Ahmed Aftab, Akbar Anum, Kunwar Digbijay, Mehta Fena
National Institute of Child Health (NICH) Karachi Pakistan.
Department of Pediatrics University of Nebraska Medical Center Omaha Nebraska USA.
Clin Case Rep. 2024 Oct 21;12(10):e9490. doi: 10.1002/ccr3.9490. eCollection 2024 Oct.
Concurrent recurrence of acute disseminated encephalomyelitis (ADEM) and poststreptococcal glomerulonephritis (PSGN) in a thalassemia intermedia patient is rare and underscores the complexity of autoimmune disorders. This case emphasizes the importance of considering ADEM in the differential diagnosis of children presenting with PSGN accompanied by neurological symptoms.
Post-streptococcal glomerulonephritis (PSGN) is a common group A streptococcal (GAS) infection sequela. The pathophysiology of PSGN involves immune complex deposition, with type 3 hypersensitivity reaction triggered by GAS. Certain neurological conditions may also arise following a GAS infection, possibly due to molecular mimicry in the brain, a pathophysiology similar to rheumatic fever, another common sequel of GAS infection. We present the case of a child with β-thalassemia intermedia who exhibited the classic triad (edema, hypertension, hematuria) of PSGN along with neurological manifestations, including a low glasgow coma scale (GCS) score and seizures. Magnetic resonance imaging (MRI) of the brain indicated changes consistent with acute disseminated encephalomyelitis (ADEM). Initially treated with methylprednisolone, the patient eventually received intravenous immunoglobulin (IVIG) due to lack of response. The patient had a good outcome, with complete resolution of all symptoms and no residual neurological deficits. This case underscores the importance of considering ADEM in the differential diagnosis for patients presenting with neurological signs and symptoms following a recent throat infection with GAS. Furthermore, given the increased risk of infection in thalassemia, patients with thalassemia who have a throat infection and neurological symptoms should be evaluated for the possible presence of ADEM.
中间型地中海贫血患者同时并发急性播散性脑脊髓炎（ADEM）和链球菌感染后肾小球肾炎（PSGN）较为罕见，凸显了自身免疫性疾病的复杂性。该病例强调了在对伴有神经系统症状的PSGN患儿进行鉴别诊断时考虑ADEM的重要性。
链球菌感染后肾小球肾炎（PSGN）是A组链球菌（GAS）感染常见的后遗症。PSGN的病理生理学涉及免疫复合物沉积，由GAS触发Ⅲ型超敏反应。GAS感染后也可能出现某些神经系统疾病，可能是由于大脑中的分子模拟，这一病理生理学与风湿热相似，风湿热是GAS感染的另一种常见后遗症。我们报告了1例中间型β地中海贫血患儿，其表现出PSGN的典型三联征（水肿、高血压、血尿）以及神经系统表现，包括低格拉斯哥昏迷量表（GCS）评分和癫痫发作。脑部磁共振成像（MRI）显示与急性播散性脑脊髓炎（ADEM）一致的变化。患者最初接受甲泼尼龙治疗，最终因无反应而接受静脉注射免疫球蛋白（IVIG）治疗。患者预后良好，所有症状完全缓解，无残留神经功能缺损。该病例强调了在对近期有GAS咽喉感染后出现神经系统体征和症状的患者进行鉴别诊断时考虑ADEM的重要性。此外，鉴于地中海贫血患者感染风险增加，对于有咽喉感染和神经系统症状的地中海贫血患者，应评估是否可能存在ADEM。
