What EEG findings are characteristic of Alpers disease?

Updated: Oct 09, 2019
  • Author: Eli S Neiman, DO, FACN; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Answer

Alpers disease, an autosomal recessive inherited disorder consisting of progressive neuronal degeneration of childhood with liver disease, has been studied by Boyd et al. [40] The onset is in early childhood and consists of intractable fits, progressive dementia, and brain atrophy. EEG studies have been carried out on 12 children with this condition. The EEGs were similar and demonstrated abnormal patterns with high-amplitude slow activity as well as lower amplitude polyspikes. The flash visual evoked potential (VEP) was usually abnormal and often asymmetrical. In the appropriate clinical setting, the neurophysiologic features may aid the clinician in the diagnosis of this disease.


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