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Doctors 'cautiously optimistic' following rare epilepsy surgery (8-17-99)

Faculty from The University of Texas Health Science Center at San Antonio are reporting success today (Aug. 19) after surgery on an Italian teenager with an extremely rare form of epilepsy. The operation, the first of its kind in San Antonio, was performed Aug. 12 at University Hospital.

The patient, 18-year-old Michele (pronounced mik-hale-ee) Angeloni from the island of Sardinia in the Mediterranean Sea, averaged about one seizure an hour for the last 11 years. He has had no seizures since neurosurgeon G. Alexander West, M.D., Ph.D., assistant professor of surgery at the Health Science Center, removed a congenital tumor at the base of his brain. The boy suffered from a variety of seizure disorders, including extremely rare gelastic seizures, which are characterized by laughter. Approximately 100 cases of gelastic seizures have been reported worldwide.

"Since the operation, the patient has had no seizures, going a day without a seizure for the first time in more than 11 years," Dr. West said. "Some of the seizure types were very quiet, so the symptoms were not recognizable to his family, but others were more severe. In fact, he suffered a grand mal seizure in the car the day before he was admitted to the hospital."

Surgery was performed after all anti-epilepsy medicines failed to control the seizures and after the Angelonis sought other opinions throughout Europe. Dr. West met the family while in Italy to give invited lectures, and family members invited him to stay in their home on Sardinia. "During my visit I witnessed many seizures throughout the day," he said. "Michele had exhausted all medical opportunities. The lesion he had, in my view, left no alternative but surgery."

Dr. West consulted with neurosurgical colleagues in Dallas and Seattle before consenting to do the surgery. Several physicians in Europe had advised the family that it was too risky to perform. The boy had a tumor, known as a hamartoma, at the base of the brain and adjacent to the hypothalamus and optic nerve. The hypothalamus controls eating, sleeping and other functions and is connected to other key control centers in the brain. Michele's tumor caused seizures that started at age 6 1/2 and intensified with hormonal changes at puberty. His cognitive development was frozen at the first-grade level after his seizures began, said his mother, Silvana Angeloni.

Michele's father, Giampaolo, a commander in the Italian Navy; his mother; and his godfather, Alfredo Lupi, paid $14,000 toward the $100,000 surgery and accompanied the boy on the trip. Dr. West and other faculty physicians waived their fees, and two companies, Radionics Inc. and The Armamentarium Inc., donated electrodes and titanium plates used in the surgery.

After arriving in Texas, the family visited with Jack Farmer, Michele's uncle in Corpus Christi. "These are unbelievable people and the devotion they have and how they deal with this boy is incredible," Dr. West said. "They are important for him and they are always there for him."

"We are very happy now. I see him well; I see him without seizures," said his mother, Silvana Angeloni, as Dr. West met with the family in the hospital room. She told a story of love and persistence in acquiring the best care for her son and eventually securing a trip to San Antonio. The family first had to receive permission from the Italian government to seek surgery in the United States.

In an initial operation Aug. 9 at University Hospital, Dr. West and his team placed strips of electrodes directly on the brain to pinpoint the boy's epileptic activity. The major surgery took place Aug. 12, when the team removed a bone above the right eye to gain a clear view of Michele's hypothalamus, then resected the hamartoma. "No one has ever done this approach—removing bone to allow minimal retraction around the brain," Dr. West said. Retraction refers to holding open a wound for the purpose of surgical resection.

"His brain was abnormally firing all the time. The abnormal activity was affecting both frontal lobes and causing his behavior to become almost psychotic. In fact, his condition had regressed since he was 6 ˝. I am cautiously optimistic that we will see an improvement not only in control of seizures but in Michele's cognitive and behavioral development."

Michele left his hospital room Aug. 16 for the first time. "When he walked down the hall, all the nurses were clapping for him," Dr. West said. "He's a very special kid who has been through a lot. He nearly died when he had a seizure while swimming in the sea. We hope this will free him to live an entirely new life of learning and remembering."

Dr. West's team members include C. Akos Szabo, M.D., from the Health Science Center's Department of Medicine; Dennis Vollmer, M.D., chief of the Surgery Department's Division of Neurosurgery; Tod Sloan, M.D., Ph.D., and Kathryn Hamilton, M.D., from the Department of Anesthesiology; faculty from the departments of Radiology and Pathology; Glenn Harper, M.D., seventh-year neurosurgical resident; and Cindy Rogers, R.N., Division of Neurosurgery, clinical coordinator. Beverly Combs, admin-istrative secretary, communicated with the Angelonis in Italy and obtained housing for the family in San Antonio with the Defense Language Institute at Lackland Air Force Base.

Michele will be on three anti-epileptic medications for the foreseeable future, down from a previous maximum of 14.

Contact: Will Sansom