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Neurological features and enzyme therapy in patients with endocrine and exocrine pancreas dysfunction due to CEL

Mette Vesterhus¹, Helge Raeder, Harald Aurlien

¹Department of Clinical Medicine, University of Bergen, Bergen, Norway.

|June 12, 2008

Abstract

Objective:

To further define clinical features associated with the syndrome of diabetes and pancreatic exocrine dysfunction due to mutations in the carboxyl-ester lipase (CEL) gene and to assess the effects of pancreatic enzyme substitution therapy.

Research Design And Methods:

Nine patients with CEL gene mutation, exocrine deficiency, and diabetes were treated and followed for 30 months.

Results:

Treatment improved symptoms in seven of nine patients. Exocrine and endocrine function assessed by fecal elastase and A1C were not affected, although fecal lipid excretion was reduced. Vitamin E was low in all patients but increased with treatment (P < 0.001 at 30 months) and improved in five subjects. A predominantly demyelinating neuropathy was seen in a majority of patients, and carpal tunnel syndrome was common.

Conclusions:

Pancreatic enzyme substitution alleviated symptoms and malabsorption and normalized vitamin E levels. Glycemic control was not significantly affected. The CEL syndrome seems associated with a demyelinating neuropathology.

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