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**15** 

*Italy* 

*2Child Neurology,* 

**Epilepsy in Mitochondrial Disorders** 

Carlo Antozzi4, Massimo Zeviani3 and Laura Canafoglia1 *1From the Department of Neurophysiopathology and Epilepsy Centre,* 

*4Myopathology, IRCCS Foundation Neurological Institute Carlo Besta, Milan,* 

Mitochondrial encephalopathies (MEs) are characterized by an extreme clinical heterogeneity since they can involve different systems and manifest at distinct ages with variable course. Many affected individuals display a cluster of clinical features that fall into discrete syndromes - among syndromic pictures, epilepsy is relevant in myoclonic epilepsy with ragged-red fibers (MERRF), mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS), neurogenic weakness with ataxia and retinitis pigmentosa (NARP), Alpers' encephalopathy and Leigh syndrome (LS). However, many patients do not fit neatly into one particular syndrome, due to "overlapping" presentations (sharing

Epilepsy is a frequent symptom also in non-syndromic patients, sometimes dominating the

Mitochondrial disorders derive from mutations of mitochondrial (mtDNA) or nuclear DNA (nDNA), which lead to the impairment of the mitochondrial respiratory chain activity or mitochondrial ATP synthesis. Mitochondrial disorders typically involve tissues or organs with high energy demand including peripheral nervous system (PNS), central nervous system (CNS), eyes, ears, heart, endocrine system, kidney, guts, and liver. Fever, infection

Generally, the genotype-phenotype correlation in mitochondrial disorders is poor, since the clinical phenotype is not only dependent on the type and pathogenicity of the DNA

A possible mechanism of inheritance is maternal transmission of the mutations located in the mtDNA. It is noteworthy that variable amounts of the mutated mtDNA (mutation load) usually coexist with wild-type molecules in the different tissues, resulting in a "heteroplasmic state". Moreover, the phenotypic expression of mtDNA mutations may be dependent on a threshold effect, which can be dissimilar in different tissues, in relation to

mutation, but it may also derive from other genetic and environmental factors.

symptoms of different syndromes) or atypical clusters of symptoms.

and stress may aggravate the neurological symptoms.

**1. Introduction** 

clinical presentation.

**2. Genetics** 

Silvana Franceschetti1, Graziella Uziel2, Eleonora Lamantea3, Gianfranco Carrara3,

*3Biochemistry and Medical Genetics,* 

