Autoimmune Neurogenic Dysphagia

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.

Overlap myositis associated with Sjögren's syndrome

“…4 It is estimated to be quite common in OM (in around 26% of patients), 5 but it is much milder than in other types of IIM. 6 Inflammatory involvement of the swallowing muscles causes dysphagia in IIM, and dysphagia is estimated to present as an initial symptom in less than 10% of OM cases. 4 It can lead to severe complications such as weight loss or aspiration pneumonia.…”

Botulinum toxin injection combined with traditional swallowing rehabilitation improved cricopharyngeal dysfunction in neuromyelitis optica spectrum disorder: A case report

“…The lesions in the medullary structure can lead to the pressure of UES, which should be decreased when swallowing, increased (21). According to the literature reported, NMOSD with MO involvement was more likely to have dysphagia (6)(7)(8)(9). Hence, with the MO involvement may influence the CPG and NTS, and then lead to the higher UES residue pressure.…”

“…Previous study indicated that dysphagia frequently occurs in NMOSD with MO involvement ( 6 – 8 ), which can be detected through questionnaires and instrumental examination ( 7 , 8 ). Also oropharyngeal swallowing disorder might lead to the pneumonia and disability ( 8 ).…”

Paraneoplastic Neurologic Syndromes

“…Neurogenic dysphagia in these patients is also common and underappreciated. 59 Patients with stiff person spectrum disorders have a higher incidence of diabetes, so screening for hyperglycemia is important (especially when administering corticosteroids or glucose-containing IVIg) (CASE 7-4). patients, investigating and treating occult atypical causes of infection (eg, varicella-zoster virus, HSV) is crucial.…”

“…Physical therapy combined with long-acting benzodiazepines and other muscle relaxers (eg, baclofen, tizanidine) and focal botulinum therapy can provide tremendous symptomatic relief, but refractory cases or those with prominent functional burden should be considered for immunotherapies (eg, corticosteroids, intravenous immunoglobulin [IVIg], plasma exchange, rituximab, mycophenolate, cyclophosphamide, methotrexate, stem cell transplant). Neurogenic dysphagia in these patients is also common and underappreciated 59 . Patients with stiff person spectrum disorders have a higher incidence of diabetes, so screening for hyperglycemia is important (especially when administering corticosteroids or glucose-containing IVIg) ( case 7-4 ) 36,49 …”