facial muscle involvement, normal or nearly normal intelli- gence, leukodystrophic appearance on CT and dystrophic mus- cle changes. Various forms of CMD may be associated with more or less marked disorders in cerebral and cerebellar devel- opment. The reports of multimodal evoked potential correlates of cerebral developmental disorders seen in this nosology are rare and confined to the more severely affected forms. The pres- ent study describes the results of visual evoked potentials (VEP), auditory brainstem responses (BAER), EEG, and CT performed on l l patients with OCMD. Three patients' EEGs were abnormal with slow and irregular background and par- oxysmal activity. In 6 patients VEP demonstrated delayed PI, latency, and in 4 BAERs showed prolonged IV interpeak laten- cy. These abnormal VEP and BAER results may indicate associ- ated subtle cortical dysplasias, hypo- or dysmyelinisation in the visual cortex and brainstem which may be extremely variable from patient to patient in such a heterogeneous disorder. Evoked potential studies may serve as a supplementary tool for detecting variable associated developmental abnormalities of visual and central auditory pathways or their cerebral targets.

267. SUBACUTE SCLEROSING PANENCEPHALITIS: DATA OF NATIONAL REGISTRY CENTER K. Yalaz, B. Anlar, Y. Renda, E. Ozdirim, S. Aysun, M. Topqu, and H. Topaloglu, Ankara, Turkey

Subacute sclerosing panencephatitis (SSPE), a latent infection of the CNS caused by the measles virus, is prevalent in countries where measles is encountered and is more frequent among chil- dren experiencing measles before 2 years of age. Since 1975, 530 patients have been registered in our center. Most patients are t0-12 years of age. The male preponderance in SSPE be- comes even more pronounced among patients older than 13 years. Recent trends in epidemiologic data are in favor of a slower, more chronic course in SSPE than the classically de- scribed progressive disease. The question of recent measles out- breaks among older children resulting in SSPE cases in adults can be answered by regular reporting of SSPE cases and careful analysis of data.

268. UNUSUAL CASE OF DUCHENNE MUSCULAR DYSTROPHY (DMD) Haluk Topaloglu, Pervin Dinner, Safiye Gtgtis, Stikrtiye Ayter, and Meral Topqu, Ankara, Turkey

A 10-year-old boy with DMD had quite unusual clinical data. He initially did not walk until 7 years of age, walked only for 9 months, and became wheelchair-bound. He had a large deletion in the dystrophin gene. Exons from 45 to 52 were deleted as shown by multiplex PCR amplification system. Deletions en- compassed the central high-frequency deletion region of the gene. He had almost no dystrophin on the sections of muscle biopsies by using Newcastle monoclonals (Dys 1, Dys 2). Be- cause there is no dystrophin in muscle biopsies, and mutations exist in hot spot regions (exons 45-52) and because of the clini- cal picture, this case is defined as a severe type of DMD. Early developmental milestones may be delayed in DMD but patients usually start to walk around ages 2 to 3 years. Delay to this extent is very unusual.

269. CASE STUDIES IN RIGID SPINE SYNDROME H. Topaloglu, K. Yalaz, S. Gtgtis. and G. Ktse. Ankara. Turkey

The rigid spine syndrome (RSS) is clinically characterized by limitation of flexion of the cervical and dorsolumbar spine, due to shortening of the erector trunci muscles without severe weak- ness. Most cases occur sporadically but there is evidence of autosomal-recessive inheritance. The time of onsel is in child- hood. Creatine kinase levels are variably elevated. Biopsy find- ings from quadriceps or erector trunci muscles may show vari- ous forms of myopathies. We report 3 cases with RSS; 2 have nemaline myopathy (2 siblings and parents are first cousins), the other has probable Emery-Dreifuss type dystrophy.

270. PROGRESSIVE DYSTONIA WITH DIURNAL VARIATION (SEGAWA DYSTONIA) IN 3 PATIENTS Kivilcim Giictiyener, Sabiha Aysun, and Pelin Adiyaman, Ankara, Turkey

Progressive dystonia with diurnal duration is an important and treatable disorder that should be considered in children present- ing with gait disturbance. We present 2 siblings, ages 16 and 9 years of age, and an unrelated girl, age 7, with diurnal fluctua- tions of motor performance due to increased dystonia and spas- ticity. In both siblings the dystonia had started in the lower extremities with twisting of one foot and gait difficulties. In the other girl the dystonia was typically least severe in the morning and increased as the day continued. All 3 patients markedly improved with low doses of carbidopa-levodopa. Understanding the clinical spectrum of this disorder is a prerequisite for de- tailed genetic analysis and appreciation of the subtle and ex- treme manifestations that will lead to greater recognition of treatable patients.

271. MRI AND EEG IN SPASTIC DIPLEGIA WITH LOW BIRTH WEIGHT Akira Onuma and Yasuko Kobayashi, Sendai, Japan

MRI and EEG were investigated in 40 children t23 males. 17 females; ages 2-14 years: mean age: 7.6 years) with spastic diplegias, whose birth weights were under 2.500 gm. In all 40 children, MRI had typical findings of periventricular leukomala- cia (PVL), such as enlargement and deformity of the posterior horn of the lateral ventricles, decreased white matter volume around the trigonum areas of the lateral ventricles and focal high-intensity areas on T2-weighted images in the periventricu- lar white matter. Epileptiform activities were observed on EEGs of 26 of 40 children. They were all focal spikes: midline only (Cz and/or Pz; 14 of 26). multifocal spikes including midtine (8 of 26) and other focal areas than midline (4 of 26). Longitudinal EEG examinations revealed that these midline spikes were recognized mainly from 4-10 years of age and gradually disap- peared after 11 years of age. These midline spikes showed clear age specificity. In conclusion, in most of the spastic diplegias with low birth weight whose MR/showed PVL, midline spikes are considered to be specific EEG findings in this type of spastic diplegia and possibly originate from diffuse, subcortical lesions.

272-274. WITHDRAWN.

410 PEDIATRIC NEUROLOGY Vol. 8 No. 5