Child Neurol Research Articles

Errata

Developmental Medicine & Child NeurologyVolume 53, Issue 5 p. 478-478 Free Access Errata This article corrects the following: Congenital lower brachial plexus palsy due to cervical ribs ARCHANA DESURKAR, KERRY MILLS, MATTHEW PITT, WAJANAT JAN, MARCO SINSI, IAN MALE, ELIZABETH WRAIGE, Volume 53Issue 2Developmental Medicine & Child Neurology pages: 188-190 First Published online: January 18, 2011 First published: 12 April 2011 https://doi.org/10.1111/j.1469-8749.2011.03985_2.xAboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat In the following article [1], the author’s name was spelled incorrectly. M. Sinsi should be M Sinisi. Reference 1 Elizabeth Wraige, Arachana Desurkar, Kerry Mills, Matthew Pitt, Wajanat Jan, Marco Sinisi, Ian Male. Congenital lower brachial plexus palsy due to cervical ribs. Dev Med Child Neurol 2010; 53: 188– 190. We apologise for this error. Volume53, Issue5May 2011Pages 478-478 ReferencesRelatedInformation

Subacute Sclerosing Panencephalitis Presenting as Mania

Subacute Sclerosing Panencephalitis Presenting as Mania

Proneural Transcription Factors Regulate Different Steps of Cortical Neuron Migration through Rnd-Mediated Inhibition of RhoA Signaling

SummaryLittle is known of the intracellular machinery that controls the motility of newborn neurons. We have previously shown that the proneural protein Neurog2 promotes the migration of nascent cortical neurons by inducing the expression of the atypical Rho GTPase Rnd2. Here, we show that another proneural factor, Ascl1, promotes neuronal migration in the cortex through direct regulation of a second Rnd family member, Rnd3. Both Rnd2 and Rnd3 promote neuronal migration by inhibiting RhoA signaling, but they control distinct steps of the migratory process, multipolar to bipolar transition in the intermediate zone and locomotion in the cortical plate, respectively. Interestingly, these divergent functions directly result from the distinct subcellular distributions of the two Rnd proteins. Because Rnd proteins also regulate progenitor divisions and neurite outgrowth, we propose that proneural factors, through spatiotemporal regulation of Rnd proteins, integrate the process of neuronal migration with other events in the neurogenic program.

Erratum

Developmental Medicine & Child NeurologyVolume 53, Issue 3 p. 280-280 Free Access Erratum This article corrects the following: Improving attitudes towards children with disabilities in a school context: a cluster randomized intervention study EMMANUELLE GODEAU, CÉLINE VIGNES, MARIANE SENTENAC, VIRGINIE EHLINGER, FÉLIX NAVARRO, HÉLÈNE GRANDJEAN, CATHERINE ARNAUD, Volume 52Issue 10Developmental Medicine & Child Neurology pages: e236-e242 First Published online: July 14, 2010 First published: 03 February 2011 https://doi.org/10.1111/j.1469-8749.2010.03908.xCitations: 1AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat In the article by Loeters et al. [1] there was an error in the first paragraph on page 605. The error was in the definition of the Cobbs angle and read, ‘However, scoliosis, which may be defined as a spinal curve of angle <10°, quantified using the Cobb method,¹ is not itself the major factor contributing to such limitations.’ The correct text is: ‘However, scoliosis, which may be defined as a spinal curve of angle >10°, quantified using the Cobb method,¹ is not itself the major factor contributing to such limitations.’ We apologise for this error. Reference 1 Loeters MJB, Maathuis CGB, Hadders-Algra M. Risk factors for emergence and progression of scoliosis in children with severe cerebral palsy: a systematic review. Dev Med Child Neurol 2010; 52: 605– 11. Wiley Online LibraryPubMedWeb of Science®Google Scholar Citing Literature Volume53, Issue3March 2011Pages 280-280 ReferencesRelatedInformation

Interferon Antagonist NSs of La Crosse Virus Triggers a DNA Damage Response-like Degradation of Transcribing RNA Polymerase II

