[A case of chronic inflammatory demyelinating polyneuropathy with recurrent ophthalmoplegia, persistent conduction block, antibody activity against gangliosides GM1].
We report a 19-year-old female with chronic inflammatory demyelinating polyneuropathy (CIDP) with recurrent ophthalmoplegia. The patient had chronic, recurrent, asymmetrical, predominantly, distal limb weakness, and numbness of extremities with recurrent external ophthalmoplegia. Ophthalmoplegia developed in each attack of distal limb weakness, and also rapidly subsided with recovery of limb weakness. Motor nerve conduction studies revealed conduction block in more than one nerve and conduction velocities were generally normal in those segments of the nerve where conduction block was not detected. Serum anti-gangliosides GM1 IgM antibody investigated by ELISA was elevated. Thin-layer chromatography immunostaining also confirmed this result. Sural nerve biopsy showed normal findings. In spite of improvement of her signs and symptoms after prednisolone therapy, multifocal conduction block was persistent. Muscle power improved in association with decreased in anti-GM1 antibody activity. There were many reports of CIDP with cranial nerve involvements, but recurrent ophthalmoplegia in CIDP is rare. It is widely accepted that serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome. However, serum anti-GQ1b IgG antibody was not detected in this case. It is unclear whether anti-GM1 antibody may play a role to pathogenesis of ophthalmoplegia or not in this case.
Department of Neurology, Kitasato University East Hospital.
M
M
Ryo
T
T
Saito
N
N
Kunii
H
H
Hasegawa
H
H
Kowa
D000328:Adult; D001323:Autoantibodies / Q000378:metabolism*; D002908:Chronic Disease; D003711:Demyelinating Diseases / Q000150:complications*; D005260:Female; D005677:G(M1) Ganglioside / Q000276:immunology*; D006801:Humans; D009431:Neural Conduction; D009886:Ophthalmoplegia / Q000150:complications*; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000503:physiopathology; D011115:Polyneuropathies / Q000150:complications*; D012008:Recurrence
D001323:Autoantibodies; D005677:G(M1) Ganglioside