CLN3 disease is a pediatric neurodegenerative condition wherein seizures are common. The most com... more CLN3 disease is a pediatric neurodegenerative condition wherein seizures are common. The most common disease‐causing variant is an ~1‐kb deletion in CLN3. We investigated seizure phenotype in relation to genotype and to adaptive behavior, MR spectroscopy and CSF biochemical markers in a CLN3 cohort. We performed seizure phenotyping using clinical history, EEG, and the Unified Batten Disease Rating Scale (UBDRS) seizure score. We assessed correlations of seizure severity with disease severity (UBDRS capability), adaptive behavior composite score (ABC; Vineland‐3), glutamate+glutamine+GABA and N‐acetylaspartate+N‐acetylaspartyl glutamate (MR spectroscopy), and CSF neurofilament light chain (NEFL) levels. In 20 participants, median age was 10.7 years (IQR = 7.8). Eighteen completed baseline EEG; 12 had a 1‐year follow‐up. Seizures were reported in 14 (8 1‐kb deletion homozygotes), with median age at onset of 10.0 (IQR = 6.8). Epileptiform discharges were noted in 15 (9 homozygotes). Bilateral tonic clonic (n = 11) and nonmotor seizures (n = 7) were most common. UBDRS seizure score correlated with age (rp = 0.50; [0.08,0.77]; P = .02), UBDRS capability (rp = −0.57; [−0.81,−0.17]; P = .009) and ABC (rp = −0.66; [−0.85,−0.31]; P = .001) scores, glutamate+glutamine+GABA (rp = −0.54; [−0.80,−0.11]; P = .02) and N‐acetylaspartate+N‐acetylaspartyl glutamate (rp = −0.54; [−0.80,−0.11]; P = .02), and CSF NEFL (rp = 0.65; [0.29,0.85]; P = .002) levels. After controlling for age, correlations with ABC and CSF NEFL remained significant. In our CLN3 cohort, seizures and epileptiform discharges were frequent and often started by age 10 years without significant difference between genotypes. ABC and CSF NEFL correlate with UBDRS seizure score, reflecting the role of seizures in the neurodegenerative process. Longitudinal evaluations in a larger cohort are needed to confirm these findings.
Objective: To study the association between HHV-6 viruses and hippocampal volumes in patients wit... more Objective: To study the association between HHV-6 viruses and hippocampal volumes in patients with mesial temporal sclerosis (MTS). Background: Human herpes virus 6 (HHV-6) may play an etiologic role in mesial temporal lobe epilepsy and MTS. Previous studies found an association with febrile status epilepticus. We have reported higher prevalence of HHV-6 in MTS compared to other epilepsy etiologies. Design/Methods: We studied prospectively 34 patients referred to the Clinical Epilepsy Section, NINDS, NIH for evaluation of intractable epilepsy, with ictal video-EEG monitoring, 1.5-T or 3T GE Signa MRI with FLAIR, T1- and T2- weighted images, and 3-D SPGR or MP-RAGE. An investigator blinded to other data manually traced hippocampi manually on each slice, before assembly into three- dimensional volumes. The main study outcome measure was the hippocampal volume asymmetry index (AI) ipsilateral and contralateral to the seizure focus compared between HHV-6 positive and negative patients. Viral DNA was isolated from fresh brain tissue. For 25 patients, viral detection was performed using quantitative real time PCR specific for HHV-6A and HHV-6B. For nine patients, viral DNA detection was performed using digital droplet PCR (DdPCR) specific for HHV-6A and HHV-6B. DdPCR is a highly sensitive and precise novel PCR technology that enables absolute quantification of target DNA molecules. Statistical analysis was performed with SPSS. Results: HHV-6B was detected in 16 of 25 patients studied with real time PCR. Among 9 studied with DdPCR, one had HHV-6A, two HHV-6B, and two both viruses. HHV-6-negative patients had significantly greater AI and lower ipsilateral volume than HHV-6 positive patients (p<0.001). On ANOVA including epilepsy duration, HHV-6 status retained a significant effect on AI (p<0.01). Conclusion: Our data suggest multiple potential etiologies for MTS. HHV6 may have a protective effect on hippocampal volume. Study Supported By: Division of Intramural Research NINDS NIH Disclosure: Dr. Theodore has received personal compensation in an editorial capacity for Epilepsy Research. Dr. Theodore holds stock and/or stock options in General Electric. Dr. Leibovitch has nothing to disclose. Dr. Billioux has nothing to disclose. Dr. Germayen has nothing to disclose. Dr. Kalikhman has nothing to disclose. Dr. Inati has nothing to disclose. Dr. Heiss has nothing to disclose. Dr. Zaghloul has nothing to disclose. Dr. Jacobson has nothing to disclose.
