Boland Allison C, Wind Alexander, Alkhoujah Mohammad
Orthopedic Surgery, Wayne State University School of Medicine, Detroit, USA.
Pediatrics, Wayne State University School of Medicine, Detroit, USA.
Cureus. 2024 Aug 14;16(8):e66870. doi: 10.7759/cureus.66870. eCollection 2024 Aug.
Propionic acidemia (PA) is a rare metabolic disorder stemming from genetic mutations, often causing hyperammonemia, acidosis, and basal ganglia issues. Its symptoms range from vomiting to neurological abnormalities, with severe cases presenting in neonates. Neurological complications including stroke-like episodes are common, requiring immediate attention. An eight-month-old boy with PA presented to the emergency department with respiratory distress, cough, and lethargy. Initial evaluation showed acidemia and elevated ammonia levels. He tested positive for rhinovirus and was diagnosed with acute viral bronchiolitis. While his respiratory symptoms improved, he developed neurological deficits, including hypotonia and weakness. Neurology consultations explored possible diagnoses such as botulism or acute inflammatory demyelinating polyneuropathy (AIDP). Imaging revealed basal ganglia abnormalities consistent with PA progression. Due to aspiration risk, he was transferred to the pediatric intensive care unit for supportive care. Despite unremarkable lumbar puncture and MRI results, new metabolic brain changes were noted, particularly in the basal ganglia. He was managed for weakness and feeding difficulties due to a metabolic stroke. After adjusting nutritional support and discussing long-term feeding options, he was discharged on day 29 with a nasogastric tube due to his inability to meet caloric goals orally. Neurological complications in PA, such as basal ganglia abnormalities and stroke-like episodes, are well-documented. Our case illustrates how an acute respiratory illness can obscure underlying neurological deficits, leading to delayed diagnosis. Symptoms resembling other conditions, such as descending hypotonia in our case, broaden the differential diagnosis to include botulism toxicity and AIDP. This report demonstrates the variety of clinical features patients with PA can present with and the importance of working up a metabolic crisis in addition to conditions with overlapping symptoms.
丙酸血症(PA)是一种由基因突变引起的罕见代谢紊乱疾病,常导致高氨血症、酸中毒和基底神经节问题。其症状从呕吐到神经异常不等,严重病例在新生儿中出现。包括类似中风发作的神经并发症很常见,需要立即关注。一名患有PA的八个月大男孩因呼吸窘迫、咳嗽和嗜睡被送往急诊科。初步评估显示有酸血症和氨水平升高。他鼻病毒检测呈阳性,被诊断为急性病毒性细支气管炎。虽然他的呼吸道症状有所改善,但出现了神经功能缺损,包括肌张力减退和虚弱。神经科会诊探讨了可能的诊断,如肉毒中毒或急性炎症性脱髓鞘性多发性神经病(AIDP)。影像学检查显示基底神经节异常与PA进展一致。由于有误吸风险,他被转至儿科重症监护病房进行支持治疗。尽管腰椎穿刺和MRI结果无异常,但注意到有新的代谢性脑部变化,尤其是在基底神经节。由于代谢性中风,他因虚弱和喂养困难而接受治疗。在调整营养支持并讨论长期喂养方案后,由于他无法通过口服达到热量目标,于第29天带着鼻胃管出院。PA中的神经并发症,如基底神经节异常和类似中风发作,已有充分记录。我们的病例说明了急性呼吸道疾病如何掩盖潜在的神经功能缺损,导致诊断延迟。类似其他病症的症状,如我们病例中的下行性肌张力减退,扩大了鉴别诊断范围,包括肉毒中毒毒性和AIDP。本报告展示了PA患者可能出现的各种临床特征,以及除了有重叠症状的病症外,对代谢危机进行检查的重要性。