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[Diffuse digestive ganglioneuromatosis].
Schweizerische Medizinische Wochenschrift
|April 28, 1984
Summary
Intestinal ganglioneuromatosis, a rare condition linked to neural crest dysfunction, can cause severe motility disorders. This pathology is associated with Multiple Endocrine Neoplasia type IIb (MEN IIb) or neurofibromatosis.
Area of Science:
- Gastroenterology and Genetics
- Neurobiology and Endocrinology
Background:
- Intestinal ganglioneuromatosis is a rare pathology stemming from neural crest dysfunction.
- It is often associated with Multiple Endocrine Neoplasia type IIb (MEN IIb) or von Recklinghausen's neurofibromatosis.
Observation:
- Presents two rare cases of intestinal ganglioneuromatosis.
- Highlights the diffuse proliferation of the peripheral autonomous nervous system.
Findings:
- Intestinal ganglioneuromatosis can lead to severe, potentially fatal, intestinal motility disorders.
- Excessive nerve growth factor production may play an etiologic role.
Implications:
- Discusses the physiopathology, including increased vasoactive intestinal polypeptide.
- Explores potential ectopic calcitonin secretion by ganglioneuromatosis.