Long-term seizure and psychiatric outcomes following laser ablation of mesial temporal structures.

OBJECTIVE: Postsurgical seizure outcome following laser interstitial thermal therapy (LiTT) for the management of drug-resistant mesial temporal lobe epilepsy (MTLE) has been limited to 2 years. Furthermore, its impact on presurgical mood and anxiety disorders has not been investigated. The objectives of this study were (1) to identify seizure outcome changes over a period ranging from 18 to 81 months; (2) to investigate the seizure-free rate in the last follow-up year; (3) to identify the variables associated with seizure freedom; and (4) to identify the impact of LiTT on presurgical mood and anxiety disorders. METHODS: Medical records of all patients who underwent LiTT for MTLE from 2013 to 2019 at the University of Miami Comprehensive Epilepsy Center were retrospectively reviewed. Demographic, epilepsy-related, cognitive, psychiatric, and LiTT-related data were compared between seizure-free (Engel Class I) and non-seizure-free (Engel Class II + III + IV) patients. Statistical analyses included univariate and multivariate stepwise logistic regression analyses. RESULTS: Forty-eight patients (mean age = 43 ± 14.2 years, range = 21-78) were followed for a mean period of 50 ± 20.7 months (range = 18-81); 29 (60.4%) achieved an Engel Class I outcome, whereas 11 (22.9%) had one to three seizures/year. Seizure-freedom rate decreased from 77.8% to 50% among patients with 24- and >61-month follow-up periods, respectively. In the last follow-up year, 83% of all patients were seizure-free. Seizure freedom was associated with having mesial temporal sclerosis (MTS), no presurgical focal to bilateral tonic-clonic seizures, and no psychopathology in the last follow-up year. Presurgical mood and/or anxiety disorder were identified in 30 patients (62.5%) and remitted after LiTT in 19 (62%). SIGNIFICANCE: LiTT appears to be a safe and effective surgical option for treatment-resistant MTLE, particularly among patients with MTS. Remission of presurgical mood and anxiety disorders can also result from LiTT.

Laser thermal ablation for mesiotemporal epilepsy: Analysis of ablation volumes and trajectories.

OBJECTIVE: To identify features of ablations and trajectories that correlate with optimal seizure control and minimize the risk of neurocognitive deficits in patients undergoing laser interstitial thermal therapy (LiTT) for mesiotemporal epilepsy (mTLE). METHODS: Clinical and radiographic data were reviewed from a prospectively maintained database of all patients undergoing LiTT for the treatment of mTLE at the University of Miami Hospital. Standard preoperative and postoperative evaluations, including contrast-enhanced magnetic resonance imaging (MRI) and neuropsychological testing, were performed in all patients. Laser trajectory and ablation volumes were computed both by manual tracing of mesiotemporal structures and by nonrigid registration of ablation cavities to a common reference system based on 7T MRI data. RESULTS: Among 23 patients with at least 1-year follow-up, 15 (65%) were free of disabling seizures since the time of their surgery. Sparing of the mesial hippocampal head was significantly correlated with persistent disabling seizures (p = 0.01). A lateral trajectory through the hippocampus showed a trend for poor seizure outcome (p = 0.08). A comparison of baseline and postoperative neurocognitive testing revealed areas of both improvement and worsening, which were not associated with ablation volume or trajectory. SIGNIFICANCE: At 1-year follow-up, LiTT appears to be a safe and effective tool for the treatment of mTLE, although a longer follow-up period is necessary to confirm these observations. Better understanding of the impact of ablation volume and location could potentially fine-tune this technique to improve seizure-freedom rates and associated neurologic and cognitive changes.

Atypical language representation in children with intractable temporal lobe epilepsy.

This study evaluated language organization in children with intractable epilepsy caused by temporal lobe focal cortical dysplasia (FCD) alone or dual pathology (temporal lobe FCD and hippocampal sclerosis, HS). We analyzed clinical, neurological, fMRI, neuropsychological, and histopathologic data in 46 pediatric patients with temporal lobe lesions who underwent excisional epilepsy surgery. The frequency of atypical language representation was similar in both groups, but children with dual pathology were more likely to be left-handed. Atypical receptive language cortex correlated with lower intellectual capacity, verbal abstract conceptualization, receptive language abilities, verbal working memory, and a history of status epilepticus but did not correlate with higher seizure frequency or early seizure onset. Histopathologic substrate had only a minor influence on neuropsychological status. Greater verbal comprehension deficits were noted in children with atypical receptive language representation, a risk factor for cognitive morbidity.

