Rare diseases · Sign or symptom
Oculomotor apraxia
HP:0000657
What it means
Ocular motor apraxia is a deficiency in voluntary, horizontal, lateral, fast eye movements (saccades) with retention of slow pursuit movements. The inability to follow objects visually is often compensated by head movements. There may be decreased smooth pursuit, and cancelation of the vestibulo-ocular reflex.
Oculomotor apraxia leads defective or absent horizontal voluntary eye movements with head thrusting to look at objects to the side as well as jerky, abnormal eye movements.
Rare diseases that can present with this41
Very common80–99%
9- Autosomal recessive cerebellar ataxia-pyramidal signs-nystagmus-oculomotor apraxia syndrome
- Duane retraction syndrome
- Isolated Joubert syndrome
- Joubert syndrome with hepatic defect
- Joubert syndrome with ocular anomaly
- Joubert syndrome with renal defect
- Ocular motor apraxia, Cogan type
- Posterior cortical atrophy
- Wieacker-Wolff syndrome
Common30–79%
16- Ataxia-intellectual disability-oculomotor apraxia-cerebellar cysts syndrome
- Ataxia-oculomotor apraxia type 4
- Ataxia-telangiectasia-like disorder
- Atypical progressive supranuclear palsy syndrome
- Autosomal recessive cerebellar ataxia due to CWF19L1 deficiency
- Autosomal recessive cerebelloparenchymal disorder type 3
- Autosomal recessive spastic paraplegia type 35
- Childhood-onset autosomal recessive slowly progressive spinocerebellar ataxia
- Early-onset spastic ataxia-myoclonic epilepsy-neuropathy syndrome
- Free sialic acid storage disease
- Joubert syndrome with Jeune asphyxiating thoracic dystrophy
- Nasu-Hakola disease
- Neurodevelopmental delay-seizures-ophthalmic anomalies-osteopenia-cerebellar atrophy syndrome
- Spinocerebellar ataxia type 29
- Spinocerebellar ataxia with axonal neuropathy type 2
- Tubulinopathy-associated dysgyria
Sometimes5–29%
13- Alacrimia-choreoathetosis-liver dysfunction syndrome
- Alternating hemiplegia of childhood
- Cerebral visual impairment
- Cortical dysgenesis with pontocerebellar hypoplasia due to TUBB3 mutation
- Distal 22q11.2 microdeletion syndrome
- Distal deletion 10q syndrome
- Early-onset autosomal dominant Alzheimer disease
- Gaucher disease
and 5 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions, and Orphanet separately records 2 diseases where this sign is specifically absent. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Defective or absent horizontal voluntary eye movements · Ocular motor apraxia
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.