Neurocase, 2015 Vol. 21, No. 2, 144–147, http://dx.doi.org/10.1080/13554794.2013.878724
Exceptional verbal intelligence after hemispherotomy in a child with Rasmussen encephalitis Catherine Grosmaitrea,b,c, Isabelle Jambaquéa,b,c, Georg Dorfmullera,b,c,d, Sebastian Rodrigoa,b,c, Dorothée Villee, Olivier Delalanded and Christine Bulteaua,b,c,d* a Inserm U663, Paris, France; bPRES Sorbonne Paris Cité, University Paris Descartes, France; cCEA, Gif sur Yvette, France; Fondation Ophtalmologique A. de Rothschild, Pediatric Neurosurgery Department, 75019 Paris, France; eHôpital Femme Mère Enfant Neuropediatric Department, 69677 Bron, France
d
(Received 3 April 2013; accepted 29 October 2013) We report a longitudinal case study of a left-handed girl who underwent left hemispherotomy at 7 years for Rasmussen encephalitis (RE). Presurgical evaluation showed mild hemiparesis, no visual defect, and light neuropsychological impairment with short-term memory weakness. Language fMRI showed a right hemispheric dominance. Postoperatively, the patient exhibited right hemiplegia and hemianopsia but preserved intellectual capacities. She became seizure-free, and antiepileptic medication was discontinued. Long-term follow-up showed very high verbal intelligence at 11 years of age (VCI of 155) and improvement in working memory as well as language and reading abilities. Furthermore, a significant visuoverbal discrepancy became increasingly pronounced. Thus, early surgical treatment of epilepsy avoided the global cognitive deterioration usually associated with RE. Finally, such a high level of verbal functioning combined with low spatial reasoning with a single hemisphere provides additional information on the neurocognitive profile of children with RE after hemispherotomy. Keywords: gifted child; hemispherotomy; Rasmussen encephalitis
Rasmussen encephalitis (RE) is a progressive inflammatory epileptic encephalopathy, characterized by partial intractable epilepsy, progressive unilateral hemispheric atrophy associated with motor and cognitive decline (Bahi-Buisson et al., 2007; Bien et al., 2005). Immunosuppressive and immunomodulatory interventions, as well as antiepileptic drugs (AEDs), have beneficial effects at an early stage but are not effective to control seizure in advanced disease. Hemispherotomy (H) is then recommended since around 60–90% of patients are subsequently seizure-free (Cross et al., 2006; Delalande et al., 2007). Cognitive functions seem to be globally stable after H (Bayard & Lassonde, 2001; Pulsifer et al., 2004), and etiologies do not have any effect on seizure outcome (Jonas et al., 2004). Delalande et al. (2007) found, however, a correlation between preoperative delay and the Vineland Adaptive Behavior score in a large series with various etiologies (developmental, acquired, or progressive). Children with a longer duration of seizures had lower performances at long-term outcome. Classically, H can be considered when hemiplegia, hemianopsia, and both language and cognitive deficits are severe and will not be aggravated by surgery. However, while H is the only way to cure RE, the crucial decision is when it should be done in order to avoid global mental deterioration. We report a 5-year longitudinal case study of a left-handed girl with RE who underwent early left H in the course of the *Corresponding author. Email: [email protected] © 2014 Taylor & Francis
disease. Strikingly, we observed exceptional verbal intelligence in this child with a single hemisphere. Our case provides additional information on outcome of the neurocognitive profile after H in children with RE. Case report Patient history MI is a left-handed, 11-year-old girl who suffered from left hemispheric RE. She comes from a monolingual Frenchspeaking family, with high potential in siblings and parents, had normal developmental milestones, behavior, and schooling until the age of 5 years when she experienced partial seizures (collapse of her right leg leading to falls). The initial MRI exhibited a focal hyperintense T2 signal over the left centro-parietal region. Other types of partial epileptic seizures occurred, and the following MRI revealed a moderate unilateral left hemispheric atrophy and a hypersignal in the insular area in favor of RE. Steroids were administrated and led to epileptic and motor improvement. During this period, MI had normal schooling and learned to read. At the age of 6.5 years, MI presented continuous partial epilepsy and right hemiparesis increased. Presurgical video EEG registered many types of seizures, which onset over the left hemisphere, with multifocal spikes and slow waves during interictal activity and no abnormality over the right
Neurocase
Figure 1. Preoperative language fMRI showing right hemispheric lateralization. Top line: activations in the right temporal gyrus during receptive tasks (left: word repetition; right: sentence listening). Bottom line: activations in the right inferior frontal gyrus and Supplementary Motor Area (SMA) during expressive tasks (left: word generation; right: sentence generation). [To view this figure in color, please see the online version of this Journal.]
