Fatigue in immune-mediated neuropathies
Introduction
Immune-mediated neuropathies include Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUSP), and multifocal motor neuropathy (MMN). Electrophysiological examination in these patients generally reveals features of a demyelinating polyneuropathy, although a subgroup of GBS patients may have predominantly axonal features (acute motor axonal neuropathy (AMAN)). Diagnostic criteria for all these neuropathies have been formulated [1], [2], [3], [4]. From a clinical point of view there is increasing evidence that these illnesses represent part of a continuum, mainly separated by the location and extent of motor and/or sensory deficit, their evolution over time, and their response to treatment [1], [2], [3], [4]. However, despite being potentially treatable, particularly in GBS, CIDP, and MMN, studies on residual deficits in these conditions have demonstrated deficits in daily and social activities and decrement in quality of life expectations [5], [6], [7], [8], [9]. Moreover, some reports have addressed less tangible complaints in these conditions like being fatigued as one of the most important causes of dysfunctioning [7], [9], [10], [11], [12], [13], [14], [15]. Since attention in these illnesses is primarily directed towards weakness and sensory disturbances, it is suggested that fatigue may have been under-recognized for years by neurologists and rehabilitation physicians.
The pathogenesis of fatigue in neuromuscular diseases is unknown, but is believed to be multifactorial and may be caused by peripheral and central processes [16], [17], [18]. This review will be highlighting the known literature on fatigue in immune-mediated disorders, particularly focusing on GBS, CIDP, MGUSP, and MMN. The impact of fatigue on daily functionality and quality of life, the suggested underlying mechanisms, and possible therapeutic interventions will be addressed as well. Finally, unanswered questions for future studies in this field will be highlighted.
Section snippets
Papers addressing fatigue in immune-mediated neuropathies
An initial paper in the late eighties briefly addressed fatigue as a residual complaint in a limited number of GBS cases. The presence of fatigue was compared between patients with neuromuscular disorders (myasthenia gravis, GBS (n = 3), myopathies, metabolic muscle disorders) versus two other groups of patients (group 1: chronic fatigue syndrome not otherwise explained; group 2: patients with major depression) using a fatigue symptoms assessment that comprises questions to determine physical and
Assessment of fatigue
Over 30 scales have been developed attempting to measure the nature, severity and impact of fatigue in a wide range of clinical populations [30]. These outcome measures vary greatly in their content and in how well they have been evaluated. The most widely used outcome measure in immune-mediated neuropathies is the unidimensional Fatigue Severity Scale (FSS) [9], [19]. This scale has demonstrated to be very brief with good scientific soundness (validity and reliability scores) [9]. However,
Impact on daily functioning and quality of life
Contradictory findings have been published regarding the impact of fatigue on disability and quality of life expectations in patients with immune-mediated neuropathies. Fatigue did not show any correlation with clinical features like general weakness, sensory deficit, duration of illness, age, or type of diagnosis in patients with GBS, CIDP, and MGUSP. In essence, the severity of fatigue was equivalent between patients with normal strength or sensory deficit and those having physical deficits
Therapeutic options of fatigue in immune-mediated neuropathies
In immune-mediated neuropathies, physical training seemed to have a positive effect on reducing fatigue [13], [25]. In patients recovered from GBS and in patients with well-established ongoing therapy suffering from CIDP with severely reported fatigue, implementation of an exercise programs demonstrated a substantial reduction of 20% of self-reported fatigue [13], [17]. This improvement had been maintained at 2-year follow-up. However, the underlying mechanism for this positive effect has not
Conclusion
Fatigue is a highly prevalent and disabling symptom in immune-mediated neuropathies with severe impact on daily living, social functioning and quality of life. The aetiology of fatigue in these disorders, like in other illnesses, has not been elucidated yet and is considered to be multi-factorial. Future studies are warranted to focus on the underlying mechanisms leading to fatigue, which could provide an insight to a better therapeutic approach, hereby improving the well being of patients with
Conflicts of interest
The authors have nothing to disclose.
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