RESUMO
Introduction: The 3A syndrome or Allgrove syndrome is an autosomic recessive disease characterized by the association of oesophagus achalasia, alacrymie and suprarenal insufficiency. Neurological disorders can appear at any time of the course's disease and would be due to a neuropathy affecting the central nervous system as well as the peripheral nervous system
Patient and methods: From1991 to 2005, we noticed 22 cases of Allgrove syndrome. The follow-up of these patients enabled us to notify the neurological abnormalities appear later in the evolution
Results: neurological symptoms appear at any time of the course's disease. All cases were backward pupil. 8 patients had a nasal voice due to palate paralysis. 7 patients had xerostomia wich is included in the dry syndrome due to the degeneration of salivary glands. One patient had a neurological motor disorder and 3 patients had a sensitive disorders with loss of cold feeling and vasomotor disorders in the hands and feet. These symptoms appear after 4 years on average after diagnosis. An orthostatics hypotension was noticed among three patients as a dysautonomic sign
Conclusion: All of these data approve that neurological disorders appear at a variable time of the course's disease. This polyneuropathy affects the oesophagus and always leads to the recurrence of the digestives signs