Doria Lamba L, De Grandis E, De Negri E, Montaldi L, Grosso P, Marazzi M G, Rossi A, Tuo P
Unità Operativa di Neuropsichiatria Infantile Dipartimento di Neuroscienze, Oftalmologia e Genetica, Sezione di Neuroscienze dello Sviluppo, Istituto G. Gaslini, Università degli Studi di Genova, Genova, Italy.
Minerva Pediatr. 2006 Jun;58(3):305-9.
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory demyelinating encephalomyelitis with often monosymptomatic abrupt onset, followed by multifocal neurologic symptomatology depending on lesion-site. Diagnosis is made on the basis of characteristic magnetic resonance imaging (MRI) signal alterations. ADEM is sensitive to steroid therapy, immunoglobulins and plasmapheresis, presents usually a monophasic course and disappears completely after 2 or 3 weeks. Resolution of MRI lesions appears usually within 6 months of presentation. We report on a 14-year-old male, admitted to our Emergency Unit because of fever and acute urinary retention with a normal neurological examination. Urinary tract ultrasonography and mictional cystography were normal; electrophysiology showed a mild involvement of the peripheral nervous system and brain and spine MRI revealed disseminated areas of increased signal on T2-weighted sequences suggestive of ADEM. Steroid therapy brought about clinical recovery in a few days. Resolution of lesions on MRI after 4 months and absence of relapses during four-year clinical follow-up confirmed definitive diagnosis. Our case is interesting because, to our knowledge, this is the first literature report with acute urinary retention as predominant symptom in monosymptomatic forms. Another peculiar feature is the absence of associated neurologic symptomatology despite MRI evidence of important brain and spine alterations.
急性播散性脑脊髓炎(ADEM)是一种免疫介导的炎症性脱髓鞘性脑脊髓炎,通常起病急骤且症状单一,随后根据病变部位出现多灶性神经症状。诊断基于特征性的磁共振成像(MRI)信号改变。ADEM对类固醇治疗、免疫球蛋白和血浆置换敏感,通常呈单相病程,2至3周后完全消失。MRI病变通常在出现症状后6个月内消退。我们报告一例14岁男性,因发热和急性尿潴留入住我院急诊科,神经系统检查正常。泌尿系统超声和排尿性膀胱造影正常;电生理检查显示外周神经系统轻度受累,脑和脊柱MRI显示T2加权序列上有散在的高信号区,提示ADEM。类固醇治疗在数天内使临床症状恢复。4个月后MRI病变消退,四年临床随访期间无复发,确诊为该病。我们的病例很有意思,据我们所知,这是首例以急性尿潴留为单一症状形式的主要症状的文献报道。另一个独特之处是,尽管MRI显示脑和脊柱有明显改变,但无相关神经症状。