Predictors of seizure-free outcome after epilepsy surgery for pediatric tuberous sclerosis complex.

PURPOSE: Variable predictors of postsurgical seizure outcome have been reported in children with tuberous sclerosis complex (TSC). We analyzed a large surgical series of pediatric TSC patients in order to identify prognostic factors crucial for selection of subjects for epilepsy surgery. METHODS: Thirty-three children with TSC who underwent excisional epilepsy surgery at Miami Children's Hospital were retrospectively reviewed. A total of 29 clinical, neuropsychological, electroencephalography (EEG), magnetic resonance imaging (MRI), and surgical variables were analyzed and related to seizure outcomes. Univariate Barnard's exact test, Wilcoxon's rank-sum test, and multivariate statistical Cox's model were used to examine the significance of associations between the variables and seizure outcome. KEY FINDINGS: Eighteen patients (55%) have been seizure-free 2 years after (final) surgery; postoperative complications occurred in five subjects (15%). Complete removal of epileptogenic tissue detected by both MRI and intracranial EEG, regional scalp interictal EEG patterns, and agreement of interictal and ictal EEG localization were the most powerful predictors of seizure-free outcome. Other significant predictors included occurrence of regional scalp ictal EEG patterns, fewer brain regions affected by tubers, presence of preoperative hemiparesis, and one-stage surgery. Remaining factors such as age at seizure onset, incidence of infantile spasms or other seizure types, duration of epilepsy, seizure frequency, mental retardation, as well as types and extent of resections did not influence outcome. SIGNIFICANCE: Perioperative features rather than preoperative variables are the most important determinants of postsurgical seizure outcome in patients with TSC. Our findings may assist in the surgical management of these patients.

Assessment and Management of Long COVID.

Almost two years into the pandemic, the scientific and healthcare communities continue to learn a great deal regarding COVID-19, the disease produced by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Broad variability during acute COVID-19 infection is seen, ranging from asymptomatic presentation to death. The vast majority of individuals who develop COVID-19 return to their pre-COVID-19 baseline within several weeks. However, a portion of patients will develop a post-COVID-19 syndrome of persistent cognitive, somatic, and behavioral symptoms. This syndrome, designated as post-acute sequelae of SARS-CoV-2 infection, is more commonly known as long COVID. The objectives of this paper are to inform psychologists regarding our current understanding of the underlying pathophysiology of COVID-19, review criteria for range of severity during acute illness, present clinical manifestations of long haul phenomena, and discuss the emerging literature base of evidence-based treatment and management approaches.

Correction to: Assessment and Management of Long COVID.

[This corrects the article DOI: 10.1007/s42843-022-00055-8.].

Low-grade focal cortical dysplasia is associated with prenatal and perinatal brain injury.

PURPOSE: Prenatal and perinatal adverse events are reported to have a pathogenetic role in focal cortical dysplasia (FCD). However, no data are available regarding the prevalence and significance of this association. A cohort of children with significant prenatal and perinatal brain injury and histologically proven mild malformations of cortical development (mMCD) or FCD was analyzed. METHODS: We retrospectively evaluated a surgical series of 200 patients with histologically confirmed mMCD/FCD. Combined historical and radiologic inclusion criteria were used to identify patients with prenatal and perinatal risk factors. Electroclinical, imaging, neuropsychological, surgical, histopathologic, and seizure outcome data were reviewed. RESULTS: Prenatal and perinatal insults including severe prematurity, asphyxia, bleeding, hydrocephalus, and stroke occurred in 12.5% of children with mMCD/FCD (n = 25). Their epilepsy was characterized by early seizure onset, high seizure frequency, and absence of seizure control. Patients with significant prenatal and perinatal risk factors had more abnormal neurologic findings, lower intelligence quotient (IQ) scores, and slower background EEG activity than mMCD/FCD subjects without prenatal or perinatal brain injury. MRI evidence of cortical malformations was identified in 74% of patients. Most patients underwent large multilobar resections or hemispherectomies; 54% were seizure-free 2 years after surgery. Histologically "milder" forms of cortical malformations (mMCD and FCD type I) were observed most commonly in our series. CONCLUSIONS: Surgically remediable low-grade cortical malformations may occur in children with significant prenatally and perinatally acquired encephalopathies and play an important role in the pathogenesis of their epilepsy. Presurgical detection of dysplastic cortex has important practical consequences for surgical planning.

