This condition is inherited in an autosomal recessive manner.
Northern epilepsy syndrome (NE), or progressive epilepsy with mental retardation (EPMR), is a subtype of neuronal ceroid lipofuscinosis and a rare disease that is regarded as a Finnish heritage disease. Unlike most Finnish heritage diseases, this syndrome has been reported only in Finland.[1] The disease is characterized by seizures in early childhood that progressively get worse until after puberty. Once the onset of seizures occurs, mental degradation is seen. This continues into adulthood, even after seizure frequency has decreased. The cause of the disease is a missense mutation on chromosome 8. The creation of a new protein occurs, and the lipid content of the brain is altered because of it. The ratio of the mutation carriers is 1:135. There is nothing that has been found to stop the progression of the disease, but symptomatic approaches, such as the use of benzodiazepines, have helped control seizures.[2][3][4]
^Krystyna E. Wiśniewski; Nanbert Zhong; Jeffrey C. Hall (2001). Batten disease: diagnosis, treatment, and research. Academic Press. p. 243. ISBN 978-0-12-017645-8. page 125
^Cite error: The named reference Prevention Genetics was invoked but never defined (see the help page).
^Cite error: The named reference Genetics Home Reference was invoked but never defined (see the help page).
^Cite error: The named reference New Member was invoked but never defined (see the help page).
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diseases in Finland. Healthcare in Finland Finnish heritage disease Northernepilepsysyndrome "Causes of Death 2009". Statistics Finland. Retrieved 13 December...
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The Daily Northwestern. Retrieved 2019-05-23. "CURE Epilepsy: Citizens United for Research in Epilepsy". www.cureepilepsy.org. Foley, Elise (2007-03-26)...
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