A senior citizen sufferin...

A 64-yr-old man had a 25-yr H/o Crohn’s disease involving multiple bowel resections for fistulae and obstruction. Since his operation, he has experienced frequent abdominal cramps and 4 to 8 stools daily. He was managed on a low-fat diet with medium-chain triglyceride, multivitamin, iron, calcium and magnesium oral supplements. On initial physical examination, the abnormal findings were marked cachexia, pigmentary degeneration of the retina and massive bilateral visual field scotomata preserving only 10% of central vision, as shown in the figure. 

He also had a generalized motor weakness in proximal than distal muscle groups and a broad-based gait with marked ataxia, brisk reflexes, and a bilateral Babinski response. In addition, proprioception was moderately diminished in all extremities. All Laboratory values were average except vitamin E. serum vitamin E levels were 0.03 mg/dl. Electroretinography revealed a marked decrease of the A and B wave amplitudes under both scotopic and photopic conditions and an increased response latency. 

His vitamin E status was corrected with 3200 mg of intramuscular tocopherol acetate. As a result, the patient’s vitamin E status eventually normalized, and during this same period, he noted a gradual improvement in his vision and gait. In addition, a neurological reassessment showed objective improvement of both his visual fields.