Wakrim Soukaina, El Mekkaoui Adel, Benlenda Othmane, Nainia Khalila, Nassik Hicham
Radiology Department, Faculty of Medicine and Pharmacy, CHU Souss Massa, Ibn Zohr University, Agadir, Morocco.
Intensive Care Anaesthesia Department, Faculty of Medicine and Pharmacy, CHU Souss Massa, Ibn Zohr University, Agadir, Morocco.
Radiol Case Rep. 2022 Aug 20;17(10):4046-4048. doi: 10.1016/j.radcr.2022.07.060. eCollection 2022 Oct.
Acute hyperammonemic encephalopathy is rare and generally is not widely known; only a few pediatric cases were found in the literature. These lesions' clinical presentation differs significantly so they can mimic other lesions. In this case report, we discuss a 5-year-old boy who presented with generalized seizures and was unconscious in an apyretic context, for which she had a cranial computed tomographic and magnetic resonance imaging, both objectified an acute hyperammonemic encephalopathy resulting from an enzyme deficiency. Magnetic resonance imaging revealed lesions throughout the cortex, with the perirolandic and occipital cortices spared. This distribution of cerebral signal abnormalities on magnetic resonance imaging with an abrupt and profound neurological disorder is secondary to hyperammonemic. The knowledge of the magnetic resonance imaging results of this entity is essential to accelerate the diagnosis, and treatment, also to prevent sequelae.
急性高氨血症性脑病较为罕见,通常并不广为人知;文献中仅发现少数儿科病例。这些病变的临床表现差异很大,因此可能会与其他病变相混淆。在本病例报告中,我们讨论了一名5岁男孩,他在无发热情况下出现全身性癫痫发作且昏迷,为此进行了头颅计算机断层扫描和磁共振成像检查,二者均显示为酶缺乏导致的急性高氨血症性脑病。磁共振成像显示整个皮质均有病变,中央前回和枕叶皮质未受累。磁共振成像上脑信号异常的这种分布情况,伴突然且严重的神经功能障碍,是高氨血症所致。了解该病症的磁共振成像结果对于加快诊断、治疗以及预防后遗症至关重要。