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Early total corpus callosotomy in a patient with cryptogenic West syndrome
Seizure 22 (2013) 320–323
Contents lists available at SciVerse ScienceDirect
Seizure journal homepage: www.elsevier.com/locate/yseiz
Case report
Early total corpus callosotomy in a patient with cryptogenic West syndrome Yuka Suzuki a,*, Hiroshi Baba b, Keisuke Toda b, Tomonori Ono b, Mika Kawabe c, Mitsumasa Fukuda c a
Graduate School of Medicine Ehime University, Community Emergency Medicine, Japan National Hospital Organization Nagasaki Medicine Center, Neurosurgery Department, Japan c Graduate School of Medicine Ehime University, Pediatrics Department, Japan b
A R T I C L E I N F O
2. Case Article history: Received 26 September 2012 Received in revised form 9 January 2013 Accepted 11 January 2013 Keywords: Cryptogenic West syndrome Corpus callosotomy Epilepsy surgery
1. Introduction West syndrome is a generalized epileptic syndrome of infancy, that is characterized by clinical spasms and hypsarrhythmia and usually has an onset age before two years old. Children affected by West syndrome have a high risk of severe psychomotor developmental delay. Infants with West syndrome without identification of underlying neurological disorder after adequate investigations are classified as ‘‘cryptogenic’’ and usually have better prognoses than children with ‘‘symptomatic’’ West syndrome.1 Early seizure cessation, particularly among cryptogenic cases, may cure the disease and allow a good neurodevelopmental outcome.2 Corpus callosotomy was first introduced as a palliative treatment for epilepsy in 1940 and is performed in patients whose seizures are not amenable to surgical focal resection. West syndrome is often medically intractable and has features of ‘‘generalized’’ epilepsy, but corpus callosotomy has not been discussed widely as a treatment strategy. Given the catastrophic nature of West syndrome, an early indication for total corpus callosotomy may be appropriate. Here, we describe a case that was diagnosed as cryptogenic West syndrome, in which the epileptogenic focus was defined after total corpus callosotomy.
* Corresponding author at: Graduate School of Medicine Ehime University, Community Emergency Medicine, 454 Shitsukawa, Toon, Ehime 7910295, Japan. Tel.: +81 89 9605930; fax: +81 89 9605485. E-mail address: [email protected] (Y. Suzuki).
An 8 month-old boy was referred to our hospital following the appearance of repetitive spastic movements. He was born naturally at 38 weeks of gestation with no complications and reached all developmental milestones until 7 months-old. About one week before referral he started having clusters of 5–7 sudden, brief spastic movements, during which he dropped his head and raised both arms. He became unable to sit and lost his smile. His interictal waking and sleep EEG showed typical hypsarrhythmia consisting of random high-voltage slow waves and spikes. His ictal EEG showed voltage attenuation superimposed with bursts of low voltage, fast activity that was often contaminated with movements artifacts (Fig. 1A). MRI showed no abnormalities and other tests were also within normal limits. The patient was diagnosed with cryptogenic West syndrome. After VitB6 administration (40 mg/kg/day), adrenocorticotrophic hormone (ACTH) (0.0125 mg/kg/day = 0.5 IU/kg/day) was started. Because of several series of epileptic spasms, ACTH dosage was increased to 0.025 mg/kg/day and valproic acid (VPA) (40 mg/kg/ day; serum concentration 80 mg/dl) was added. Seizures ceased completely after five days and an EEG showed age-appropriate background activity without any epileptiform discharges. At one year old, the patient started having a series of similar epileptic spasms and he again showed developmental regression. His EEG showed typical hypsarrhythmia, but MRI and interictal and ictal ECD-single photon emission computed tomography (SPECT)findings were unremarkable (Fig. 2A-1). Increasing the VPA dose (serum concentration 120 mg/dl), nitrazepam, zonisamide, and lamotrigine were all tried with no response. At the age of 14 months, total callosotomy was performed at the National Hospital Organization Nagasaki Medical Center. After corpus callosotomy all spasms lateralized to the left side of the body. During seizures, he suddenly looked up and raised his left arm. Ictal EEG showed polyspikes and waves followed by brief voltage attenuation predominantly over the right hemisphere (Fig. 1B). Interictally, polyspike-wave discharges were expressed in the right fronto-central region. An ictal ECD-SPECT study demonstrated hyperperfusion in the right frontal lobe (Fig. 2A2). Interictal FDG-positron emission tomography (PET) showed hypometabolism in the right hemisphere (Fig. 2B). Based on these localized findings, right frontal resection was performed two months after corpus callosotomy. The resected lesion was
