Acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis

months) were included in the study. All had limb deformities associated with a nonfixed joint contracture that had not responded to physical therapy. ...

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months) were included in the study. All had limb deformities associated with a nonfixed joint contracture that had not responded to physical therapy. Clinical assessment of passive and active muscle tone was performed using a modified Ashworth scale. The range of motion to passive movement was measured with a manual goniometer. Botulinum toxin was injected directly into the muscle at several sites. The postinjection scores of hypertonia were significantly lower (P < .01) and the range of motion values increased significantly (P < .001). Functional improvement was manifested by decreased scissoring in all 6 children with adductor muscles injected; all 6 children with knee flexor muscles injected were able to straighten the knees. The 3 children with injected gastrocnemius muscle were able to achieve heel-strike barefooted. This study provides evidence that the injection of botulinum toxin intramusculary in selected skeletal muscles decreases muscle tone and contractures, and increases range of motion and motor function. 104. RELATIONSHIP BETWEEN EEG BACKGROUND ACTIVITY AND RELAPSE Kazuhisa Hongou, Tohru Konishi, Kaori Masuko, Yoshihiro Naganuma, Miyako Murakami, Miwa Yamatani, Sinnichi Yagi, and Toshio Okada, Toyama, Japan

We retrospectively studied the relationship between EEG background activity and prognosis of epileptic seizures in childhood epilepsy. The subjects were 54 children with epilepsy, ages 5-18 years old, who had been free from epileptic seizures for more than 3 years and then gradually diminished and discontinued antiepileptic drugs (AEDs). Ten patients relapsed after diminution of AEDs and the remaining 44 patients have not relapsed for more than 1 year after discontinuation of AEDs. We evaluated EEGs (01 derivation), which were recorded every year from the final seizure to diminution of AEDs, and compared the development of EEG background activity between groups with relapses and those without relapses. The mean frequency significantly increased after final seizures in patients without relapses, but that finding did not change in those with relapses. The relative power of delta most remarkably decreased and that of alpha-1 increased in symptomatic partial epilepsy without relapse. Our study suggested that the evaluation of serial EEGs on AED therapy was a useful predictor of outcome, especially in symptomatic partial epilepsy. 105. AGE CHANGES OF EEG BACKGROUND ACTIVITIES AND EVENT-RELATED POTENTIALS (P300) IN EPILEPSY Tohru Konishi, Kaori Masuko, Yoshihiro Naganuma, Kazuhisa Hongou, Miyako Murakami, Miwa Yamatani, and Shinichi Yagi, Toyama, Japan CNS maturation by means of power spectral analysis of EEG and the development of cognitive function by means of event-related potentials (ERPs) were evaluated in 100 patients with childhood epilepsy and 33 normal controls. (1) In normal controls, mean frequency of EEG was in good proportion to age and depended on decreasing delta and theta powers and increasing alpha-2. P300 latency was also inversely proportional to age. (2) The significant EEG slowing and prolongation of P300 latency were recognized in patients with epilepsies. These abnormalities were most marked in patients with symptomatic partial epilepsies,

112 PEDIATRIC NEUROLOGY Vol. 11 No. 2

mild in cryptogenic partial epilepsies or idiopathic generalized epilepsies, and minimal in idiopathic partial epilepsy (BECT). The age changes of two tests were strikingly different between epileptic syndromes. (3) The changes of EEG parameters and P300 latency was almost parallel in normal controls. The correlation between the two tests in BECT were similar to those in normal controls, but showed some discrepancies in the other epileptic syndromes. The change of P300 latency tended to be smaller than that of EEG in patients with epilepsies. These resuits suggest that some CNS dysfunction and cognitive impairment exist widely in children with epilepsy, and manifest characteristic age changes according to epileptic syndromes. 106. ACUTE DISSEMINATED ENCEPHALOMYELITIS Sarenur Tiitiinc~io~lu, Hasan Ytiksel, Nilgiin Yiinten, and Fahr~ Tercan, Bornova-Izmir, Turkey Acute disseminated encephalomyelitis (ADEM) is a rare, acute inflammatory, demyelinating, monophasic illness of the CNS [1]. It develops within 5-14 days following an infectious disease (e.g., measles, mumps, or upper respiratory tract infection) or vaccination (e.g., diphtheria, tetanus, or rabies) [2]. Perivascular mononuclear cell infiltration and demyelination are prominent pathologic findings. Clinical findings are confined to acute widespread CNS dysfunction like coma, convulsion, hemiplegia, or cranial nerve involvement. Cranial MRI discloses numerous discrete and periventricular high-signal lesions within the white matter of the cerebral hemispheres on T2-weighted images. Corticosteroids are helpful in recovery of clinical and radiological findings [2-5]. Four patients with ADEM, ages 6 months to 6 years, are presented. Three of them were given dexamethasone for 2 weeks. Although the youngest patient demonstrated full recovery, the others had incomplete recovery. These patients had neurologic impairment, such as hemiparesis, dystonia/athetosis, and optic atrophy. References: [1] Aluond EC. Disseminated encephalitis: Its variations in form and their relationship to other disease of nervous system. In: Koetsier JC, ed. Handbook of clinical neurology. Vol. 3 (47): Demyelinating disease. Amsterdam: North Holland, 1985;467-502. [2] Scott WA, Grosmann RR, Goldberg H, et al: MR diagnosis of acute disseminated encephalomyelitis. J Comput Assist Tomogr 1986;10:798-801. [3] Karen SC, Todd MH, Richard RS, Mary KE: Gadolonium enhancement in acute disseminated encephalomyelitis. J Comput Assist Tomogr 1991;15:673-5. [4] Hiller DH, Robb SA, Onmerod IEC, et al: MRI of white-matter disease of childhood. Dev Med Child Neurol 1990;32:97-107. [5] Broich K, Norwich D, Aloui A: HMPAO-spect and MRI in acute disseminated encephalomyelitis. J Nucl Med 1991 ;32:1897-900. 107. EFFICACY OF VIGABATRIN IN INTRACTABLE EPILEPSY Sajun Chung and Minyoung Park, Seoul, Korea Thirty children with various types of intractable epilepsy were treated with vigabatrin as single-blind, add-on therapy. Before entering the study, each patient had an initial baseline observation period of 2 months, during which time the patient was maintained on pre-existing antiepileptic drugs. The starting dose was 40-50 mg/kg/day, increasing to 80-100 mg/kg/day depending on the clinical response. Responders were defined as those patients exhibiting a greater than 50% reduction in seizure fre-