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

Patient Presentations of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

CIDP typically presents with progressive symmetric proximal and distal weakness, large fiber sensory loss, and areflexia, with clinical nadir reached more than 8 weeks after symptom onset, distinguishing it from acute inflammatory demyelinating neuropathies like Guillain-Barré syndrome. 1

Classic CIDP Presentation

• Progressive or relapsing course over at least 2 months, unlike Guillain-Barré syndrome which reaches maximum disability within 2-4 weeks 2
• Symmetric weakness affecting both proximal and distal muscle groups, often affecting all four limbs 1, 3
• Large fiber sensory loss with decreased or absent deep tendon reflexes 1
• Affects both proximal and distal regions, in contrast to length-dependent "stocking-and-glove" distribution seen in other neuropathies 2
• Pain is reported in approximately 42% of patients, more frequently than previously recognized 4

Sensory Manifestations

• Distal paresthesias or sensory loss that can progress proximally 5
• Sensory ataxia variant occurs in approximately 12% of patients 4
• Vibration and proprioception deficits are common, particularly in patients with monoclonal gammopathy 4
• Some patients may present with predominantly sensory symptoms before motor involvement becomes apparent 6

Motor Manifestations

• Progressive weakness typically starting in the legs and potentially spreading to arms and cranial muscles 5
• Pure motor variant occurs in approximately 10% of patients, with motor signs and without sensory signs/symptoms at diagnosis 3, 4
• Motor predominant CIDP can present with upper and lower limb weakness, with both distal and proximal involvement 3
• Weakness can significantly impact mobility and activities of daily living 6

CIDP Variants

• MADSAM (Multifocal Acquired Demyelinating Sensory And Motor) variant presents with asymmetric or multifocal sensorimotor deficits rather than the symmetric pattern seen in typical CIDP 2
• Pure motor CIDP (PM-CIDP) presents without sensory symptoms and with normal sensory nerve conduction studies 3
• Motor predominant CIDP (MPred-CIDP) presents primarily with motor symptoms but has abnormal sensory nerve action potential amplitudes 3
• Mononeuritis multiplex pattern occurs in approximately 9% of patients 4
• Paraparetic pattern (predominantly lower limb involvement) occurs in about 4% of patients 4
• Relapsing acute Guillain-Barré syndrome-like pattern occurs in approximately 16% of patients 4

Electrophysiological Features

• Conduction block is the most common electrodiagnostic abnormality, detected in at least one nerve in 73% of patients 4
• F-wave abnormalities are present in approximately 88% of patients with motor CIDP 3
• Only about 31% of patients have pure demyelinating neuropathy; the majority have some degree of axonal change 4
• Demyelinating changes on electrodiagnostic testing are a key diagnostic feature 6

Diagnostic Challenges

• CIDP can be misdiagnosed as polymyalgia rheumatica due to symmetric and proximal weakness of the legs along with elevated inflammatory markers 7
• Symptoms can mimic other conditions, requiring careful clinical assessment and electrodiagnostic testing for accurate diagnosis 6
• Lack of a specific diagnostic biomarker makes diagnosis dependent on clinical suspicion, findings, and demonstration of demyelinating changes 6

Associated Conditions

• Approximately 47% of patients with motor CIDP have an associated inflammatory or infectious disease or neoplasia 3
• Some patients may have monoclonal gammopathy of uncertain significance (MGUS) 4

Disease Course and Prognosis

• CIDP has a progressive or relapsing course 2
• Pure motor CIDP patients tend to have poorer outcomes compared to motor predominant CIDP 3
• Response to treatment varies, with approximately 66% of patients responding to one of the three main therapies (plasma exchange, IVIG, or steroids) 4
• Some patients who fail initial therapy may benefit from alternative treatments 4

Early recognition of these clinical presentations is crucial as delays in diagnosis and treatment can result in significant disability and morbidity 1.