CIDP: DESCRIPTION OF FOUR PARTICULAR CASES

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Chronic inflammatory demyelinating polineuropathy (CIDP) is an acquired polineuropathy, clinically heterogeneous, and is associated with demyelination of spinal roots and peripheral nerves with a monophasic, progressive or relapsing course.We describe four patients affected by CIDP with different clinical pictures.The first patient, a 54‐year‐old man, complained of a 10 year paresthesia, with a slight wasting and weakening of the right leg, where he showed the absence of deep tendon reflexes and a reduction of the vibration sense.The second patient, a 21‐year‐old woman, had distal paresthesia and mild weakness restricted to the left hand. Deep tendon reflexes were reduced and vibration sense was normal.The third patient, a 41‐year‐old man, with a 5 year history of muscle cramps more frequent in the upper limbs, showed mild weakness of the right forearm, absence of distal deep tendon reflexes, and a reduction of vibration sense in the lower limbs.The last patient, a 20‐year‐old man, showed a marked increase of CPK level, a severe and progressive proximal and distal hypotrophy in the upper and lower limbs, and respiratory distress. Deep tendon reflexes were absent.Nerve conduction studies showed a reduction of sensory and motor velocity, which was not homogeneous among the patients and among the explored nerves of each patients. Proximal motor conduction blocks were present only in three cases. CSF parameters were normal and antibodies anti nerve were absent in all patients.The first three patients were treated with IVIg with a clear clinical and electrophysiological improvement, the last patient showed a moderate improvement, only after cyclosporin A treatment.These four cases confirm that CIDP is clinically heterogeneous with a variable spectrum of symptoms and severity, from a minimal clinical evidence to a severe clinical picture as in our fourth patient.