La Crosse encephalitis virus (LACV) is a mosquito-borne member of the negative-strand RNA virus family Bunyaviridae. We have previously shown that the virulence factor NSs of LACV is an efficient inhibitor of the antiviral type I interferon system. A recombinant virus unable to express NSs (rLACVdelNSs) strongly induced interferon transcription, whereas the corresponding wt virus (rLACV) suppressed it. Here, we show that interferon induction by rLACVdelNSs mainly occurs through the signaling pathway leading from the pattern recognition receptor RIG-I to the transcription factor IRF-3. NSs expressed by rLACV, however, acts downstream of IRF-3 by specifically blocking RNA polymerase II-dependent transcription. Further investigations revealed that NSs induces proteasomal degradation of the mammalian RNA polymerase II subunit RPB1. NSs thereby selectively targets RPB1 molecules of elongating RNA polymerase II complexes, the so-called IIo form. This phenotype has similarities to the cellular DNA damage response, and NSs was indeed found to transactivate the DNA damage response gene pak6. Moreover, NSs expressed by rLACV boosted serine 139 phosphorylation of histone H2A.X, one of the earliest cellular reactions to damaged DNA. However, other DNA damage response markers such as up-regulation and serine 15 phosphorylation of p53 or serine 1524 phosphorylation of BRCA1 were not triggered by LACV infection. Collectively, our data indicate that the strong suppression of interferon induction by LACV NSs is based on a shutdown of RNA polymerase II transcription and that NSs achieves this by exploiting parts of the cellular DNA damage response pathway to degrade IIo-borne RPB1 subunits.

Tourette's Syndrome and Type 2 Bipolar Disorder

Back to table of contents Previous article Next article LettersFull AccessTourette's Syndrome and Type 2 Bipolar DisorderLuís Fonseca, Joana Damásio, Marina Magalhães, and Joaquim DuarteLuís FonsecaPsychiatry and Mental Health Department, Braga Hospital, Braga, PortugalSearch for more papers by this author, Joana DamásioNeurology Department, Porto Hospital Centre, Porto, PortugalSearch for more papers by this author, Marina MagalhãesNeurology Department, Porto Hospital Centre, Porto, PortugalSearch for more papers by this author, and Joaquim DuartePsychiatry and Mental Health Department, Braga Hospital, Braga, PortugalSearch for more papers by this authorPublished Online:1 Jan 2011AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Tourette's syndrome is an inherited, childhood-onset neuropsychiatric disorder characterized by a fluctuating course of multiple chronic motor and phonic tics. Around 88% of Tourette's syndrome patients have comorbidity, the most common being attention-deficit/hyperactivity disorder, followed by obsessive compulsive disorder.1Case ReportA 34-year-old male patient with a history of Tourette's syndrome was seen for acute onset of insomnia, irritability, and overspending. He also presented slight neck jerking. He had been medicated with 600 mg of sulpiride and 10 mg of haloperidol for Tourette's syndrome. At this time, 500 mg of valproate was prescribed with symptomatic improvement. Antipsychotic medication was maintained. Four months later he presented with depressive mood, anhedonia, insomnia, loss of appetite, and diminished ability to concentrate of 1 month duration. Fluvoxamine was prescribed and titrated to 100 mg while the other drugs were maintained. Again he improved.Nine months later, the patient was seen for acute onset of insomnia, overspending, dysphoric mood, sexual disinhibition, and megalomaniac thoughts, and he was admitted to our unit. A brain MRI and thorough lab work returned normal results. Valproate was titrated to 1,000 mg, and we added 300 mg of quetiapine. Haloperidol and sulpiride were stopped without the worsening of neck jerking or the onset of new tics. Three weeks later, he presented no psychiatric symptoms and was discharged home.At the age of 13, our patient had acutely developed cervical motor tics. He started haloperidol, titrated to 6 mg, with symptomatic improvement. When he turned 18 the motor tics worsened, new ones involving the superior limbs appeared, and the onset of vocal tics (shouting repeatedly during his speech and coprolalia) occurred. Haloperidol augmentation to 10 mg improved these.Until the age of 23, the tics presented a waxing and waning course. At this age, cervical and mouth tics slightly improved and others began (scratching the genitalia, clothes pruning). Haloperidol was then stopped, and sulpiride titrated to 600 mg improved these symptoms. A slight neck jerking remained up to the time he presented to our unit.The clinical picture that emerged 9 months after the depressive syndrome was clearly a hypomanic state. The diagnosis of bipolar disorder type II could then be confidently made.2Hypomania could be considered an iatrogenic effect of antidepressant medication. However, it is unlikely due to the antimanic effect of valproate and sulpiride3 and the 9 months that had lapsed since the antidepressant was prescribed.DiscussionComorbidity between Tourette's syndrome and bipolar disorder, although infrequent, has been reported.4 A modulation defect of the mesencefalic nuclea in certain areas of the cortical-limbic system, ventral striatum, and sensory motor cortical regions could explain the association. The onset of mania or bipolar disorder in neuropsychiatric disorders with predominant subcortical dysfunction (e.g., Huntington's) further supports it.1,5The onset of vocal tics at the age of 18 fulfilled diagnostic criteria for Tourette's syndrome.5 Currently, neck jerking is the only remaining tic. This is consistent with the known decrease of tic frequency and intensity during adolescence in about two-thirds of children with Tourette's syndrome, as well as its frequent remission in adulthood.6Psychiatry and Mental Health Department, Braga Hospital, Braga, PortugalNeurology Department, Porto Hospital Centre, Porto, PortugalPsychiatry and Mental Health Department, Braga Hospital, Braga, Portugal1. Robertson M: Mood disorders and Gilles de la Tourette's syndrome: an update on prevalence, etiology, comorbidity, clinical associations and implications. J Psychosom Res 2006; 61:349–358Crossref, Medline, Google Scholar2. Muller-Oerlinghausen B , Berghofer A , Bauer M: Bipolar disorder. Lancet 2002; 19:359241–247Crossref, Google Scholar3. Cristhie J , Whalley L , Hunter R, et al.: Sulpiride treatment of acute mania with a comparison of the effects of plasma hormone concentrations of lithium and sulpiride treatment. J Affective Disord 1989; 16:115–120Crossref, Medline, Google Scholar4. Berthier M , Kulisevsky J , Campos V: Bipolar disorder in adult patients with Tourette's syndrome: a clinical study. Biol Psychiatry 1998; 43:364–370Crossref, Medline, Google Scholar5. Harris K , Singer H: Tic disorders: neural circuits, neurochemistry and neuroimmunology. J Child Neurol 2006; 21:678–689Crossref, Medline, Google Scholar6. Bloch M , Leckman J , Zhu H, et al.: Caudate volumes in childhood predict symptom severity in adults with Tourette syndrome. Neurology 2005; 65:1253–1258Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited ByNone Volume 23Issue 1 Winter 2011Pages E26-E26 Metrics PDF download History Published online 1 January 2011 Published in print 1 January 2011