1059-1311/$ – see front matter ß 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.seizure.2013.01.009
Y. Suzuki et al. / Seizure 22 (2013) 320–323
321
Fig. 1. (A) Example of an ictal EEG recorded during preoperative EEG-video monitoring. The ictal pattern is characterized by diffuse voltage suppression superimposed with bursts of low voltage beta range activities. The EEG was recorded by 21 electrodes using the 10–20 system and is represented in monopolar reference. (B) Example of an ictal EEG recorded during routine EEG-video monitoring after corpus callosotomy. The ictal pattern is characterized by high voltage polyspike wave discharges followed by brief voltage attenuation over the right hemisphere. The EEG was recorded by 19 electrodes using the 10–20 system and is represented in monopolar reference. Arrowheads indicate times at which an ictal event was noted by observers.
determined by intraoperative electrocorticography. Surgical specimens showed preserved cortical lamination and prominent gliosis in the subcortical areas. Subsequently, he has been seizure-free for three years. Before corpus callosotomy at 16 months old his developmental age was 10 months, he had no language, showed autistic behavior, and his developmental quotient (DQ) was 62.5 on the Kinder Infant Development Scale. Six months after surgery, his behavior clearly changed and he was able to sit still for 30 min watching TV, speak several words, and follow simple verbal commands, although the DQ was only 66.7. Post surgically, he showed ‘‘catch-up’’ developmental progress, and his DQ improved to 72.7, 80.4, and 81.8 yearly over three years. At his current age of 4 years 7 month old, 3 years after surgery, his language is beyond the telegraphic stage with some Japanese grammatical errors. 3. Discussion Total corpus callosotomy revealed previously unperceivable seizure foci in our patient diagnosed with cryptogenic West
syndrome, and led to successful surgical lesional resection. Surgical treatment for symptomatic West syndrome is effective for controlling seizures and cessation of spasms, and early surgery for catastrophic epilepsy such as West syndrome has been shown to have positive effects on developmental outcomes.2 Structural abnormalities also strongly influence the prognosis in patients with West syndrome.1,3 Therefore, the importance of evaluation to determine resectable epileptogenic lesions in medically intractable West syndrome cannot be stressed enough. Corpus callosotomy is most effective in patients with atonic seizures with drop attacks, and has not been established as a standard surgical strategy for cryptogenic West syndrome. Clear improvements with corpus callosotomy also occur for other types of seizures, such as tonic seizures and secondarily generalized seizures.4 Pinard et al.5 first reported seizure reductions and developmental improvements in patients with post-West syndrome after corpus callosotomy. Baba and colleagues performed early total corpus callosotomy in patients with West syndrome without a clear resectable region, of whom 75% showed significant developmental improvement during three-year follow-up.6
322
Y. Suzuki et al. / Seizure 22 (2013) 320–323
Fig. 2. (A-1) Interictal ECD-single photon emission computed tomography (SPECT) performed prior to corpus callosotomy. Perfusion is symmetrically distributed without localizing findings. (A-2) Ictal and interictal ECD-SPECT after corpus callosotomy. Ictal ECD-SPECT showed hyperperfusion in the right frontal region (arrowheads) and interictal ECD-SPECT showed hypoperfusion in a similar region (small arrows). (B) Interictal FDG-positron emission tomography (PET). Interictal FDG PET showed cortical hypometabolism (large arrows) in right frontotemporal regions.
