Triple-A or Allgrove syndrome is a rare multisystem disease classically 
associated with esophageal achalasia, adrenal insufficiency and alacrima. Here, 
we describe the poorly understood neurological characteristics often associated 
with this condition, through the clinical and electrophysiological analysis of 
eight patients. All patients were genetically confirmed and had a mutation in 
the ALADIN gene. They all displayed a classical picture of Triple-A syndrome: 
all suffered from achalasia and alacrima and half of them from adrenal 
insufficiency. However, all harbored a neurological picture characterized by a 
recognizable pattern of peripheral neuropathy. Other neurological features 
included cognitive deficits, pyramidal syndrome, cerebellar dysfunction, 
dysautonomia, neuro-ophthalmological signs and bulbar and facial symptoms. This 
neurological picture was prominent in all patients and misled the initial 
diagnosis in six of them, which had a late onset. We then review the previous 
neurological reports of this disease, to improve the understanding of this rare 
condition. Diagnosis of late-onset Triple-A syndrome is difficult when the 
clinical picture is mainly neurological and when endocrine or gastrointestinal 
signs are minor. The characteristics of the peripheral neuropathy, among other 
neurological signs, can be of help.