A Case Report of Acute Motor Axonal Neuropathy in a Girl Presenting with Bilateral Claw Hand

A 10-year-old girl with normal development presented with difficulty in extending her little and ring fingers of both hands for five days. By the third day, she also felt calf pain and struggled to get up from the floor by the fourth day, though her condition did not worsen further. She remained ambulatory and showed no signs of weakness in her trunk, neck, bulbar, or respiratory muscles. There were no indications of altered consciousness, seizures, unusual movements, sensory issues, or bowel/bladder problems.

During the examination, her vital signs were stable, but a neurological assessment showed bilateral clawing of her fourth and fifth fingers and weak hand grip. Muscle strength was rated 4/5 in her finger flexors and extensors, while strength in other joints was normal. Moreover, her deep tendon reflexes were not present, while plantar reflex was flexor on both sides. 

Nerve conduction studies indicated acute motor axonal polyneuropathy (AMAN). While the antinuclear antibody (ANA) test was negative, serum tests showed positive anti-GM1 antibodies. An MRI of the cervical spine was normal. The girl was treated with intravenous immunoglobulin (2 g/kg) and showed gradual improvements. At a two-week follow-up, there were no signs of proximal girdle weakness, and remarkable improvement was observed in her clawing, with full strength (5/5) in both finger extensor and flexor muscles.

This case highlights AMAN as a subtype of Guillain-Barré syndrome (GBS), an acute immune-mediated condition affecting peripheral nerves, which is more commonly seen in tropical regions. Isolated or predominantly bilateral claw hand in pediatric GBS is uncommon. This patient emphasizes the importance of clinical assessments in diagnosing the AMAN subtype of GBS, particularly in areas with limited access to advanced testing. 

Source: Krishnenthu, K.B., Pavithran, K., Mohammed, M.T.P. et al. Bilateral Claw Hand in Pediatric Guillain Barré Syndrome. Indian J Pediatr. 2024. https://doi.org/10.1007/s12098-024-05385-7