The Experts below are selected from a list of 504 Experts worldwide ranked by ideXlab platform
Ruth Ottman - One of the best experts on this subject based on the ideXlab platform.
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evaluation of depression risk in lgi1 mutation carriers
Epilepsia, 2010Co-Authors: Gary A Heiman, Ruth Ottman, Kay Kamberakis, Richard Gill, Sergey Kalachikov, Timothy A Pedley, Allen W HauserAbstract:Depression is the most common comorbid condition in epilepsy, affecting 20–55% of patients (Mendez et al., 1986; Edeh & Toone 1987; Robertson et al., 1987; Baker et al., 1996; Jacoby et al., 1996; Lambert & Robertson 1999; Hesdorffer et al., 2000; Hesdorffer et al., 2006). The cause of this comorbidity is unknown. Possible explanations include psychosocial consequences of epilepsy, treatment side-effects, seizure manifestations, or common neurobiologic mechanisms. While studies have consistently shown an increased risk of depression after epilepsy onset (Edeh & Toone 1987; Jacoby et al., 1996), some data also suggest that depressed individuals have an increased risk of developing epilepsy (Forsgren & Nystrom 1990; Hesdorffer et al., 2000; Hesdorffer et al., 2006). This bidirectional relationship supports the hypothesis of overlapping mechanisms for the two disorders (Kanner 2003; Kanner 2006; LaFrance et al., 2008), which might be mediated by a shared genetic susceptibility or by common environmental risk factors. The comorbidity of epilepsy with depression is especially striking for temporal lobe epilepsy (TLE) (Edeh & Toone 1987; Perini et al., 1996; Hesdorffer et al., 2000; Quiske et al., 2000; Piazzini et al., 2001; Gaitatzis et al., 2004), suggesting limbic system involvement for both disorders (Kanner & Balabanov 2002; Briellmann et al., 2007; Kanner 2008). So far, only one gene has been identified with a major influence on susceptibility to a form of TLE: the leucine-rich, glioma inactivated 1 gene (LGI1). Mutations in LGI1 have been identified in approximately 50% of families with autosomal dominant partial epilepsy with auditory features (ADPEAF), and have a penetrance of about 67% (Rosanoff & Ottman 2008). ADPEAF is an idiopathic focal epilepsy syndrome with auditory symptoms or Receptive Aphasia as major ictal manifestations (Ottman et al., 1995; Poza et al., 1999; Winawer et al., 2000; Winawer et al., 2002; Michelucci et al., 2003). These symptoms strongly suggest localization to the lateral temporal lobe, and hence the syndrome has also been called autosomal dominant lateral temporal lobe epilepsy. Psychiatric symptoms, including depression and suicide, were reported in one ADPEAF family with an LGI1 mutation (Chabrol et al., 2007). We took advantage of this gene identification to investigate the hypothesis of a shared genetic susceptibility to epilepsy and depression. Specifically, we investigated whether mutations in LGI1, which are known to have a strong effect on the risk for epilepsy, also raise the risk for depressive symptoms. Although the penetrance of mutations in ADPEAF families is relatively high, some family members with mutations remain unaffected with epilepsy. Hence in assessing the possible effects of LGI1 mutations on depression, we studied mutation carriers with and without epilepsy separately, to distinguish the effects of epilepsy or its treatment from those of mutations per se.