We report the case of a multifocal dysembryoplastic neuroepithelial tumor (DNT) in a 7-year-old g... more We report the case of a multifocal dysembryoplastic neuroepithelial tumor (DNT) in a 7-year-old girl with local tumor regrowth 6 years later. The tumor was localized in the right parietal lobe extending from the cortex into the periventricular white matter. After subtotal resection of a histopathologically confirmed DNT we observed unexpected tumor progression in long-term follow-up. Therefore, a second surgery was performed when the patient was 14 years of age. In neuropathological examination of the second specimen the tumor showed an increased cellularity and pleomorphism, microvascular proliferations, an elevated proliferative activity (MIB1-index focally up to 10%) and cellular atypia not typical for WHO grade I DNT. Furthermore, MRI studies showed additional supratentorial and infratentorial lesions which remained stable over years and are also well consistent with DNTs. Thus, an unusual form of a DNT with multifocal lesions, local regrowth and morphological transformation is supposed.
CLN3 disease is a pediatric neurodegenerative condition wherein seizures are common. The most com... more CLN3 disease is a pediatric neurodegenerative condition wherein seizures are common. The most common disease‐causing variant is an ~1‐kb deletion in CLN3. We investigated seizure phenotype in relation to genotype and to adaptive behavior, MR spectroscopy and CSF biochemical markers in a CLN3 cohort. We performed seizure phenotyping using clinical history, EEG, and the Unified Batten Disease Rating Scale (UBDRS) seizure score. We assessed correlations of seizure severity with disease severity (UBDRS capability), adaptive behavior composite score (ABC; Vineland‐3), glutamate+glutamine+GABA and N‐acetylaspartate+N‐acetylaspartyl glutamate (MR spectroscopy), and CSF neurofilament light chain (NEFL) levels. In 20 participants, median age was 10.7 years (IQR = 7.8). Eighteen completed baseline EEG; 12 had a 1‐year follow‐up. Seizures were reported in 14 (8 1‐kb deletion homozygotes), with median age at onset of 10.0 (IQR = 6.8). Epileptiform discharges were noted in 15 (9 homozygotes). Bilateral tonic clonic (n = 11) and nonmotor seizures (n = 7) were most common. UBDRS seizure score correlated with age (rp = 0.50; [0.08,0.77]; P = .02), UBDRS capability (rp = −0.57; [−0.81,−0.17]; P = .009) and ABC (rp = −0.66; [−0.85,−0.31]; P = .001) scores, glutamate+glutamine+GABA (rp = −0.54; [−0.80,−0.11]; P = .02) and N‐acetylaspartate+N‐acetylaspartyl glutamate (rp = −0.54; [−0.80,−0.11]; P = .02), and CSF NEFL (rp = 0.65; [0.29,0.85]; P = .002) levels. After controlling for age, correlations with ABC and CSF NEFL remained significant. In our CLN3 cohort, seizures and epileptiform discharges were frequent and often started by age 10 years without significant difference between genotypes. ABC and CSF NEFL correlate with UBDRS seizure score, reflecting the role of seizures in the neurodegenerative process. Longitudinal evaluations in a larger cohort are needed to confirm these findings.
Background: fMRI language tasks readily identify frontal language areas; temporal activation has ... more Background: fMRI language tasks readily identify frontal language areas; temporal activation has been less consistent. No studies have compared clinical visual judgment to quantitative region of interest (ROI) analysis.Objective: To identify temporal language areas in patients with partial epilepsy using a reading paradigm with clinical and ROI interpretation.Methods: Thirty patients with temporal lobe epilepsy, aged 8 to 56 years, had 1.5-T fMRI. Patients silently named an object described by a sentence compared to a visual control. Data were analyzed with ROI analysis from t-maps. Regional asymmetry indices (AI) were calculated ([L−R]/[L+R]) and language dominance defined as >0.20. t-Maps were visually rated by three readers at three t thresholds. Twenty-one patients had intracarotid amobarbital test (IAT).Results: The fMRI reading task provided evidence of language lateralization in 27 of 30 patients with ROI analysis. Twenty-five were left dominant, two right, one bilateral, ...
