Episode 52: Neurological Aspects of Systemic Autoimmune Diseases
Dec 24, 2022
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Explore the neurological aspects of systemic autoimmune diseases like lupus, rheumatoid arthritis, and sarcoidosis. Learn about the manifestations of these conditions, including myelitis, optic neuritis, and cognitive dysfunction. Dive into the dangers of TNF alpha inhibitors in patients with demyelinating disorders like MS.
Neuropsychiatric events and strokes are common in lupus patients.
Sarcoidosis can affect any organ system with specific clinical presentations like brainstem syndrome.
Behcet's disease presents with oral ulcers, retinal involvement, and brainstem syndrome.
IgG4 related disease involves fibrosis from plasma cell infiltration and affects multiple organs.
TNF alpha inhibitors can induce demyelinating disorders impacting MS and optic neuritis.
Deep dives
Lupus: Multisystemic Autoimmune Disease with Neurologic Complications
Lupus, a chronic autoimmune disease affecting multiple organs, mainly in women, presents with various clinical features such as oral ulcers, arthritis, and neuropsychiatric manifestations. Diagnostic criteria include positive ANA and specific antibodies like anti-double-stranded DNA. Neurological complications like neuropsychiatric events and strokes are common in lupus patients.
Sarcoidosis: Multiorgan Disorder involving Nervous System Impact
Sarcoidosis, characterized by non-caseating granulomas, can affect any organ system with some specific clinical presentations like lupus perneal, uveoporotic fever, and brainstem syndrome due to vasculovestulitis involving mainly the cranial nerves. Diagnostic imaging may show characteristic nodular enhancements or T2 hyperintensities in the brainstem.
Behcet's Disease: Vasculitis Disorder with Neurological Involvement
Behcet's disease, defined by vasculitis affecting blood vessels, presents with oral ulcers, retinal and venous thrombosis, and involvement of the central nervous system, often manifesting as brainstem syndrome and myelitis. MRI findings may reveal T2 hyperintensities or cerebral venous sinus thrombosis.
IgG4 Related Disease: Lymphoplasmacytic Infiltration and Neurological Impact
IgG4 related disease involves fibrosis from plasma cell infiltration and affects multiple organs, primarily through inflammation and fibrosis. Neurological involvement often includes orbital pseudotumor and hypertrophic pachymeningitis, with MRI showing smooth dural enhancement in the latter.
TNF Alpha Inhibitor Toxicity: Demyelinating Disorders and MS Exacerbation
TNF alpha inhibitors used in rheumatology can lead to central and peripheral demyelinating disorders, exacerbating multiple sclerosis and optic neuritis. Complications are associated with drugs like tannercept and infliximab, with varied onset ranging from days to years post-treatment.
Summary for Beshette's Disease: Multisystem Inflammation and Nervous System Impact
Beshette's disease is a vasculitis disorder manifesting as oral ulcers, retinal involvement, and brainstem syndrome. MRI may show dural enhancement and often, hypopituitarism. Peripheral involvement can lead to sensory motor neuropathy and hypopituitarism.
IgG4 Related Disease Neurological Presentations: Orbitopathy and Pachymeningitis
IgG4 related disease can involve the orbit with pseudotumors and the meninges with pachymeningitis, displaying smooth dural enhancement on imaging.
TNF Alpha Inhibitor Toxicity: Risk of Demyelinating Disorders
TNF alpha inhibitors can cause central and peripheral demyelinating syndromes, exacerbating multiple sclerosis, often presenting as an MS-like syndrome or optic neuritis with varying recovery outcomes.
Special Mention: Behcet's Disease and IgG4 Related Disease: Neurological Considerations
Behcet's disease manifests with oral ulcers and CNS involvement like brainstem syndrome, while IgG4 related disease leads to fibrosis in various organs, presenting distinct neurological features like orbital pseudotumor.
Summary for TNF Alpha Inhibitor Toxicity and Special Mentions
TNF alpha inhibitors can induce demyelinating disorders impacting MS, while Behcet's disease shows vasculitis symptoms and IgG4 related disease presents with lymphoplasmacytic infiltration causing orbital and pachymeningitis complications.
A discussion of the neurological aspects of rheumatological disorders such as systemic lupus erythematosis, rheumatoid arthritis, sarcoidosis and others. With Drs. Aaron Bower and Ariel Lefland.
Note: This podcast is intended solely as an educational tool for learners, especially neurology residents. The contents should not be interpreted as medical advice.
References and Additional Reading: 1. Pawate S. Sarcoidosis and the Nervous System. CONTINUUM: Lifelong Learning in Neurology. 2020;26(3):695. doi:10.1212/CON.0000000000000855 2. Pavlakis PP. Rheumatologic Disorders and the Nervous System. CONTINUUM: Lifelong Learning in Neurology. 2020;26(3):591. do:10.1212/CON.0000000000000856 3.Karam C. Peripheral Neuropathies Associated With Vasculitis and Autoimmune Connective Tissue Disease. CONTINUUM: Lifelong Learning in Neurology. 2020;26(5):1257. doi:10.1212/CON.0000000000000917
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