Penfield's syndrome is characterized by attacks of epileptic seizures caused by a tumour pressing on the hypothalamus, accompanied by vegetative manifestations: congestion of face, sialorrhea, perspiration, tears, exophthalmos, tachycardia, polypnea, restlessness, and logorrhea. Prevalent in males; onset at 6 or 7 years of age.
In 1929 Penfield described peculiar attacks characterized by a wide variety of symptoms and manifestations that he designated as "diencephalic autonomic epilepsy." Signs and symptoms consisted of prodromal restlessness, sudden vasodilatation of skin in area supplied by the cervical sympathetic nerves, sudden rise in blood pressure, lacrimation, diaphoresis, salivation, dilatation or contraction of pupil, sometimes protrusion of eyes, increased rate and pressure of pulse, marked retardation of respiratory rate, elicitability of pilomotor reflex, and rarely loss of consciousness. These features were followed by disappearance of superficial blush and fall of blood pressure, slowing and weakening of pulse, hiccupping, transient shivering, and Cheyne-Stokes respiration.
These symptoms are characteristic of disturbance of hypothalamic function and are considered to be due to a focal epileptic discharge. Penfield's patient died in an attack. Autopsy revealed a tumour in the third ventricle.
- W. Penfield:
Diencephalic autonomic epilepsy.
Archives of Neurology and Psychiatry, Chicago, 1929; 22: 358-374.
- Robert H. Durham:
Encyclopedia of Medical Syndromes.
New York : Paul B. Hoeber Inc., 1960. 628 pages.