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altered language processing in autosomal dominant partial epilepsy with auditory features
Neurology, 2008Co-Authors: Ruth Ottman, L Rosenberger, Anto Bagic, K Kamberakis, Eva K Ritzl, A M Wohlschlager, Sadat Shamim, Susumu Sato, Clarissa J LiewAbstract:Background: Autosomal dominant partial epilepsy with auditory features (ADPEAF) is an idiopathic focal epilepsy syndrome with auditory symptoms or Receptive Aphasia as major ictal manifestations, frequently associated with mutations in the leucine-rich, glioma inactivated 1 ( LGI1 ) gene. Although affected subjects do not have structural abnormalities detected on routine MRI, a lateral temporal malformation was identified through high resolution MRI in one family. We attempted to replicate this finding and to assess auditory and language processing in ADPEAF using fMRI and magnetoencephalography (MEG). Methods: We studied 17 subjects (10 affected mutation carriers, 3 unaffected carriers, 4 noncarriers) in 7 ADPEAF families, each of which had a different LGI1 mutation. Subjects underwent high-resolution structural MRI, fMRI with an auditory description decision task (ADDT) and a tone discrimination task, and MEG. A control group comprising 26 volunteers was also included. Results: We found no evidence of structural abnormalities in any of the 17 subjects. On fMRI with ADDT, subjects with epilepsy had significantly less activation than controls. On MEG with auditory stimuli, peak 2 auditory evoked field latency was significantly delayed in affected individuals compared to controls. Conclusions: These findings do not support the previous report of a lateral temporal malformation in autosomal dominant partial epilepsy with auditory features (ADPEAF). However, our fMRI and magnetoencephalography data suggest that individuals with ADPEAF have functional impairment in language processing.
Robert Rothermel - One of the best experts on this subject based on the ideXlab platform.
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episodic Receptive Aphasia in a child with landau kleffner syndrome pet correlates
Brain & Development, 2006Co-Authors: Aimee F Luat, Harry T Chugani, Eishi Asano, Csaba Juhasz, Gary Trock, Robert RothermelAbstract:We report a four-year-old boy with Landau-Kleffner Syndrome (LKS) characterized by episodic Receptive Aphasia lasting for few weeks followed by gradual recovery of baseline language functions. Neuropsychological evaluation during an episode showed severe impairment in verbal skills and comprehension, but relative preservation of non-verbal skills. Although he could carry on a conversation during remission, neuropsychological evaluation demonstrated findings reflective of Receptive language deficits. Prolonged EEG disclosed frequent sharp-wave activity in the left and right temporal regions but no electrographic seizures. Glucose metabolism PET scan during the fourth episode of Aphasia revealed intense hypermetabolism in the left temporal neocortex. Awake EEG during the PET tracer uptake period showed 6.0 spikes/min in the left temporal region and 4.0 spikes/min in the right. A repeat PET scan during remission showed hypometabolism in the left temporal cortex. Awake EEG during the FDG uptake period showed 16.0 spikes/min in the right temporal region and 0.3 spikes/min in the left. During his fifth aphasic episode, EEG (without PET) showed 0.16 spikes/min in the right temporal region and none in the left. Intermittent short episodes of predominantly Receptive Aphasia with near-total recovery between episodes can be one of the clinical presentations of LKS. This case illustrates the dynamic changes of glucose metabolism in the temporal lobe during episodes of Aphasia and remission in a case with LKS. The clinical course of our patient with transient EEG and PET findings suggest that glucose metabolism in LKS cannot be attributed solely to interictal epileptiform activities on scalp EEG.
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case report episodic Receptive Aphasia in a child with landau kleffner syndrome pet correlates
2006Co-Authors: Aimee F Luat, Harry T Chugani, Eishi Asano, Gary Trock, Robert RothermelAbstract:We report a four-year-old boy with Landau–Kleffner Syndrome (LKS) characterized by episodic Receptive Aphasia lasting for few weeks followed by gradual recovery of baseline language functions. Neuropsychological evaluation during an episode showed severe impairment in verbal skills and comprehension, but relative preservation of non-verbal skills. Although he could carry on a conversation during remission, neuropsychological evaluation demonstrated findings reflective of Receptive language deficits. Prolonged EEG disclosed frequent sharp-wave activity in the left and right temporal regions but no electrographic seizures. Glucose metabolism PET scan during the fourth episode of Aphasia revealed intense hypermetabolism in the left temporal neocortex. Awake EEG during the PET tracer uptake period showed 6.0 spikes/min in the left temporal region and 4.0 spikes/min in the right. A repeat PET scan during remission showed hypometabolism in the left temporal cortex. Awake EEG during the FDG uptake period showed 16.0 spikes/min in the right temporal region and 0.3 spikes/min in the left. During his fifth aphasic episode, EEG (without PET) showed 0.16 spikes/min in the right temporal region and none in the left. Intermittent short episodes of predominantly Receptive Aphasia with near-total recovery between episodes can be one of the clinical presentations of LKS. This case illustrates the dynamic changes of glucose metabolism in the temporal lobe during episodes of Aphasia and remission in a case with LKS. The clinical course of our patient with transient EEG and PET findings suggest that glucose metabolism in LKS cannot be attributed solely to interictal epileptiform activities on scalp EEG. 2006 Elsevier B.V. All rights reserved.