Cognitive outcomes following laser interstitial therapy for mesiotemporal epilepsies.

OBJECTIVE: To provide a review of cognitive outcomes across a full neuropsychological profile in patients who underwent laser interstitial thermal therapy (LiTT) for mesiotemporal epilepsy (mTLE). METHODS: We examined cognitive outcomes following LiTT for mTLE by reviewing a consecutive series of 26 patients who underwent dominant or nondominant hemisphere procedures. Each patient's pre- and postsurgical performance was examined for clinically significant change (>1SD improvement or decline on standardized scores), with a neuropsychologic battery that included measures of language, memory, executive functioning, and processing speed. RESULTS: Presurgical performance was largely consistent with previous research, where patients suffering from dominant hemisphere epilepsies demonstrated deficits in verbal learning and memory, whereas patients with nondominant hemisphere scored lower on visually mediated tests. Case-by-case review comparing presurgical to postsurgical scores revealed clinically significant improvement in both dominant and nondominant patients in learning and memory and other aspects of cognition such as processing speed and executive functioning. Of the few patients who did experience clinically significant decline following LiTT, a greater proportion had undergone dominant hemisphere procedures. CONCLUSIONS: Compared with the outcome literature of dominant open anterior temporal lobectomies (ATLs), where postsurgical decline has been documented in up to 40%-60% of cases, our LiTT case series exhibited a much lower incidence of postoperative language or verbal memory decline. Moreover, promising rates of postoperative improvements were also observed across multiple cognitive domains. Future studies exploring cognitive outcomes following LiTT should include comprehensive neuropsychological findings, rather than only select domains, as clinically significant change can occur in areas other than those typically associated with mesiotemporal structures.

Different features of histopathological subtypes of pediatric focal cortical dysplasia.

OBJECTIVE: Focal cortical dysplasia (FCD) is the most frequent pathological finding in pediatric epilepsy surgery patients. Several histopathological types of FCD are distinguished. The aim of the study was to define distinctive features of FCD subtypes. METHODS: We retrospectively reviewed clinical, electroencephalographic, magnetic resonance imaging, neuropsychological, and surgical variables, and seizure outcome data in 200 children. Cortical malformations were histopathologically confirmed in all patients, including mild malformation of cortical development type II (mMCD) in 36, FCD type Ia in 55, FCD type Ib in 39, FCD type IIa in 35, and FCD type IIb in 35 subjects. RESULTS: Perinatal risk factors were more frequent in mMCD/FCD type I than FCD type II. Children with FCD type IIb had more localized ictal electroencephalographic patterns and magnetic resonance imaging changes. Increased cortical thickness, abnormal gyral/sulcal patterns, gray/white matter junction blurring, and gray matter signal abnormality in fluid-attenuated inversion recovery and T2-weighted sequences occurred more often in FCD type II, were infrequent in FCD type I, and rare in mMCD. Lobar hypoplasia/atrophy was common in FCD type I. Hippocampal sclerosis was most frequent in FCD type I. Neuropsychological testing demonstrated no significant differences between the groups. There was a trend toward better surgical outcomes in FCD type II compared with FCD type I patients. INTERPRETATION: Different histopathological types of mMCD/FCD have distinct clinical and imaging characteristics. The ability to predict the subtype before surgery could influence surgical planning. Invasive electroencephalographic study should be considered when mMCD/FCD type I is expected based on noninvasive tests.

Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Mesial Temporal Epilepsy: A Case Series Analysis of Outcomes and Complications at 2-Year Follow-Up.

BACKGROUND: Laser interstitial thermal therapy (LITT) presents an important new minimally invasive tool in the management of drug-resistant mesial temporal epilepsy (MTE). However, because of its relative novelty, not much is known about long-term seizure freedom rates. The objective of this study was to evaluate the postsurgical seizure outcome following LITT after a minimum follow-up period of 2 years. METHODS: Medical records of all patients who underwent LITT for MTE from 2013 to 2018 at our comprehensive epilepsy center under a single surgeon were retrospectively reviewed. Data related to demographics, presurgical evaluations, and seizure outcome were compared between seizure-free (SF) and non-seizure-free (NSF) patients. RESULTS: In all, 26 patients were identified with at least 2 years of follow-up. Mean age was 43.8 years ± 11.6 years, and 46.2% were female. After a mean follow-up time of 42.9 months (range, 24.3-58.8 months), 61.5% (16/26) were free of disabling seizures, and 26.9% (7/26) had only rare disabling seizures. Whereas seizure-freedom rates between patients with and without mesial temporal sclerosis (MTS) were not statistically different (68% vs. 43%, P = 0.23), NSF patients without MTS had a shorter median time to first seizure than did NSF patients with MTS (0.55 month vs. 10 months, log-rank test P = 0.007). Postoperative complications occurred in 2 patients (7.7%), consisting of 1 permanent and 1 transient homonymous hemianopia. CONCLUSIONS: LITT appears to be a safe and effective initial surgical option for treatment-resistant MTE. Among patients who have seizures after treatment, those without MTS appear to have seizures earlier than those with MTS.

