[Allgrove syndrome. Report on a family].

[triple a syndrome]

Triple A or Allgrove syndrome is a rare autosomal recessive disease with alacrima , achalasia , and ACTH-resistant adrenal insufficiency . It is usually associated with neurological disorders . Recently , mutations in the AAAS , a candidate gene mapped to chromosome 12 q 13 , were identified . We report a family with seven affected siblings . All of them have signs of alacrima , four were operated on for achalasia , five have neurological abnormalities including cranial nerve abnormalities , amyotrophic lateral sclerosis , pyramidal syndrome , distal motor neuropathy , and amyotrophy , and two have adrenal insufficiency . Triple A syndrome should be considered in any young patient with alacrima .

Diseases
Validation

Diseases presenting "amyotrophic lateral sclerosis" symptom

adrenomyeloneuropathy

alexander disease

cadasil

dystrophic epidermolysis bullosa

inclusion body myositis

liposarcoma

locked-in syndrome

neuralgic amyotrophy

pyruvate dehydrogenase deficiency

sneddon syndrome

triple a syndrome

x-linked adrenoleukodystrophy

This symptom has already been validated