Paroxysmal type neuroocular syndrome-2 (NOC2) is characterized by daily paroxysmal spells characterized by eye deviation or nystagmus with abnormal head posturing apparent from birth or early infancy. The episodes tend to be triggered after sleeping, and most patients show improvement of the ocular symptoms over time. Affected individuals also have hypotonia, mild developmental delay, dysarthria, and gait ataxia; most have mildly impaired intellectual development. Seizures are not observed.
For a discussion of genetic heterogeneity of neuroocular syndrome, see NOC1 (619539). [from
OMIM]