Patients with generalized epilepsy persisting after West syndrome can also benefit from complete callosotmy.7 These findings suggest that corpus callosotomy may ameliorate frequent generalized or rapidly secondarily generalized seizures in West syndrome. Traditionally, anterior two-thirds or total corpus callosotomy have been performed. Comparing these methods, total callosotomy showed better seizure reduction in patients after West syndrome,7 whereas only one of 13 patients after West syndrome benefited from anterior callosotomy and 12 required additional second stage surgery.7 Spencer reported that seizure types including tonic, atonic, and generalized tonic–clonic are markedly decreased or totally eliminated in 80–90% of patients after total corpus callosum disconnection, but in 50% or less after partial resection.5 Thus, patients with post-West syndrome showed better seizure control and behavioral improvement without disconnection syndrome after total and two-staged total corpus callosotomy, compared with anterior resection.5,8 Our patient showed a good first response to the standard dose of ACTH. Therefore, he was a good candidate for a second course of ACTH therapy. However, patients who relapse after the first course of ACTH therapy often show re-relapse after a second course.9,10 Seizure recurrence with as short an interval as that in our patient strongly suggested medically intractable seizures11 and the effective treatment window for West syndrome is limited because of its age dependency. Furthermore, a second course of ACTH can be given after surgery, if necessary. For all of these reasons, we selected one-step total corpus callosotomy for treatment of relapsed spams, so as not to lose the treatment window. Total corpus callosotomy was performed without major sequelae or neurological deficits.
Prior to corpus callosotomy, neither interictal and ictal EEG nor neuroimaging indicated a localized epileptic focus in our patient. It was only after disconnecting the corpus callosum that a confined epileptic focus was defined. Prior to corpus callosotomy, clinical presentation, EEG, interictal SPECT, and MRI all pointed to cryptogenic West syndrome. We did not perform ictal SPECT or PET and it is possible that these studies might have defined a localized focus without corpus callosotomy, and that lesionectomy itself would have been sufficient to control the seizures. Surgical specimens revealed prominent white matter gliosis and preserved cortical structures, and MRI may not detect this kind of dysplastic area. Pinard et al.5 found that bilateral hypsarrhythmia in West syndrome patients altered after total corpus callosotomy to become unilateral hypsarrhythmia with asymmetrical spasms. Three patients with medically intractable frequent generalized seizures were shown to have a regional ictal zone after corpus callosotomy.5 Thus, an absence of localized findings in EEG and neuroimaging does not imply the absence of epileptic foci in some patients, and we recommend though preoperative work-up including interictal SPECT or PET. The pathophysiology of developmental comorbidities in West syndrome has not been well established. It is also unclear whether early intervention and seizure cessation can prevent developmental regression.12 Several studies have shown that a short treatment lag is correlated with a more favorable developmental outcome in patients with both symptomatic and cryptogenic West syndrome.1,12 Some recommend early surgery before development of severe psychomotor delay.1,6,12 Our patient showed dramatic behavioral changes and began to speak some words several months after surgery. This observation supports the view that early
Y. Suzuki et al. / Seizure 22 (2013) 320–323
seizure cessation leads to better developmental prognosis in some patients with West syndrome. Total corpus callosotomy in our patient revealed previously undetectable epileptogenic foci, and subsequent resection improved his seizures and developmental outcome. West syndrome has a catastrophic nature with frequent generalized or rapidly secondarily generalized seizures and many patients suffer severe developmental delays. In light of this finding, we recommend consideration of total corpus callosotomy in patients with cryptogenic West syndrome that is medically intractable or shows seizure-recurrence without much treatment lag. Failure of initial treatment and early seizure relapse may indicate focal epileptogenic foci, in such cases we recommend performing neurofunctional studies such as PET and SPECT for preoperative evaluation. We also advocate the importance of repeat evaluation after callosotomy. Preoperatively, the absence of localized findings in neuroimaging and functional imaging may not mean the absence of resectable epileptic foci in patients with West syndrome. Conflict of interest None of the authors has any conflict of interest to disclose. We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.
