Addison Pernicious Anemia Syndrome

Addison Pernicious Anemia Syndrome (Pernicious Anemia Syndrome;
Vitamin B12 Deficiency Anemia; Macrocytic Anemia; Biermer Syndrome) 28

General: Autosomal dominant; female preponderance; onset between ages 30 and 50 years; lack of intrinsic factor normally produced in the fundus of stomach and important for absorption of vitamin Bl2 in the intestinal tract; infrequent ocular involvement.

Ocular: Central scotoma, centrocecal scotomata, and field contractions in a few cases; retinal hemorrhages (round with white center) at the posterior pole; both retina and disk may have a whitish, hazy appearance; optic neuritis (ischemic); optic atrophy; palsies of extraocular muscles; ocular hypotony; cataract; bilateral, slowly progressive optic neuropathy, unclear etiology.

Clinical: Megaloblastic anemia (chronic and progressive); hypochlorhydria; glossitis; stomatitis; constipation or diarrhea; paresthesias and numbness; incoordination; ataxia; sphincter malfunction.

Biermer A. Form von Progressiver Pernizioser Anamie. Korresp Bl Schweiz Arzt 1872; 2:15.

Fisher JM, Taylor KB. A comparison of autoimmune phenomena in pernicious anemia and chronic atrophic gastritis. N Engl J Med 1965; 272:499.

Geeraets WJ. Ocular syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Magalini SI, Scrascia E. Dictionary of medical syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

Miller NR, ed. Walsh and Hoyt's clinical neuro-ophthalmology, vol. 1, 4th ed. Baltimore: Williams & Wilkins, 1982.