Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 82

Abstract

A 69‐year‐old right‐handed man was recently referred to our institution with a two‐month history of diplopia and ptosis in his left eye: the ocular symptoms typically fluctuates during the day, being least severe in the morning and worsening as the day progress or while reading or driving. Neostigmine injection produced a clear improvement of weakness. The serum concentration of anti‐AChR antibody was elevated > 8.4 nmol/L (NV < 0.4); a chest CT scan was unremarkable with no evidence of mediastinal mass. The clinical picture and laboratory tests support the diagnosis of ocular myasthenia. His past medical history was insignificant until the age of 59. At that time, he complained with a diffuse, slightly asymmetrical muscle weakness both in the upper and lower limbs and sensory symptoms of glove and stocking distribution (numbness). A progressive course over months was seen with hand and anterior leg muscles hypertrophy becoming evident. Strength was decreased distally greater than proximally in the upper and lower extremities. Deep tendon reflexes were absent. The cerebrospinal fluid demonstrated a protein level of 72 mg/dl (NV < 45 mg/dl). Nerve conduction study showed a multifocal sensory‐motor polyneuropathy with both conduction block/temporal dispersion of the C‐MAP and slowing of the velocity. A diagnosis of chronic inflammatory polyneuropathy (CIDP) was considered and the patient was treated with steroids, plasmapheresis and azathiaprine: after a slight improvement, the condition has been stabilized. Both MG and CIDP have been individually associated with disorders of presumed autoimmune pathogenesis. Their concurrence is rarely found (less than 10 cases to the best of our knowledge): although the existence of this syndrome can be strongly supported by the clinical, electrophysiological and laboratory data, a common basic abnormality of immune regulation is still unknown.