Introduction:

Acute inflammatory demyelinating polyneuropathy (AIDP), also known as Guillain-Barré syndrome (GBS), is a rare but serious autoimmune disorder characterized by rapidly progressive weakness, sensory disturbances, and areflexia due to demyelination of peripheral nerves. It is considered a medical emergency requiring prompt diagnosis and treatment. In this article, we delve into the causes, symptoms, diagnosis, and treatment of acute inflammatory demyelinating polyneuropathy.

Causes of Acute Inflammatory Demyelinating Polyneuropathy:

• Autoimmune Response: AIDP is thought to result from an aberrant autoimmune response triggered by various factors, including preceding infections such as Campylobacter jejuni, cytomegalovirus (CMV), Epstein-Barr virus (EBV), Mycoplasma pneumoniae, or Zika virus, among others.
• Molecular Mimicry: Molecular mimicry, where antigens from infectious agents resemble components of peripheral nerve myelin, may lead to cross-reactive immune responses targeting peripheral nerves, resulting in demyelination and subsequent nerve dysfunction.

Symptoms of Acute Inflammatory Demyelinating Polyneuropathy:

• Muscle Weakness: AIDP typically presents with rapidly progressive symmetric muscle weakness, initially affecting the lower extremities and ascending to involve the upper extremities and respiratory muscles, leading to respiratory failure in severe cases.
• Sensory Disturbances: Patients may experience sensory abnormalities such as paresthesias (tingling, numbness), dysesthesias (abnormal sensations), and sensory loss, often following the pattern of ascending weakness.
• Areflexia: AIDP is characterized by areflexia, with absent or diminished deep tendon reflexes (such as the patellar reflex and Achilles reflex) due to nerve conduction block and demyelination of sensory and motor nerve fibers.
• Autonomic Dysfunction: Some individuals with AIDP may develop autonomic dysfunction, manifesting as cardiovascular instability (such as orthostatic hypotension, tachycardia), gastrointestinal dysmotility, and bladder dysfunction.

Diagnosis of Acute Inflammatory Demyelinating Polyneuropathy:

• Clinical Evaluation: Diagnosis of AIDP is based on clinical features, including rapidly progressive symmetric weakness, areflexia, and sensory disturbances, in the absence of alternative explanations.
• Electrodiagnostic Studies: Nerve conduction studies (NCS) and electromyography (EMG) can help confirm the diagnosis by demonstrating features of demyelination, such as prolonged distal motor latencies, slowed nerve conduction velocities, and temporal dispersion of compound muscle action potentials (CMAPs).
• Cerebrospinal Fluid Analysis: Lumbar puncture and analysis of cerebrospinal fluid (CSF) may reveal elevated protein levels (albuminocytologic dissociation) without pleocytosis, supporting the diagnosis of AIDP.
• Imaging Studies: Magnetic resonance imaging (MRI) of the spine may be performed to rule out alternative diagnoses and assess for nerve root enhancement characteristic of AIDP.

Treatment of Acute Inflammatory Demyelinating Polyneuropathy:

• Intravenous Immunoglobulin (IVIG): Intravenous immunoglobulin is the preferred first-line treatment for AIDP, administered at a high dose (usually 2 g/kg body weight) over several days, which helps modulate the immune response and promote remyelination of peripheral nerves.
• Plasma Exchange (Plasmapheresis): Plasma exchange may be considered as an alternative or adjunctive therapy for patients with AIDP, particularly in cases of severe weakness or when IVIG is contraindicated.
• Supportive Care: Supportive measures such as respiratory support (including mechanical ventilation if necessary), pain management, deep vein thrombosis prophylaxis, and physical rehabilitation are essential components of management for patients with AIDP.
• Monitoring and Follow-Up: Close monitoring of respiratory function, neurological status, and complications (such as autonomic dysfunction and infections) is essential, with regular follow-up assessments to track recovery and address ongoing needs.

Conclusion:

Acute inflammatory demyelinating polyneuropathy (AIDP) is a rare autoimmune disorder characterized by rapidly progressive weakness, sensory disturbances, and areflexia due to demyelination of peripheral nerves. Early recognition, prompt diagnosis, and initiation of appropriate treatment are crucial for optimizing outcomes and minimizing complications in patients with AIDP.

Hashtags: #AIDP #GuillainBarreSyndrome #PeripheralNeuropathy #AutoimmuneDisorder