hemisphere. She was treated by clobazam, oxcarbazepine, levetiracetam, and stiripentol and corticoids but developed Cushing syndrome. Whatever the treatment, seizures occurred daily and motor deficit increased. She was referred to our institution for left H according to the vertical parasagittal surgical procedure (Delalande et al., 2007). Preoperative evaluation showed moderate right hemiparesis with motor heminegligence; she was unable to attend school due to severe epileptic seizures. Visual field remained normal. Language fMRI showed right hemispheric lateralization networks during sentence listening, semantic word generation, and verb generation (Figure 1). Neuropsychological evaluation showed slight impairment in working memory but no intellectual disabilities and normal reading abilities. Left H was performed at the age of 6 years 11 months and was followed by a right hemianopsia and a right hemiplegia. Oral language and reading abilities remained stable. The child became seizure-free and AED were stopped (Engel’s Class I).
Neuropsychological evaluation Table 1 summarizes the neuropsychological findings of presurgical and postoperative assessment. Informed patient and parent consent was obtained for participation in neuropsychological evaluations and reassessment for identifying postoperative changes.
145
Verbal and nonverbal intellectual functions were evaluated pre- and postoperatively on the Wechsler scale. At presurgery, general intellectual assessment revealed a very high verbal comprehension index (VCI of 120), although the perceptual reasoning index (PRI) was average. After surgery, a significant visuoverbal discrepancy appeared and became increasingly pronounced (36 points at 0.8 years postoperative, 66 points at 2.9 years, and 71 points at 4.2 years). In fact, MI exhibited exceptional verbal intelligence well above the norm (superior or gifted range), combined with low spatial reasoning. MI obtained an exceptional score of 19 on the vocabulary subtest during all the follow-up while marked progress on comprehension and similarities subtests was also observed. Working memory index and processing speed index evidenced a subnormal or low average neuropsychological condition presurgically. After surgery, there was marked improvement in the working memory index and “Digit span” went up dramatically after surgery. Speed processing decreased to the lower normal range both before and after surgery. Language assessment comprised the expressive and receptive language tasks of the NEPSY (Korkman, Kirk, & Kemp, 2003) and the BILO (Bilan Informatisé du Langage Oral) (Khomsi, Khomsi, Pasquet, & ParbeauGueno, 2007). Presurgical evaluation showed efficient verbal abilities except on the rapid naming task. After surgery, language performances improved with time and MI performed well on all verbal tasks. The French reading test L’Alouette (Lefabvrais, 1967) was administered to determine a reading age. Reading performances were well above normal during follow-up. We evaluated spatial perception using the NEPSY Arrows and Visual attention subtests (Korkman et al., 2003) and the Rey figure. MI showed no evidence of spatial perception deficit but had slight visual attention problems and poor visual memory. The Child Behavioral Checklist (Achenbach, 1991) provided information about behavioral and emotional adaptation. MI did not exhibit any behavioral or social impairment during follow-up. Discussion Our study concerns a child who underwent left H for RE with slight neuropsychological impairment in the preoperative course and was followed up for 5 years. The preoperative cognitive profile may be due to the severity of epilepsy and progressive encephalopathy in RE. She experienced at least five seizures/day (right hemibody clonic seizures) with continuous partial epilepsy of the right hand and was treated by steroids, levetiracetam, clobazam, stiripentol, and carbamazepine. Interictal
