Dysgammaglobulinemia in steroid-dependent optic neuritis: response to gammaglobulin treatment.
At the age of 12, a prematurely born boy with an otherwise unremarkable past medical history developed bilateral optic neuritis associated with transverse myelopathy. Over the ensuing 3 years, recurrent bouts of optic neuritis OU, with dyschromatopsia, and acuity and field loss (arcuate, central, and paracentral scotomas) were controlled with increasing doses of corticosteroids. However, the patient became steroid-dependent and experienced recurrent optic neuritis during multiple attempts at tapering the steroids. He developed optic atrophy and steroid complications, including cushingnoid features and growth maturation delay. Immunoglobulin G subclass 2 and 3 deficiencies were the only serologically detectable abnormalities. Administration of intravenous gammaglobulin (25 g monthly) allowed discontinuation of steroids without further ophthalmic or neurologic disease. Following steroid withdrawal and institution of gammaglobulin, the patient grew 6 inches within 2 years, regaining his vision, retrieving his stature, and normalizing his psychosocial development.
Frohman
L P
LP
Department of Ophthalmology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark 07103-2714.
Cook
S D
SD
Bielory
L
L
D000293:Adolescent; D004406:Dysgammaglobulinemia / Q000150:complications* / Q000188:drug therapy; D004679:Encephalomyelitis / Q000150:complications; D006130:Growth Disorders; D006801:Humans; D017099:IgG Deficiency; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D009902:Optic Neuritis / Q000188:drug therapy*; D011241:Prednisone / Q000009:adverse effects*; D014792:Visual Acuity
D016756:Immunoglobulins, Intravenous; D011241:Prednisone