Homovanillic acid and 5-hydroxyindoleacetic acid levels in cerebrospinal fluid of patients with progressive myoclonus epilepsy

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Abstract
The possibility of disturbed dopamine and serotonin metabolism in progressive myoclonus epilepsy (PME) occurring in Finland (a type of PME without Lafora bodies) was examined. Both HVA [homovanillic acid] and 5-HIAA [5-hydroxyindoleacetic acid] basal concentrations in the CSF and their increase after oral probenecid administration were studied in 19 PME patients and in 19 age- and sex-matched control patients. The control patients had grand mal epilepsy but not myoclonus or ataxia. The HVA basal value was significantly reduced and that of 5-HIAA was also slightly reduced in the PME patients compared to the values of the epileptic controls or to those of 26 nonepileptic controls. HVA and 5-HIAA concentrations seemed to correlate with the PME severity. The most severely affected patients generally had the lowest values. After oral probenecid this trend was seen when the HVA and 5-HIAA increases were expressed per .mu.g CSF probenecid, i.e., the mildly affected PME group showed higher increases in response to probenecid than the most severely affected PME group. The PME patients had higher probenecid levels in the CSF than the epileptic controls.