146 Table 1.
C. Grosmaitre et al. Results of the prospective neuropsychological assessment.
Test Age (years)
Presurgical assessment 6.8
Postsurgery 1 (+1 year) 8
Postsurgery 2 (+2.1 years)
Postsurgery 3 (+4.1 years)
9.8
11.1
Measures of intelligence outcome WISC IV (Wechsler Intelligence Scale for Children) Verbal comprehension index (VCI) 120 Vocabulary* 19 Similarities* 12 Comprehension* 9 Perceptual reasoning index (PRI) 109 Block design* 11 Matrix reasoning* 12 Picture concepts* 11 Working memory index (WMI) 85 Digit span* 4 Letter-number sequencing* 11 Processing speed index (PSI) 81 Coding* 6 Symbol search* 7
138 19 13 16 102 11 9 11
154 19 19 17 88 10 7 8 106 11 11 81 8 5
155 19 19 18 84 9 6 8 115 14 11 73 6 4
13 14 13 12
12
Measures of language outcome NEPSY (Korkman) Verbal fluency* Comprehension of instructions* Phonological process* Rapid naming* BILO (Khomsi) Oral comprehension§ Lexical judgment§ Syntactic judgment§ Words Repetition§ Picture naming§ Syntactic drafting§ Reading age (Lefabvrais)
11 9 10 6
14
−0.16 −0.11 +1.13 +0.62 +1.34 +1.68 8 years
8.6 years
12 10
11
+1.29 +0.22 +0.83 +0.82 +1.89 +1.39 12.4 years
Measures of visuospatial outcome NEPSY (Korkman) Arrow – orientation perception* Visual attention* Rey-Osterrieth Copy of the Complex Figure§
−05
10 7
9
+0.88
+1.11
52 32 48
59 42 57
Measures of behavior outcome CBCL (Achenbach)# Internalizing Externalizing Total
59 44 54
Notes: BILO: oral comprehension (efficiency of comprehension strategies, in particular inferential), lexical judgment (precision of the semantic, lexical representations, nouns and verbs), syntactic judgment (quality of the morphosyntax in reception and sensitivity to the linguistic standard), word repetition (quality of the phonology in production), picture naming (lexicon in production: availability of nouns and verbs), and syntactic drafting (production of statements: morphosyntactic skill in production). *Standard scores. § Z scores. #Achenbach: a T score equal to or above 65 on any of the subscales is considered to denote pathology.
abnormalities and ictal EEG discharges were limited to the right abnormalities and did not spread to the contralateral hemisphere. Neuropsychological follow-up showed an
exceptional intellectual outcome (VCI of 155) with an increase in verbal functions in the upper range within the postoperative course.
Neurocase H cured epilepsy, and all antiepileptic drugs were withdrawn within the six postoperative months. In fact, fMRI showed right language dominance and the decision to perform surgery as soon as possible was uncontroversial. Furthermore, her left H afforded seizure control and avoided any interference of the epileptic hemisphere on her dominant right hemisphere. Preoperatively, working memory index and processing speed index were in the low average range and there was a discrepancy between verbal and nonverbal efficiency. Presurgically, she had a VCI of 120 with 19 in vocabulary, which is part of crystallized intelligence. Similarities and comprehension, linked to fluid intelligence, were weaker and progressed much more after surgery. MI’s verbal working memory improved after hemispherotomy, which may contribute to high verbal proficiency. On the other hand, speed processing remained low, but MI had right hemiplegia and visual hemianopsia, a neurological condition that can be associated with poor oculomotor performance and lower attention resources. Finally, MI’s postoperative intellectual profile is characterized by a marked visuoverbal discrepancy with poor perceptive reasoning index that stagnated during followup. There is evidence of the preservation of MI’s verbal skills combined with low spatial reasoning, which is characteristic of a “traditional right hemisphere strength” (Liégeois, Cross, Polkey, Harkness, & Vargha-Khadem, 2008). It is interesting to report two distinct cognitive areas that followed opposite trajectories after surgery in our atypical case report. Nevertheless, no study has yet described such an exceptional verbal postoperative outcome in a child with a single hemisphere (Bayard & Lassonde, 2001). Preoperatively, short-term memory weakness may be attributed to the cognitive slowdown commonly observed in RE, and it is tempting to speculate that our patient may regain a level that was most likely present prior to the onset of disease. MI was undoubtedly a child with an initially high potential, and surgery was performed before she clearly deteriorated. The short preoperative delay, as well as her young age at surgery, was also an essential factor in preserving her intellectual potential (Delalande et al., 2007; Jonas et al., 2004). Finally, the case of this verbally exceptionally gifted child suggests the possibility of a more optimistic outcome after surgical treatment than the cognitive decline expected in RE.
