The Burden of Neurosarcoidosis: Essential Approaches to Early Diagnosis and Treatment

Abstract

Neurosarcoidosis (NS) is an often severe, destructive manifestation with a likely under-reported prevalence of 5 to 15% of sarcoidosis cases, and in its active phase demands timely treatment intervention. Clinical signs and symptoms of NS are variable and wide-ranging, depending on anatomical involvement. Cranial nerve dysfunction, cerebrospinal parenchymal disease, aseptic meningitis, and leptomeningeal disease are the most commonly recognized manifestations. However, non-organ-specific potentially neurologically driven symptoms, such as fatigue, cognitive dysfunction, and small fiber neuropathy, appear frequently.Heterogeneous clinical presentations and absence of any single conclusive test or biomarker render NS, and sarcoidosis itself, a challenging definitive diagnosis. Clinical suspicion of NS warrants a thorough systemic and neurologic evaluation hopefully resulting in supportive extraneural physical exam and/or tissue findings. Treatment targets the severity of the manifestation, with careful discernment of whether NS reflects active potentially reversible inflammatory granulomatous disease versus inactive postinflammatory damage whereby functional impairment is unlikely to be pharmacologically responsive. Non-organ-specific symptoms are poorly understood, challenging in deciphering reversibility and often identified too late to respond to conventional immunosuppressive/pharmacological treatment. Physical therapy, coping strategies, and stress reduction may benefit patients with all disease activity levels of NS.This publication provides an approach to screening, diagnosis, disease activity discernment, and pharmacological as well as nonpharmacological treatment interventions to reduce disability and protect health-related quality of life in NS.

Sarcoidosis Illuminations on Living During COVID-19: Patient Experiences of Diagnosis, Management, and Survival Before and During the Pandemic

“…The groups expressed that respiratory and multi-organ system conditions, like sarcoidosis, historically received limited public awareness, research attention, and funding; and that the COVID-19 pandemic may lead to a greater appreciation of and knowledge in respiratory and multi-organ system diseases. Further expressed was the hope that knowledge gained in sarcoidosis (1,(11)(12)(13)(14)(15)(16)) might be useful in understanding the quality-of-life and therapeutic realms of COVID-19 treatment and recovery.…”

Health-Related Quality of Life (HRQoL) in Sarcoidosis: Diagnosis, Management, and Health Outcomes

“…Fatigue and small fiber neuropathy play a role in cognitive failure [92], with the presence of cognitive failure being an independent predictor of fatigue [40]. Interestingly, the only factor associated with a significantly higher level of cognition was recent TNFalpha-inhibition [93] Again, such findings re-assert the question of sub-radiological and treatable sarcoidosis being present [94]. In addition to optimally suppressing sarcoidosis, psychological interventions that focus on coping, stress reduction, cognitive-behavioral therapy, and mindfulness-based cognitive therapy can support cognitive function.…”

“…SFN-related chest pain in sarcoidosis, though should be investigated for cardiopulmonary involvement accordingly, occurs with an often described painful, gripping that can restrict chest expansion. However, SFN is much more extensive and includes autonomic dysfunction, hypohidrosis or hyperhidrosis, gastrointestinal disturbances, e.g., diarrhea, constipation or gastroparesis, micturition disturbances, sicca symptoms (dry eyes and/or dry mouth), blurry vision with accommodation problems, hot flushes, orthostatic dizziness, sexual dysfunction, cardiac palpitations/(pre)syncope [94,97]. SFN occurs as frequently as 86% in northern Europe [23].…”

“…Systemic inflammatory disease may generate overwhelming fatigue whereby treating the underlying inflammatory condition can provide relief. Suboptimal treatment of sarcoidosis can also result in lingering fatigue, and possibly cognitive dysfunction [94]. Prior active inflammatory disease might result in residual changes to the muscular and vascular infrastructure, potentially resulting in a lower exercise capacity and physical fatigue.…”

Sarcoidosis and frailty: recognizing factors that foster holistic resilience

“…Conceptually important to frailty, sarcoidosis is associated with significant impairment and impedance of psychosocial and emotional well-being due to disease-related symptoms, medication toxicities/intolerances and stress on major life areas, such as social interactions, education, family, employment, financial and community engagement [3,5,6 ▪▪ ,7]. Fear and anxiety about the uncertain disease trajectory and its complications cause stress and contribute to the burden of sarcoidosis [1,2,3,6 ▪▪ ].…”