Twenty-two patients with absence seizures and other seizure types were treated with valproic acid and followed up for 1 year. Results were excellent with more than 75% improvement in 80% of patients with absence seizures, in 40% of those with tonic-clonic seizures, in all of those with myoclonic seizures, and in 43% of those with partial seizures. Fifty-seven percent of the patients had more than a 75% reduction in the total number of paroxysmal spike-wave discharges, and 62% had more than a 75% reduction in the number of spike-wave discharges lasting longer than 3 seconds. Photosensitivity and activation by hyperventilation decreased. More patients achieved good EEG control in 1 year than in 10 weeks.

Twenty-five patients with absence seizures were treated with valproic acid in doses from 17 to 62.5 mg per kilogram per day. Nineteen patients experienced reduction of spike and wave discharges; in 11 it was greater than 75 percent. Twenty-one patients had a reduction of the total time of spike and wave discharge. Four patients had increase of spike and wave discharges. Nineteen patients had fewer absence seizures. There was no correlation between plasma concentration of valproic acid and EEG change, but clinical improvement occurred when plasma levels of valproic acid reached 50 to 60 microgram per milliliter.

Chronic valproate therapy induces symptomatic tremor in about 10% of patients. We studied the effects of propranolol, amantadine, diphenhydramine, benztropine, and cyproheptadine on these tremors in 19 patients by using serial accelerometric recordings. Propranolol was clearly the most therapeutic. Amantadine was moderately effective, but cyproheptadine, diphenhydramine, and benztropine gave little or no relief.

No abstract

A 68-year-old male, diagnosed 1 year previously as having myelofibrosis, developed hemolysis, red cell inclusions, and 37% Hb H. The alpha/beta globin synthetic ratio for circulating reticulocytes, determined by 3H-leucine incorporation and globin chain separation by carboxymethylcellulose chromatography in urea, was 0.049. When total RNA was purified from peripheral blood cells and translated in a wheat germ cell-free translation system, the alpha/beta ratio of the translation products was 0.26, indicating mRNA as a major cause of the globin synthetic imbalance. This study demonstrates that myelofibrosis is one setting in which acquired Hb H disease occurs; that the synthetic imbalance may be extreme; and that it can be associated with an imbalance in the activities of specific globin mRNAs.