323
References 1. Riikonen RS. Favourable prognostic factors with infantile spasms. European Journal of Paediatric Neurology 2010;14:13–8. 2. Jonas R, Asarnow RF, LoPresti C, Yudovin S, Koh S, Wu JY, et al. Surgery for symptomatic infant-onset epileptic encephalopathy with and without infantile spasms. Neurology 2005;64:746–50. 3. Osborne JP, Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, et al. The underlying etiology of infantile spasms (West syndrome): information from the United Kingdom Infantile Spasms Study (UKISS) on contemporary causes and their classification. Epilepsia 2010;51:2168–74. 4. Spencer SS. Corpus callosum section and other disconnection procedures for medically intractable epilepsy. Epilepsia 1988;28:S85–99. 5. Pinard JM, Delalande O, Plouin P, Dulac O. Callosotomy in West syndrome suggests a cortical origin of hypsarrhythmia. Epilepsia 1993;34:780–7. 6. Baba H, Ono T, Toda K, Baba S, Ono K. Surgical outcome of corpus callosotomy for intractable epilepsy with West syndrome. Proceedings of 62nd American Epilepsy Society Annual Meeting. 2008. Abst. 2.282. 7. Pinard JM, Delalande O, Chiron C, Soufflet C, Plouin P, Kim Y, et al. Callosotomy for epilepsy after West syndrome. Epilepsia 1999;12:1727–34. 8. Clarke DF, Wheless JW, Chacon MM, Breier J, Koenig MK, McManis M, et al. Corpus callosotomy: a palliative therapeutic technique may help identify resectable epileptogenic foci. Seizure 2007;16:545–53. 9. Fujii A, Oguni H, Hirano Y, Shioda M, Osawa M. A long-term, clinical study on symptomatic infantile spasms with focal features. Brain and Development 2012. Epub ahead of print. 10. Okanishi T, Sugiura C, Satio Y, Maegaki Y, Ohno K, Togari H. Long-term weekly ACTH therapy for relapsed West syndrome. Pediatric Neurology 2008;38:445–9. 11. Panzica F, Binelli S, Canafoglia L, Casazza M, Freri E, Granata T, et al. ICTAL EEG fast activity in West syndrome: from onset to outcome. Epilepsia 2007;48:2101–10. 12. Karvelas G, Lortie A, Scantlebury MH, Duy PT, Cossette P, Carmant L. A retrospective study on aetiology based outcome of infantile spasms. Seizure 2009;18:197–201.
Contents lists available at SciVerse ScienceDirect
Seizure journal homepage: www.elsevier.com/locate/yseiz
Case report
Early total corpus callosotomy in a patient with cryptogenic West syndrome Yuka Suzuki a,*, Hiroshi Baba b, Keisuke Toda b, Tomonori Ono b, Mika Kawabe c, Mitsumasa Fukuda c a
Graduate School of Medicine Ehime University, Community Emergency Medicine, Japan National Hospital Organization Nagasaki Medicine Center, Neurosurgery Department, Japan c Graduate School of Medicine Ehime University, Pediatrics Department, Japan b
A R T I C L E I N F O
2. Case Article history: Received 26 September 2012 Received in revised form 9 January 2013 Accepted 11 January 2013 Keywords: Cryptogenic West syndrome Corpus callosotomy Epilepsy surgery
1. Introduction West syndrome is a generalized epileptic syndrome of infancy, that is characterized by clinical spasms and hypsarrhythmia and usually has an onset age before two years old. Children affected by West syndrome have a high risk of severe psychomotor developmental delay. Infants with West syndrome without identification of underlying neurological disorder after adequate investigations are classified as ‘‘cryptogenic’’ and usually have better prognoses than children with ‘‘symptomatic’’ West syndrome.1 Early seizure cessation, particularly among cryptogenic cases, may cure the disease and allow a good neurodevelopmental outcome.2 Corpus callosotomy was first introduced as a palliative treatment for epilepsy in 1940 and is performed in patients whose seizures are not amenable to surgical focal resection. West syndrome is often medically intractable and has features of ‘‘generalized’’ epilepsy, but corpus callosotomy has not been discussed widely as a treatment strategy. Given the catastrophic nature of West syndrome, an early indication for total corpus callosotomy may be appropriate. Here, we describe a case that was diagnosed as cryptogenic West syndrome, in which the epileptogenic focus was defined after total corpus callosotomy.
