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Visual abnormalities and occipital EEG discharges: risk factors for West syndrome.

Seventeen infants (7 boys and 10 girls) with visual abnormalities (such as poor tracking and following and nystagmoid eye movement) and occipital slow activities with irregular polyspikes on EEG were studied. Two thirds of the children showed development of West syndrome with hypsarrhythmia on follow-up EEG. In 1 child, hypsarrhythmia was evident on the follow-up EEG, but no clinical seizures occurred. Because most of the children in our series developed West syndrome, patients with visual abnormalities associated with occipital EEG discharges in early infancy may be at high risk for West syndrome. Occipital abnormalities in early infancy may relate to the occurrence of West syndrome.

Child, Preschool↗

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A human B-cell CLL model established by transplantation of JOK-1 cells into SCID mice and an anti-leukemia efficacy of fludarabine phosphate.

The present study was carried out to establish a human chronic lymphocytic leukemia (CLL) mouse model by transplantation of a JOK-1 human CLL cell line into SCID (severe combined immunodeficient) mice and to examine anti-leukemic effects of fludarabine phosphate, a prodrug of 9-beta-D-arabinofuranosyl-2-fluoroadenine (2F-ara-A). In vitro cytotoxic screening pattern of 2F-ara-A differed from those of other anticancer agents. Intraperitoneal inoculation with JOK-1 cells in SCID mice allowed the cells to infiltrate into a variety of organs including the liver and thymus, and resulted in the death of the mice with a median survival time of 29.5 days, associated with hepatomegaly, splenomegaly and enlarged lymph nodes. The ascitic cells expressing the human B-lymphocytic cell surface antigen CD19 actually grew after a latent period of 15 days. In this model, twice daily administration of fludarabine phosphate at a dose of 135 mg/kg for 5 days prolonged the survival time of the mice for considerably longer period than once-a-day treatment. Fludarabine phosphate in the doubled course of twice daily increased life span of 32.9%, which was in a similar range to that of doxorubicin. Thus, intraperitoneal inoculation of the human JOK-1 CLL cells into SCID mice seems to serve as an animal model potentially for human leukemia, suggesting that transplantation and subsequent infiltration processes of human CLL cells is useful measures to explore mechanistic aspects for drug-induced modulation of the tumor progression.

Animals↗