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[Diffuse digestive ganglioneuromatosis].

R Chabloz, G Chapuis, P Saraga

Schweizerische Medizinische Wochenschrift

|April 28, 1984

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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.

Related Experiment Videos

Explores potential ectopic calcitonin secretion by ganglioneuromatosis.