Optic nerve atrophy in adrenoleukodystrophy detectable by optic coherence tomography
| Autor: | Helen V. Danesh-Meyer, Taras Papchenko, Brian T. Grainger |
|---|---|
| Rok vydání: | 2009 |
| Předmět: |
Male
Retinal Ganglion Cells endocrine system Pathology medicine.medical_specialty genetic structures Nerve fiber layer Blindness Nerve Fibers Myelinated Atrophy Predictive Value of Tests Physiology (medical) Optic Nerve Diseases medicine Humans Vision test Adrenoleukodystrophy Retina Optic disc pallor business.industry Vision Tests Optic Nerve General Medicine Anatomy Middle Aged medicine.disease Magnetic Resonance Imaging eye diseases Ganglion medicine.anatomical_structure Neurology Optic nerve Disease Progression Surgery sense organs Neurology (clinical) business Tomography Optical Coherence Demyelinating Diseases |
| Zdroj: | Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia. 17(1) |
| ISSN: | 1532-2653 |
| Popis: | The adrenoleukodystrophies (ALDs) are a group of metabolic disorders characterised by the accumulation of very long-chain fatty acids in all tissues. The two most frequent ALD phenotypes are adult-onset adrenomyeloneuropathy (AMN) and childhood cerebral ALD. Visual system involvement in the adult phenotype is well described as impairment of visual function and optic disc pallor on clinical examination accompanied by demyelination of the optic nerves seen on MRI. Thinning of the retinal nerve fiber layer and ganglion cell death has been described in a neonatal form of ALD. Our patient provides evidence, through ocular coherence tomography scanning of the retina, that such degenerative changes also underlie the visual dysfunction seen in the AMN phenotype. |
| Databáze: | OpenAIRE |
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