A 12 year old boy with multiple lentigines (Leopard) syndrome in association with Gerstmann syndrome and CT abnormalities is reported from the Department of Pediatrics and Pediatric Neurology, Beilinson Medical Center, Petah Tikva and Tel Aviv University, Sackler School of Medicine, Petah Tikva, Israel. Learning difficulties were first observed at five years of age. He had a single simple febrile convulsion at three years of age. Several hundred hyperpigmented skin lesions over the face, trunk, and extremities had appeared gradually after birth and had increased in number and size. Mild dysmorphic features included hypertelorism, epicanthal folds, cubitus valgus, and pterygium colli. A systolic cardiac murmur was indicative of pulmonic stenosis and was accompanied by EKG changes. The signs of Gerstmann syndrome included dyscalculia, left/right disorientation, finger agnosia, and dysgraphia. His IQ score was 86. Cranial CT showed dilatation of the left lateral ventricle especially in the occipital horn and mild atrophy of the left parietal lobe. [1]

COMMENT. The term Leopard is a mnemonic acronym for the features of the syndrome which may include: L - lentigines, E -EKG abnormalities, O - ocular hypertelorism, P - pulmonary stenosis, A - abnormal genitalia, R - retardation of growth, D -deafness. Mild mental retardation has been reported in patients with Leopard syndrome. Perhaps the “A” in the mnemonic should stand for acalculia in place of “abnormal genitalia”. Some neuroradiologists would report the mild asymmetry of ventricles on the CT as a variant of normal and, in the absence of abnormalities of the white and gray matter, the diagnosis of parietal lobe atrophy may be questionable. As the author suggests, Gerstmann syndrome in children may be more common than indicated in the literature. In my experience this syndrome is not infrequent in children presenting with attention deficit disorders and normal CT scans are not unusual. It is possible that the MRI may be more revealing of associated cortical defects.