What is the differentiation between Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and Guillain-Barré Syndrome (GBS)?

From the Guidelines

Differentiation between Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and Guillain-Barré Syndrome (GBS) is crucial for optimal management, as CIDP is a chronic condition requiring long-term immunomodulatory therapy, whereas GBS is an acute condition that can be effectively treated with IVIG or plasma exchange during the acute phase only. The key differences between CIDP and GBS lie in their clinical course, treatment, and prognosis. CIDP progresses over at least 8 weeks, while GBS reaches its nadir within 4 weeks 1. CIDP typically presents with symmetric proximal and distal weakness, sensory symptoms, and areflexia that fluctuates or steadily progresses over months, whereas GBS presents with rapidly progressive ascending weakness, sensory symptoms, and areflexia that typically reaches maximum severity within 2-4 weeks, often following a respiratory or gastrointestinal infection.

Some of the key features that distinguish CIDP from GBS include:

• Clinical course: CIDP is chronic, while GBS is acute
• Treatment: CIDP requires long-term immunomodulatory therapy, while GBS is treated with IVIG or plasma exchange during the acute phase only
• Prognosis: CIDP has a variable prognosis, while GBS is typically monophasic with recovery over weeks to months
• CSF analysis: Both conditions show albuminocytologic dissociation, but protein elevation may be higher in CIDP
• Nerve conduction studies: CIDP shows demyelinating features that may be patchy, while GBS typically shows uniform slowing

According to a recent study published in Nature Reviews Neurology in 2021, the treatment of GBS requires a multidisciplinary approach, including supportive medical care and immunotherapy, with intravenous immunoglobulin (0.4 g/kg for 5 days) and plasma exchange (usually five sessions at 200–250 ml/kg) being proven and equally effective treatments 2. However, the study also highlights the challenges and prospects of managing GBS in low-income and middle-income countries, where access to these treatments may be limited.

In terms of diagnosis, a ten-step guideline for the diagnosis and management of GBS has been developed, which includes early recognition and diagnosis, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae 1. This guideline is based on current literature and expert consensus and provides a globally applicable framework for the diagnosis and management of GBS.

Overall, accurate differentiation between CIDP and GBS is essential for optimal management and treatment, and a multidisciplinary approach, including supportive medical care and immunotherapy, is crucial for improving outcomes in patients with GBS.

From the Research

Differentiation between CIP and GBS

• The differentiation between Critical Illness Polyneuropathy (CIP) and Guillain-Barré Syndrome (GBS) can be challenging, but several studies have identified key differences between the two conditions 3, 4, 5, 6, 7.
• Electrophysiological studies, such as nerve conduction studies and needle electromyography, can provide definitive evidence for polyneuropathy and help differentiate between CIP and GBS 4, 5.
• CIP is characterized by reductions in the amplitudes of compound muscle and sensory nerve action potentials, while GBS is characterized by demyelination and conduction block 4, 5.
• The presence of sensory nerve conduction studies can help differentiate between CIP and GBS, as CIP typically shows reduced sensory nerve action potentials, while GBS shows preserved or slightly reduced sensory nerve action potentials 4, 5.
• Ultrasound and neurophysiological measurements can also be used to differentiate between GBS and acute-onset chronic inflammatory demyelinating polyradiculoneuritis (CIDP), with GBS showing nerve enlargement and ultrasonic sensory sparing pattern (uSSP) 6.
• The clinical presentation, electrophysiological features, and cerebrospinal fluid results can also help differentiate between CIP and GBS, with CIP typically presenting with a more subacute onset and GBS presenting with a more acute onset 4, 7.
• The presence of anti-GM1 antibodies and molecular mimicry has been postulated as a possible mechanism triggering GBS, while CIP is thought to be related to critical illness and sepsis 4, 7.