Kumarasiri Ishara, Samararathna Ruwan, Sandakelum Udara, Muthukumarana Oshanie, Balasubramaniam Reha, Mettananda Sachith
University Paediatrics Unit, Colombo North Teaching Hospital, Ragama 11010, Sri Lanka.
Department of Paediatrics, Faculty of Medicine, University of Kelaniya, Thalagolla Road, Ragama 11010, Sri Lanka.
Case Rep Pediatr. 2022 Feb 8;2022:5492155. doi: 10.1155/2022/5492155. eCollection 2022.
Acute disseminated encephalomyelitis is a rare inflammatory demyelinating disease characterized by acute onset polyfocal neurological deficits associated with encephalopathy. It commonly presents with fever, meningism, seizures, ataxia, motor deficits, and bladder dysfunction. Although cranial neuropathies, including optic neuritis and facial nerve palsies, have previously been reported, children presenting with bilateral ptosis is extremely rare. Here, we report a 3-year-old child with acute disseminated encephalomyelitis presenting with acute onset bilateral ptosis due to involvement of the single central levator subnucleus of the oculomotor nerve. . A 3-year-old Sri Lankan boy presented with drooping of the upper eyelids for three days and unsteady gait for two days. He did not have seizures, blurring of vision, limb weakness, swallowing or breathing difficulties, or bladder dysfunction. On examination, he had bilateral ptosis, gait ataxia, and dysmetria. His vision, eye movements, and examination of other cranial nerves were normal. MRI brain revealed high signal intensities involving the subcortical white matter of parietal and occipital lobes, midbrain in the area of single central levator subnucleus of the oculomotor nerve, cerebellar vermis, and right cerebellar hemisphere. Based on the clinical features suggesting polyfocal neurological involvement of the midbrain and cerebellum and characteristic MRI findings, the diagnosis of acute disseminated encephalomyelitis was made. He responded well and rapidly to high-dose intravenous methylprednisolone and showed a complete clinical and radiological recovery.
This case report describes a rare presentation of acute disseminated encephalomyelitis, bilateral ptosis due to involvement of the single central levator subnucleus of the oculomotor nerve. It highlights that the presenting manifestations of acute disseminated encephalomyelitis can be subtle and vary; however, timely diagnosis and treatment result in complete recovery.
急性播散性脑脊髓炎是一种罕见的炎症性脱髓鞘疾病，其特征为急性起病的多灶性神经功能缺损并伴有脑病。它通常表现为发热、脑膜刺激征、癫痫发作、共济失调、运动功能缺损及膀胱功能障碍。虽然此前已有包括视神经炎和面神经麻痹在内的颅神经病变的报道，但以双侧上睑下垂为表现的儿童极为罕见。在此，我们报告一名3岁儿童，因动眼神经单一中央提上睑肌亚核受累而出现急性起病的双侧上睑下垂，诊断为急性播散性脑脊髓炎。一名3岁斯里兰卡男孩出现上睑下垂3天，步态不稳2天。他没有癫痫发作、视力模糊、肢体无力、吞咽或呼吸困难或膀胱功能障碍。检查发现他有双侧上睑下垂、步态共济失调和辨距不良。他的视力、眼球运动及其他颅神经检查均正常。脑部MRI显示顶叶和枕叶皮质下白质、动眼神经单一中央提上睑肌亚核所在区域的中脑、小脑蚓部及右小脑半球有高信号强度。基于提示中脑和小脑多灶性神经受累的临床特征及典型的MRI表现，诊断为急性播散性脑脊髓炎。他对大剂量静脉注射甲基强的松龙反应良好且迅速，临床和影像学表现完全恢复。
本病例报告描述了急性播散性脑脊髓炎一种罕见的表现形式，即动眼神经单一中央提上睑肌亚核受累导致的双侧上睑下垂。它强调急性播散性脑脊髓炎的临床表现可能不明显且多样；然而，及时诊断和治疗可实现完全康复。
