Purpose: Allgrove syndrome, also known as “triple A” syndrome, is characterized by adrenal insufficiency, achalasia, and alacrimia. When neurological signs are also present, the condition is referred to as “4 A” syndrome. Methods: We conducted a retrospective analysis of three patients with 4 A syndrome confirmed genetically. A complete neurological exam was carried out by an experimented neurologist. Results: Herein, we describe the neurological characteristics often associated with this condition, through the clinical and electrophysiological analysis of three patients. All patients exhibited a mutation in AAAS, the gene coding for ALADIN. While these individuals presented with the classic features of triple-A syndrome, neurological symptoms were not prominent. Conclusion The neurological manifestations of Allgrove syndrome have historically been overlooked and inadequately explored. Due to the condition’s rarity and substantial phenotypic heterogeneity, only recently have a variety of symptoms been recognized and described.

Methods: A retrospective study was conducted using the data of all patients hospitalized for TS and BS in the infectious diseases department between 1991 and 2018. Results: Among a total of 117 patients, 73 had TS (62.4%) and 44 had BS (37.6%). Females were significantly more affected with TS than males (56.2% vs. 22.7%, p<0.001). Fever (72.7% vs. 45.2%, p=0.004) and sweating (72.7% vs. 47.9%, p=0.009) were significantly more frequent among patients with BS. The median erythrocyte sedimentation rate was significantly higher in the TS group (median, 70 mm/hr; interquartile range [IQR], 45–103 mm/hr) than in the BS group (median, 50 mm/hr; IQR, 16–75 mm/hr) (p=0.003). Thoracic involvement was significantly more frequent in the TS group (53.4% vs. 34.1%, p=0.04), whereas lumbar involvement was significantly more frequent in the BS group (72.7% vs. 49.3%, p=0.01). Initial imaging findings revealed significantly higher frequencies of posterior vertebral arch involvement, vertebral compaction, and spinal cord compression in the TS group. Percutaneous abscess drainage (20.5% vs. 2.3%, p=0.005) and surgical treatment (17.8% vs. 2.3%, p=0.01) were more frequently indicated in the TS group, with a significant difference. Conclusions A combination of clinical, laboratory, and radiological features can be used to distinguish between TS and BS while these patients await diagnosis confirmation.