147
Acknowledgments We thank Professor Olivier Dulac for referring the child to our institution. We also thank Dorothée Leunen for her contribution to the electrophysiological evaluation. None of the authors have any conflict of interest.
References Achenbach, T. M. (1991). Manual for the child behavior checklist and youth self-report. Burlington: University of Vermont Department of Psychiatry. Bahi-Buisson, N., Villanueva, V., Bulteau, C., Delalande, O., Dulac, O., Chiron, C., & Nabbout, R. (2007). Long term response to steroid therapy in Rasmussen encephalitis. Seizure, 6, 485–492. doi:10.1016/j.seizure.2007.03.002 Bayard, S., & Lassonde, M. (2001). Cognitive, sensory and motor adjustment to hemispherectomy. In I. Jambaqué, M. Lassonde, & O. Dulac (Eds.), Neuropsychology of childhood epilepsy (pp. 229–244). New York, NY: Kluwer Academic. Bien, C. G., Granata, T., Antozzi, C., Cross, J. H., Dulac, O., Kurthen, M., & Elger, C. E. (2005). Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement. Brain: A Journal of Neurology, 128, 454–471. doi:10.1093/brain/awh415 Cross, J. H., Jayakar, P., Nordli, D., Delalande, O., Duchowny, M., Wieser, H. G., … Mathern, G. (2006). International league against epilepsy, subcommission for paediatric epilepsy surgery; Commissions of neurosurgery and paediatrics. Proposed criteria for referral and evaluation of children for epilepsy surgery: Recommendations of the subcommission for pediatric epilepsy surgery. Epilepsia, 47, 952–959. Delalande, O., Bulteau, C., Dellatolas, G., Fohlen, M., Jalin, C., Buret, V., … Jambaque, I. (2007). Vertical parasagittal hemispherotomy: Surgical procedures and clinical long-term outcomes in a population of 83 children. Neurosurgery, 60, 19–32. Jonas, R., Nguyen, S., Hu, B., Asarnow, R. F., LoPresti, C., Curtiss, S., & Mathern, G. W. (2004). Cerebral hemispherectomy: Hospital course, seizure, developmental, language, and motor outcomes. Neurology, 62, 1712–1721. Khomsi, A., Khomsi, J., Pasquet, F., & Parbeau-Gueno, A. (2007). BILO, Bilan Informatisé du Langage Oral. Paris: Editions du Centre de Psychologie Appliquée. Korkman, M., Kirk, S., & Kemp, S. (2003). NEPSY. Bilan Neuropsychologique de l’Enfant: Adaptation française. Paris: Les Editions du Centre de Psychologie Appliquée. Lefabvrais, A. (1967). Test de l’Alouette: Test d’analyse de la lecture et de la dyslexie. Paris: Editions du Centre de Psychologie Appliquée. Liégeois, F., Cross, H., Polkey, C., Harkness, W., & VarghaKhadem, F. (2008). Language after hemispherectomy in childhood: Contributions from memory and language. Neuropsychologia, 46, 3101–3107. doi:10.1016/j. neuropsychologia.2008.07.001 Pulsifer, M. B., Brandt, J., Salorio, C. F., Vining, E. P., Carson, B. S., & Freeman, J. M. (2004). The cognitive outcome of hemispherectomy in 71 children. Epilepsia, 45, 243–254. doi:10.1111/j.0013-9580.2004.15303.x
Copyright of Neurocase (Psychology Press) is the property of Psychology Press (UK) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Exceptional verbal intelligence after hemispherotomy in a child with Rasmussen encephalitis Catherine Grosmaitrea,b,c, Isabelle Jambaquéa,b,c, Georg Dorfmullera,b,c,d, Sebastian Rodrigoa,b,c, Dorothée Villee, Olivier Delalanded and Christine Bulteaua,b,c,d* a Inserm U663, Paris, France; bPRES Sorbonne Paris Cité, University Paris Descartes, France; cCEA, Gif sur Yvette, France; Fondation Ophtalmologique A. de Rothschild, Pediatric Neurosurgery Department, 75019 Paris, France; eHôpital Femme Mère Enfant Neuropediatric Department, 69677 Bron, France
d
