A profile of childhood neuropathies at a university hospital in Oman

Area of Science:

• Pediatric Neurology
• Neuroscience
• Clinical Medicine

Background:

• Childhood neuropathies present with diverse symptoms including acute flaccid paralysis and chronic weakness.
• Early diagnosis and management are crucial for improving outcomes in pediatric neurological disorders.

Purpose of the Study:

• To comprehensively analyze childhood neuropathies (under 14 years) focusing on their profile, clinical presentation, and management strategies.
• To identify the most prevalent types of neuropathies in children and assess the proportion of treatable and preventable cases.

Main Methods:

• A mixed-methods approach combining prospective evaluation of acute flaccid paralysis cases (1992-2000) and retrospective chart reviews for other neuropathies.
• Inclusion criteria encompassed children with acute flaccid paralysis, longstanding weakness, neuroregression, or neuropathy symptoms secondary to anti-cancer drugs.
• Data were collected from hospital medical records, pediatric neurology outpatient clinics, and the neurophysiology laboratory.

Main Results:

• Eighty-two children were diagnosed with peripheral neuropathy, with a near-equal gender distribution (39 males, 43 females).
• Acute Guillain-Barre syndrome was the most frequent diagnosis (45.1%), followed by various genetic neuropathies including hereditary motor and sensory neuropathy (20.7%).
• Other identified neuropathies included chronic inflammatory demyelinating neuropathy (6.1%) and vincristine-induced neuropathy (3.5%).

Conclusions:

• Acute Guillain-Barre syndrome is the predominant acquired neuropathy in the studied pediatric population.
• A significant proportion of childhood neuropathies are treatable (54.7%), highlighting the importance of timely intervention.
• Preventable genetic neuropathies constitute a substantial portion (45.3%), emphasizing the need for genetic counseling and early detection strategies.