Sindrome di Parry-Romberg: aspetti neuropsicologici e neuroradiologici

Abstract Parry-Romberg syndrome is a rare disease characterized by progressive atrophy of the tissues of one half of the face; an involvement of the central nervous system occurs infrequently. Various pathogenetic hypotheses have been advanced: hereditary, inflammatory, infective, lesion of central or peripheral sympathetic nervous system, immunological, vascular malformation, localized scleroderma, and trauma. Atrophy generally started before the age of 30 years; trigeminal neuralgia, seizures or facial trauma are generally antecedents of the facial hemiatrophy. Cerebral atrophy omolateral to the facial hemiatrophy is rarely reported; white matter alterations, calcifications, vascular malformations, or hamartomas are also described. Cognitive deficits were previously reported only in 2 patients with facial hemiatrophy associated with seizures. We report neuropsychological and neuroradiological features in a case of left facial hemiatrophy with no neurological symptoms. Verbal performances impairment was observed; brain MR showed left cerebral atrophy with no white matter or cortical abnormalities. The alterations of cognitive pattern in our patient were related to the side of cerebral atrophy. We retain that a neuropsychological evaluation is necessary in patients affected by Parry-Romberg disease to verify the frequence of selective cognitive deficits although in absence of signs and symptoms of central nervous system involvement.