Objective: To study the association between HHV-6 viruses and hippocampal volumes in patients wit... more Objective: To study the association between HHV-6 viruses and hippocampal volumes in patients with mesial temporal sclerosis (MTS). Background: Human herpes virus 6 (HHV-6) may play an etiologic role in mesial temporal lobe epilepsy and MTS. Previous studies found an association with febrile status epilepticus. We have reported higher prevalence of HHV-6 in MTS compared to other epilepsy etiologies. Design/Methods: We studied prospectively 34 patients referred to the Clinical Epilepsy Section, NINDS, NIH for evaluation of intractable epilepsy, with ictal video-EEG monitoring, 1.5-T or 3T GE Signa MRI with FLAIR, T1- and T2- weighted images, and 3-D SPGR or MP-RAGE. An investigator blinded to other data manually traced hippocampi manually on each slice, before assembly into three- dimensional volumes. The main study outcome measure was the hippocampal volume asymmetry index (AI) ipsilateral and contralateral to the seizure focus compared between HHV-6 positive and negative patients. Viral DNA was isolated from fresh brain tissue. For 25 patients, viral detection was performed using quantitative real time PCR specific for HHV-6A and HHV-6B. For nine patients, viral DNA detection was performed using digital droplet PCR (DdPCR) specific for HHV-6A and HHV-6B. DdPCR is a highly sensitive and precise novel PCR technology that enables absolute quantification of target DNA molecules. Statistical analysis was performed with SPSS. Results: HHV-6B was detected in 16 of 25 patients studied with real time PCR. Among 9 studied with DdPCR, one had HHV-6A, two HHV-6B, and two both viruses. HHV-6-negative patients had significantly greater AI and lower ipsilateral volume than HHV-6 positive patients (p<0.001). On ANOVA including epilepsy duration, HHV-6 status retained a significant effect on AI (p<0.01). Conclusion: Our data suggest multiple potential etiologies for MTS. HHV6 may have a protective effect on hippocampal volume. Study Supported By: Division of Intramural Research NINDS NIH Disclosure: Dr. Theodore has received personal compensation in an editorial capacity for Epilepsy Research. Dr. Theodore holds stock and/or stock options in General Electric. Dr. Leibovitch has nothing to disclose. Dr. Billioux has nothing to disclose. Dr. Germayen has nothing to disclose. Dr. Kalikhman has nothing to disclose. Dr. Inati has nothing to disclose. Dr. Heiss has nothing to disclose. Dr. Zaghloul has nothing to disclose. Dr. Jacobson has nothing to disclose.
We report the case of a multifocal dysembryoplastic neuroepithelial tumor (DNT) in a 7-year-old g... more We report the case of a multifocal dysembryoplastic neuroepithelial tumor (DNT) in a 7-year-old girl with local tumor regrowth 6 years later. The tumor was localized in the right parietal lobe extending from the cortex into the periventricular white matter. After subtotal resection of a histopathologically confirmed DNT we observed unexpected tumor progression in long-term follow-up. Therefore, a second surgery was performed when the patient was 14 years of age. In neuropathological examination of the second specimen the tumor showed an increased cellularity and pleomorphism, microvascular proliferations, an elevated proliferative activity (MIB1-index focally up to 10%) and cellular atypia not typical for WHO grade I DNT. Furthermore, MRI studies showed additional supratentorial and infratentorial lesions which remained stable over years and are also well consistent with DNTs. Thus, an unusual form of a DNT with multifocal lesions, local regrowth and morphological transformation is supposed.
