[Response to high dose corticosteroids in a girl with bilateral optic neuritis]. / Respuesta a altas dosis de corticosteroides en una niña con neuritis óptica bilateral.

INTRODUCTION: Optic neuritis is rare in childhood. Frequently (35-52% of all cases depending on the series) they have, during their clinical course, foci of demyelination leading to the clinical picture of multiple sclerosis (MS). Since 1993, the optic neuritis study group has recommended treatment with high doses of corticosteroids, since this seemed to stop progression, improve long-term results and delay the appearance of MS. The course of our patient was better than we expected. CLINICAL CASE: A 10 year old prepubertal girl complained of progressive loss of vision and slight pain in the right eye for 26 days before admission to hospital. On examination there was obvious papillitis of the right ocular fundus with total loss of the pupillary light reflex, together with consensual hyporeflexia of the left eye. Study of the visual evoked potentials (VEP) showed that there was marked delay of the P-100 wave, and a lower amplitude in the right eye. Magnetic resonance imaging did not show any demyelinated focus. Serological testing for neurotropic viruses was negative. CONCLUSIONS: After the initial phase of intravenous treatment (third day) there was subjective recovery of vision and the pupillary light reflex returned. VEP studies showed marked recovery. Thirty days after treatment was started there was almost complete subjective and VEP recovery. This rapid progress, as compared to that of other paediatric cases published, suggests a mechanism involving decompression of the optic nerve.

Rett's syndrome: a neurophysiological study.

Thirteen girls with Rett's syndrome were evaluated with wakefulness and sleep polygraphic records and EMG studies. The main EEG findings were paroxysmal activity during sleep, multifocal in younger and unifocal in older girls, and "pseudorhythmic flattening". In the opinion of the authors, the changes with age are related to evolution of the disease or maturational changes of CNS. The affectation of the peripheral nervous system was demonstrated in almost half the patients.

Intoxication caused by ingestion of rape oil denatured with aniline.

One hundred seventy children, 16 months to 14 years of age, were intoxicated by the ingestion of rape oil denatured with aniline. The most frequent findings were fever, itch, adenopathy, hepatomegaly, and varied exanthems. A few weeks after the onset of the disease, more than a third of the patients experienced a syndrome similar to scleroderma. After more than nine months, three patients were found to be seriously impaired. Two girls died of measles contracted during the course of the intoxication. Prednisone caused dramatic and rapid symptomatic relief.