(Received 3 April 2013; accepted 29 October 2013) We report a longitudinal case study of a left-handed girl who underwent left hemispherotomy at 7 years for Rasmussen encephalitis (RE). Presurgical evaluation showed mild hemiparesis, no visual defect, and light neuropsychological impairment with short-term memory weakness. Language fMRI showed a right hemispheric dominance. Postoperatively, the patient exhibited right hemiplegia and hemianopsia but preserved intellectual capacities. She became seizure-free, and antiepileptic medication was discontinued. Long-term follow-up showed very high verbal intelligence at 11 years of age (VCI of 155) and improvement in working memory as well as language and reading abilities. Furthermore, a significant visuoverbal discrepancy became increasingly pronounced. Thus, early surgical treatment of epilepsy avoided the global cognitive deterioration usually associated with RE. Finally, such a high level of verbal functioning combined with low spatial reasoning with a single hemisphere provides additional information on the neurocognitive profile of children with RE after hemispherotomy. Keywords: gifted child; hemispherotomy; Rasmussen encephalitis
Rasmussen encephalitis (RE) is a progressive inflammatory epileptic encephalopathy, characterized by partial intractable epilepsy, progressive unilateral hemispheric atrophy associated with motor and cognitive decline (Bahi-Buisson et al., 2007; Bien et al., 2005). Immunosuppressive and immunomodulatory interventions, as well as antiepileptic drugs (AEDs), have beneficial effects at an early stage but are not effective to control seizure in advanced disease. Hemispherotomy (H) is then recommended since around 60–90% of patients are subsequently seizure-free (Cross et al., 2006; Delalande et al., 2007). Cognitive functions seem to be globally stable after H (Bayard & Lassonde, 2001; Pulsifer et al., 2004), and etiologies do not have any effect on seizure outcome (Jonas et al., 2004). Delalande et al. (2007) found, however, a correlation between preoperative delay and the Vineland Adaptive Behavior score in a large series with various etiologies (developmental, acquired, or progressive). Children with a longer duration of seizures had lower performances at long-term outcome. Classically, H can be considered when hemiplegia, hemianopsia, and both language and cognitive deficits are severe and will not be aggravated by surgery. However, while H is the only way to cure RE, the crucial decision is when it should be done in order to avoid global mental deterioration. We report a 5-year longitudinal case study of a left-handed girl with RE who underwent early left H in the course of the *Corresponding author. Email: [email protected] © 2014 Taylor & Francis
disease. Strikingly, we observed exceptional verbal intelligence in this child with a single hemisphere. Our case provides additional information on outcome of the neurocognitive profile after H in children with RE. Case report Patient history MI is a left-handed, 11-year-old girl who suffered from left hemispheric RE. She comes from a monolingual Frenchspeaking family, with high potential in siblings and parents, had normal developmental milestones, behavior, and schooling until the age of 5 years when she experienced partial seizures (collapse of her right leg leading to falls). The initial MRI exhibited a focal hyperintense T2 signal over the left centro-parietal region. Other types of partial epileptic seizures occurred, and the following MRI revealed a moderate unilateral left hemispheric atrophy and a hypersignal in the insular area in favor of RE. Steroids were administrated and led to epileptic and motor improvement. During this period, MI had normal schooling and learned to read. At the age of 6.5 years, MI presented continuous partial epilepsy and right hemiparesis increased. Presurgical video EEG registered many types of seizures, which onset over the left hemisphere, with multifocal spikes and slow waves during interictal activity and no abnormality over the right
Neurocase
Figure 1. Preoperative language fMRI showing right hemispheric lateralization. Top line: activations in the right temporal gyrus during receptive tasks (left: word repetition; right: sentence listening). Bottom line: activations in the right inferior frontal gyrus and Supplementary Motor Area (SMA) during expressive tasks (left: word generation; right: sentence generation). [To view this figure in color, please see the online version of this Journal.]
