Ocular manifestations of neurologic disease in early childhood
AffiliationOphthalmology (Eye & Ear Hospital)
Access StatusOpen Access
© 2017 Dr. Francis Billson
Neuro-ophthalmology is a medical discipline focusing on diseases of the central nervous system that may affect the visual sensory or motor systems or pupillary reflexes. Neuro-ophthalmologists are often part of the academic medical team and the eyes may be involved in a variety of medical disciplines. Paediatric ophthalmology adds precision to diagnostic challenges arising from the complexity of foetal and early development of the visual system. The first chapter of this thesis deals with normal neuronal, vascular and immunological development of the retina. The second chapter deals with a selection of visuo-motor disturbances in the neonate and early childhood, including the difficulties faced by the paediatric neuro-ophthalmologist in diagnosis in the context of children, particularly in the early years of life where development adds a profound challenge to the interpretation of conditions and determining whether conditions are progressive or static: • Chapter 2.1 deals with the objective measurement of visual fields by directly recording from the visual (occipital) cortex. • Chapter 2.2 deals with confusion and possible mismanagement of visual path glioma and the importance of objective perimetry in clarifying diagnosis and determining management, particularly in early childhood. • Chapter 2.3 deals with the further problem of regional giantism associated with neurofibromatosis and presenting in early childhood with buphthalmos. • Chapter 2.4 discusses the publication of an early series of cases of optic nerve hypoplasia at a time when there were relatively few reported in the literature, however the condition is now recognised as the commonest cause of blindness at birth. • Chapter 2.5 discusses optic neuropathy and the importance of distinguishing ‘functional’ loss of vision from pathologic conditions such as drug toxicity. • Chapter 2.6 discusses two unusual cases of pathological papilledema in childhood, treated with sheath splitting surgery in its early developmental phase. • Chapter 2.7 deals with a series of cases of Aicardi’s syndrome, the first to appear in the English literature. • Chapter 2.8 deals with strabismus and amblyopia and demonstrates the vulnerability of the developing visual system to abnormal visual environmental influences and motor disturbances. • Chapter 2.9 deals with congenital nystagmus and its differential diagnosis. • Chapter 2.10 discusses the developmental causes of supra-nuclear disturbances of gaze, their differential diagnosis and the importance of excluding tumours of the visual path in early childhood. The final chapter highlights and summarises the major contributions to paediatric neuro-ophthalmology of the topics covered in this thesis and the importance of development adding complexity to the interpretation of diagnosis.
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