* Corresponding author at: Graduate School of Medicine Ehime University, Community Emergency Medicine, 454 Shitsukawa, Toon, Ehime 7910295, Japan. Tel.: +81 89 9605930; fax: +81 89 9605485. E-mail address: [email protected] (Y. Suzuki).
An 8 month-old boy was referred to our hospital following the appearance of repetitive spastic movements. He was born naturally at 38 weeks of gestation with no complications and reached all developmental milestones until 7 months-old. About one week before referral he started having clusters of 5–7 sudden, brief spastic movements, during which he dropped his head and raised both arms. He became unable to sit and lost his smile. His interictal waking and sleep EEG showed typical hypsarrhythmia consisting of random high-voltage slow waves and spikes. His ictal EEG showed voltage attenuation superimposed with bursts of low voltage, fast activity that was often contaminated with movements artifacts (Fig. 1A). MRI showed no abnormalities and other tests were also within normal limits. The patient was diagnosed with cryptogenic West syndrome. After VitB6 administration (40 mg/kg/day), adrenocorticotrophic hormone (ACTH) (0.0125 mg/kg/day = 0.5 IU/kg/day) was started. Because of several series of epileptic spasms, ACTH dosage was increased to 0.025 mg/kg/day and valproic acid (VPA) (40 mg/kg/ day; serum concentration 80 mg/dl) was added. Seizures ceased completely after five days and an EEG showed age-appropriate background activity without any epileptiform discharges. At one year old, the patient started having a series of similar epileptic spasms and he again showed developmental regression. His EEG showed typical hypsarrhythmia, but MRI and interictal and ictal ECD-single photon emission computed tomography (SPECT)findings were unremarkable (Fig. 2A-1). Increasing the VPA dose (serum concentration 120 mg/dl), nitrazepam, zonisamide, and lamotrigine were all tried with no response. At the age of 14 months, total callosotomy was performed at the National Hospital Organization Nagasaki Medical Center. After corpus callosotomy all spasms lateralized to the left side of the body. During seizures, he suddenly looked up and raised his left arm. Ictal EEG showed polyspikes and waves followed by brief voltage attenuation predominantly over the right hemisphere (Fig. 1B). Interictally, polyspike-wave discharges were expressed in the right fronto-central region. An ictal ECD-SPECT study demonstrated hyperperfusion in the right frontal lobe (Fig. 2A2). Interictal FDG-positron emission tomography (PET) showed hypometabolism in the right hemisphere (Fig. 2B). Based on these localized findings, right frontal resection was performed two months after corpus callosotomy. The resected lesion was
1059-1311/$ – see front matter ß 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.seizure.2013.01.009
Y. Suzuki et al. / Seizure 22 (2013) 320–323
321
Fig. 1. (A) Example of an ictal EEG recorded during preoperative EEG-video monitoring. The ictal pattern is characterized by diffuse voltage suppression superimposed with bursts of low voltage beta range activities. The EEG was recorded by 21 electrodes using the 10–20 system and is represented in monopolar reference. (B) Example of an ictal EEG recorded during routine EEG-video monitoring after corpus callosotomy. The ictal pattern is characterized by high voltage polyspike wave discharges followed by brief voltage attenuation over the right hemisphere. The EEG was recorded by 19 electrodes using the 10–20 system and is represented in monopolar reference. Arrowheads indicate times at which an ictal event was noted by observers.
