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Jeavons Syndrome

Jeavons syndrome (JS), a generalized epilepsy of presumed genetic origin, is characterized by eyelid myoclonia with or without absence seizures.¹ EEG paroxysms of high amplitude polyspike discharges (3-6 Hz) lasting for 1-6 seconds induced by eye closure (fixation-off sensitivity) and “intermittent photic stimulation” (photosensitivity) are diagnostic.¹ The prevalence of JS among generalized epilepsies is 7.3-12.9%,² predominantly affecting females age 2-14 years.¹ Dysfunction of the alpha generator is the hypothesized pathomechanism.¹ The differential includes juvenile myoclonic epilepsy, childhood absence epilepsy, and facial tics.¹ Valproic acid and then levetiracetam, clonazepam, and lamotrigine are treatment options.¹ Commercially available blue Z1 lenses can also help with photosensitivity.¹ Eyelid myoclonia may persist, but photosensitivity can decrease with age.¹ The overall prognosis is good.

References:

1. Hannawi Y, Satpute SS, Maheshwari A. Mystery case: eyelid myoclonia with absences in an adult patient. Neurology. 2014;82(8):e63-e64. doi:10.1212/WNL.0000000000000139

2. Covanis A. Jeavons syndrome – updated review. Journal of Epileptology. 2015;23(2):113-123.

Rebecca John, MBBS, Kannur Medical College, Kerala, India, Neurology rotation, Houston Medical Clerkships, Sugar Land, Texas.

Dr. John reports no disclosures.