Toward a deeper and more comprehensive understanding of neurological symptoms related to Allgrove syndrome: a case report.

Introduction and importance: Allgrove syndrome (AS) is a rare autosomal recessive disorder caused by Case presentation: We report a 13-year-old boy with AS on oral hydrocortisone who presented with recurrent vomiting for 1 month. Neurological examination revealed below-average intellectual function, decreased muscle bulk, mild weakness, bilateral hyperreflexia, wide-based ataxic gait, intention tremor, dysarthria, and positive finger-nose test. Cranial nerves and sensation were intact. Laboratory investigations showed elevated ACTH due to poor hydrocortisone absorption, and endoscopy confirmed achalasia. He was switched to intravenous hydrocortisone and underwent Heller myotomy with Nissen fundoplication, resulting in resolution of vomiting and normalization of ACTH, though neurological symptoms persisted. Clinical discussion: AS often presents first with alacrima, followed by achalasia, causing dysphagia and vomiting, and adrenal insufficiency developing in childhood, which can be life-threatening. Neurological manifestations may include ataxia, tremor, hyperreflexia, and cognitive impairment. Our case highlights the impact of achalasia on hydrocortisone absorption and the need for early multidisciplinary intervention. Conclusion: Early recognition, long-term follow-up, and proper care are crucial to prevent adrenal crises and manage neurological complications, improving outcomes and quality of life in affected patients.