Epilepsia, 32(5):668471, zyxwvutsrqpon 1991 Raven Press, Ltd., New York zyxwvutsrq 0 International League Against Epilepsy zyxwvutsrqpo Outcome for West Syndrome Following Surgical Treatment Basim M. Uthman, *S. A. Reid, B. J. Wilder, TM. R. Andriola, and SAhmad A. Beydoun zyxw Departments of Neurology and *Neurosurgery, VA Medical Center, and University of Florida; ?Department of Neurology, School of Medicine, State University of Stony Brook, Stony Brook, New York; and $Department of Neurology, University of Michigan Medical School, Ann Arbor, Michigan, U.S.A. Summary: We report the case of an 18-month-old child with infantile spasms and a hypsarrhythmic electroen- cephalogram (EEG) pattern associated with a porenceph- alic cyst. Surgical removal of the cyst and its surrounding tissue was performed following failure of medical ther- apy. Postoperatively, the patient has been free of infantile spasms for 12 months and the EEG has normalized. He Infantile spasms, when associated with neurolog- ical deficits or due to a known cause, are usually difficult to treat and have an overall poor prognosis. Although many etiologies have been reported, they have rarely been associated with treatable struc- tural lesions of the brain. We report a child with infantile spasms, a hyps- arrhythmic electroencephalogram (EEG) pattern, and a unilateral porencephalic cyst. The patient failed medical therapy. Surgical removal of the cyst and its surrounding tissue was performed and he became seizure free, except for rare myoclonic jerks upon awakening. The EEG has become nor- mal. CASE PRESENTATION The boy is the product of an uneventful full-term pregnancy, delivered by Cesarean section because of prolonged labor and failure to progress. His mother did not notice any developmental abnormal- ities. He was first evaluated at 4 months of age for infantile spasms that occurred in clusters several Received April 1990; revision accepted August 1990. Address correspondence and reprint requests to Dr. B. M. Uthman at Neurology Service zyxwvutsrqp (127), Veterans Affairs Medical Center, Gainesville, FL 32608, U.S.A. has been maintained on the same preoperative antiepilep tic medications. This case suggests that surgical treat- ment is helpful in selected patients with infantile spasms and focal CNS lesions. Key Words: West syndrome- Infantile spasms-Hypsarrhythmia-Neurosurgery- Electroencephalography. times daily. Each cluster lasted 20-30 min. No focal seizures were reported. His neurologic examination revealed a left hemiparesis and hypertonia as well as a left visual field deficit. Computed tomography (CT) of the brain showed an area of encephaloma- lacia in the right parietotemporal area. His EEG revealed a hypsarrhythmic pattern that did not re- spond to pyridoxine. Metabolic studies, toxo- plasma, rubella, cytomegalovirus, and herpes titers, and cerebrospinal fluid examination were normal. He was given a course of adrenocorticotropin (ACTH) that resulted in a transient cessation of spasms for the next 3 months. At 8 months of age, 7 weeks following discontinuation of ACTH treat- ment, he developed episodes of unexplained staring and intense crying that were controlled by clonaze- pam (CZP), 0.5 mg, b.i.d. At 10 months of age, the infantile spasms recurred. They consisted of a brief tonic extension of both arms and generalized stiff- ening of the body followed by tonic elevation of both legs and deviation of the eyes and head to the left. He also developed left focal tonic seizures that lasted zyxw 1&15 s. CZP was increased to toxicity; ad- dition of carbamazepine (CBZ), valproate (VPA), and phenytoin (PHT) failed to control his seizures. At 17 months of age, he was readmitted to the hos- pital because of an increase in frequency of spasms as well as development of generalized tonic-clonic 668