Acquired onset of third, fourth, and sixth cranial nerve palsies in children and adolescents

Abstract

Purpose: To describe the causes of third, fourth, and sixth cranial nerve palsies in children and adolescents.

Methods: In this retrospective case series, a total of 66 patients aged 0-19 years with third, fourth, and sixth cranial nerve palsies seen in strabismus and neuro-ophthalmic practice from 2010 to 2017 were included. Causes of palsies were determined based on clinical assessment, high-resolution magnetic resonance imaging (MRI), and laboratory work-up.

Results: Thirty-five patients had sixth cranial nerve palsy, 14 patients had third cranial nerve palsy (7 partial, 7 complete), 13 patients had fourth cranial nerve palsy, and 4 patients had combined cranial nerve palsies in this study. Neoplasia involving central nervous system was one of the most common causes of third, fourth, and sixth cranial nerve palsies both in children (age: 0-14 years) and adolescents (age: 15-19 years) (20% and 31%, respectively). Overall, neoplasia (23%) was the most common cause of acute third, fourth, and sixth cranial nerve palsies, followed by idiopathic cause (14%), inflammation (11%), and non-aneurysmal vascular contact (11%). Neoplasia was also the most common cause of sixth and third cranial nerve palsies (25% and 29%, respectively). The most common cause of fourth cranial nerve palsy was late decompensation in congenital fourth cranial nerve palsy (46%).

Conclusions: A substantial proportion of pediatric and juvenile patients had serious pathologies for third, fourth, and sixth cranial nerve palsies. If nerve palsies are indicated, prompt diagnosis of etiologies using high-resolution MRI with contrast and laboratory work-up are important for this disease population.

Fig. 1

An infiltrative mass lesion causing bilateral sixth nerve palsy (asterisk). A 3-year-old girl presented with intermittent head turn to the right side for 3 months and mild esodeviation for 3 weeks. The patient’s guardian reported that the deviation had been improved continuously before presentation. The patient had esodeviation of 12 prism diopters and mild abduction limitation in both eyes. Immediate magnetic resonance imaging was performed. Although the patient was found to have large infiltrative mass involving brainstem and cerebellar parenchyma, her clinical presentation was mild bilateral sixth nerve palsy only

Fig. 2

A test sheet of a 5-year-old girl who presented with acute-onset double vision owing to acquired fourth nerve palsy. Observation of change in usual life including writing and head posture could be an important clue to provide medical attention to children with third, fourth, or sixth cranial nerve palsies. Her teacher in the kindergarten first noticed that the patient started to write sentences twice, one in the original line and one in the oblique to the original line (arrows). The patient stated that she suddenly started to see double images separated vertically and obliquely. She had acquired unilateral fourth nerve palsy. Through thorough work-up including full history taking, magnetic resonance imaging, and blood work, the patient was determined to be idiopathic. Her symptom and sign spontaneously resolved completely within 1 week