Case history

Case history

A 55-year-old man presents with a 4-month history of numbness in both feet and hands. Soon after this numbness, he developed symmetrical leg and arm weakness. The weakness progressed such that on the day of admission he could not lift himself up from the commode or raise his arms over his head. He notes mild shortness of breath, but no bowel or bladder incontinence, dysarthria, dysphagia, or diplopia. General examination is normal. Neurological examination shows normal mental status and cranial nerves. He has symmetrical 2-3/5 (Medical Research Council [MRC] scale) strength proximally and 3-4/5 strength distally in his arms. His legs have 3-4/5 strength proximally and 4-5/5 strength distally. Sensation is moderately decreased in a stocking-glove distribution to pin, touch, and vibration, with mild proprioceptive loss in his toes. Deep tendon reflexes are absent and Babinski's reflex is negative. Co-ordination is intact except as related to weakness. His gait is hesitant with mild lordosis but otherwise normal. He sways mildly in the Romberg position.

Other presentations

While the most common presentation of typical CIDP is of chronic progressive symmetrical weakness, patients may present with a relapsing and remitting or stepwise course, which are more common with earlier age of onset.[3]​​[4]​​[5][6]​​ Subacute presentation over 4-8 weeks may make distinction between CIDP and Guillain-Barre syndrome difficult.[3][5][7]​​​​ A subacute monophasic presentation is more common in children, adolescents, and young adults, who often respond well to immunomodulating therapy.[8][9]

There are several CIDP variants. Pure sensory presentation may occur, with distal sensory loss and pain or with a sensory ataxia, but no muscle weakness.​​ In sensory-predominant CIDP, motor demyelination is found on electrodiagnostic studies and weakness may develop as the course becomes more chronic.[1]

Patients with pure motor CIDP have relatively symmetrical proximal and distal weakness, but normal sensation clinically and electrodiagnostically. Care must be taken to differentiate from multifocal motor neuropathy, in which weakness is asymmetrical and mainly affects the upper limbs. Corticosteroids may cause deterioration in patients with motor CIDP.​​[1]

Distal CIDP (also called distal acquired demyelinating symmetrical neuropathy) must be distinguished from the neuropathy associated with myelin-associated glycoprotein.[1][10][11]​​​ Besides the lack of proximal weakness, this presentation differs from classic CIDP in that there is often a monoclonal protein, cerebrospinal fluid protein is usually not elevated, the course is rarely relapsing/remitting, and there is often a poor response to immunosuppressive medication (when a monoclonal protein is present).[10]​​

CIDP that presents with multifocal asymmetrical weakness and numbness that begins distally, often in one limb, and spreads proximally to involve several limbs is termed multifocal CIDP (also known as multifocal demyelinating neuropathy with persistent conduction block, Lewis-Sumner syndrome; multifocal acquired demyelinating sensory and motor neuropathy [MADSAM]; multifocal inflammatory demyelinating neuropathy).[1][12][13]

Rare cases may present with asymmetrical weakness and sensory loss only of the arms, known as focal CIDP or focal upper limb demyelinating neuropathy.[1][14]​​

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