:Study Objective:Specific taste quality deficits in ALS has not heretofore been described.METHOD:Case Study: A 71 year old right handed female presented with a two year course of progressive reduction in strength in her hands, arms and legs with difficulty tying shoe laces, opening jars, writing and walking. She described nocturnal muscle spasms involving all extremities. Gradually, over eight months prior to presentation, all food began to taste bad and horribly bitter. Associated with no appetite and a seven pounds weight loss.RESULTS:Abnormalities in Neurological examination: Cranial Nerve (CN) examination: CN IX and X: Gag absent bilaterally. Motor examination: Bulk: atrophy in thenar and hypothenar eminences and intrinsics in both upper extremities. Percussion induced fasciculation and myotonia in both shoulders and arms. Fasciculation of tongue with percussion myotonia of tongue. Strength: Intrinsic 4/5 in both upper extremities, 3/5 in abductor policis brevis bilaterally, 3/5 right gastrocnemius soleus, 4/5 bilateral anterior tibialis. Drift testing: left abductor digiti minimi sign. Gait: Heel and toe walking unstable with circumduction of left leg. Tandem gait unstable. Cerebellar: Holmes rebound phenomena positive in the left upper extremity. Deep tendon reflexes: 1+ left brachioradialis. 1+ left triceps. 3+ right ankle jerks. 0 left ankle jerk. Positive jaw jerk. Chemosensory Testing: Normosmia to: Alcohol Sniff Test (46), Pocket Smell Test (3/3) and Retronasal Smell Index (9). Taste Quadrant Testing: ageusia in the palate to sodium chloride and citric acid. Ageusia throughout the palate, tongue and whole mouth to sucrose and quinine hydrochloride. Fungiform papillae count: left 18, right 20 (normal). Lip biopsy (normal). MRI: T2 flair in bilateral corticospinal tracts, left greater than right in the spinal cord and the brain. EMG: fibrillation, positive waves with fasciculation in all four extremities. Voluntary contraction with polyphasic unstable motor unit action potentials.CONCLUSION:While Lang found no taste loss in ALS (Lang, 2011), Pelletier found reduction in intensity of taste to all modalities in different sectors of the tongue, but paradoxically demonstrated normogeusia in whole mouth taste perception (Pelletier, 2013). Pathological specimens of those with ALS revealed degeneration in the nucleus parabrachialis medialis and tractus trigeminothalamicus dorsalis (Oyanagi, 2015), suggesting that taste deficit may be due to central white matter abnormalities. Sweet taste is localized in the most posterior and rostral aspect of the right insular cortex, immediately adjacent to bitter (Prinster, 2017), suggesting a neighborhood effect phenomena. Weight loss in ALS may be due to sensory distortion and secondary impairment of appetite. It would be worthwhile to investigate those with ALS for evidence of otherwise overlooked gustatory deficits, correction of which may improve appetite and nutritional state.

中文翻译：

---

101 肌萎缩侧索硬化症 (ALS) - 不仅仅是运动疾病？ALS 中孤立的苦味和甜味丧失

：研究目的：迄今为止尚未描述 ALS 中的特定味觉质量缺陷。方法：案例研究：一名 71 岁的右手女性，其手、手臂和腿的力量逐渐减弱，系鞋带困难，为期两年鞋带，打开罐子，写作和走路。她描述了涉及所有四肢的夜间肌肉痉挛。渐渐地，在展示前八个月，所有的食物都开始尝起来很糟糕，而且苦得可怕。与食欲不振和体重减轻 7 磅有关。结果：神经系统检查异常：颅神经 (CN) 检查：CN IX 和 X：双侧没有呕吐物。运动检查：大块：双上肢大鱼际和小鱼际隆起和内在肌萎缩。敲击引起肩部和手臂的肌束震颤和肌强直。舌震颤伴叩击性舌肌强直。力量：双上肢固有 4/5，双侧外展短肌 3/5，右侧腓肠肌 3/5，双侧胫骨前肌 4/5。漂移测试：左外展肌小指征。步态：脚跟和脚趾走路不稳定，左腿环绕。串联步态不稳定。小脑：左上肢福尔摩斯反弹现象阳性。深腱反射：1+左肱桡肌。1+左三头肌。3+ 右脚踝抽搐。0 左脚踝抽搐。积极的下巴抽搐。化学感觉测试：Normosmia 至：酒精嗅探测试 (46)、口袋气味测试 (3/3) 和鼻后气味指数 (9)。味觉象限测试：对氯化钠和柠檬酸的味觉。整个上颚、舌头和整个嘴巴对蔗糖和盐酸奎宁的失声。菌状乳头数：左 18，右 20（正常）。唇活检（正常）。MRI：双侧皮质脊髓束 T2 水平，脊髓和大脑左侧大于右侧。肌电图：纤颤，四肢肌束的正波。具有多相不稳定运动单位动作电位的自愿收缩。结论：虽然 Lang 发现 ALS 没有味觉丧失（Lang，2011），但 Pelletier 发现舌头不同区域的所有方式的味觉强度降低，但矛盾的是，整个嘴巴都表现出正常的味觉味觉（Pelletier，2013）。ALS 患者的病理标本显示内侧臂旁核和背三叉丘脑的退化（Oyanagi，2015），这表明味觉缺陷可能是由于中央白质异常所致。甜味位于右岛叶皮层的最后面和喙侧，紧邻苦味（Prinster，2017），表明存在邻域效应现象。ALS 的体重减轻可能是由于感觉扭曲和继发性食欲减退。对 ALS 患者进行调查以寻找其他被忽视的味觉缺陷的证据是值得的，纠正这些缺陷可能会改善食欲和营养状态。