[Frequency of Trypanosoma cruzi infection in patients with implanted pacemaker]. / Frecuencia de anticuerpos anti-Trypanosoma cruzi en pacientes portadores de marcapasos de la Clínica San Pedro Claver de Bogotá.

INTRODUCTION: In Colombia the impact of infections of Trypanosoma cruzi are known to produce chronic cardiopathy and expressed by bradycardia. In Colombia the extent and impact of these infections has not been examined. OBJECTIVE: The current study aimed to determine the prevalence of T. cruzi infection as measured by serology, in a population of patients with cardiopathy that required a permanent pacemaker as treatment for cardiac rhythm abnormalities and conduction blocking. MATERIALS AND METHODS: A cross sectional study sampled 332 patients from the pacemaker clinic at the San Pedro Claver Hospital in Bogotá, Colombia, for one year (2004-2005). Epidemiological and clinical data were obtained through interviews and physical examination. Serological tests consisted of indirect inmunofluorescence assay and ELISA. Statistical analyses were accomplished with chi-square and Students t tests. RESULTS: Of patients with pacemakers, 17.1% had anti-T. cruzi antibodies (seropositive). At the time when the pacemaker was implanted, chronic Chagas disease patients were younger (55+/-13 years) than those patients with cardiopathy (60+/-17 years) with no anti-T. cruzi antibodies (p<0.01). The seropositive group was aware of the Chagas disease vector (83.6%) in contrast to the seronegative group (39.6%, p<0.001). In 60% of the patients of the seropositive group, no clinical signs of the disease were apparent. The geographical origin of the seropositive group were traced to regions in Colombia known to be endemic for Chagas disease transmission. CONCLUSION: Chagas disease prevalence is high in Colombian patients who required a permanent cardiac pacemaker. Chronic Chagas disease patients required pacemaker implant at a younger age in contrast with patients with other cardiac pathologies. The clinical recognition of Chagas disease associated with cardiopathy is low despite the epidemiological data.

Near-infrared spectroscopy to define cognitive frontal lobe functions.

Near-infrared spectroscopy (NIRS) monitors changes in the regional cerebral oxygenation (rSO) and has been used to study cerebral physiologic functions in normal states and during epileptic seizures. Yet, the limitations and pitfalls of the technique are not fully understood. The authors evaluated NIRS changes over the frontal lobes during language tasks known to be associated with the integrity of the dominant frontal lobe in 17 normal adults (handedness: 14 right, 3 left). Recording protocol involved a baseline (3 minutes) with the subject relaxed and thinking of a blue sky and a second baseline during voluntary mouth movements mimicking speech. Two standardized neuropsychological word-generation tasks (controlled word-association tests: CFL and Animals) were then administered (4 minutes total) followed again by the two baseline procedures. Mouth movement without verbalization increased rSO values in excess of 2 SD of baseline fluctuations in 70% of the subjects. A t-test comparison of these baseline measurements was statistically significant (P < 0.0001). A general linear model repeated-measures procedure was then used to statistically examine NIRS increments during the cognitive tasks above and beyond the contribution produced by mouth movements during the second baseline. Compared to the second baseline, rSO further increased significantly during the word generation tasks (left frontal F value = 21.4, P < 0.0001; right frontal F value = 15.2, P < 0.001), confirming the utility of the technique. There was no apparent difference related to handedness. These findings corroborate the usefulness of NIRS to demonstrate focal cerebral activation during an "executive" language task. However, interpretation of changes can be confounded by extracerebral factors such as mouth movement, a finding of particular relevance in NIRS clinical applications.

Mild cognitive impairment: new neuropsychological and pharmacological target.

