Annals of Indian Academy of Neurology
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Year : 2013  |  Volume : 16  |  Issue : 2  |  Page : 266-268

A case of late-onset allgrove syndrome presenting with predominant autonomic dysfunction

1 Department of Endocrinology, KPC Medical College and Hospital, 1F, Raja Subodh Chandra Mullick Road, Jadavpur, Kolkata, West Bengal, India
2 Department of General Medicine, Peerless Hospital, 360, Panchasayar, Kolkata, West Bengal, India

Correspondence Address:
Shakya Bhattacharjee
Department of General Medicine, Peerless Hospital, 360, Panchasayar, Kolkata - 700 094, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-2327.112494

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Allgrove Syndrome or triple A syndrome is a rare familial multisystem disorder characterized by achalasia, alacrima and adrenal insufficiency. The objective was to describe a case of 4A syndrome where autonomic dysfunction was the presenting feature. A 22-year-old male presented with erectile dysfunction and loss of spontaneous morning erections for six months. He was having nocturnal diarrhea and recurrent postural dizziness for three months. He was found to have hyperpigmentation at pressure points, postural hypotension and other features of autonomic dysfunction. Laboratory investigations and imaging studies revealed hypoadrenalism, achalasia, alacrima and peripheral neuropathy. Autonomic neuropathy-related features persisted even after correction of hypoadrenalism. Based on clinical features and investigation he was diagnosed as a case of 4A syndrome presenting with autonomic dysfunction. Allgrove or 4A syndrome should be considered as a rare differential diagnosis of someone presenting with features of autonomic neuropathy.

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