[Neurophysiological aspects of Proteus syndrome]

The Proteus Syndrome was defined in 1983 by Wiedeman. However, the first case mentioned in the literature was that of Joseph Merrick, the Elephant Man, presented by Sir Frederick Treves in 1884. It is a rare pathological condition. Its multiple clinical features include; partial gigantism of hands and/or feet, pigmented nevi, hemihypertrophy of the body, tumors, skeletal anomalies, growth disorders and visceral anomalies. Hereditary transmission has not been clearly defined. Diagnosis and treatment require the participation of experts from several medical and surgical specialties.

We present a case sent to our hospital for the surgical correction of cranio-facial malformations. Epileptic crises post-operatively indicated the need for neurological and neuro-physiological study. This was done by means of conventional electro-encephalography: brainstem, somato-sensorial and visual auditory evoked potentials, together with imaging techniques which showed the structural and functional asymmetry of the central nervous system at both cerebral and brainstem levels.

Few neuro-physiological studies are included in the literature we reviewed for this paper. Therefore we do not know whether the functional anomalies of the central nervous system which we describe should be considered to be part of the syndrome.