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Neurological manifestations in autoinflammatory diseases
Antonio Uccelli1, Marco Gattorno2
1Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health Unit, University of Genoa; Ospedale Policlinico San Martino, IRCCS, Genoa; and Centre of Excellence for Biomedical Research (CEBR), University of Genoa, Italy.
Clinical and Experimental Rheumatology
|May 10, 2018
Summary
Autoinflammatory diseases (AIDs) involve innate immune system dysregulation causing inflammation. Neurological issues like meningitis, stroke, and hearing loss can manifest as rare but serious complications of AIDs.
Area of Science:
- Immunology
- Neurology
- Genetics
Background:
- Autoinflammatory diseases (AIDs) are a group of disorders characterized by innate immune system dysregulation.
- These conditions lead to systemic inflammation affecting various organs and tissues.
- Neurological manifestations can be a significant feature in some AIDs.
Purpose of the Study:
- To review the spectrum of neurological manifestations associated with autoinflammatory diseases.
- To highlight the role of innate immunity in CNS involvement in AIDs.
- To provide a comprehensive overview of rare neurological complications in AIDs.
Main Methods:
- Literature review of neurological manifestations in autoinflammatory diseases.
- Analysis of case studies and clinical data.
- Synthesis of current understanding of CNS involvement in AIDs.
Main Results:
- Neurological manifestations in AIDs are variable and can include chronic aseptic meningitis, brain atrophy, sensorineural hearing loss, and early-onset strokes.
- Other reported neurological issues include mental retardation, cerebellitis, ataxia, and severe encephalopathy with brain calcifications.
- Central nervous system involvement is a rare complication, stemming from systemic or local immune responses involving innate immunity cells.
Conclusions:
- Neurological complications are rare but significant manifestations of autoinflammatory diseases.
- Understanding these neurological aspects is crucial for comprehensive patient management.
- Further research is needed to elucidate the mechanisms underlying CNS involvement in AIDs.
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