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Paroxysmal involuntary eye movements

MedGen UID:
Concept ID:
Synonym: Abnormal eye movements, paroxysmal
HPO: HP:0007704


Sudden-onset episode of abnormal, involuntary eye movements. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVParoxysmal involuntary eye movements

Conditions with this feature

Encephalopathy due to GLUT1 deficiency
MedGen UID:
Concept ID:
Disease or Syndrome
The phenotypic spectrum of glucose transporter type 1 deficiency syndrome (Glut1 DS) is now known to be a continuum that includes the classic phenotype as well as paroxysmal exercise-induced dyskinesia and epilepsy (previously known as dystonia 18 [DYT18]) and paroxysmal choreoathetosis with spasticity (previously known as dystonia 9 [DYT9]), atypical childhood absence epilepsy, myoclonic astatic epilepsy, and paroxysmal non-epileptic findings including intermittent ataxia, choreoathetosis, dystonia, and alternating hemiplegia. The classic phenotype is characterized by infantile-onset seizures, delayed neurologic development, acquired microcephaly, and complex movement disorders. Seizures in classic early-onset Glut1 DS begin before age six months. Several seizure types occur: generalized tonic or clonic, focal, myoclonic, atypical absence, atonic, and unclassified. In some infants, apneic episodes and abnormal episodic eye-head movements similar to opsoclonus may precede the onset of seizures. The frequency, severity, and type of seizures vary among affected individuals and are not related to disease severity. Cognitive impairment, ranging from learning disabilities to severe intellectual disability, is typical. The complex movement disorder, characterized by ataxia, dystonia, and chorea, may occur in any combination and may be continuous, paroxysmal, or continual with fluctuations in severity influenced by environmental factors such as fasting or with infectious stress. Symptoms often improve substantially when a ketogenic diet is started.

Recent clinical studies


Bose R, Banerjee AD
Pediatr Neurosurg 2019;54(6):436-440. Epub 2019 Oct 7 doi: 10.1159/000503114. PMID: 31590166
Califano L, Melillo MG, Mazzone S, Vassallo A
Acta Otorhinolaryngol Ital 2008 Dec;28(6):287-91. PMID: 19205592Free PMC Article


Quade A, Thiel A, Kurth I, Holtgrewe M, Elbracht M, Beule D, Eggermann K, Scholl UI, Häusler M
Eur J Paediatr Neurol 2020 Mar;25:181-186. Epub 2019 Nov 20 doi: 10.1016/j.ejpn.2019.11.002. PMID: 31810576


Bose R, Banerjee AD
Pediatr Neurosurg 2019;54(6):436-440. Epub 2019 Oct 7 doi: 10.1159/000503114. PMID: 31590166

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