Neuroradiological and eye movement correlates in children with intermittent saccade failure: "ocular motor apraxia"

Ocular motor apraxia (OMA) is a clinical sign involving the intermittent inability to initiate saccades, and a failure of quick phases during optokinetic nystagmus (OKN) and vestibular nystagmus (VN). Some patients have no other associated abnormalities (idiopathic), whereas others have a variety of neurological conditions. We quantified the severity of the saccade failure and correlated it with neuro-radiological and other oculomotor findings in 62 children (aged 17 days - 14 years). Saccades, smooth pursuit, OKN and VN were recorded using electrooculography and the extent of "locking up" (absent quick phases during OKN and VN) was measured. Saccades were usually hypometric. Pursuit and OKN gains were normal in the majority of the idiopathic cases but were low in those with other neurological conditions. Twenty-four patients had essentially normal scans, whereas 38 had abnormal scans: Delayed myelination, cerebellar abnormalities (particularly involving the vermis), and agenesis of the corpus callosum were the most common findings. A significant positive correlation was present between increasing neuro-radiological deficits and severity of "locking up" during OKN. Principal component analysis showed that brainstem and cerebellar vermis abnormalities were the main factors involved. A pathophysiological basis of OMA is discussed in the light of animal and clinical studies.