Background: fMRI language tasks readily identify frontal language areas; temporal activation has ... more Background: fMRI language tasks readily identify frontal language areas; temporal activation has been less consistent. No studies have compared clinical visual judgment to quantitative region of interest (ROI) analysis.Objective: To identify temporal language areas in patients with partial epilepsy using a reading paradigm with clinical and ROI interpretation.Methods: Thirty patients with temporal lobe epilepsy, aged 8 to 56 years, had 1.5-T fMRI. Patients silently named an object described by a sentence compared to a visual control. Data were analyzed with ROI analysis from t-maps. Regional asymmetry indices (AI) were calculated ([L−R]/[L+R]) and language dominance defined as >0.20. t-Maps were visually rated by three readers at three t thresholds. Twenty-one patients had intracarotid amobarbital test (IAT).Results: The fMRI reading task provided evidence of language lateralization in 27 of 30 patients with ROI analysis. Twenty-five were left dominant, two right, one bilateral, ...
fMRI language tasks reliably identify language areas in presurgical epilepsy patients, but activa... more fMRI language tasks reliably identify language areas in presurgical epilepsy patients, but activation using single paradigms may disagree with the intracarotid amobarbital test (IAT). To determine whether a panel of fMRI tasks targeting different aspects of language processing increases accuracy in determining hemisphere language dominance. Twenty-six patients age 12 to 56 years, predominantly with temporal lobe epilepsy, were studied using whole-brain 1.5 T fMRI (echo planar imaging, blood oxygenation level-dependent) with three task categories using a block design: verbal fluency, reading comprehension, and auditory comprehension. fMRI t maps were visually rated at three thresholds. All patients had assessment of language lateralization by IAT. fMRI showed left dominance in 21 patients, right dominance in 2, and bilateral activation in 2; raters disagreed over a left vs right bilateral rating in 1 patient. There was full agreement between IAT and fMRI in 21 of 25 patients (IAT failed in 1). In three instances of partial disparity with IAT, the fMRI panel showed consistent findings across raters. Agreement between raters was excellent (partial disagreement in only one patient); the panel of tasks was superior to any single task for interrater agreement (Cramer V 0.93 [range 0.91 to 1.0] vs 0.72 [range 0.60 to 0.86]). A panel of fMRI language paradigms may be more accurate for evaluating partial epilepsy patients than a single task. A panel of tasks reduces the likelihood of nondiagnostic findings, improves interrater reliability, and helps confirm language laterality.
CLN3 disease is a pediatric neurodegenerative condition wherein seizures are common. The most com... more CLN3 disease is a pediatric neurodegenerative condition wherein seizures are common. The most common disease‐causing variant is an ~1‐kb deletion in CLN3. We investigated seizure phenotype in relation to genotype and to adaptive behavior, MR spectroscopy and CSF biochemical markers in a CLN3 cohort. We performed seizure phenotyping using clinical history, EEG, and the Unified Batten Disease Rating Scale (UBDRS) seizure score. We assessed correlations of seizure severity with disease severity (UBDRS capability), adaptive behavior composite score (ABC; Vineland‐3), glutamate+glutamine+GABA and N‐acetylaspartate+N‐acetylaspartyl glutamate (MR spectroscopy), and CSF neurofilament light chain (NEFL) levels. In 20 participants, median age was 10.7 years (IQR = 7.8). Eighteen completed baseline EEG; 12 had a 1‐year follow‐up. Seizures were reported in 14 (8 1‐kb deletion homozygotes), with median age at onset of 10.0 (IQR = 6.8). Epileptiform discharges were noted in 15 (9 homozygotes). Bilateral tonic clonic (n = 11) and nonmotor seizures (n = 7) were most common. UBDRS seizure score correlated with age (rp = 0.50; [0.08,0.77]; P = .02), UBDRS capability (rp = −0.57; [−0.81,−0.17]; P = .009) and ABC (rp = −0.66; [−0.85,−0.31]; P = .001) scores, glutamate+glutamine+GABA (rp = −0.54; [−0.80,−0.11]; P = .02) and N‐acetylaspartate+N‐acetylaspartyl glutamate (rp = −0.54; [−0.80,−0.11]; P = .02), and CSF NEFL (rp = 0.65; [0.29,0.85]; P = .002) levels. After controlling for age, correlations with ABC and CSF NEFL remained significant. In our CLN3 cohort, seizures and epileptiform discharges were frequent and often started by age 10 years without significant difference between genotypes. ABC and CSF NEFL correlate with UBDRS seizure score, reflecting the role of seizures in the neurodegenerative process. Longitudinal evaluations in a larger cohort are needed to confirm these findings.
