What are the typical patient presentations of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)?

Clinical Presentations of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

CIDP typically presents with progressive or relapsing symmetric proximal and distal weakness of upper and lower limbs, sensory involvement of at least two limbs, and decreased or absent deep tendon reflexes, with symptoms developing over at least 2 months. 1, 2

Core Clinical Features

• CIDP has a progressive or relapsing course over at least 2 months, distinguishing it from Guillain-Barré syndrome which reaches maximum disability within 2-4 weeks 1
• Progressive weakness typically starts in the legs and potentially spreads to arms and cranial muscles 1
• Distal paresthesias or sensory loss can progress proximally 1
• Decreased or absent deep tendon reflexes are a hallmark finding 2
• CIDP affects both proximal and distal regions, unlike drug-induced neuropathies which typically present with a length-dependent "stocking-and-glove" distribution 1

CIDP Variants

• The MADSAM variant (Multifocal Acquired Demyelinating Sensory And Motor neuropathy, also known as Lewis-Sumner syndrome) presents with asymmetric or multifocal sensorimotor deficits rather than the symmetric pattern seen in typical CIDP 1, 3
• Pure motor CIDP (PM-CIDP) occurs in approximately 10% of cases, with motor signs and without sensory signs/symptoms at diagnosis 4, 5
• Sensory ataxic variant accounts for about 12% of cases, with predominant sensory ataxia 4
• Some patients present with a mononeuritis multiplex pattern (9%) or paraparetic pattern (4%) 4
• About 16% of patients present with relapsing acute Guillain-Barré syndrome-like episodes 4

Sensory Manifestations

• Pain is reported in approximately 42% of patients, which is more frequent than previously recognized 4
• Patients may describe paresthesias (abnormal sensations) or sensory loss 2
• Sensory symptoms typically begin distally and can progress proximally 1
• Vibration sense and proprioception may be particularly affected in some variants 5

Motor Manifestations

• Symmetric proximal and distal weakness is the classic presentation 2
• At the most severe disease stage, 94% of patients with motor CIDP have both upper and lower limb weakness 5
• Distal and proximal weakness in all 4 limbs occurs in 56% of motor CIDP patients 5
• Progressive weakness typically begins in the legs and may spread to arms and cranial muscles 1

Disease Course

• CIDP can follow a progressive, relapsing-remitting, or monophasic course 2
• The majority of motor CIDP patients show a progressive clinical course 5
• Patients with pure motor CIDP tend to have poorer outcomes compared to those with motor-predominant CIDP 5

Associated Findings

• Approximately 47% of motor CIDP patients have an associated inflammatory or infectious disease or neoplasia 5
• Conduction blocks (82%) and F-wave abnormalities (88%) are the most frequent electrophysiological findings in motor CIDP 5
• Cerebrospinal fluid analysis typically shows elevated protein with normal cell count (albuminocytologic dissociation) 6

Diagnostic Challenges

• Diagnosis is often difficult due to the absence of specific lab findings and definitive criteria 6
• Electromyography (EMG)/nerve conduction studies and cerebrospinal fluid (CSF) analysis are key diagnostic tools 6
• Conduction block is the most common EMG abnormality, detected in at least one nerve in 73% of patients 4
• Only 31% of patients have a pure demyelinating neuropathy; the majority have some degree of axonal change 4

Pitfalls and Caveats

• CIDP can be mistaken for other neuropathies, leading to delays in correct diagnosis and treatment 2
• Several conditions can masquerade as neuropathy, including entrapments, fasciitis, and claudication 7
• Autoimmune nodopathies are no longer considered CIDP variants according to updated guidelines, as patients with these disorders do not meet hallmark CIDP criteria 2
• The diagnosis should be reconsidered if there is no response to immunomodulatory treatment 2

Understanding the varied clinical presentations of CIDP is crucial for early diagnosis and appropriate treatment, which can significantly impact long-term outcomes and quality of life for affected patients.