[A case of Miller Fisher syndrome with gadolinium-enhancing lesions in the cranial nerves and the cauda equina on magnetic resonance imaging].
MRI findings for a patient with Miller Fisher syndrome (MFS) are reported. A 25-year-old woman presented with blephaloptosis, double vision, and gait disturbance after antecedent upper respiratory infection. Neurologic examination on admission revealed bilateral blephaloptosis and restriction of extraocular movement. She was unable to maintain an upright posture, nor able to walk because of severe truncal ataxia. Mild limb ataxia was also seen. The tendon reflexes were generally absent and she had hypesthesia in her shoulder and upper limbs. On the next day, her eyes became fixed in the central position. Based on these characteristic symptoms, she was diagnosed as having MFS and underwent plasmapheresis therapy six times. Although ataxia gradually reduced, ophthalmoplegia did not improve and severe symmetric facial nerve palsy appeared. Antiganglioside IgG antibodies (anti-GQ1b and GD1b) were detected in the sera on admission. Spinal fluid examination on day 18 revealed 5 cells/microliter and protein 171 mg/dl. T1-weighted images with Gd-DTPA on day 15 demonstrated enhancement of the posterior nerve roots of the cauda equina. MRI on day 32 revealed swelling and enhancement of the bilateral oculomotor nerves, as well as the facial nerves and the abducens nerves. Marked improvement of ophthalmoplegia followed high dose intravenous immunoglobulin therapy. Gd-enhanced MRI after recovery showed no enhancement of the cauda equina, nor the cranial nerves. These findings well correlated with her clinical features. Although abnormal Gd-enhancement of the cranial nerves or cauda equina in several neurological disorders has been documented, similar findings have been rarely reported in MFS. In our patient, IgG anti-GQ1b and GD1b antibodies were detected and the distribution of Gd-enhanced lesions was compatible with the distribution of the involved gangliosides. We suggest that contrast enhanced MRI is useful for identifying affected cranial nerves and cauda equina nerve roots. In addition, MRI may play a significant role in disclosing the pathophysiology of this disease.
Hattori
M
M
Department of Neurology, Tosei General Hospital.
Takada
K
K
Yamada
K
K
Kamimoto
K
K
Mitake
S
S
D000328:Adult; D001323:Autoantibodies / Q000097:blood; D002420:Cauda Equina / Q000473:pathology*; D003391:Cranial Nerves / Q000473:pathology*; D005260:Female; D019786:Gadolinium DTPA; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D019846:Miller Fisher Syndrome / Q000175:diagnosis* / Q000628:therapy; D016896:Treatment Outcome
D001323:Autoantibodies; D005732:Gangliosides; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D019786:Gadolinium DTPA