associated to gluten free diet in the early phase of treatment of celiac disease patients with gastrointestinal symptoms. Material and methods: Prospective, randomized study. 18 adnlt patients with newly diagnosed celiac disease were enrolled into the stndy if they had a clear malabsorption syndrome. Before starting gluten free diet (GFD) all patients underwent clinical evaluation, laboratory tests, upper and lower endoscopy with jejunal and rectal biopsy respectively, and a visual analogue scale for well-being. They were randomly assigned to two treatments: gluten-free diet or gluten-free diet plus budesonide 3 mg bis in die, for 4 weeks. In order to anticipate the activation of budesonide in the npper intestinal segments, patients were advised to open the capsnles and suspend the pellets in a small qnantity of water and to take omeprazole 20 mg/die at least two hour prior administration of budesonide. Results: In our series, budesonide treatment induced a greater increase of body weight, a decrease of number of bowel movements per day and of stool weight than GFD alone. Well being analogue scale scores were higher in patients treated with both GFD and bndesonide when compared to those treated with GFD alone. Conclusions: Budesonide shows efficacy in the treatment of symptomatic adult celiac patients. The efficacy might be increased by a pharmaceutical formnla active in first segments of ilenm.
PA.80 PRIMARY DUODENAL MALT LYMPHOMA AND HELICOBACTER PYLORI ERADICATION A. Savio*, D. Giacomin, G. Viviani, B. Kildani, A. Zappella, F. Pirali
Ospedale S. Orsola Fatebenefratelli, Brescia Background and aim: A few cases of regression of extra-gastric low grade MALT lymphoma after eradication of Helicobacter pylori have been reported so far. Six ont of the 9 duodenal cases reported showed regression after antibacterial therapy while the other 3 showed persistence with dissemination after 10 months in one. Material and methods: We report a case of primary duodenal low grade