hemisphere. She was treated by clobazam, oxcarbazepine, levetiracetam, and stiripentol and corticoids but developed Cushing syndrome. Whatever the treatment, seizures occurred daily and motor deficit increased. She was referred to our institution for left H according to the vertical parasagittal surgical procedure (Delalande et al., 2007). Preoperative evaluation showed moderate right hemiparesis with motor heminegligence; she was unable to attend school due to severe epileptic seizures. Visual field remained normal. Language fMRI showed right hemispheric lateralization networks during sentence listening, semantic word generation, and verb generation (Figure 1). Neuropsychological evaluation showed slight impairment in working memory but no intellectual disabilities and normal reading abilities. Left H was performed at the age of 6 years 11 months and was followed by a right hemianopsia and a right hemiplegia. Oral language and reading abilities remained stable. The child became seizure-free and AED were stopped (Engel’s Class I).
Neuropsychological evaluation Table 1 summarizes the neuropsychological findings of presurgical and postoperative assessment. Informed patient and parent consent was obtained for participation in neuropsychological evaluations and reassessment for identifying postoperative changes.
145
Verbal and nonverbal intellectual functions were evaluated pre- and postoperatively on the Wechsler scale. At presurgery, general intellectual assessment revealed a very high verbal comprehension index (VCI of 120), although the perceptual reasoning index (PRI) was average. After surgery, a significant visuoverbal discrepancy appeared and became increasingly pronounced (36 points at 0.8 years postoperative, 66 points at 2.9 years, and 71 points at 4.2 years). In fact, MI exhibited exceptional verbal intelligence well above the norm (superior or gifted range), combined with low spatial reasoning. MI obtained an exceptional score of 19 on the vocabulary subtest during all the follow-up while marked progress on comprehension and similarities subtests was also observed. Working memory index and processing speed index evidenced a subnormal or low average neuropsychological condition presurgically. After surgery, there was marked improvement in the working memory index and “Digit span” went up dramatically after surgery. Speed processing decreased to the lower normal range both before and after surgery. Language assessment comprised the expressive and receptive language tasks of the NEPSY (Korkman, Kirk, & Kemp, 2003) and the BILO (Bilan Informatisé du Langage Oral) (Khomsi, Khomsi, Pasquet, & ParbeauGueno, 2007). Presurgical evaluation showed efficient verbal abilities except on the rapid naming task. After surgery, language performances improved with time and MI performed well on all verbal tasks. The French reading test L’Alouette (Lefabvrais, 1967) was administered to determine a reading age. Reading performances were well above normal during follow-up. We evaluated spatial perception using the NEPSY Arrows and Visual attention subtests (Korkman et al., 2003) and the Rey figure. MI showed no evidence of spatial perception deficit but had slight visual attention problems and poor visual memory. The Child Behavioral Checklist (Achenbach, 1991) provided information about behavioral and emotional adaptation. MI did not exhibit any behavioral or social impairment during follow-up. Discussion Our study concerns a child who underwent left H for RE with slight neuropsychological impairment in the preoperative course and was followed up for 5 years. The preoperative cognitive profile may be due to the severity of epilepsy and progressive encephalopathy in RE. She experienced at least five seizures/day (right hemibody clonic seizures) with continuous partial epilepsy of the right hand and was treated by steroids, levetiracetam, clobazam, stiripentol, and carbamazepine. Interictal