Utility of language comprehension tests for unintelligible or non‐speaking children with cerebral palsy: a systematic review

to identify the use and utility of language comprehension tests for unintelligible or non-speaking children with severe cerebral palsy (CP). severe CP was defined as severe dysarthria (unintelligible speech) or anarthria (absence of speech) combined with severe limited mobility, corresponding to Gross Motor Function Classification System levels IV to V. An electronic search in the databases of PubMed, PsychInfo, Embase, and CINAHL was made of studies published between January 1965 and December 2008. Indexing terms and free-text terms for 'cerebral palsy', 'language', and 'instrumentation' were used. Studies were included when (1) the focus was to investigate comprehension of spoken language of children (0-18 y) with severe CP, and (2) language tests were described. twelve standardized tests and five experimental instruments were identified. All standardized tests were developed for children without limited mobility. Only the Peabody Picture Vocabulary Test - Revised was frequently used and feasible for older children with severe CP (> 9y). The other tests were used occasionally. To establish utility, adaptations of standardized test procedures were necessary. language comprehension tests for children with severe CP are scarce. A language comprehension test specifically designed for these children is warranted. Cite this as: Dev Med Child Neurol 52: e267-e277.

Erratum

Developmental Medicine & Child NeurologyVolume 52, Issue 8 p. 784-784 Free Access Erratum This article corrects the following: Probable trigeminal autonomic cephalgia in a 3-month-old male infant IRENE VAZ, Volume 52Issue 4Developmental Medicine & Child Neurology pages: 400-402 First Published online: December 9, 2009 First published: 08 July 2010 https://doi.org/10.1111/j.1469-8749.2010.03739.xAboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat In the following article [1] figure 1 was incorrectly published in black and white. Please find the figure in colour below along with the figure legend. (1) [ Right-sided flushing of the face, conjunctival redness, drooping of the eyelid, and discharge from the nostril. ] References 1 Vaz I. Probable trigeminal autonomic cephalgia in a 3-month-old male infant. Dev Med Child Neurol 2010; 52: 400– 2. Wiley Online LibraryCASPubMedWeb of Science®Google Scholar We apologise for this error. Volume52, Issue8August 2010Pages 784-784 ReferencesRelatedInformation

