xDeletions on the short arm of chromosome 4 cause Wolf-Hirschhorn syndrome
(WHS) and Pitt-Rogers-Danks syndrome (PRDS). WHS is associated with severe
growth and mental retardation, microcephaly, a characteristic facies and co
ngenital malformations. The PRDS phenotype is similar to WHS but generally
less severe. Seizures occur in the majority of WHS and PRDS patients. Sgro
et al. [17] described a stereotypic electroclinical pattern in four unrelat
ed WHS patients, consisting of intermittent bursts of 2-3 Hz high voltage s
low waves with spike wave activity in the parietal areas during drowsiness
and sleep associated with myoclonic jerks. We report a patient with PRDS an
d the typical EEG pattern and review 14 WHS patients with similar EEG findi
ngs reported in the literature.
Conclusion Awareness and recognition of the characteristic electroclinical
findings in Wolf-Hirschhorn syndrome and Pitt-Rogers-Danks syndrome might h
elp in the early diagnosis of such patients.