Abstract Details

Title 2 cases of hypertrophic CIDP associated to other autoimmune neurological disorders: Myasthenia Gravis and Multiple Sclerosis.

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 12-025

Objective NA

Background Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) associated to Myasthenia Gravis (MG) or Multiple Sclerosis (MS) is scarcely described. Different from MG, MS and CIDP share some fundamental immunological principles. In both MS and CIDP, dysfunction of immune tolerance mechanisms causes humoral and cellular autoimmunity to the complex of the myelin sheath and axon.

Design/Methods NA

Results Case 1: 46-year-old man presenting with paresthesia, pain and slowly progressive weakness with distally predominance on limbs followed by sensitive ataxia and areflexia. CSF showed elevated protein level without pleiocytosis and signs of diffuse demyelination on electromyoneurogram (EMNG). On MRI, cranial nerves, cervical and lumbar roots and plexus with severe thickness was observed. Treated with Intravenous Immunoglobulins, prednisolone and Azatioprine. After 9 years, patient presented ptosis, dyplopia, dysphagia, dysphonia and positive antibodies (AchR) for MG. Case 2: 52-year-old woman presenting with spastic paraparesis, loss of sensitivity on lower limbs and hands, areflexia and bilateral Babinski. CSF showed elevated protein level without pleiocytosis and presence of oligoclonal bands. Severe demyelinating neuropathy was observed at EMNG. MRI: difuse demyelination in CNS and severe thickness of lumbar roots and plexus. Iniciated treatment with mensal Intravenous immunoglobulin, Prednisolone, Azatioprine and Fingolimod presenting in 18 moths significant clinical improvement.

Conclusions Combination of CIDP and MG is rare. It is speculated that both Th1 and Th2 cells are involved in their coexistence. The abnormal activation of immune system produces cross-reacting antibodies, which attack the AchR of neuromuscular junctions and the myelin protein of peripheral nerves. Also, the occurrence of CIDP associated to MS may be fortuitous. However, there is growing evidence of common risk factors, such as immunopathogenetic mechanisms, including shared myelin antigens, common immunologic factors, endothelial alterations, and altered expression of adhesion molecules. Both cases have association with autoimmune diseases, corroborating the literature, therefore more studies about this correlation are still necessary.

Authors/Disclosures
Victor Mantelatto Bonsi PRESENTER	Victor Mantelatto Bonsi has nothing to disclose.
André Luiz Guimarães de Queiroz	André Luiz Guimarães de Queiroz has nothing to disclose.
Karlla Danielle Ferreira Lima	Karlla Danielle Ferreira Lima has nothing to disclose.
	No disclosure on file
	No disclosure on file
Alex Baeta	Alex Baeta has nothing to disclose.

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