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Multiple cerebral lesions in a patient with refractory celiac disease:A case report

World Journal of Gastroenterology 2020年第47期26卷 7584-7592页

作者： Lena Horvath Georg Oberhuber Andreas Chott Maria Effenberger Herbert Tilg Eberhard Gunsilius Dominik Wolf Sarah Iglseder Department of Internal Medicine V(Hematology and Medical Oncology) Medical University InnsbruckInnsbruck 6020Austria InnPath GmbH Institute of PathologyInnsbruck 6020Austria ottakring clinic Institute of Pathology and MicrobiologyVienna 1160Austria Department of Internal Medicine I(Gastroenterology HepatologyEndocrinology and Metabolism)Medical University InnsbruckInnsbruck 6020Austria Department of Neurology Medical University InnsbruckInnsbruck 6020Austria

BACKGROUND Enteropathy-associated T cell lymphoma(EATL)is an aggressive intestinal T cell lymphoma derived from intraepithelial lymphocytes,which occurs in individuals with celiac disease(CD).Cerebral involvement is an extremely rare condition and as described so far,lymphoma lesions may present as parenchymal predominantly supratentorial or leptomeningeal *** describe a case of EATL with multifocal supra-and infratentorial brain involvement in a patient with refractory celiac disease(RCD).CASE SUMMARY A 58-years old man with known CD developed ulcerative jejunitis and was diagnosed with RCD type *** months later he presented with subacute cerebellar symptoms(gait ataxia,double vision,dizziness).Cranial magnetic resonance imaging(MRI)revealed multifocal T2 hyperintense supra-and infratentorial *** studies of blood and cerebrospinal fluid were inconspicuous for infectious,inflammatory or autoimmune diseases.18Ffluorodeoxyglucose-positron emission tomography/computed tomography(18FDG-PET/CT)scan showed a suspect hypermetabolic lesion in the left upper abdomen and consequent surgical jejunal resection revealed the diagnosis of *** the diagnostic work-up,neurological symptoms aggravated and evolved refractory to high-dosage *** MRI scans showed progressive cerebral lesions,highly suspicious for lymphoma and methotrexate chemotherapy was ***,clinically the patient responded only ***,cerebral biopsy confirmed the diagnosis of cerebral involvement of *** the poor prognosis and deterioration of the performance status,best supportive care was *** patient passed away three weeks after *** EATL with cerebral involvement must be considered as a possible differential diagnosis in patients with known RCD presenting with neurological symptoms.

关键词： Enteropathy-associated T cell lymphoma Brain neoplasm Celiac disease Cerebellar syndrome Case report

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