Lupus erythematosus and Miller-Fisher syndrome.
OBJECTIVE
To compare the clinical course of an unusual case of Miller-Fisher syndrome in systemic lupus erythematosus with therapeutic interventions, in particular with plasma exchanges.


METHODS
The clinical state and laboratory and electrophysiologic parameters were controlled for over a year and related to therapeutic attempts with immunoglobulins, steroids, and plasma exchanges.


METHODS
Medical intensive care unit of a university hospital.


METHODS
A 17-year-old black female student with known systemic lupus erythematosus who developed ataxia, are flexia, and ophthalmoplegia (Miller-Fisher syndrome) and later became tetraplegic and required full mechanical ventilatory support.


RESULTS
High-dose immunoglobulin treatment combined with corticosteroid pulse therapy was not beneficial. However, plasma exchange (performed five times over a period of 4 months) was followed by a striking clinical improvement within hours after each plasma exchange.


CONCLUSIONS
Plasma exchange appears to remove a yet unknown agent producing a distal motor nerve conduction block and is efficacious in severe neuropathy associated with Miller-Fisher syndrome in lupus erythematosus.
Bingisser
R
R
Department of Internal Medicine, University Hospital, Zürich, Switzerland.
Speich
R
R
Fontana
A
A
Gmür
J
J
Vogel
B
B
Landis
T
T
D000293:Adolescent; D001259:Ataxia / Q000150:complications* / Q000628:therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000628:therapy; D008775:Methylprednisolone / Q000627:therapeutic use; D009886:Ophthalmoplegia / Q000150:complications* / Q000628:therapy; D010951:Plasma Exchange; D011782:Quadriplegia / Q000150:complications; D012021:Reflex, Abnormal; D013577:Syndrome
D016756:Immunoglobulins, Intravenous; D003520:Cyclophosphamide; D008775:Methylprednisolone