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Published in: World Journal of Pediatrics 3/2018

01-06-2018 | Original Article

Clinical characteristics of two cohorts of infantile spasms: response to pyridoxine or topiramate monotherapy

Authors: Jiao Xue, Ping Qian, Hui Li, Ye Wu, Hui Xiong, Yue-Hua Zhang, Zhi-Xian Yang

Published in: World Journal of Pediatrics | Issue 3/2018

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Abstract

Background

Infantile spasms (IS) was an epileptic disease with varied treatment widely among clinicians. Here, we aimed to compare and analyze the clinical characteristics of IS response to pyridoxine or topiramate monotherapy (TPM control IS).

Methods

The clinical manifestations, treatment processes and outcomes were analyzed in 11 pyridoxine responsive IS and 17 TPM-control IS.

Results

Of the 11 patients with pyridoxine responsive IS, nine were cryptogenic/idiopathic. Age of seizure onset was 5.36 ± 1.48 months. Spasms were controlled within a week in most of the patients. At the last follow-up, EEG returned to normal in 8. Psychomotor development was normal in 6, mild delay in 3, severe delay in 2. Of the 17 patients with TPM-control IS, 10 were cryptogenic/idiopathic. The age of seizure onset was 5.58 ± 2.09 months. All patients were controlled within a month. At the last follow-up, EEG was normal in 10. Psychomotor development was normal in 8, mild delay in 5, severe delay in 4. Genetic analysis did not show any meaningful results.