determined by intraoperative electrocorticography. Surgical specimens showed preserved cortical lamination and prominent gliosis in the subcortical areas. Subsequently, he has been seizure-free for three years. Before corpus callosotomy at 16 months old his developmental age was 10 months, he had no language, showed autistic behavior, and his developmental quotient (DQ) was 62.5 on the Kinder Infant Development Scale. Six months after surgery, his behavior clearly changed and he was able to sit still for 30 min watching TV, speak several words, and follow simple verbal commands, although the DQ was only 66.7. Post surgically, he showed ‘‘catch-up’’ developmental progress, and his DQ improved to 72.7, 80.4, and 81.8 yearly over three years. At his current age of 4 years 7 month old, 3 years after surgery, his language is beyond the telegraphic stage with some Japanese grammatical errors. 3. Discussion Total corpus callosotomy revealed previously unperceivable seizure foci in our patient diagnosed with cryptogenic West
syndrome, and led to successful surgical lesional resection. Surgical treatment for symptomatic West syndrome is effective for controlling seizures and cessation of spasms, and early surgery for catastrophic epilepsy such as West syndrome has been shown to have positive effects on developmental outcomes.2 Structural abnormalities also strongly influence the prognosis in patients with West syndrome.1,3 Therefore, the importance of evaluation to determine resectable epileptogenic lesions in medically intractable West syndrome cannot be stressed enough. Corpus callosotomy is most effective in patients with atonic seizures with drop attacks, and has not been established as a standard surgical strategy for cryptogenic West syndrome. Clear improvements with corpus callosotomy also occur for other types of seizures, such as tonic seizures and secondarily generalized seizures.4 Pinard et al.5 first reported seizure reductions and developmental improvements in patients with post-West syndrome after corpus callosotomy. Baba and colleagues performed early total corpus callosotomy in patients with West syndrome without a clear resectable region, of whom 75% showed significant developmental improvement during three-year follow-up.6
322
Y. Suzuki et al. / Seizure 22 (2013) 320–323
Fig. 2. (A-1) Interictal ECD-single photon emission computed tomography (SPECT) performed prior to corpus callosotomy. Perfusion is symmetrically distributed without localizing findings. (A-2) Ictal and interictal ECD-SPECT after corpus callosotomy. Ictal ECD-SPECT showed hyperperfusion in the right frontal region (arrowheads) and interictal ECD-SPECT showed hypoperfusion in a similar region (small arrows). (B) Interictal FDG-positron emission tomography (PET). Interictal FDG PET showed cortical hypometabolism (large arrows) in right frontotemporal regions.
Patients with generalized epilepsy persisting after West syndrome can also benefit from complete callosotmy.7 These findings suggest that corpus callosotomy may ameliorate frequent generalized or rapidly secondarily generalized seizures in West syndrome. Traditionally, anterior two-thirds or total corpus callosotomy have been performed. Comparing these methods, total callosotomy showed better seizure reduction in patients after West syndrome,7 whereas only one of 13 patients after West syndrome benefited from anterior callosotomy and 12 required additional second stage surgery.7 Spencer reported that seizure types including tonic, atonic, and generalized tonic–clonic are markedly decreased or totally eliminated in 80–90% of patients after total corpus callosum disconnection, but in 50% or less after partial resection.5 Thus, patients with post-West syndrome showed better seizure control and behavioral improvement without disconnection syndrome after total and two-staged total corpus callosotomy, compared with anterior resection.5,8 Our patient showed a good first response to the standard dose of ACTH. Therefore, he was a good candidate for a second course of ACTH therapy. However, patients who relapse after the first course of ACTH therapy often show re-relapse after a second course.9,10 Seizure recurrence with as short an interval as that in our patient strongly suggested medically intractable seizures11 and the effective treatment window for West syndrome is limited because of its age dependency. Furthermore, a second course of ACTH can be given after surgery, if necessary. For all of these reasons, we selected one-step total corpus callosotomy for treatment of relapsed spams, so as not to lose the treatment window. Total corpus callosotomy was performed without major sequelae or neurological deficits.
