TABLE A. The frequency of afferent visual system abnormalities in disorders associated with pediatric chronic ataxia.
| Disorder | Number of patients | Decreased visual acuity | Abnormal visual fields | Abnormal pupillary exam | Abnormal optic discs |
|---|---|---|---|---|---|
| Angelman syndrome | 16 | 1 | |||
| Ataxia telangiectasia | 13 | 2 | |||
| Postnatal stroke† | 11 | 1 | 1 | ||
| Friedreich ataxia | 7 | 1 | |||
| Acetazolamide-responsive episodic ataxia | 7 | 1 | |||
| Neuronal ceroid lipofuscinosis | 6 | 4 | 1 | 3 | 5 |
| Hypoxic ischemic encephalopathy following birth | 5 | 1 | 1 | ||
| Neuronal migration disorder | 5 | 1 | |||
| Severe epilepsy syndrome | 5 | 2 | |||
| Brain trauma | 2 | ||||
| Dandy-Walker syndrome | 2 | 1 | |||
| Marinesco-Sjogren syndrome | 2 | 1 | |||
| Multiple sclerosis | 2 | 1 | 2 | ||
| Prematurity | 2 | 1 | |||
| Neuronopathic Gaucher disease | 1 | 1 | |||
| Hypomyelinating leukodystrophy of unknown etiology | 1 | 1 | 1 | ||
| Pontocerebellar hypoplasia type 3 | 1 | 1 | |||
| Wernicke’s encephalopathy | 1 | 1 | |||
| Acute disseminated encephalomyelitis | 1 |
†Eight patients had postnatal ischemic stroke and three had postnatal hemorrhagic stroke. No neuro-ophthalmological signs were reported in the single patient with the prenatal ischemic stroke.