From the Guidelines
AIDP (Acute Inflammatory Demyelinating Polyneuropathy) is the most common form of Guillain-Barré syndrome, and its treatment primarily involves immunotherapy with either intravenous immunoglobulin (IVIG) or plasma exchange (PLEX), which should be started within the first two weeks of symptom onset for best results, as supported by the most recent and highest quality study 1.
Key Characteristics of AIDP
- AIDP is an autoimmune disorder where the immune system attacks the peripheral nerves, typically beginning with weakness and tingling in the extremities that rapidly progresses upward, potentially affecting breathing and heart rate in severe cases.
- It is the most common subtype of Guillain-Barré syndrome, accounting for 90-95% of cases in some regions, as reported in a study from 2021 1.
- The clinical presentation of AIDP is heterogeneous, and diagnosis is based on patient history, neurological, electrophysiological, and cerebrospinal fluid (CSF) examinations, as outlined in a guideline for the diagnosis and management of Guillain-Barré syndrome 1.
Treatment and Management
- Immunotherapy with IVIG at 2g/kg divided over 2-5 days or PLEX with 5 exchanges over 1-2 weeks is the primary treatment for AIDP, with both treatments being equally effective, as stated in the guideline 1.
- Supportive care, including respiratory monitoring, prevention of complications like deep vein thrombosis, and physical therapy, is crucial for managing AIDP.
- Most patients begin recovery within 2-4 weeks after symptom plateau, though complete recovery may take months to years, as noted in the study from 2019 1.
Pathogenesis and Outcome
- AIDP is believed to result from molecular mimicry, where antibodies produced against an infection (often Campylobacter jejuni) cross-react with peripheral nerve components, causing demyelination and conduction block in the peripheral nerves.
- The outcome of AIDP can vary, with some patients experiencing a full recovery, while others may be left with residual weakness or disability, as reported in the study from 2021 1.
- Mortality rates for AIDP are estimated to be around 3-10%, even with the best medical care available, as stated in the guideline 1.
From the Research
Definition and Description of AIDP
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is an immune-mediated polyneuropathy usually triggered by infections or vaccinations 2.
- AIDP is also known as Guillain-Barré syndrome, which is the most common form of Guillain-Barré syndrome in Western countries 3.
Clinical Presentation and Diagnosis
- AIDP can manifest with ascending hypotonia, weakness, pain, and areflexia in the lower extremities 2.
- The clinical presentation, laboratory, and neurophysiological studies support the diagnosis of AIDP 2.
- Electrophysiological descriptions of changes in abnormalities suggestive of demyelination after an AIDP episode are rare, but studies have shown that abnormalities suggestive of demyelination can worsen on subsequent examinations and persist for long periods after the acute episode 3.
Treatment and Management
- Intravenous immunoglobulin (IvIg) and plasma exchange (PE) are commonly used treatments for AIDP 4, 5.
- Studies have shown that IvIg can be more effective than PE in reducing the length of hospitalization and improving motor recovery 4.
- The mechanism by which IvIg acts is less clear, but possibilities include antiidiotypic antibody effect, complement absorption, and downregulation of immunoglobulin production 5.
Prognosis and Outcome
- The prognosis for AIDP is generally favorable, with most patients experiencing significant improvement or complete recovery 2, 4.
- However, some patients may experience persistent abnormalities suggestive of demyelination for long periods after the acute episode, despite clinical improvement 3.
- Further study is needed to determine the role of IvIg and PE in the treatment of AIDP and to explore other potential treatments, such as immunosuppressive or immunomodulatory agents 5, 6.