The authors report two cases of Brazilian children with most of the common syndromic features of Proteus syndrome, such as asymmetric overgrowth of tissues, skin abnormalities, hypotonia and mental retardation. In both patients, a refractory epilepsy, compatible with Ohtahara syndrome, as well as hemimegalencephaly, with asymmetric distribution of facial fat, were also diagnosed.

Alpha coma, an EEG pattern characterized by diffuse or widespread rhythmic activity in the alpha frequency band, is typically recorded in patients with profound coma and is frequently associated with severe neurological conditions. The most common etiologic factors of this pattern are hypoxic-ischemic encephalopathy, encephalitis, head trauma, metabolic disorders, and drug overdose. Reports of alpha coma pattern in children are relatively common. Clinical significance, both in children and adults, is variable, and highly dependent on etiology. The objective of this article is to report a clinical case of alpha coma pattern in a child with neuroblastoma. The EEG pattern was recorded during the evolution of treatment, secondary to complicating septic encephalopathy. The alpha coma pattern was replaced by a normal trace following a favorable outcome after sepsis resolution.