Polymyositis with cranial neuropathy has been described rarely. This paper reports 3 cases of polymyositis with trigeminal or facial neuropathy. Patients presented with muscular weakness, myalgia, rhabdomyolysis, endomysial infiltration with necrosis and regeneration on biopsy, and in 2 cases a myopathic EMG pattern. Two patients additionally had Sjögren’s syndrome with antinuclear antibodies. Anti-JO1 antibodies were found in 1 patient.

One patient did well with corticosteroid therapy alone. Another improved with the addition of immunoglobulins. The third developed macrocheilia, facial diplegia, antibodies against voltage-gated potassium channels and a neuromyotonic paraneoplastic syndrome. He died after a year despite treatment with corticosteroids and immunoglobulins. The patients all fulfilled diagnostic criteria for polymyositis clinically and in terms of EMG, biochemical and pathological features. In the two with Sjögren’s syndrome the question whether the polymyositis with cranial neuropathy was primary or secondary remains unresolved.

A cranial neuropathy in polymyositis indicates a screen for cancer and connective tissue disease.

22 references.