Objective: To study the association between HHV-6 viruses and hippocampal volumes in patients wit... more Objective: To study the association between HHV-6 viruses and hippocampal volumes in patients with mesial temporal sclerosis (MTS). Background: Human herpes virus 6 (HHV-6) may play an etiologic role in mesial temporal lobe epilepsy and MTS. Previous studies found an association with febrile status epilepticus. We have reported higher prevalence of HHV-6 in MTS compared to other epilepsy etiologies. Design/Methods: We studied prospectively 34 patients referred to the Clinical Epilepsy Section, NINDS, NIH for evaluation of intractable epilepsy, with ictal video-EEG monitoring, 1.5-T or 3T GE Signa MRI with FLAIR, T1- and T2- weighted images, and 3-D SPGR or MP-RAGE. An investigator blinded to other data manually traced hippocampi manually on each slice, before assembly into three- dimensional volumes. The main study outcome measure was the hippocampal volume asymmetry index (AI) ipsilateral and contralateral to the seizure focus compared between HHV-6 positive and negative patients. Viral DNA was isolated from fresh brain tissue. For 25 patients, viral detection was performed using quantitative real time PCR specific for HHV-6A and HHV-6B. For nine patients, viral DNA detection was performed using digital droplet PCR (DdPCR) specific for HHV-6A and HHV-6B. DdPCR is a highly sensitive and precise novel PCR technology that enables absolute quantification of target DNA molecules. Statistical analysis was performed with SPSS. Results: HHV-6B was detected in 16 of 25 patients studied with real time PCR. Among 9 studied with DdPCR, one had HHV-6A, two HHV-6B, and two both viruses. HHV-6-negative patients had significantly greater AI and lower ipsilateral volume than HHV-6 positive patients (p<0.001). On ANOVA including epilepsy duration, HHV-6 status retained a significant effect on AI (p<0.01). Conclusion: Our data suggest multiple potential etiologies for MTS. HHV6 may have a protective effect on hippocampal volume. Study Supported By: Division of Intramural Research NINDS NIH Disclosure: Dr. Theodore has received personal compensation in an editorial capacity for Epilepsy Research. Dr. Theodore holds stock and/or stock options in General Electric. Dr. Leibovitch has nothing to disclose. Dr. Billioux has nothing to disclose. Dr. Germayen has nothing to disclose. Dr. Kalikhman has nothing to disclose. Dr. Inati has nothing to disclose. Dr. Heiss has nothing to disclose. Dr. Zaghloul has nothing to disclose. Dr. Jacobson has nothing to disclose.
We report the case of a multifocal dysembryoplastic neuroepithelial tumor (DNT) in a 7-year-old g... more We report the case of a multifocal dysembryoplastic neuroepithelial tumor (DNT) in a 7-year-old girl with local tumor regrowth 6 years later. The tumor was localized in the right parietal lobe extending from the cortex into the periventricular white matter. After subtotal resection of a histopathologically confirmed DNT we observed unexpected tumor progression in long-term follow-up. Therefore, a second surgery was performed when the patient was 14 years of age. In neuropathological examination of the second specimen the tumor showed an increased cellularity and pleomorphism, microvascular proliferations, an elevated proliferative activity (MIB1-index focally up to 10%) and cellular atypia not typical for WHO grade I DNT. Furthermore, MRI studies showed additional supratentorial and infratentorial lesions which remained stable over years and are also well consistent with DNTs. Thus, an unusual form of a DNT with multifocal lesions, local regrowth and morphological transformation is supposed.
