Autoimmune Neurogenic Dysphagia.

Panos Stathopoulos,Marinos C Dalakas

doi:10.1007/s00455-021-10338-9

Abstract

Autoimmune neurogenic dysphagia refers to manifestation of dysphagia due to autoimmune diseases affecting muscle, neuromuscular junction, nerves, roots, brainstem, or cortex. Dysphagia is either part of the evolving clinical symptomatology of an underlying neurological autoimmunity or occurs as a sole manifestation, acutely or insidiously. This opinion article reviews the autoimmune neurological causes of dysphagia, highlights clinical clues and laboratory testing that facilitate early diagnosis, especially when dysphagia is the presenting symptom, and outlines the most effective immunotherapeutic approaches. Dysphagia is common in inflammatory myopathies, most prominently in inclusion body myositis, and is frequent in myasthenia gravis, occurring early in bulbar-onset disease or during the course of progressive, generalized disease. Acute-onset dysphagia is often seen in Guillain–Barre syndrome variants and slowly progressive dysphagia in paraneoplastic neuropathies highlighted by the presence of specific autoantibodies. The most common causes of CNS autoimmune dysphagia are demyelinating and inflammatory lesions in the brainstem, occurring in patients with multiple sclerosis and neuromyelitis optica spectrum disorders. Less common, but often overlooked, is dysphagia in stiff-person syndrome especially in conjunction with cerebellar ataxia and high anti-GAD autoantibodies, and in gastrointestinal dysmotility syndromes associated with autoantibodies against the ganglionic acetyl-choline receptor. In the setting of many neurological autoimmunities, acute-onset or progressive dysphagia is a potentially treatable condition, requiring increased awareness for prompt diagnosis and early immunotherapy initiation.

Highlights

Dysphagia, manifested as difficulty swallowing solids or liquids, is a symptom of variable severity, from mild to severe, which affects quality of life and may lead to lifethreatening complications including malnutrition, weight loss, and aspiration pneumonia
We focus on common autoimmune neurological diseases, such as inflammatory autoimmune myopathies, myasthenia gravis, autoimmune cranial neuropathies in the spectrum of Guillain–Barre syndrome and multiple sclerosis, and less common like neuromyelitis optica, Stiff-person spectrum disorders and ganglionic acetyl-choline receptor autoantibody-related autoimmunities
Dysphagia most commonly occurs in Inclusion Body Myositis (IBM), but it is not uncommon in DM, anti-SSOM, and necrotizing autoimmune myositis (NAM)/immune-mediated necrotizing myositis (IMNM); an elevated creatine kinase (CK) raises suspicion, and a muscle biopsy confirms the diagnosis

Summary

Introduction

Dysphagia, manifested as difficulty swallowing solids or liquids, is a symptom of variable severity, from mild to severe, which affects quality of life and may lead to lifethreatening complications including malnutrition, weight loss, and aspiration pneumonia. Autoimmune causes of dysphagia can be gastroenterological, such as IgG4-related disease and eosinophilic esophagitis; dermatological, such as pemphigus vulgaris and bullous pemphigoid; rheumatologic, such as scleroderma, Sjogren’s syndrome, systemic lupus erythematosus, rheumatoid arthritis, Behcet disease, ANCA-associated vasculitis, or granulomatosis with polyangiitis; and neurologic. In this opinion article, we review the autoimmune neurological causes of dysphagia, highlight clinical clues and laboratory tests that facilitate early diagnosis, especially when dysphagia is the presenting symptom, and outline the most effective immunotherapeutic approaches.

Autoimmune ganglionopathies and autonomic nervous system disorders

Findings

Summary and Conclusion