Della Cella G, Beluschi C, Cipollina F
Divisione Pediatria, Ospedale di Chiavari (GE), Italia.
Pediatr Med Chir. 1991 Jul-Aug;13(4):427-30.
The Authors report a case of coeliac disease which first appeared in a boy of 4, suffering from a seizure disorder. The bulky mass of fatty faeces led towards gastroenteric investigations (xylose-test, jejunum biopsy). The atrophy of the villi was clearly shown by the biopsy and a coeliac disease was easily diagnosed. The boy was prescribed a coeliac diet and no fits of generalized convulsions occurred during three years follow-up. Yet, while he was given a challenge free-diet, they started to occur. A computerized axial tomography (TAC) carried out when he was eight and another when fifteen, evidenced bilateral, intracranial calcifications, cortical-subcortical, in the blood vessels, symmetrically located in the occipital region. The anticonvulsive therapy, started when he was 4, has never been interrupted. Now A.M. is 21 and still following an anticonvulsive polytherapy. Many tests were performed. The result of folic acid dosage carried out when he was 16, and badly follow a coeliac diet, was less than 2 ng/ml. A modification in the neurological symptomatology was noted during his puberal phase: fits of convulsions changed into daily crises of mind-failures. This feature is still present in his adult age. The case is reported for its clinical characteristics of neurological symptomatology associated with coeliac disease. The iconographical documentation evidences endocranial calcifications frequently connected with coeliac disease.
作者报告了一例乳糜泻病例,该病例首次出现在一名患有癫痫症的4岁男孩身上。大量油腻粪便促使进行胃肠检查(木糖试验、空肠活检)。活检清楚地显示了绒毛萎缩,乳糜泻很容易诊断出来。该男孩被规定了乳糜泻饮食,在三年的随访中没有发生全身性惊厥发作。然而,当给他无麸质饮食挑战时,惊厥发作又开始出现。他8岁时进行了计算机断层扫描(CT),15岁时又进行了一次,结果显示双侧颅内血管皮质下钙化,对称位于枕部。他4岁时开始的抗惊厥治疗从未中断。现在A.M. 21岁,仍在接受多种抗惊厥治疗。进行了许多检查。他16岁时,在严格遵循乳糜泻饮食的情况下,叶酸剂量检测结果低于2 ng/ml。在他青春期,神经系统症状有所改变:惊厥发作转变为每日的意识丧失危机。这种特征在他成年后仍然存在。报告该病例是因为其与乳糜泻相关的神经系统症状学的临床特征。影像学记录显示颅内钙化经常与乳糜泻有关。