CLN3 disease is a pediatric neurodegenerative condition wherein seizures are common. The most com... more CLN3 disease is a pediatric neurodegenerative condition wherein seizures are common. The most common disease‐causing variant is an ~1‐kb deletion in CLN3. We investigated seizure phenotype in relation to genotype and to adaptive behavior, MR spectroscopy and CSF biochemical markers in a CLN3 cohort. We performed seizure phenotyping using clinical history, EEG, and the Unified Batten Disease Rating Scale (UBDRS) seizure score. We assessed correlations of seizure severity with disease severity (UBDRS capability), adaptive behavior composite score (ABC; Vineland‐3), glutamate+glutamine+GABA and N‐acetylaspartate+N‐acetylaspartyl glutamate (MR spectroscopy), and CSF neurofilament light chain (NEFL) levels. In 20 participants, median age was 10.7 years (IQR = 7.8). Eighteen completed baseline EEG; 12 had a 1‐year follow‐up. Seizures were reported in 14 (8 1‐kb deletion homozygotes), with median age at onset of 10.0 (IQR = 6.8). Epileptiform discharges were noted in 15 (9 homozygotes). Bilateral tonic clonic (n = 11) and nonmotor seizures (n = 7) were most common. UBDRS seizure score correlated with age (rp = 0.50; [0.08,0.77]; P = .02), UBDRS capability (rp = −0.57; [−0.81,−0.17]; P = .009) and ABC (rp = −0.66; [−0.85,−0.31]; P = .001) scores, glutamate+glutamine+GABA (rp = −0.54; [−0.80,−0.11]; P = .02) and N‐acetylaspartate+N‐acetylaspartyl glutamate (rp = −0.54; [−0.80,−0.11]; P = .02), and CSF NEFL (rp = 0.65; [0.29,0.85]; P = .002) levels. After controlling for age, correlations with ABC and CSF NEFL remained significant. In our CLN3 cohort, seizures and epileptiform discharges were frequent and often started by age 10 years without significant difference between genotypes. ABC and CSF NEFL correlate with UBDRS seizure score, reflecting the role of seizures in the neurodegenerative process. Longitudinal evaluations in a larger cohort are needed to confirm these findings.
Background: fMRI language tasks readily identify frontal language areas; temporal activation has ... more Background: fMRI language tasks readily identify frontal language areas; temporal activation has been less consistent. No studies have compared clinical visual judgment to quantitative region of interest (ROI) analysis.Objective: To identify temporal language areas in patients with partial epilepsy using a reading paradigm with clinical and ROI interpretation.Methods: Thirty patients with temporal lobe epilepsy, aged 8 to 56 years, had 1.5-T fMRI. Patients silently named an object described by a sentence compared to a visual control. Data were analyzed with ROI analysis from t-maps. Regional asymmetry indices (AI) were calculated ([L−R]/[L+R]) and language dominance defined as >0.20. t-Maps were visually rated by three readers at three t thresholds. Twenty-one patients had intracarotid amobarbital test (IAT).Results: The fMRI reading task provided evidence of language lateralization in 27 of 30 patients with ROI analysis. Twenty-five were left dominant, two right, one bilateral, ...
Objective: To study the association between HHV-6 viruses and hippocampal volumes in patients wit... more Objective: To study the association between HHV-6 viruses and hippocampal volumes in patients with mesial temporal sclerosis (MTS). Background: Human herpes virus 6 (HHV-6) may play an etiologic role in mesial temporal lobe epilepsy and MTS. Previous studies found an association with febrile status epilepticus. We have reported higher prevalence of HHV-6 in MTS compared to other epilepsy etiologies. Design/Methods: We studied prospectively 34 patients referred to the Clinical Epilepsy Section, NINDS, NIH for evaluation of intractable epilepsy, with ictal video-EEG monitoring, 1.5-T or 3T GE Signa MRI with FLAIR, T1- and T2- weighted images, and 3-D SPGR or MP-RAGE. An investigator blinded to other data manually traced hippocampi manually on each slice, before assembly into three- dimensional volumes. The main study outcome measure was the hippocampal volume asymmetry index (AI) ipsilateral and contralateral to the seizure focus compared between HHV-6 positive and negative patients. Viral DNA was isolated from fresh brain tissue. For 25 patients, viral detection was performed using quantitative real time PCR specific for HHV-6A and HHV-6B. For nine patients, viral DNA detection was performed using digital droplet PCR (DdPCR) specific for HHV-6A and HHV-6B. DdPCR is a highly sensitive and precise novel PCR technology that enables absolute quantification of target DNA molecules. Statistical analysis was performed with SPSS. Results: HHV-6B was detected in 16 of 25 patients studied with real time PCR. Among 9 studied with DdPCR, one had HHV-6A, two HHV-6B, and two both viruses. HHV-6-negative patients had significantly greater AI and lower ipsilateral volume than HHV-6 positive patients (p<0.001). On ANOVA including epilepsy duration, HHV-6 status retained a significant effect on AI (p<0.01). Conclusion: Our data suggest multiple potential etiologies for MTS. HHV6 may have a protective effect on hippocampal volume. Study Supported By: Division of Intramural Research NINDS NIH Disclosure: Dr. Theodore has received personal compensation in an editorial capacity for Epilepsy Research. Dr. Theodore holds stock and/or stock options in General Electric. Dr. Leibovitch has nothing to disclose. Dr. Billioux has nothing to disclose. Dr. Germayen has nothing to disclose. Dr. Kalikhman has nothing to disclose. Dr. Inati has nothing to disclose. Dr. Heiss has nothing to disclose. Dr. Zaghloul has nothing to disclose. Dr. Jacobson has nothing to disclose.