Lipid second messengers and related enzymes in vertebrate rod outer segments

Rod outer segments (ROSs) are specialized light-sensitive organelles in vertebrate photoreceptor cells. Lipids in ROS are of considerable importance, not only in providing an adequate environment for efficient phototransduction, but also in originating the second messengers involved in signal transduction. ROSs have the ability to adapt the sensitivity and speed of their responses to ever-changing conditions of ambient illumination. A major contributor to this adaptation is the light-driven translocation of key signaling proteins into and out of ROS. The present review shows how generation of the second lipid messengers from phosphatidylcholine, phosphatidic acid, and diacylglycerol is modulated by the different illumination states in the vertebrate retina. Findings suggest that the light-induced translocation of phototransduction proteins influences the enzymatic activities of phospholipase D, lipid phosphate phosphatase, diacylglyceride lipase, and diacylglyceride kinase, all of which are responsible for the generation of the second messenger molecules.

Response

Dr. Eeg-Olofsson addresses an issue important to physicians caring for pediatric patients with epilepsy, and in particular those children whose electroencephalogram recordings depict centrotemporal spikes. This issue refers to the clinical question of whether a “classic” form of rolandic epilepsy exists, along with atypical presentations of the disease [ [1] Lundberg S. Eeg-Olofsson O. Rolandic epilepsy: A challenge in terminology and classification. Eur J Paediatr Neurol. 2003; 7: 239-241 Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar ] in a continuum [ [2] Kramer U. Atypical presentations of benign epilepsy with centrotemporal spikes: A review. J Child Neurol. 2008; 23: 785-790 Crossref PubMed Scopus (55) Google Scholar ], or whether the clinical variants represent different diseases related to specific, though as yet undefined, genetic etiologies, or to anatomic malformations in some cases. Rolandic EpilepsyPediatric NeurologyVol. 42Issue 3PreviewTo the Editor: Full-Text PDF

Systematic Review of Interventions for Low Bone Mineral Density in Children With Cerebral Palsy

To systematically review the efficacy of interventions to improve low bone mineral density (LBMD) in children and adolescents with cerebral palsy (CP). We performed a systematic search for published randomized, controlled trials (RCTs) and controlled clinical trials (CCTs) of children with CP (aged 0-20 years, all Gross Motor Function Classification System [GMFCS] levels) who received various medical and physical interventions for LBMD compared with no intervention or standard care. Study validity was evaluated by using the Physiotherapy Evidence Database (PEDro) scale. Standardized mean differences (SMDs) were calculated when data were sufficient. Eight of 2034 articles met the inclusion criteria (6 RCTs, 2 CCTs). There were 3 trials of bisphosphonates, one of which (Henderson RC, Lark RK, Kecskemethy HH, Miller F, Harcke HT, Bachrach SJ. J Pediatr. 2002;141[5]:644-651) revealed a large and significant effect on BMD in 1 of 3 sites in the distal femur (SMD: 1.88 [95% confidence interval (CI): 0.52-3.24]). There were 3 trials of weight-bearing through varying approaches, one of which (Caulton JM, Ward KA, Alsop CW, Dunn G, Adams JE, Mughal MZ. Arch Dis Child. 2004;89[2]:131-135) showed a large and significant effect on the lumbar spine when increasing static standing time (SMD: 1.03 [95% CI: 0.21-1.85]). The administration of vitamin D and calcium produced a large, nonsignificant effect on BMD in the lumbar spine (Jekovec-Vrhovsek M, Kocijancic A, Prezelj J. Dev Med Child Neurol. 2000;42[6]:403-405) (SMD: 0.88 [95% CI: -0.07 to 1.84). Growth hormone combined with vitamin D and/or calcium resulted in effects comparable with vitamin D and/or calcium on BMD in the lumbar spine (Ali O, Shim M, Fowler E, et al. J Clin Endocrinol Metab. 2007;92[3]:932-937) (SMD 0 [95% CI: -1.24 to 1.24]). Important effects on LBMD have been observed in small and diverse cohorts of children with CP. It is unclear whether small sample sizes or variable treatment responses account for nonsignificant findings. Additional large RCTs are needed of both physical and medical approaches.

