Subtle Esophageal Motility Alterations in Parkinsonian Syndromes: Synucleinopathies vs. Tauopathies

Abstract

Background Esophageal dysfunction is a frequent phenomenon in Parkinson's disease during all disease stages, but data about esophageal involvement in atypical parkinsonian syndromes as well as possible differences between alpha‐synucleinopathies and tauopathies, including causative links to the origin of the dysfunction, are lacking so far. Objective To describe esophageal alternation patterns in different parkinsonian syndromes and to look for differences supporting the hypothesis of alpha‐synuclein aggregation being linked to gastrointestinal impairment in parkinsonian syndromes. Methods We performed an analysis and comparison of esophageal high‐resolution manometry examination parameters in n = 10 patients with Parkinson's disease, n = 10 patients with multiple system atrophy (both alpha‐synucleinopathies), n = 10 patients with progressive supranuclear palsy (tauopathy), and n = 10 age‐matched controls. Additionally, pharyngeal dysphagia was evaluated by an endoscopic examination of swallowing in all three patient groups. Results Statistically significant lower values for esophageal peristalsis and distal contractile integral were found for both groups of alpha‐synucleinopathies (Parkinson's disease and multiple system atrophy) in comparison to the patients with tauopathy (progressive supranuclear palsy group), as well as the age‐matched controls, where pathological pharyngeal findings were similar in all patient groups. Conclusions Subtle esophageal motility alterations in parkinsonian syndromes seem to be limited to alpha‐synucleinopathies, but are not measurable in tauopathies, indicating a causative connection between pathological alpha‐synuclein aggregation in gastrointestinal tissues and esophageal involvement.

Diagnosis and treatment of neurogenic dysphagia – S1 guideline of the German Society of Neurology

“…For gastroenterological disorders of the oesophagus, an assessment based on the Chicago Classification is common [ 88 ]. Only in recent years, HRM was used for the assessment of the oesophageal motility in patients with neurological diseases, especially Parkinson syndromes [ 89 ], inflammatory myopathies [ 90 ] and Morbus Huntington [ 91 ]. In neurology HRM is particularly important in patients with opening disorders of the UES, e.g., as a result of myopathies or strategic brainstem infarctions.…”

Assessment of Gastrointestinal Autonomic Dysfunction: Present and Future Perspectives

“…Esophageal motor dysfunction is present in half of all patients with type 1 DM and dysphagia [ 53 ]. In addition, esophageal dysmotility is frequently seen in the α-synucleinopathies, most often as generally reduced peristalsis with ineffective swallows [ 31 , 33 ]. Absent or impaired esophageal activity is documented in POTS with conventional esophageal manometry and with high-resolution esophageal manometry in the Ehlers–Danlos Syndrome, hypermobility type [ 54 , 55 ].…”

“…Pan-enteric dysmotility has been documented, and the abnormalities in each segment of the GI tract are presented in Figure 1 . Aperistalsis and uncoordinated contractions are common in the esophagus [ 7 , 31 ]. Gastric dysmotility presents as delayed or accelerated gastric emptying time and reduced postprandial accommodation [ 21 , 30 ].…”

Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment

“…Although the oropharyngeal phases of swallowing are most impaired in MSA, subtle esophageal dysmotility may also occur. Main dysfunctions observed by means of VFSS and high-resolution manometry, even in patients who were asymptomatic, included food stagnation in the esophagus, reduced esophageal peristalsis, hypomotility of the distal esophagus, abnormally high upper esophageal sphincter (UES) pressure and uncoordinated proximal esophageal contraction pressure during swallowing and resting [23,27,28].…”

“…High-resolution manometry is useful to assess disorders of esophageal peristalsis [20,23,27,28]. Also, electrokinesigraphic studies of the oropharyngeal phases of swallowing have been used to evaluate pathophysiological mechanisms of oropharyngeal dysphagia in MSA [30,49].…”