J Pediatr Neurol 2(1): 15-19 (2004)
Roberto H. Caraballo, Ricardo O. Cersósimo, Fernanda Garro, Karina Kesler, Natalio Fejerman
Servicio de Neurología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina
We report the results of patients with Down syndrome (DS) and West syndrome (WS) treated with vitamin B6 and discuss the mechanisms of pyridoxine in WS. Twenty patients with DS and WS were referred to our service for infantile spasms (IS) between February 1990 and December 2002. WS was diagnosed on the basis of clinical and electroencephalogram features. Thirteen patients were treated conservatively while seven patients (four girls and three boy) were treated according to the new alternative treatment scheme with vitamin B6. The dose of oral pyridoxine was between 200 and 400 mg/day (25-50 mg/kg/day). Five patients initiated the treatment with vitamin B6 for IS two weeks and two patients four weeks after starting to receive antiepileptic drugs. One of them, who initially received intravenous pyridoxine 200 mg/day during three days with a partial control of IS, responded well to oral vitamin B6 400 mg/day associated to valproic acid five months later. Mean age at the beginning of pyridoxine treatment was 9.5 months, ranging from 3 to 14 months. The time between the onset of spasms and vitamin B6 administration ranged from 2 to 4 weeks. Cessation of spasms was obtained within 2 weeks of treatment in four patients. Their electroencephalograms became normal as soon as the spasms disappeared. In four patients whose spasms ceased, pyridoxine was discontinued after 24 and 30 months and clinical spasms did not recur. These four children did not experience any other type of seizure after a mean time of follow-up of 6 years (range 1-10 years) without any type of treatment. In the fifth patient, cessation of spasms was obtained within four weeks the second time she received vitamin B6, five months after the first treatment. She still receives with pyridoxine 400 mg/day (40 mg/kg/day) after twelve months of follow up and has had no spasms since. Two patients did not respond well either to pyridoxine or to vigabatrin, adrenocorticotropic hormone, valproic acid or benzodiazepines. The patients had no adverse events during treatment with pyridoxine. We consider an oral dose of 200-400 mg/day (25-50 mg/kg/day) of pyridoxine either in monotherapy or combined with classic antiepleptic drugs to be the first choice of therapy in patients with IS and DS. Furthermore, patients who initially do not respond well to pyridoxine could be treated again and for a longer period.
Journal of Mental Deficiency Research 29(3): 233-240 (1985 Sep)
A double blind study of vitamin B6 in Down's syndrome infants: I. Clinical and biochemical results
Coleman, Mary et al.
Georgetown University School of Medicine
19 infants with Down's Syndrome participated in a double-blind study of the clinical effects of pharmacological doses of vitamin B6 administration, starting under 8 wks of age and continuing until 3 yrs of age. 10 Ss received the vitamin and 9 the placebo. No statistically significant differences were found between the 2 groups in mental age, height, weight, cranial circumference, or tongue protrusion. Vitamin B6 significantly elevated whole blood 5-hydroxytryptamine during the 1st yr. A study of side effects conducted on a larger open population of 400 Down's Syndrome patients (from infants to aged 12 yrs) found vitamin B6 to be relatively safe when administered over long periods of time, with photosensitive blisters as the major complication.
Journal of Mental Deficiency Research 29(3): 241-246 (1985 Sep)
A double blind study of vitamin B6 in Down's syndrome infants: II. Cortical auditory evoked potentials.
Frager, Joseph; Barnet, Ann; Weiss, Ira; Coleman, Mary Montefiore
Hospital & Medical Center, Dept of Medicine, New York, NY
Recorded cortical auditory evoked potentials (CAEPs) at 1 and at 3 yrs of age in 19 children with Down's Syndrome participating in a double-blind trial of vitamin B6 and placebo that was begun in early infancy and continued for 3 yrs. CAEPs have previously been shown to have abnormally high amplitude in Down's Syndrome patients. The CAEPs of the Ss in the B6 treated and placebo groups were compared. Only minor effects were found in the CAEPs recorded at 1 yr of age. At 3 yrs of age, however, comparison of the B6 treated group and the placebo group revealed significant differences in both amplitudes and latencies of CAEP components. Peak-to-peak amplitudes of prominent components were significantly lower in B6 treated Ss than in their placebo controls. Amplitude correlated in some cases with whole blood serotonin levels. Latencies for several prominent evoked peaks were significantly longer in B6 treated Ss. Findings suggest a difference in neurodevelopmental trajectories that seems to be a pharmacological effect of B6 administration. (17 ref)