The Structure of the Mammalian RNase H2 Complex Provides Insight into RNA·DNA Hybrid Processing to Prevent Immune Dysfunction

The mammalian RNase H2 ribonuclease complex has a critical function in nucleic acid metabolism to prevent immune activation with likely roles in processing of RNA primers in Okazaki fragments during DNA replication, in removing ribonucleotides misinserted by DNA polymerases, and in eliminating RNA.DNA hybrids during cell death. Mammalian RNase H2 is a heterotrimeric complex of the RNase H2A, RNase H2B, and RNase H2C proteins that are all required for proper function and activity. Mutations in the human RNase H2 genes cause Aicardi-Goutières syndrome. We have determined the crystal structure of the three-protein mouse RNase H2 enzyme complex to better understand the molecular basis of RNase H2 dysfunction in human autoimmunity. The structure reveals the intimately interwoven architecture of RNase H2B and RNase H2C that interface with RNase H2A in a complex ideally suited for nucleic acid binding and hydrolysis coupled to protein-protein interaction motifs that could allow for efficient participation in multiple cellular functions. We have identified four conserved acidic residues in the active site that are necessary for activity and suggest a two-metal ion mechanism of catalysis for RNase H2. An Okazaki fragment has been modeled into the RNase H2 nucleic acid binding site providing insight into the recognition of RNA.DNA junctions by the RNase H2. Further structural and biochemical analyses show that some RNase H2 disease-causing mutations likely result in aberrant protein-protein interactions while the RNase H2A subunit-G37S mutation appears to distort the active site accounting for the demonstrated substrate specificity modification.

A Pressing Headache

A Pressing Headache

Combination of Linkage Mapping and Microarray-Expression Analysis Identifies NF-κB Signaling Defect as a Cause of Autosomal-Recessive Mental Retardation

Autosomal-recessive inheritance accounts for nearly 25% of nonsyndromic mental retardation (MR), but the extreme heterogeneity of such conditions markedly hampers gene identification. Combining autozygosity mapping and RNA expression profiling in a consanguineous Tunisian family of three MR children with mild microcephaly and white-matter abnormalities identified the TRAPPC9 gene, which encodes a NF-kappaB-inducing kinase (NIK) and IkappaB kinase complex beta (IKK-beta) binding protein, as a likely candidate. Sequencing analysis revealed a nonsense variant (c.1708C>T [p.R570X]) within exon 9 of this gene that is responsible for an undetectable level of TRAPPC9 protein in patient skin fibroblasts. Moreover, TNF-alpha stimulation assays showed a defect in IkBalpha degradation, suggesting impaired NF-kappaB signaling in patient cells. This study provides evidence of an NF-kappaB signaling defect in isolated MR.

CMIP and ATP2C2 Modulate Phonological Short-Term Memory in Language Impairment

Specific language impairment (SLI) is a common developmental disorder characterized by difficulties in language acquisition despite otherwise normal development and in the absence of any obvious explanatory factors. We performed a high-density screen of SLI1, a region of chromosome 16q that shows highly significant and consistent linkage to nonword repetition, a measure of phonological short-term memory that is commonly impaired in SLI. Using two independent language-impaired samples, one family-based (211 families) and another selected from a population cohort on the basis of extreme language measures (490 cases), we detected association to two genes in the SLI1 region: that encoding c-maf-inducing protein (CMIP, minP = 5.5 × 10−7 at rs6564903) and that encoding calcium-transporting ATPase, type2C, member2 (ATP2C2, minP = 2.0 × 10−5 at rs11860694). Regression modeling indicated that each of these loci exerts an independent effect upon nonword repetition ability. Despite the consistent findings in language-impaired samples, investigation in a large unselected cohort (n = 3612) did not detect association. We therefore propose that variants in CMIP and ATP2C2 act to modulate phonological short-term memory primarily in the context of language impairment. As such, this investigation supports the hypothesis that some causes of language impairment are distinct from factors that influence normal language variation. This work therefore implicates CMIP and ATP2C2 in the etiology of SLI and provides molecular evidence for the importance of phonological short-term memory in language acquisition.