Prior to corpus callosotomy, neither interictal and ictal EEG nor neuroimaging indicated a localized epileptic focus in our patient. It was only after disconnecting the corpus callosum that a confined epileptic focus was defined. Prior to corpus callosotomy, clinical presentation, EEG, interictal SPECT, and MRI all pointed to cryptogenic West syndrome. We did not perform ictal SPECT or PET and it is possible that these studies might have defined a localized focus without corpus callosotomy, and that lesionectomy itself would have been sufficient to control the seizures. Surgical specimens revealed prominent white matter gliosis and preserved cortical structures, and MRI may not detect this kind of dysplastic area. Pinard et al.5 found that bilateral hypsarrhythmia in West syndrome patients altered after total corpus callosotomy to become unilateral hypsarrhythmia with asymmetrical spasms. Three patients with medically intractable frequent generalized seizures were shown to have a regional ictal zone after corpus callosotomy.5 Thus, an absence of localized findings in EEG and neuroimaging does not imply the absence of epileptic foci in some patients, and we recommend though preoperative work-up including interictal SPECT or PET. The pathophysiology of developmental comorbidities in West syndrome has not been well established. It is also unclear whether early intervention and seizure cessation can prevent developmental regression.12 Several studies have shown that a short treatment lag is correlated with a more favorable developmental outcome in patients with both symptomatic and cryptogenic West syndrome.1,12 Some recommend early surgery before development of severe psychomotor delay.1,6,12 Our patient showed dramatic behavioral changes and began to speak some words several months after surgery. This observation supports the view that early
Y. Suzuki et al. / Seizure 22 (2013) 320–323
seizure cessation leads to better developmental prognosis in some patients with West syndrome. Total corpus callosotomy in our patient revealed previously undetectable epileptogenic foci, and subsequent resection improved his seizures and developmental outcome. West syndrome has a catastrophic nature with frequent generalized or rapidly secondarily generalized seizures and many patients suffer severe developmental delays. In light of this finding, we recommend consideration of total corpus callosotomy in patients with cryptogenic West syndrome that is medically intractable or shows seizure-recurrence without much treatment lag. Failure of initial treatment and early seizure relapse may indicate focal epileptogenic foci, in such cases we recommend performing neurofunctional studies such as PET and SPECT for preoperative evaluation. We also advocate the importance of repeat evaluation after callosotomy. Preoperatively, the absence of localized findings in neuroimaging and functional imaging may not mean the absence of resectable epileptic foci in patients with West syndrome. Conflict of interest None of the authors has any conflict of interest to disclose. We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.
323
References 1. Riikonen RS. Favourable prognostic factors with infantile spasms. European Journal of Paediatric Neurology 2010;14:13–8. 2. Jonas R, Asarnow RF, LoPresti C, Yudovin S, Koh S, Wu JY, et al. Surgery for symptomatic infant-onset epileptic encephalopathy with and without infantile spasms. Neurology 2005;64:746–50. 3. Osborne JP, Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, et al. The underlying etiology of infantile spasms (West syndrome): information from the United Kingdom Infantile Spasms Study (UKISS) on contemporary causes and their classification. Epilepsia 2010;51:2168–74. 4. Spencer SS. Corpus callosum section and other disconnection procedures for medically intractable epilepsy. Epilepsia 1988;28:S85–99. 5. Pinard JM, Delalande O, Plouin P, Dulac O. Callosotomy in West syndrome suggests a cortical origin of hypsarrhythmia. Epilepsia 1993;34:780–7. 6. Baba H, Ono T, Toda K, Baba S, Ono K. Surgical outcome of corpus callosotomy for intractable epilepsy with West syndrome. Proceedings of 62nd American Epilepsy Society Annual Meeting. 2008. Abst. 2.282. 7. Pinard JM, Delalande O, Chiron C, Soufflet C, Plouin P, Kim Y, et al. Callosotomy for epilepsy after West syndrome. Epilepsia 1999;12:1727–34. 8. Clarke DF, Wheless JW, Chacon MM, Breier J, Koenig MK, McManis M, et al. Corpus callosotomy: a palliative therapeutic technique may help identify resectable epileptogenic foci. Seizure 2007;16:545–53. 9. Fujii A, Oguni H, Hirano Y, Shioda M, Osawa M. A long-term, clinical study on symptomatic infantile spasms with focal features. Brain and Development 2012. Epub ahead of print. 10. Okanishi T, Sugiura C, Satio Y, Maegaki Y, Ohno K, Togari H. Long-term weekly ACTH therapy for relapsed West syndrome. Pediatric Neurology 2008;38:445–9. 11. Panzica F, Binelli S, Canafoglia L, Casazza M, Freri E, Granata T, et al. ICTAL EEG fast activity in West syndrome: from onset to outcome. Epilepsia 2007;48:2101–10. 12. Karvelas G, Lortie A, Scantlebury MH, Duy PT, Cossette P, Carmant L. A retrospective study on aetiology based outcome of infantile spasms. Seizure 2009;18:197–201.