From the Guidelines
Definition of Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
Acute inflammatory demyelinating polyneuropathy (AIDP), also known as Guillain-Barré syndrome, is a rare autoimmune disorder characterized by rapid onset of muscle weakness and paralysis.
- It is an inflammatory disease of the peripheral nervous system (PNS) and is the most common cause of acute flaccid paralysis, with an annual global incidence of approximately 1–2 per 100,000 person-years 1.
- AIDP is thought to be caused by an aberrant immune response to infections that results in damage to peripheral nerves, although the pathogenesis is not fully understood 1.
- The clinical presentation of AIDP is heterogeneous, and several distinct clinical variants exist, including a pure motor variant, paraparetic variants, and Miller Fisher syndrome (MFS) 1.
Diagnosis and Treatment
- Diagnosis of AIDP is based on the patient history and neurological, electrophysiological, and cerebrospinal fluid (CSF) examinations 1.
- Treatment typically involves plasma exchange or intravenous immunoglobulin (IVIG) at a dose of 2g/kg administered over 2-5 days, with corticosteroids such as prednisone 60mg daily for 1-2 weeks also considered in some cases 1.
- Supportive care, including pain management with medications like gabapentin 300-1200mg daily and physical therapy, is also essential to manage symptoms and promote recovery.
- In severe cases, prompt recognition of symptoms is essential to prevent respiratory insufficiency due to affected cervical nerve roots, and treatment may involve IVIG and/or plasma exchange or selective separation 1.
Clinical Features and Outcome
- The severity of AIDP is highly variable, ranging from mild distal limb weakness to complete paralysis, respiratory failure, and even death 1.
- About 20% of patients with AIDP develop respiratory failure and require mechanical ventilation, and cardiac arrhythmias and blood pressure instability can occur owing to involvement of the autonomic nervous system 1.
- The outcome of AIDP is generally favorable, with 60–80% of patients able to walk independently 6 months after disease onset, with or without treatment 1.
From the Research
Definition and Characteristics of Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
- AIDP is characterized by the autoimmune destruction of Schwann cells with resultant myelin degradation 2
- It is the most common subtype of Guillain-Barré Syndrome (GBS) 2, 3, 4
- AIDP is an immune-mediated polyneuropathy usually triggered by infections or vaccinations 3
Clinical Presentation
- Symptom onset is typically 1-3 weeks following an upper respiratory or gastrointestinal illness 2
- Rapidly progressive ascending areflexic motor paralysis is a common symptom 2, 5
- Lower cranial nerves are often involved, leading to bulbar weakness and respiratory compromise 2
- Autonomic dysregulation is common and must be managed carefully to avoid potentially fatal autonomic dysregulation 2
- Initial complaint can be lower back pain in up to 66% of GBS cases 2
Diagnosis and Treatment
- Diagnosis is largely clinical, but diagnostic tools are available to confirm the diagnosis, prognosticate, detect variant forms, and rule out mimics 5
- Intravenous Immunoglobulin (IVIg) and/or plasmapheresis coupled with supportive management is the mainstay of GBS treatment 2, 3, 4
- The majority of patients make a full recovery in up to one year 2, 3