‘User views of Emergency Health Care Plans for disabled children and young people’

Hand function in children with cerebral palsy after upper-limb tendon transfer and muscle release. Dev Med Child Neurol 1998; 40: 612–21. 14. Dahlin LB, Komoto Tufvesson Y, Salgeback S. Surgery of the spastic hand in cerebral palsy. Improvement in stereognosis and hand function after surgery. J Hand Surg 1998; 23: 334–39. 15. Matsuo T, Matsuo A, Hajime T, Fukumoto S, Chen W, Iwamoto Y. Release of flexors and intrinsic muscles for finger spasticity in cerebral palsy. Clin Orthop Relat Res 2001; 384: 162–68.

Glycoengineered Acid α-Glucosidase With Improved Efficacy at Correcting the Metabolic Aberrations and Motor Function Deficits in a Mouse Model of Pompe Disease

Improving the delivery of therapeutics to disease-affected tissues can increase their efficacy and safety. Here, we show that chemical conjugation of a synthetic oligosaccharide harboring mannose 6-phosphate (M6P) residues onto recombinant human acid alpha-glucosidase (rhGAA) via oxime chemistry significantly improved its affinity for the cation-independent mannose 6-phosphate receptor (CI-MPR) and subsequent uptake by muscle cells. Administration of the carbohydrate-remodeled enzyme (oxime-neo-rhGAA) into Pompe mice resulted in an approximately fivefold higher clearance of lysosomal glycogen in muscles when compared to the unmodified counterpart. Importantly, treatment of immunotolerized Pompe mice with oxime-neo-rhGAA translated to greater improvements in muscle function and strength. Treating older, symptomatic Pompe mice also reduced tissue glycogen levels but provided only modest improvements in motor function. Examination of the muscle pathology suggested that the poor response in the older animals might have been due to a reduced regenerative capacity of the skeletal muscles. These findings lend support to early therapeutic intervention with a targeted enzyme as important considerations in the management of Pompe disease.

Aphasic or amnesic status epilepticus detected on PET but not EEG

To describe five patients with ictal aphasia and one patient with ictal amnesia, who had focal positron emission tomography (PET) hypermetabolism but no clear ictal activity on electroencephalography (EEG). (18)F-Fluorodeoxyglucose (FDG)-PET scans with concomitant EEG were obtained in five patients with suspected ictal aphasia or ictal amnesia without ictal activity on EEG. We reviewed medical history, EEG, imaging data, and treatment outcome. Brain magnetic resonance imaging (MRI) showed no structural abnormalities in any of the patients. EEG showed left temporal irregular delta activity in three patients, with aphasia and nonspecific abnormalities in two other patients, all without clear ictal pattern. All patients demonstrated focal hypermetabolism on PET scan. The hypermetabolism was in the left frontotemporal region in patients with ictal aphasia and in the bilateral hippocampal region in the patient with amnesia. Three patients who received intravenous benzodiazepines during their episodes had transient clinical improvement. With antiepileptic drug (AED) treatment, symptoms gradually resolved in all patients. Concomitant resolution of PET hypermetabolism was documented in three patients who had follow up scans. One patient with ictal aphasia later developed recurrent episodes, each with recurrent PET hypermetabolism. This patient and one other patient required immune-modulating therapy in addition to AEDs. FDG-PET imaging should be considered as a diagnostic tool in patients with suspected ictal aphasia or amnesia, who fail to show clear evidence of ictal activity on EEG.

Erratum

Ghofrani M, Mahvelati F, Tonekaboni H. Headache as a sole manifestation in nonconvulsive status epilepticus. J Child Neurol. 2007;22:660-662. (DOI: 10.1177/0883073807303252) The article, Headache as a Sole Manifestation in Nonconvulsive Status Epilepticus, was published in Volume 21, Issue 11 (DOI: 10.1177/08830738060210111801), and it was mistakenly published a second time in Volume 22, Issue 5. This article has been removed from the online version of the second issue in which it appeared. We regret the error and apologize for any inconvenience.

MAP'ing CNS Development and Cognition: An ERKsome Process

The ERK MAP kinase signaling cascade plays critical roles in brain development, learning, memory, and cognition. It has recently been appreciated that mutation or deletion of elements within this signaling pathway leads to developmental syndromes in humans that are associated with impaired cognitive function and autism. Here, we review recent studies that provide insight into the biological roles of the ERKs in the brain that may underlie the cognitive deficits seen in these syndromes.