Ribichini Emanuela, Pallotta Nadia, Badiali Danilo, Carlucci Maria, Ceccanti Marco, Cambieri Chiara, Libonati Laura, Corazziari Enrico Stefano, Ruoppolo Giovanni, Inghilleri Maurizio
Department of Translational and Precision Medicine, Sapienza University, Rome, Italy.
Neuromuscular Disorders Unit, Department of Human Neurosciences, Sapienza University, Rome, Italy.
Front Neurol. 2025 Jan 13;15:1509917. doi: 10.3389/fneur.2024.1509917. eCollection 2024.
BACKGROUND/AIMS: Oro-pharyngeal dysfunction has been reported in Amyotrophic Lateral Sclerosis (ALS). We aimed to assess ALS patients upper gastrointestinal (GI) motor activity and GI symptoms according to bulbar and spinal onset and severity of ALS.
ALS bulbar (B) and spinal (S) patients with ALS Functional Rating Scale (ALSFRS-r) ≥35, bulbar sub-score ≥10, and Forced Vital Capacity (FVC) >50%, underwent to: Fiberoptic Endoscopic Evaluation of Swallowing (FEES); esophageal manometry; gastric emptying; Rome symptom questionnaire. Medical Research Council Scale for Muscle Strength (MRC) was performed for the upper and lower limbs. Mann-Whitney's U, Fisher's ranks test, Pearson's test was used.
Thirteen ALS patients were included (6 F; mean age 61.2 ± 13.7 years, range: 37-87), 5 with B and 8 with S onset (ALSFRS-R score 39.5 ± 4.9, MRC score 128.6 ± 23.3, disease duration 22.8 ± 17.9 months). FEES detected a high dysphagia score in 5 patients with no difference between S and B phenotype. Lower esophageal sphincter pressure was normal in all patients. Esophageal dysmotility was observed in three S and two B onset patients. Upper esophageal sphincter (UES) pressure was higher in all ALS patients. UES spasms and delayed gastric emptying were detected in two B and one S and in two B and four S patients, respectively. There was no correlation between esophagogastric motor abnormalities and clinical characteristics of ALS, nor GI symptoms.
The presence of UES spasm and the delayed gastric emptying in a subgroup of ALS patients may suggest the role of ANS dysfunction in ALS.
背景/目的：已有报道称肌萎缩侧索硬化症（ALS）患者存在口咽功能障碍。我们旨在根据ALS的延髓和脊髓起病情况及严重程度，评估ALS患者的上消化道（GI）运动活性和GI症状。
ALS功能评定量表（ALSFRS-r）≥35、延髓亚评分≥10且用力肺活量（FVC）>50%的ALS延髓型（B）和脊髓型（S）患者接受了以下检查：纤维内镜吞咽评估（FEES）；食管测压；胃排空；罗马症状问卷。对上下肢进行医学研究委员会肌力量表（MRC）检查。采用曼-惠特尼U检验、费舍尔秩和检验、皮尔逊检验。
纳入13例ALS患者（6例女性；平均年龄61.2±13.7岁，范围：37-87岁），5例为延髓起病，8例为脊髓起病（ALSFRS-R评分39.5±4.9，MRC评分128.6±23.3，病程22.8±17.9个月）。FEES在5例患者中检测到高吞咽困难评分，S型和B型表型之间无差异。所有患者的食管下括约肌压力均正常。在3例脊髓起病和2例延髓起病的患者中观察到食管运动障碍。所有ALS患者的食管上括约肌（UES）压力均较高。分别在2例延髓起病和1例脊髓起病患者以及2例延髓起病和4例脊髓起病患者中检测到UES痉挛和胃排空延迟。食管胃运动异常与ALS的临床特征及GI症状之间均无相关性。
一部分ALS患者中存在UES痉挛和胃排空延迟，这可能提示自主神经功能障碍在ALS中的作用。
