Bassen-Kornzweig syndrome Health Article

Expectations (prognosis)

The outcome is related to the degree and progression of neurological and visual problems. Severe forms of the disease lead to irreversible neurologic disease before age 30.

Complications

• Blindness
• Mental deterioration
• Loss of function of peripheral nerves, ataxia

Calling your health care provider

Call if your infant or child exhibits symptoms of this disease.

Prevention

High doses of fat soluble vitamins may be able to slow progression of some problems such as degeneration of the retina and decreased vision.

References

Rodriguez-Oquendo A, Kwiterovich Jr PO. Dyslipidemias. In: Fernandes J, Saudubray J-M, van den Berghe G, Walter JH, eds. Inborn metabolic diseases: diagnosis and treatment. Germany: Springer; 2006:400-401.