146 Table 1.
C. Grosmaitre et al. Results of the prospective neuropsychological assessment.
Test Age (years)
Presurgical assessment 6.8
Postsurgery 1 (+1 year) 8
Postsurgery 2 (+2.1 years)
Postsurgery 3 (+4.1 years)
9.8
11.1
Measures of intelligence outcome WISC IV (Wechsler Intelligence Scale for Children) Verbal comprehension index (VCI) 120 Vocabulary* 19 Similarities* 12 Comprehension* 9 Perceptual reasoning index (PRI) 109 Block design* 11 Matrix reasoning* 12 Picture concepts* 11 Working memory index (WMI) 85 Digit span* 4 Letter-number sequencing* 11 Processing speed index (PSI) 81 Coding* 6 Symbol search* 7
138 19 13 16 102 11 9 11
154 19 19 17 88 10 7 8 106 11 11 81 8 5
155 19 19 18 84 9 6 8 115 14 11 73 6 4
13 14 13 12
12
Measures of language outcome NEPSY (Korkman) Verbal fluency* Comprehension of instructions* Phonological process* Rapid naming* BILO (Khomsi) Oral comprehension§ Lexical judgment§ Syntactic judgment§ Words Repetition§ Picture naming§ Syntactic drafting§ Reading age (Lefabvrais)
11 9 10 6
14
−0.16 −0.11 +1.13 +0.62 +1.34 +1.68 8 years
8.6 years
12 10
11
+1.29 +0.22 +0.83 +0.82 +1.89 +1.39 12.4 years
Measures of visuospatial outcome NEPSY (Korkman) Arrow – orientation perception* Visual attention* Rey-Osterrieth Copy of the Complex Figure§
−05
10 7
9
+0.88
+1.11
52 32 48
59 42 57
Measures of behavior outcome CBCL (Achenbach)# Internalizing Externalizing Total
59 44 54
Notes: BILO: oral comprehension (efficiency of comprehension strategies, in particular inferential), lexical judgment (precision of the semantic, lexical representations, nouns and verbs), syntactic judgment (quality of the morphosyntax in reception and sensitivity to the linguistic standard), word repetition (quality of the phonology in production), picture naming (lexicon in production: availability of nouns and verbs), and syntactic drafting (production of statements: morphosyntactic skill in production). *Standard scores. § Z scores. #Achenbach: a T score equal to or above 65 on any of the subscales is considered to denote pathology.
abnormalities and ictal EEG discharges were limited to the right abnormalities and did not spread to the contralateral hemisphere. Neuropsychological follow-up showed an
exceptional intellectual outcome (VCI of 155) with an increase in verbal functions in the upper range within the postoperative course.
Neurocase H cured epilepsy, and all antiepileptic drugs were withdrawn within the six postoperative months. In fact, fMRI showed right language dominance and the decision to perform surgery as soon as possible was uncontroversial. Furthermore, her left H afforded seizure control and avoided any interference of the epileptic hemisphere on her dominant right hemisphere. Preoperatively, working memory index and processing speed index were in the low average range and there was a discrepancy between verbal and nonverbal efficiency. Presurgically, she had a VCI of 120 with 19 in vocabulary, which is part of crystallized intelligence. Similarities and comprehension, linked to fluid intelligence, were weaker and progressed much more after surgery. MI’s verbal working memory improved after hemispherotomy, which may contribute to high verbal proficiency. On the other hand, speed processing remained low, but MI had right hemiplegia and visual hemianopsia, a neurological condition that can be associated with poor oculomotor performance and lower attention resources. Finally, MI’s postoperative intellectual profile is characterized by a marked visuoverbal discrepancy with poor perceptive reasoning index that stagnated during followup. There is evidence of the preservation of MI’s verbal skills combined with low spatial reasoning, which is characteristic of a “traditional right hemisphere strength” (Liégeois, Cross, Polkey, Harkness, & Vargha-Khadem, 2008). It is interesting to report two distinct cognitive areas that followed opposite trajectories after surgery in our atypical case report. Nevertheless, no study has yet described such an exceptional verbal postoperative outcome in a child with a single hemisphere (Bayard & Lassonde, 2001). Preoperatively, short-term memory weakness may be attributed to the cognitive slowdown commonly observed in RE, and it is tempting to speculate that our patient may regain a level that was most likely present prior to the onset of disease. MI was undoubtedly a child with an initially high potential, and surgery was performed before she clearly deteriorated. The short preoperative delay, as well as her young age at surgery, was also an essential factor in preserving her intellectual potential (Delalande et al., 2007; Jonas et al., 2004). Finally, the case of this verbally exceptionally gifted child suggests the possibility of a more optimistic outcome after surgical treatment than the cognitive decline expected in RE.