Mild cognitive impairment (MCI) is increasingly being conceptualized in the literature as a cognitive disturbance representing a transitional phase between normal aging and dementia. The operational definitions of MCI provide an opportunity for neuropsychologists to detect subtle deficit and monitor cognitive status sequentially in order to determine rate and degree of progression. More importantly, clinical and neuropsychological studies are needed that can better characterize which MCI patients are at greatest risk for conversion to dementia. Preliminary data has also designated MCI as a potential indicator for initiation of pharmacotherapy, with the objective of decelerating rate of progression to dementia. Current criteria and clinical issues related to MCI are discussed, with the objective of better familiarizing clinicians with this syndrome and fostering ongoing investigations.

The bacterial microbiome in paired vaginal and vestibular samples from women with vulvar vestibulitis syndrome.

Composition of the bacterial microbiome in the vagina and vestibule from 30 women with vulvar vestibulitis syndrome (VVS) and 15 healthy controls were compared by pyrosequencing 16S rRNA gene amplicons. Vaginal concentrations of interleukin (IL)-1ß were determined by ELISA. Questionnaires elicited clinical and symptom data. Eighteen genera were detected in vaginal samples, and 23 genera were identified in vestibule samples, from women with VVS. The genera at both sites and the mean number of genera in subjects with VVS were largely similar to those in control subjects. However, differences were noted including higher proportions of Streptococcus and Enterococcus in women with VVS. Furthermore, Lactobacillus iners was more frequently identified in women with VVS while L. crispatus was more frequent in the control women. The dominant bacterial genera in the vagina closely paralleled the dominant genera present in the corresponding vestibular sample in both groups, leading us to postulate that vaginal secretions are an important source of bacteria present on the vestibule. Vaginal IL-1ß levels were similar and varied depending on the dominant bacteria. We conclude in this pilot study that no major differences are apparent in the vagina and vestibule between women with or without VVS, except for an increased prevalence of Streptococcus and L. iners in some women with VVS.

Early seizure onset and dysplastic lesion extent independently disrupt cognitive networks.

OBJECTIVE: To determine the most important factors influencing neuropsychological performance in children with intractable epilepsy due to focal cortical dysplasia and the nature of the interaction among significant variables. METHODS: Surgical patients with histologically verified focal cortical dysplasia were retrospectively evaluated to determine the impact of histopathology, extent of lobar involvement, hemispheric laterality, age at onset, and duration of epilepsy on cognitive functioning. A composite neuropsychological variable was obtained by transforming data from 5 major cognitive domains using principal components analysis. Multiple regression was used to examine the unique contributions of predictor variables on composite cognition and Full Scale IQ. Data were qualitatively evaluated for nonstatistical trends. RESULTS: Poor cognitive outcomes were associated with early age at onset of epilepsy (AOE) and widespread dysplastic involvement. Extent of dysplasia and AOE together accounted for 35% of Full Scale IQ variance, and 21% of composite cognitive performance. Each factor contributed independently to cognitive dysfunction. CONCLUSIONS: Early AOE disrupts critical periods of development and leads to poor cognitive outcome, but children with multilobar dysplasia are likely to have diminished cognitive skills regardless of AOE. Later AOE is not expected to mitigate deficits because of widespread pathology, nor would a localized lesion be likely to mollify the developmental deficits resulting from early AOE.

Frontal activation and connectivity using near-infrared spectroscopy: verbal fluency language study.

Near infrared spectroscopy (NIRS) is an optical technique with high temporal resolution and reasonably good spatial resolution, which allows non invasive measurement of the blood oxygenation of tissue. The current work is focused in assessing and correlating brain activation, connectivity and cortical lateralization of the frontal cortex in response to language-based stimuli, using NIRS. Experimental studies were performed on 15 normal right-handed adults, wherein the participants were presented with a verbal fluency task. The hemodynamic responses in the pre- and anterior frontal cortex were assessed in response to a Word generation task in comparison to the baseline random Jaw movement and Rest conditions. The functional connectivity analysis was performed using zero-order correlations and the cortical lateralization was evaluated as well. An increase in oxy- and a decrease in deoxy-hemoglobin were observed during verbal fluency task in the frontal cortex. Unlike in the pre-frontal cortex, the hemodynamic response in the anterior frontal during verbal fluency task was not significantly different from that during random Jaw movement. Bilateral activation and symmetrical connectivity were observed in the pre-frontal cortex, independent of the stimuli presented. A left cortical dominance and asymmetry connectivity was observed in the anterior frontal during the verbal fluency task. The work is focused to target the pediatric epileptic populations in the future, where understanding the brain functionality (activation, connectivity, and dominance) in response to language is essential as a part of the pre-surgical evaluation in a clinical environment.

