Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and sensory neuropathy have also been described,89 together with ataxia, optic atrophy and impaired intelligence, "I indicating that the disorder has a wide spectrum of clinical manifestations. This paper describes the findings in a multicentre collaborative study carried out to ascertain the incidence of disordered neurological and automatic funciton in the syndrome. MethodsIn order to obtain information on patients with the adrenal insufficiency/alacrima/achalasia syndrome in the UK, questionnaires were sent to 39 senior members of the British Society for Paediatric Endocrinology asking for information on any cases under their care. Thirty seven questionnaires were returned, and six cases with the disorder were identified in the UK (cases 4, 5, 6, 13, 15, 17 described below).To increase the number of patients available for study, members of the European Society for Paediatric Endocrinology known to have had patients with the disorder under their care were invited to participate in the study and were sent questionnaires asking for information on the presence of adrenal insufficiency, alacrima, achalasia of the cardia, abnormal neurological findings, tests of autonomic function, evidence of intellectual impairment, skin manifestations, and family history.In some families siblings have died in early infancy, probably as a result of adrenal insufficiency. These have not been included in the study as little information is available on their neurological status.

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