Discussion on inflammatory polyneuropathies, focusing on Guillaume Bresindrum and its clinical presentation. Exploring AIDP pathophysiology, examination findings, diagnostic testing, and treatment options. Various variants of Guillain-Barre syndrome discussed, including clinical differences, nerve conduction studies, and antibody-associated syndromes.
22:35
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Quick takeaways
Inflammatory polyneuropathies, such as acute inflammatory demyelinating polyridiculoneuropathy (AIDP), are often characterized by progressive weakness and loss of reflexes, and can be caused by autoantibodies that disrupt the myelin sheath leading to sensory symptoms and autonomic dysfunction.
Different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including Lewis-Sumner syndrome, multifocal motor neuropathy, and distal symmetric sensory neuropathy (DADS), have distinct clinical features and may require specific treatment approaches involving steroids and intravenous immunoglobulin therapy.
Deep dives
Key Points about Acute Inflammatory Demyelinating Polyridiculoneuropathy
Acute inflammatory demyelinating polyridiculoneuropathy (AIDP) is characterized by progressive flaccid weakness, usually starting distally and progressing proximally, along with aroflexia and loss of reflexes. The typical clinical vignette involves a patient with a history of antecedent infection, such as diarrheal illness or viral upper respiratory infection. AIDP is caused by autoantibodies that disrupt the myelin sheath, particularly ganglyside antibodies. Physical examination findings include sensory symptoms, parasthesias, relative symmetry of symptoms, and evidence of autonomic dysfunction. Lumbar puncture with increased protein without a concurrent increase in cellularity is a diagnostic test, along with electromyography and nerve conduction studies that show conduction block and decreased compound muscle action potential. The main treatment options are intravenous immunoglobulin therapy and plasma forasis, with monitoring of respiratory status being crucial.
Insights into Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) typically has a longer time course of progression, lasting at least eight weeks. Electromyography and nerve conduction studies reveal similar findings to AIDP, such as F-wave prolongation, temporal dispersion, and reduced conduction velocity. Treatment for CIDP involves steroids and maintenance intravenous immunoglobulin therapy. Other variants of CIDP include Lewis-Sumner syndrome, multifocal motor neuropathy, distal symmetric sensory neuropathy (DADS), and poems syndrome (polyneuropathy, organomegaly, endocrineopathy, M-protein spike syndrome). Each variant has distinct clinical features and may require specific treatment approaches.
Overview of Guillain-Barre Syndrome (GBS) and Antibody-Associated Syndromes
Guillain-Barre syndrome (GBS) is an umbrella term for various autoimmune neuropathies characterized by progressive weakness and areflexia. The GQ1B antibody is associated with Miller-Fisher syndrome (ophthalmoparesis, ataxia, areflexia) and can also be linked to Bickerstaff brainstem encephalitis. Axonal variants of GBS, such as acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN), tend to have more severe presentations, prolonged F-wave absence, and reduced compound muscle action potential. Autoantibodies like anti-MAG (myelin associated glycoprotein) and vegF (vascular endothelial growth factor) are found in other GBS variants like DADS and Poems syndrome. Treatment options depend on the specific variant and may involve steroids, intravenous immunoglobulins, or hematological referral.
A overview of some of the most common and important inflammatory polyneuropathies, with Drs. Kevin Yan and Kevin Wilson.
Note: This podcast is intended solely as an educational tool for learners, especially neurology residents. The contents should not be interpreted as medical advice.
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