Status atual do tratamento em uma sessão para fobias específicas / Current status of one-session treatment for specific phobias

O tratamento em uma sessão (OST) é uma forma de exposição intensa para o tratamento das fobias especificas. O OST combina técnicas de exposição ao vivo, reestruturação cognitiva, modelagem participativa e reforço positivo em uma única sessão de 03 horas. O presente trabalho teve como objetivo analisar os artigos indexados nas bases de dados Medline e Psycinfo no período entre 2009 e 2020, identificando estudos sobre a eficácia do OST nas fobias especificas. A amostra final foi constituída por 13 artigos. Todos os artigos atestaram a eficácia positiva dessa forma de tratamento nos diversos estímulos fóbicos tratados. Uma maior divulgação do OST deve ser incentivada no Brasil, para que as pessoas acometidas por esse transtorno de ansiedade possam se beneficiar dos efeitos muito bem estabelecidos dessa forma de tratamento.(AU)

One-session treatment (OST) is a form of massed exposure therapy for the treatment of specific phobias. OST combines in vivo exposure techniques, cognitive restructuring, participant modeling and positive reinforcement in a single session of 03 hours. This study aimed to examine the articles indexed on the databases of Medline and Psycinfo in the period between 2009 and 2020, to identifying studies on the effectiveness of OST in specific phobias. The final sample consisted of 13 articles. Articles attested to the positive effectiveness of this form of treatment in the various specific phobic disorders. Greater dissemination of OST should be encouraged in Brazil, so that people affected by this anxiety disorder can benefit from the well-established effects of this form of treatment.(AU)

Prevalence of metabolic syndrome in a predominantly cuban, psychiatrically ill, and homeless population.

OBJECTIVE: This study examined the prevalence of metabolic syndrome among a group of psychiatric outpatients enrolled in a homeless program that is located in a predominantly Hispanic geographic area of South Florida. METHOD: Data for this retrospective, cross-sectional analysis were obtained from a record review of 122 adult patients who received full medical and psychiatric assessments based on DSM-IV criteria during participation in our homeless program from January 2009 to May 2009. The primary outcome measure was the presence of metabolic syndrome. RESULTS: The prevalence of metabolic syndrome within this population was 29.5%. Elevated waist circumference (48.5%) and elevated blood pressure (44.3%) were the 2 most frequent risk factors for the syndrome. Mean length of homelessness was 3.93 years, with no significant relationship noted between the presence of metabolic syndrome and duration of homelessness. Ninety-three percent of the subjects had been diagnosed with either schizophrenia or a mood disorder, and 61% had been treated with an atypical antipsychotic for at least 2 months over the preceding year. Our sample was predominantly Hispanic (79.5%), with Cuban Americans comprising 95% of that group. Among Hispanics, the prevalence rate of metabolic syndrome was 28.9%. CONCLUSIONS: Within our sample, homeless individuals compared to the general adult population in the United States seem to be at equal risk for metabolic syndrome. Although other studies have suggested an increased prevalence for metabolic syndrome among Hispanics, the obtained rate for our particular Hispanic sample was consistent with estimated prevalence of non-Hispanic individuals in the United States. Intervention programs rendering services to this population should include routine screening for presence of cardiovascular risk factors constituting metabolic syndrome.

Agenesis of the arcuate fasciculi in congenital bilateral perisylvian syndrome: a diffusion tensor imaging and tractography study.

OBJECTIVE: To describe the absence of the arcuate fasciculi in 2 cases of congenital bilateral perisylvian syndrome (CBPS). DESIGN: Case series. SETTING: Pediatric referral hospital-based study. PATIENTS: Two patients with CBPS, referred to our institution as candidates for surgical treatment of epilepsy. Intervention Diffusion tensor imaging (1.5-T scanner; 15 encoding directions; b = 800 s/mm(2)) and deterministic tractography of the main projection and association tracts. MAIN OUTCOME MEASURES: Neuropsychology evaluation; fractional anisotropy, apparent diffusion coefficients, and anatomical aspect of the tracts. RESULTS: Absence of the arcuate fasciculus was observed in both subjects. Ancillary findings were complete absence of the superior longitudinal fasciculi in 1 case and underdevelopment in the other. Low fractional anisotropy of the left inferior occipitofrontal fasciculus was found in both cases. The same tract was maloriented in 1 of the cases. CONCLUSION: Agenesis of the arcuate fasciculus may accompany CBPS.