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Neuro-Opthalmic Signs in Children with Ataxia
Causes of Ataxia
- Hypoxic-Ischemia enchephalopathy
- Episodic ataxia
- Neuronal ceroid lipofucinosis
- Neuronal migration disorder
- Ischemic stroke
- Friedreich ataxia
- Mitochondrial Disease
- Severe epilepsy
- Ataxia telangiectasia
Common Associations
| Neuronal-ophthalmic Finding | Frequency |
|---|---|
| Afferent | |
| Decreased visual acuity | 9% |
| Abnormal optic discs | 9% |
| Abnormal pupils | 3% |
| Abnormal Visual Fields | 1% |
| Efferent | |
| Strabismus | 30% |
| Pathologic nysagmus | 28% |
| Gaze evoked nystagmus | 15% |
| Impaired smooth pursuit | 23% |
| Hypometric saccades | 10% |
| Hypermetric saccades | 2% |
| Impaired Ductions | 2% |
| Ataxia Disorder | Most Common Association |
|---|---|
| Hypoxic-Ischemic encephalopathy | Strabismus |
| Episotic ataxia | Nystagmus |
| Neuronal ceroid lipofuscinosis | Abnormal Optic discs |
| Joubert Syndrome | Strabismus |
| Neuronal Migration Disorder | Strabismus |
| Ischemic Stroke | Nystagmus |
| Friedreich Ataxia | Impaired Saccades |
| Mitochondrial Disease | Strabismus/Nystagmus |
| Severe epilepsy | Decreased Visual acuity |
| Ataxia Telangiectasia | Impaired smooth pursuit |
| Angelman Syndrome | Strabismus |
- Adapted from Salman et. al. Neuroophthalmology. 2015;39(3):125-131