[A case of progressive hemifacial and hemispheric atrophy with multiple hemi-intracerebral calcifications presenting with occipital lobe epilepsy].

ABSTRACT

A 30-year-old man with progressive hemifacial atrophy is described. He had right hemifacial atrophy and epileptic seizures first noted at the age of about 15 years. Examination revealed atrophy of the right half of the tongue, skin pigmentation in the right neck, grizzled hair on the right side of the head, and left upper temporal homonymous hemianopsia. CT and MRI revealed multiple intracerebral calcifications, and EEG showed spike discharges predominantly in the right occipital lobe, ipsilateral to the hemifacial atrophy. The epileptic seizures were associated with visual hallucinations that are characteristic of occipital epilepsy. A skin biopsy obtained from the pigmented region in the right neck showed chronic inflammatory changes consisting of severe atrophy of the epidermis, dermis, and fatty tissue, marked proliferation of collagen fibers, and perivascular infiltration by round cells and giant phagocytes. Previous descriptions on the pathogenesis of hemiatrophy of the face and brain were reviewed in relation to the present case.