147
Acknowledgments We thank Professor Olivier Dulac for referring the child to our institution. We also thank Dorothée Leunen for her contribution to the electrophysiological evaluation. None of the authors have any conflict of interest.
References Achenbach, T. M. (1991). Manual for the child behavior checklist and youth self-report. Burlington: University of Vermont Department of Psychiatry. Bahi-Buisson, N., Villanueva, V., Bulteau, C., Delalande, O., Dulac, O., Chiron, C., & Nabbout, R. (2007). Long term response to steroid therapy in Rasmussen encephalitis. Seizure, 6, 485–492. doi:10.1016/j.seizure.2007.03.002 Bayard, S., & Lassonde, M. (2001). Cognitive, sensory and motor adjustment to hemispherectomy. In I. Jambaqué, M. Lassonde, & O. Dulac (Eds.), Neuropsychology of childhood epilepsy (pp. 229–244). New York, NY: Kluwer Academic. Bien, C. G., Granata, T., Antozzi, C., Cross, J. H., Dulac, O., Kurthen, M., & Elger, C. E. (2005). Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement. Brain: A Journal of Neurology, 128, 454–471. doi:10.1093/brain/awh415 Cross, J. H., Jayakar, P., Nordli, D., Delalande, O., Duchowny, M., Wieser, H. G., … Mathern, G. (2006). International league against epilepsy, subcommission for paediatric epilepsy surgery; Commissions of neurosurgery and paediatrics. Proposed criteria for referral and evaluation of children for epilepsy surgery: Recommendations of the subcommission for pediatric epilepsy surgery. Epilepsia, 47, 952–959. Delalande, O., Bulteau, C., Dellatolas, G., Fohlen, M., Jalin, C., Buret, V., … Jambaque, I. (2007). Vertical parasagittal hemispherotomy: Surgical procedures and clinical long-term outcomes in a population of 83 children. Neurosurgery, 60, 19–32. Jonas, R., Nguyen, S., Hu, B., Asarnow, R. F., LoPresti, C., Curtiss, S., & Mathern, G. W. (2004). Cerebral hemispherectomy: Hospital course, seizure, developmental, language, and motor outcomes. Neurology, 62, 1712–1721. Khomsi, A., Khomsi, J., Pasquet, F., & Parbeau-Gueno, A. (2007). BILO, Bilan Informatisé du Langage Oral. Paris: Editions du Centre de Psychologie Appliquée. Korkman, M., Kirk, S., & Kemp, S. (2003). NEPSY. Bilan Neuropsychologique de l’Enfant: Adaptation française. Paris: Les Editions du Centre de Psychologie Appliquée. Lefabvrais, A. (1967). Test de l’Alouette: Test d’analyse de la lecture et de la dyslexie. Paris: Editions du Centre de Psychologie Appliquée. Liégeois, F., Cross, H., Polkey, C., Harkness, W., & VarghaKhadem, F. (2008). Language after hemispherectomy in childhood: Contributions from memory and language. Neuropsychologia, 46, 3101–3107. doi:10.1016/j. neuropsychologia.2008.07.001 Pulsifer, M. B., Brandt, J., Salorio, C. F., Vining, E. P., Carson, B. S., & Freeman, J. M. (2004). The cognitive outcome of hemispherectomy in 71 children. Epilepsia, 45, 243–254. doi:10.1111/j.0013-9580.2004.15303.x
Copyright of Neurocase (Psychology Press) is the property of Psychology Press (UK) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