We report the case of a multifocal dysembryoplastic neuroepithelial tumor (DNT) in a 7-year-old g... more We report the case of a multifocal dysembryoplastic neuroepithelial tumor (DNT) in a 7-year-old girl with local tumor regrowth 6 years later. The tumor was localized in the right parietal lobe extending from the cortex into the periventricular white matter. After subtotal resection of a histopathologically confirmed DNT we observed unexpected tumor progression in long-term follow-up. Therefore, a second surgery was performed when the patient was 14 years of age. In neuropathological examination of the second specimen the tumor showed an increased cellularity and pleomorphism, microvascular proliferations, an elevated proliferative activity (MIB1-index focally up to 10%) and cellular atypia not typical for WHO grade I DNT. Furthermore, MRI studies showed additional supratentorial and infratentorial lesions which remained stable over years and are also well consistent with DNTs. Thus, an unusual form of a DNT with multifocal lesions, local regrowth and morphological transformation is supposed.
Background: fMRI language tasks readily identify frontal language areas; temporal activation has ... more Background: fMRI language tasks readily identify frontal language areas; temporal activation has been less consistent. No studies have compared clinical visual judgment to quantitative region of interest (ROI) analysis.Objective: To identify temporal language areas in patients with partial epilepsy using a reading paradigm with clinical and ROI interpretation.Methods: Thirty patients with temporal lobe epilepsy, aged 8 to 56 years, had 1.5-T fMRI. Patients silently named an object described by a sentence compared to a visual control. Data were analyzed with ROI analysis from t-maps. Regional asymmetry indices (AI) were calculated ([L−R]/[L+R]) and language dominance defined as >0.20. t-Maps were visually rated by three readers at three t thresholds. Twenty-one patients had intracarotid amobarbital test (IAT).Results: The fMRI reading task provided evidence of language lateralization in 27 of 30 patients with ROI analysis. Twenty-five were left dominant, two right, one bilateral, ...
fMRI language tasks reliably identify language areas in presurgical epilepsy patients, but activa... more fMRI language tasks reliably identify language areas in presurgical epilepsy patients, but activation using single paradigms may disagree with the intracarotid amobarbital test (IAT). To determine whether a panel of fMRI tasks targeting different aspects of language processing increases accuracy in determining hemisphere language dominance. Twenty-six patients age 12 to 56 years, predominantly with temporal lobe epilepsy, were studied using whole-brain 1.5 T fMRI (echo planar imaging, blood oxygenation level-dependent) with three task categories using a block design: verbal fluency, reading comprehension, and auditory comprehension. fMRI t maps were visually rated at three thresholds. All patients had assessment of language lateralization by IAT. fMRI showed left dominance in 21 patients, right dominance in 2, and bilateral activation in 2; raters disagreed over a left vs right bilateral rating in 1 patient. There was full agreement between IAT and fMRI in 21 of 25 patients (IAT failed in 1). In three instances of partial disparity with IAT, the fMRI panel showed consistent findings across raters. Agreement between raters was excellent (partial disagreement in only one patient); the panel of tasks was superior to any single task for interrater agreement (Cramer V 0.93 [range 0.91 to 1.0] vs 0.72 [range 0.60 to 0.86]). A panel of fMRI language paradigms may be more accurate for evaluating partial epilepsy patients than a single task. A panel of tasks reduces the likelihood of nondiagnostic findings, improves interrater reliability, and helps confirm language laterality.
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