Conclusions

The clinical characteristics and disease courses of pyridoxine responsive IS and TPM-control IS were similar, which possibly clued for a same pathogenic mechanism. Pyridoxine should be tried first in all IS patients, even in symptomatic cases. If patients were not responsive to pyridoxine, TPM could be tried.
Literature
1.
go back to reference West WJ. On a peculiar form of infantile convulsions. Lancet. 1841;1:724–5.CrossRef West WJ. On a peculiar form of infantile convulsions. Lancet. 1841;1:724–5.CrossRef
5.
go back to reference Ohtahara S, Yamatogi Y, Ohtsuka Y. Vitamin B(6) treatment of intractable seizures. Brain Dev. 2011;33:783–9.CrossRefPubMed Ohtahara S, Yamatogi Y, Ohtsuka Y. Vitamin B(6) treatment of intractable seizures. Brain Dev. 2011;33:783–9.CrossRefPubMed
6.
go back to reference Coursin DB. Convulsive seizures in infants with pyridoxine-deficiency diet. J Am Med Assoc. 1954;154:406–8.CrossRefPubMed Coursin DB. Convulsive seizures in infants with pyridoxine-deficiency diet. J Am Med Assoc. 1954;154:406–8.CrossRefPubMed
7.
8.
go back to reference Mills PB, Struys E, Jakobs C, Plecko B, Baxter P, Baumgartner M, et al. Mutations in antiquitin in individuals with pyridoxine-dependent seizures. Nat Med. 2006;12:307–9.CrossRefPubMed Mills PB, Struys E, Jakobs C, Plecko B, Baxter P, Baumgartner M, et al. Mutations in antiquitin in individuals with pyridoxine-dependent seizures. Nat Med. 2006;12:307–9.CrossRefPubMed
10.
go back to reference Gümüş H, Kumandaş S, Per H. Levetiracetam monotherapy in newly diagnosed cryptogenic West syndrome. Pediatr Neurol. 2007;37:350–3.CrossRefPubMed Gümüş H, Kumandaş S, Per H. Levetiracetam monotherapy in newly diagnosed cryptogenic West syndrome. Pediatr Neurol. 2007;37:350–3.CrossRefPubMed
12.
go back to reference Riikonen R. Recent advances in the pharmacotherapy of infantile spasms. CNS Drugs. 2014;28:279–90.CrossRefPubMed Riikonen R. Recent advances in the pharmacotherapy of infantile spasms. CNS Drugs. 2014;28:279–90.CrossRefPubMed
13.
go back to reference Ohtsuka Y, Ogino T, Asano T, Hattori J, Ohta H, Oka E. Long-term follow-up of vitamin B6-responsive West syndrome. Pediatr Neurol. 2000;23:202–6.CrossRefPubMed Ohtsuka Y, Ogino T, Asano T, Hattori J, Ohta H, Oka E. Long-term follow-up of vitamin B6-responsive West syndrome. Pediatr Neurol. 2000;23:202–6.CrossRefPubMed
14.
go back to reference Kong WJ, Zhang YJ, Gao Y, Liu X, Gao K, Xie H, et al. SCN8A mutations in Chinese children with early onset epilepsy and intellectual disability. Epilepsia. 2015;56:431–8.CrossRefPubMed Kong WJ, Zhang YJ, Gao Y, Liu X, Gao K, Xie H, et al. SCN8A mutations in Chinese children with early onset epilepsy and intellectual disability. Epilepsia. 2015;56:431–8.CrossRefPubMed
15.
go back to reference Ohtsuka Y, Matsuda M, Ogino T, Kobayashi K, Ohtahara S. Treatment of the West syndrome with high-dose pyridoxal phosphate. Brain Dev. 1987;9:418–21.CrossRefPubMed Ohtsuka Y, Matsuda M, Ogino T, Kobayashi K, Ohtahara S. Treatment of the West syndrome with high-dose pyridoxal phosphate. Brain Dev. 1987;9:418–21.CrossRefPubMed
16.
go back to reference Hosain SA, Merchant S, Solomon GE, Chutorian A. Topiramate for the treatment of infantile spasms. J Child Neurol. 2006;21:17–9.CrossRefPubMed Hosain SA, Merchant S, Solomon GE, Chutorian A. Topiramate for the treatment of infantile spasms. J Child Neurol. 2006;21:17–9.CrossRefPubMed
17.
go back to reference Tsuji T, Okumura A, Ozawa H, Ito M, Watanabe K. Current treatment of West syndrome in Japan. J Child Neurol. 2007;22:560–4.CrossRefPubMed Tsuji T, Okumura A, Ozawa H, Ito M, Watanabe K. Current treatment of West syndrome in Japan. J Child Neurol. 2007;22:560–4.CrossRefPubMed
18.
go back to reference Baxter P. Pyridoxine-dependent and pyridoxine-responsive seizures. Dev Med Child Neurol. 2001;43:416–20.CrossRefPubMed Baxter P. Pyridoxine-dependent and pyridoxine-responsive seizures. Dev Med Child Neurol. 2001;43:416–20.CrossRefPubMed
19.
go back to reference Güveli BT, Çokar Ö, Dörtcan N, Benbir G, Demirbilek V, Dervent A. Long-term outcomes in patients with West syndrome: an outpatient clinical study. Seizure. 2015;25:68–71.CrossRefPubMed Güveli BT, Çokar Ö, Dörtcan N, Benbir G, Demirbilek V, Dervent A. Long-term outcomes in patients with West syndrome: an outpatient clinical study. Seizure. 2015;25:68–71.CrossRefPubMed
20.
go back to reference Pavone P, Striano P, Falsaperla R, Pavone L, Ruggieri M. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013. Brain Dev. 2014;36:739–51.CrossRefPubMed Pavone P, Striano P, Falsaperla R, Pavone L, Ruggieri M. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013. Brain Dev. 2014;36:739–51.CrossRefPubMed
22.
go back to reference Stockler S, Plecko B, Gospe SM Jr, Coulter-Mackie M, Connolly M, van Karnebeek C, et al. Pyridoxine dependent epilepsy and antiquitin deficiency: clinical and molecular characteristics and recommendations for diagnosis, treatment and follow-up. Mol Genet Metab. 2011;104:48–60.CrossRefPubMed Stockler S, Plecko B, Gospe SM Jr, Coulter-Mackie M, Connolly M, van Karnebeek C, et al. Pyridoxine dependent epilepsy and antiquitin deficiency: clinical and molecular characteristics and recommendations for diagnosis, treatment and follow-up. Mol Genet Metab. 2011;104:48–60.CrossRefPubMed
Metadata
Title
Clinical characteristics of two cohorts of infantile spasms: response to pyridoxine or topiramate monotherapy
Authors
Jiao Xue
Ping Qian
Hui Li
Ye Wu
Hui Xiong
Yue-Hua Zhang
Zhi-Xian Yang
Publication date
01-06-2018
Publisher
Childrens Hospital, Zhejiang University School of Medicine
Published in
World Journal of Pediatrics / Issue 3/2018
Print ISSN: 1708-8569
Electronic ISSN: 1867-0687
DOI
https://doi.org/10.1007/s12519-018-0127-9

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