Lambert-Eaton myasthenic syndrome with ophthalmoparesis and pseudoblepharospasm

Muscle Nerve. 1999 Dec;22(12):1727-30. doi: 10.1002/(sici)1097-4598(199912)22:12<1727::aid-mus19>3.0.co;2-k.

Abstract

We report a patient initially diagnosed as having ocular myasthenia gravis who showed progressive ophthalmoparesis and pseudoblepharospasm together with positive acetylcholine receptor antibodies. Repeated evaluation with high-frequency repetitive stimulation revealed an incremental response and elevated titers of antibodies against presynaptic calcium channels, confirming Lambert-Eaton myasthenic syndrome. Systemic evaluation revealed no malignant neoplasm but revealed euthyroid Hashimoto's disease. Immunomodulative therapy including plasma exchange and administration of an immunosuppressent (azathioprine) combined with a potassium-channel blocker (3,4-diaminopyridine) reduced the ocular abnormalities. We conclude that the ocular manifestations in this patient were probably caused by Lambert-Eaton myasthenic syndrome.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / blood
  • Blepharospasm / etiology*
  • Diagnosis, Differential
  • Electrodiagnosis
  • Humans
  • Lambert-Eaton Myasthenic Syndrome / complications
  • Lambert-Eaton Myasthenic Syndrome / diagnosis*
  • Male
  • Middle Aged
  • Muscle Weakness / complications
  • Myasthenia Gravis / diagnosis
  • Ophthalmoplegia / etiology*
  • Plasma Exchange
  • Receptors, Cholinergic / immunology
  • Receptors, Cholinergic / metabolism
  • Thyroiditis, Autoimmune / complications
  • Ulnar Nerve / physiopathology

Substances

  • Autoantibodies
  • Receptors, Cholinergic