To send content items to your account,
please confirm that you agree to abide by our usage policies.
If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account.
Find out more about sending content to .
To send content items to your Kindle, first ensure firstname.lastname@example.org
is added to your Approved Personal Document E-mail List under your Personal Document Settings
on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part
of your Kindle email address below.
Find out more about sending to your Kindle.
Note you can select to send to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi.
‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
Progressive myoclonus epilepsy of the Unverricht-Lundborg type (EPM1) is an autosomal recessive neurodegenerative disorder that has the highest incidence among the progressive myoclonus epilepsies worldwide. The primary therapeutic approaches for EPM1 patients include rehabilitation and symptomatic pharmacologic management. Pharmacologic intervention includes: valproic acid, clonazepam, high doses of piracetam, levetiracetam and topiramate and zonisamide. Several case reports suggest that treatment of EPM1 patients with the antioxidant N-acetylcysteine (NAC) alleviates key features of the disorder including dysarthria, ataxia, and seizures. Although the role of oxidative stress in EPM1-linked neuronal degeneration is not completely understood, a specific decrease in cerebellar defenses against oxidative stress and a concomitant increase in lipid peroxidation occurs in the mouse model for EPM1. Myoclonic seizures can also be easily misdiagnosed as tonic-clonic seizures or even pseudoepileptic seizures, especially as the majority of the myoclonic movements are not time-locked to electroencephalogram (EEG) discharges.