Aimee F Luat - One of the best experts on this subject based on the ideXlab platform.
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episodic Receptive Aphasia in a child with landau kleffner syndrome pet correlates
Brain & Development, 2006Co-Authors: Aimee F Luat, Harry T Chugani, Eishi Asano, Csaba Juhasz, Gary Trock, Robert RothermelAbstract:We report a four-year-old boy with Landau-Kleffner Syndrome (LKS) characterized by episodic Receptive Aphasia lasting for few weeks followed by gradual recovery of baseline language functions. Neuropsychological evaluation during an episode showed severe impairment in verbal skills and comprehension, but relative preservation of non-verbal skills. Although he could carry on a conversation during remission, neuropsychological evaluation demonstrated findings reflective of Receptive language deficits. Prolonged EEG disclosed frequent sharp-wave activity in the left and right temporal regions but no electrographic seizures. Glucose metabolism PET scan during the fourth episode of Aphasia revealed intense hypermetabolism in the left temporal neocortex. Awake EEG during the PET tracer uptake period showed 6.0 spikes/min in the left temporal region and 4.0 spikes/min in the right. A repeat PET scan during remission showed hypometabolism in the left temporal cortex. Awake EEG during the FDG uptake period showed 16.0 spikes/min in the right temporal region and 0.3 spikes/min in the left. During his fifth aphasic episode, EEG (without PET) showed 0.16 spikes/min in the right temporal region and none in the left. Intermittent short episodes of predominantly Receptive Aphasia with near-total recovery between episodes can be one of the clinical presentations of LKS. This case illustrates the dynamic changes of glucose metabolism in the temporal lobe during episodes of Aphasia and remission in a case with LKS. The clinical course of our patient with transient EEG and PET findings suggest that glucose metabolism in LKS cannot be attributed solely to interictal epileptiform activities on scalp EEG.
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case report episodic Receptive Aphasia in a child with landau kleffner syndrome pet correlates
2006Co-Authors: Aimee F Luat, Harry T Chugani, Eishi Asano, Gary Trock, Robert RothermelAbstract:We report a four-year-old boy with Landau–Kleffner Syndrome (LKS) characterized by episodic Receptive Aphasia lasting for few weeks followed by gradual recovery of baseline language functions. Neuropsychological evaluation during an episode showed severe impairment in verbal skills and comprehension, but relative preservation of non-verbal skills. Although he could carry on a conversation during remission, neuropsychological evaluation demonstrated findings reflective of Receptive language deficits. Prolonged EEG disclosed frequent sharp-wave activity in the left and right temporal regions but no electrographic seizures. Glucose metabolism PET scan during the fourth episode of Aphasia revealed intense hypermetabolism in the left temporal neocortex. Awake EEG during the PET tracer uptake period showed 6.0 spikes/min in the left temporal region and 4.0 spikes/min in the right. A repeat PET scan during remission showed hypometabolism in the left temporal cortex. Awake EEG during the FDG uptake period showed 16.0 spikes/min in the right temporal region and 0.3 spikes/min in the left. During his fifth aphasic episode, EEG (without PET) showed 0.16 spikes/min in the right temporal region and none in the left. Intermittent short episodes of predominantly Receptive Aphasia with near-total recovery between episodes can be one of the clinical presentations of LKS. This case illustrates the dynamic changes of glucose metabolism in the temporal lobe during episodes of Aphasia and remission in a case with LKS. The clinical course of our patient with transient EEG and PET findings suggest that glucose metabolism in LKS cannot be attributed solely to interictal epileptiform activities on scalp EEG. 2006 Elsevier B.V. All rights reserved.
