Abstracts

Rationale: Prader-Willi syndrome is a chromosomal disorder caused by absence of expression of the paternal genes in the 15q11_ q13 chromosome region. This case is discussed to illustrate unusual EEG pattern in case of Prader Willi SyndromeMethods: Description of case and EEGResults: Presented here is the case of 12 years old girl with Prader –Willi Syndrome due to typical chromosome deletion and cognitive delay who had history suggestive of complex partial seizures starting around age of 4 years. Episodes were described as staring with unresponsiveness. She had normal MRI apart from microcephaly and initial EEG showed multifocal sharp waves and spikes predominantly over right parietotemporal region. Repeat EEG done at 5 years of age was reported as showing theta frequency variance, which was deemed as likely non-epileptiform phenomenon. She was treated with Keppra for period of time, which was subsequently discontinued because of non-compliance. Family was reluctant for medication as clinical events were apparently improved. The girl was brought back to neurology clinic at the age of 12 years due to episodes of head dropping as noted by teachers although family denied seeing at home. EEG did show runs of notched theta activity predominantly in T7, P7 derivatives often synchronous and occasionally vacillating with T8, P8 with no ictal correlate. Electrographic findings in Prader –Willi syndrome described in literature include 1) polyspike and wave and short burst (wang et.al 2005) 2) Focal paroxysmal discharges, persistent high amplitude 4-6 Hz activities (Wang et.al 2005) 3) Focal paroxysmal discharges, normal, or multifocal spikes (Kumada et al., 2005, Fan et al., 2009) EEG in our case did generate wide differentials among our group ranging from possible subclinical status to psychomotor variant. Although SREDA (Subclinical rhythmic EEG discharge of adults) is not unheard of in children, it couldn’t be substantiated, as EEG was abnormal at baseline with voltage asymmetry.Conclusions: Although it is a interesting finding ,we need series of cases with similar electrographic findings before considering it as one of distinct EEG pattern in children with Prader-Willi Syndrome.