The results of surgical removal of a porencephalic cyst in an 18-month old child with infantile spasms and hypsarrhythmia are reported from the Departments of Neurology and Neurosurgery, VA Medical Center and University of Florida; the State University of Stony Brook, NY; and University of Michigan Medical School, Ann Arbor, MI. Infantile spasms were evaluated first at 4 months of age. The seizures were generalized but the neurologic examination showed a left hemiparesis and a left visual field deficit. ACTH provided a transient control of spasms for 3 months. At 18 months the seizures had become refractory to medical treatment. Postoperatively, seizures have been controlled for 12 months and the use of the paretic extremities has improved. Preoperative anti-epileptic medications were continued. 
COMMENT. This report indicates that infantile spasms and hypsarrhythmia of a diffuse pattern may be associated with a focal cerebral lesion amenable to surgery. Infantile spasms associated with COFS syndrome (cerebro-oculo-facial-skeletal syndrome) in a 3-month-old child is reported from the Department of Neurology, New York Medical College, New York, NY . COFS syndrome is a rare autosomal recessive condition characterized by microcephaly, microphthalmia and/or cataracts, neurogenic arthrogryposis and multiple congenital anomalies. The infantile spasms and hypsarrhythmia resolved during ACTH therapy.