Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 58

Abstract

GBS is characterized by ascending motor paresis, hypo/areflexia, minimal objective sensory symptoms, electrophysiological evidence of demyelination, CSF albuminocytologic dissociation. Several related syndromes are regarded as variants being the pathogenesis considered an autoimmune attack on peripheral nerve antigens. A 42‐year‐old veterinary was admitted because of severe myalgias following one‐day high fever. Twelve hours later, he experienced dysphonia, dysphagia, and difficulty in chewing. Clinical examination showed: normal eye movements, bilateral facial weakness, tongue paralysis, limb and trunk sensory ataxia. Muscle strength was minimally affected and deep jerks were weakened. Sensory testing revealed distal loss for pin‐prick, vibration, and position sense in the four extremities. Normal laboratory included emato‐urinary tests, CK, rheumatology and endocrine profiles, microbiological and viral screenings. Search for enteric Campylobacter jejuni was negative. CSF protein was 160 mg/dl (normal < 45). In serum, antiGQ1b IgG were elevated. On admission, electrophysiology was normal. Twenty days later, in motor nerves conduction velocity was normal whereas evidence of demyelination was limited to prolonged F‐waves and terminal latencies. IVIG (0.4 g/kg/daily for five days) produced transient improvement. Because of relapse of sensory symptoms, 10 days later a course of PE was performed with benefit. Patient was followed clinically during six months showing almost complete recovery. In conclusion, we report an acute neuropathy with benign course, high protein in CSF, predominant involvement of sensory fibers, electrodiagnostic evidence of demyelination. The nosologic position of this case seems to fit the criteria of GBS sensory variant. The detection in serum of antiGQ1b IgG proposes similarities with FS and with ataxic GBS suggesting a continuity and a possible common autoimmune mechanism attacking either Schwann cells or motor axons.