detailed characteristics of DMD/BMD and other dystrophinop- athies worked out in close collaboration between molecular genetists, histopathomorphologists, neurologists, and other specialists.

48. COMPLETE CALLOSOTOMY IN INTRACTABLE EPILEPSY Chuan-Zhong Fu and Mei-Qian Wu, Suzhou, China

Forty-five patients (28 boys, 17 girls) with intractable epilepsy were treated from 1985 to 1991. Complete callosotomy was performed on the patients. The age of patients ranged from 4-14 years, among whom the average age at onset of seizures was 5.5 years and the time between onset and surgery averaged 6.3 years. Postoperatively, all patients were followed for longer than 1 year. Thirty-six patients (80%) were seizure-free. Seven pa- tients (l 5.5%) had fewer seizures and 2 (4.5%) were unchanged. There was no significant change in the Wechsler intelligence scale scores before and after surgery. However, the shorter the time between seizure onset and surgery, the greater the likeli- hood of improvement in verbal and perceptual IQ. Social out- come was significantly improved and a large percentage of pa- tients were either in school or employed. Early consideration of total callosotomy in children with medically refractory seizures is recommended.

49. CARBOPLATIN AND VP-16 IN TREATMENT OF HIGH STAGE MEDULLOBLASTOMA, PINEOBLAS- TOMA, AND SUPRATENTORIAL PRIMITIVE NEURO- ECTODERMAL TUMORS: A PRELIMINARY REPORT E.H. Kovnar, R.L. Heideman, S.J. Kellie, E.C. Douglass, C.A. Greenwald, J.F. Kuttesch, J.J. Ochs, J.W. Langston, R.A. Sanford, J.J. Jenkins, D.L. Fairclough, and L.E. Kun, Memphis, Tennessee

Adjuvant chemotherapy in newly diagnosed medulloblastoma (MB) has led to significant improvements in disease-free sur- vival in patients with incompletely resected or disseminated dis- ease. Cisplatin (CDDP) is one of the most active agents in MB and other primitive neuroectodermal tumors (PNETs). Dose- limiting toxicities, including hearing loss and renal tubular in- jury, have prompted investigation of potentially less toxic ana- logues. Based on previous institutional experience with CDDP and etoposide (VP-16) [J Clin Oncol 1990;8:330-6], we under- took a preirradiation trial of carboplatin (CARBO) and VP-16 in newly diagnosed patients with MB (n = 7), pineobtastoma (n = 2), and supratentorial PNET (n = 1). All patients had at least one site of radiographically measurable disease following initial surgery. CARBO (350 mg/M 2) and VP-16 (100 mg/M 2) were given on days 1 and 2 of each course. CARBO/VP-16 was repeated at 21-28-day intervals for a total of 4 courses prior to irradiation (XRT). Responses to CARBO/VP-16 were graded on the basis of change in the product of maximal cross sectional diameters (PMCSD) on serial MRI and included 1 complete response (CR: complete resolution of all tumor), 5 partial response (PR: > 50% reduction in PMCSD), 2 stable disease (SD: < 25% change in PMCSD), and 2 progressive disease (PD: > 25% increase in PMCSD). Grade 4 neutropenia (< 500/I.tl) occurred after 20 of 46 courses of therapy and Grade 4 throm- bocytopenia (< 25,000 ~tl) followed 8 of 48 courses. Mild hear- ing loss occurred in only 1 patient; there was no detectable renal

toxicity. All patients began XRT within 38 days ,~l starting the final course of chemotherapy. Patients with localized disease received conventionally fractionated XRT to a dose <+t 35 Gy tt~ the neuraxis and 54 Gy to the primary tumor ~h~me. Patient~ with disseminated disease received hyperfractionated XRT con- sisting of 48.4 Gy to the neuraxis, 60.5 Gy to x~¢:dular spinal metastases and 66 Gy to the primary lumor volu~e, Acute ef- fects attributable to XRT were limited to anore×ia and radio- epidermitis. At a median of 17 months from diagnosis ~range: 4-29 mos), 9 of 10 patients remain alive and fiee of disease. In summary, CARBO/VP-16 as used in the present report has sig- nificant activity, achieving objective responses (GR or PR) in 6 of 10 patients. These results are comparable to those reported by others using higher dose CARBO (500 mg/M 2 x 2) and VP-16 (100 mg/M 2 x 3) [Castello et al. Am J Pediatr Hemalol Oncol 1990; 12:297-300]. Manageable hematologic toxicity and lack of significant ototoxicity or renal toxicity suggest CARBO and VP-16 as an attractive combination for future phase I!1 MB trials.

50. ELECTRON MICROSCOPIC FINDINGS OF HU- MAN SKIN IN METACHROMATIC AND GLOBOID CELL LEUKODYSTROPHIES Hans H. Goebel, Seiji Kimura, and Irene Warlo, Mainz, Germany, and Yokohama, Japan

The two classic lysosomal leukodystrophies, i.e., metachromatic (MLD) and globoid cell (GCL) types, are morphologically marked by demyelination in the central and peripheral nervous systems and by lysosomal storage in respective myelin-produc- ing cells. Peripheral nerve and skin biopsies offer diagnostic recognition provided that the skin contains myelinated axons. Our studies on dermal tissues in MLD and GCL disclosed addi- tional ultrastructural abnormalities in sweat gland epithelial cells, inclusions characteristic of MLD and GCL, respectively, which, to our knowledge, do not occur in other lysosomal disor- ders. Based on tissue samples from several patients afflicted with MLD or GCL, we suggest that skin biopsies are of diag- nostic aid in MLD and GCL even when myelinated axons are absent, but sweat glands are present in the biopsied tissue speci- mens. Although aggregates of sweat glands are more frequently and more abundantly encountered in biopsied skin, especially when retrieved from sweat gland-rich areas, the presence of der- mal myelinated axons, their number and density are largely for- tuitous. Therefore, skin biopsy appears to be an adequate diag- nostic procedure to morphologically secure MLD and GCL, respectively.

51. SYMPTOMATIC POLYMORPHISM OF ELECTRI- CAL STATUS EPILEPTICUS DURING SLEEP Stella M. Ferraro, Ra61 Moreno, Claudio Podestd, Maria E. Mazzola, Horacio Encabo, and Julio Castafio, Buenos Aires, Argentina

The first description of "Subclinical electrical status epilepticus induced by sleep in children" was published by Patry et al. [1971] and afterwards by Tassinari et al. [1982] under the title of Electrical status epilepticus during sleep (ESES). This condi- tion, now incorporated in different epilepsy classifications (1985-1988-1989), were observed in patients with and without

356 PEDIATRIC NEUROLOGY Vol. 8 No. 5