Ptosis without Ophthalmoplegia in Acute Motor Axonal Neuropathy Variant of Guillain-Barré Syndrome in Children: A Case Series

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. The clinical variants include Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP), Acute Motor Sensory Axonal Neuropathy (AMSAN), Acute Motor Axonal Neuropathy (AMAN) and Miller-Fisher syndrome. Cranial nerve involvement can occur in approximately 50% of patients with GBS, but it is rarely reported in the AMAN variant. Ptosis as a presenting symptom is extremely rare in the AMAN variant. Autonomic dysfunction is also rarely reported in the AMAN variant. In the present case series, authors hereby, report five children (3 males, 2 females, aged 4-11 years) diagnosed with the AMAN variant of GBS, who presented with early onset of ptosis and rapid progression of flaccid weakness. Autonomic dysfunction, in the form of tachycardia, hypertension and excessive diaphoresis, was a prominent feature in four of the cases. The average time to reach the nadir of weakness was 4.2 days and the average duration of hospital stay was 58 days. The average GBS disability score at discharge was four. Nerve conduction studies were suggestive of the AMAN variant of GBS in all five cases. All children were treated with Intravenous Immunoglobulin (IVIg) and all required mechanical ventilation, with an average duration of 35.8 days. All five cases presented in a short period of three months, from November 2022 to February 2023.