Merry Chen - One of the best experts on this subject based on the ideXlab platform.
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hsv 2 encephalitis presenting as episodic Receptive Aphasia p4 313
Neurology, 2014Co-Authors: Mohamad Ezzeldin, Ahmed Yassin, Merry ChenAbstract:OBJECTIVE:To review the case of a patient presenting with episodic Receptive Aphasia with rapid response to Keppra and acyclovir. BACKGROUND:The annual incidence of HSV encephalitis is approximately 1.2 cases/ million population/year. HSV2 is a rare cause of encephalitis outside the neonatal period and relative incidences ranging from 1.6% to 6.5%. HSV-2 encephalitis typically produces a global encephalitis with significant neurologic impairment. We present an atypical case of HSV 2 encephalitis presenting as focal seizures manifested by episodic Receptive Aphasia, without electrographic or MRI findings. DESIGN/METHODS:Retrospective review of a case including the clinical course, diagnostic studies, and treatment approach. RESULTS:78 year old RHM, with a history of non-small cell lung cancer and bony metastasis to the thoracic spine, on chemotherapy and steroids, presents with episodic Receptive Aphasia. He had two episodes prior to hospitalization, each lasting a few hours with no other neurological complaints. He was fairly normal between episodes. Neurological examination was non-focal. MRI brain with and without contrast was unremarkable. He had another episode while in the hospital and a routine EEG during the episode that was unremarkable. He was started on Keppra for presumed focal seizures. Lumbar puncture was done and CSF was positive for HSV 2 by PCR. The patient had no oral or genital herpetic lesions. He was treated with intravenous Acyclovir for two weeks followed by Valacyclovir. He improved after starting treatment and has not had further episodes. CONCLUSIONS:HSV type 2 is a rare cause of encephalitis in adults. Our patient was elderly, had cancer, on chemotherapy and steroids which likely brought on an indolent past infection. Focal seizures presenting as episodic Receptive Aphasia can be the only symptom of this infection. Being able to recognize these episodes as focal seizures, determining the cause, and timely treatment can decrease mortality/morbidity. Disclosure: Dr. Ezzeldin has nothing to disclose. Dr. Yassin has nothing to disclose. Dr. Chen has nothing to disclose.
Elina Liukkonen - One of the best experts on this subject based on the ideXlab platform.
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neuropsychological characteristics of five children with the landau kleffner syndrome dissociation of auditory and phonological discrimination
Journal of The International Neuropsychological Society, 1998Co-Authors: Marit Korkman, Marjaliisa Granstrom, Kati Appelqvist, Elina LiukkonenAbstract:The Landau-Kleffner Syndrome (LKS) is characterized by acquired Receptive Aphasia and EEG abnormality with onset between the ages of 3 and 8 years. This study presents neuropsychological assessments in 5 children with LKS. The aims were (1) to specify the neuropsychological deficits characteristic of these children; and (2) to clarify the nature of the Receptive Aphasia by comparing nonverbal and verbal auditory discrimination. Receptive Aphasia was present in all children. Retardation, poor motor coordination, hyperkinesia, and conduct problems were frequent but variable. All children exhibited a dissociation between the discrimination of environmental sounds and phonological auditory discrimination, the latter being more impaired than the former. This suggests that the primary deficit of the Receptive Aphasia is an impairment of auditory phonological discrimination rather than a generalized auditory agnosia.
