Shobe Seid Mohammed, Melka Dereje, Mulugeta Mesfin, Adane Leul
Department of Radiology, Addis Ababa University School of Medicine, Addis Ababa, Ethiopia.
Department of Neurology, Addis Ababa University School of Medicine, Addis Ababa, Ethiopia.
Radiol Case Rep. 2025 Jun 12;20(9):4338-4341. doi: 10.1016/j.radcr.2025.05.044. eCollection 2025 Sep.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by degeneration of motor neurons, with the tongue often involved in clinical presentation. In this case, a 60-year-old female presented with progressive choking episodes and speech slurring over 9 months, exhibiting dysarthria, prominent tongue atrophy, fasciculations, and hyperreflexia. Needle electromyography (EMG) showed diffuse chronic neurogenic changes with signs of active denervation changes prominent on the tongue and right arm with normal sensory nerve studies. Magnetic resonance imaging (MRI) brain imaging revealed a Diffuse T1 Weighted image (T1WI) hyperintense of tongue known as "bright tongue sign" indicating fatty infiltration of tongue muscles, consistent with neurogenic atrophy. This case underscores the importance of recognizing this characteristic tongue hyperintensity as a valuable radiological clue in diagnosing bulbar-onset ALS and highlights the potential for early diagnosis to improve patient management and outcomes.
肌萎缩侧索硬化症（ALS）是一种致命的神经退行性疾病，其特征为运动神经元变性，临床症状常累及舌头。在本病例中，一名60岁女性在9个月内出现进行性呛咳发作和言语不清，表现为构音障碍、明显的舌肌萎缩、肌束震颤和反射亢进。针极肌电图（EMG）显示弥漫性慢性神经源性改变，舌部和右臂有明显的失神经活动改变迹象，感觉神经检查正常。脑部磁共振成像（MRI）显示舌部在弥散T1加权像（T1WI）上呈高信号，即“亮舌征”，提示舌肌脂肪浸润，符合神经源性萎缩。本病例强调了将这一特征性舌部高信号识别为诊断延髓起病型ALS的重要影像学线索的重要性，并突出了早期诊断对改善患者管理和预后的潜力。
