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Biomedical subjects

Ruben Kuzniecky

Publications and source records attributed to Ruben Kuzniecky.

11 recordsLinked to original sources

Verbal memory outcome in patients with normal preoperative verbal memory and left mesial temporal sclerosis.

PURPOSE: Previous studies have shown that structural integrity (i.e., presence/absence of mesial temporal sclerosis (MTS)) of the left mesial temporal lobe is associated with verbal memory outcome following left anterior temporal lobectomy (ATL). However, the functional integrity of the left temporal lobe, as exemplified by preoperative verbal memory performance, has also been associated with verbal memory outcome following surgery. We investigated the risk of verbal memory loss in patients with known structural abnormality (i.e., left mesial temporal sclerosis by MRI) and normal preoperative verbal memory performance who undergo left ATL. METHODS: Seventeen patients with left temporal lobe epilepsy, MRI-based exclusive left MTS, and normal preoperative verbal memory were identified. Normal verbal memory was defined as performance on both Acquisition (learning across trials 1-5) and Retrieval (long delayed free recall) portions of the California Verbal Learning Test (CVLT) above a T score of 40 (>16%ile). Postoperative verbal memory outcome was established by incorporating standardized regression-based (SRB) change scores. RESULTS: Postoperative declines across both CVLT Retrieval T scores and Acquisition T scores (average 20% and average 15% declines from baseline scores, respectively) were measured for the group. The average CVLT Retrieval SRB change score was -2.5, and the average CVLT Acquisition SRB change score was -1.0. A larger proportion of patients demonstrated postoperative declines on Retrieval scores than Acquisition scores (64.7% vs 17.6%, respectively). CONCLUSIONS: Even in the presence of left MTS, patients exhibiting normal presurgical verbal memory are at risk for verbal memory declines following ATL. These results suggest that the functional integrity of the left mesial temporal lobe may play an important role in the verbal memory outcome in this patient group.

Adolescent↗

Clinical applications of MR spectroscopy in epilepsy.

1H and 31P spectroscopy detects relevant metabolite changes in patients with TLE. Numerous studies confirm reduction in NAA and in the ratio of PCr/Pi. In his 1999 review, Kuzniecky concluded that proton MRS, using single-voxel or chemical shift imaging, lateralizes temporal lobe epilepsy in 65% to 96% of cases, with bilateral changes seen in 35% to 45% of cases, whereas phosphorus MRS shows a lateralizing PCr/Pi ratio in 65% to 75% of the TLE patients. There are indications that these changes are reversible with seizure treatment. Improvements in MRS technology, such as the ability to calculate absolute concentrations, to account for differences be-tween gray and white matter and to achieve better spectral resolution by use of a higher magnetic field strength, will now allow more extensive use of this technique for patients with epilepsy.

Amino Acids↗

Self-report of cognitive abilities in temporal lobe epilepsy: cognitive, psychosocial, and emotional factors.

Self-report of cognitive functioning using the Multiple Abilities Self-Report Questionnaire (MASQ) was examined in 57 left (LTLE) and 36 right (RTLE) temporal lobe epilepsy patients. The MASQ is a 38-item self-report measure assessing five domains of self-perceived cognitive functioning: Language, Visual-Perceptual Abilities, Verbal Memory, Visual-Spatial Memory, and Attention/Concentration. Overall, LTLE patients self-reported more cognitive difficulties across all domains. Language was the only domain to emerge as a robust indicator of seizure lateralization (LTLE patients reporting more problems). Neuropsychological test performance did not emerge as a significant predictor for any domain, whereas measures of psychosocial and emotional functioning accounted for a significant but modest amount of variance in all of them. The results suggest caution in using such self-report measures as an ecological extension of objective testing, but suggest a role in assessing self-appraisal of deficits.

Adolescent↗

Confirmatory factor analysis of the california verbal learning test in patients with epilepsy: relationship to clinical and neuropathological markers of temporal lobe epilepsy.

Latent constructs involved in California Verbal Learning Test (D. C. Delis, J. H. Kramer, E. Kaplan, & B. A. Ober, 1987) performance were examined using confirmatory factor analysis in 388 epilepsy surgery candidates. Eight factor models were compared. A single-factor model was examined, along with 7 models accommodating constructs of auditory attention, inaccurate recall, and delayed recall in different combinations. The retained model consisted of 3 correlated factors: Auditory Attention. Verbal Learning, and Inaccurate Recall. Validity of this factor structure was examined in a subsample of patients with left and right temporal lobe epilepsy. All 3 factors were related to seizure focus and magnetic resonance imaging hippocampal volume. Only Verbal Learning was related to hippocampal neuropathology, supporting the distinction between learning and attention in the factor structure.

Adolescent↗

Catastrophic focal epilepsy.

Focal epilepsy can present with a rapidly progressing course of intractable epilepsy in children. We present a typical example of such a patient with focal seizures due to a frontal lobe cortical lesion of developmental origin. MRI and SPECT revealed abnormalities in the right frontal lobe. Surgical resection resulted in excellent outcome.

Electroencephalography↗

Temporal lobectomy in congenital porencephaly associated with hippocampal sclerosis.

BACKGROUND: Clinical and neuroimaging features of patients with epilepsy and coexisting extratemporal porencephaly and hippocampal sclerosis have been previously described. OBJECTIVE: To present the clinical characteristics and surgical outcome of 6 patients with intractable epilepsy and coexisting extratemporal porencephaly and hippocampal sclerosis. PATIENTS AND METHODS: Twenty-four patients with porencephaly and epilepsy were studied. Of these, 6 had an epileptogenic focus in the temporal region. All patients underwent video electroencephalogram monitoring, magnetic resonance imaging studies, and neuropsychological evaluation. Of the subset of patients with temporal lobe epilepsy, 1 patient underwent intracranial electroencephalogram monitoring. Temporal lobe resection was performed in 5 patients. Outcomes were evaluated using the Engel classification. RESULTS: Freedom from seizures was achieved in all patients. Pathologic analysis of the resected tissue confirmed the presurgical diagnosis of mesial temporal sclerosis. CONCLUSION: Patients with extratemporal porencephaly and intractable seizures should be evaluated early and be considered for temporal lobectomy if clinical, magnetic resonance imaging, and electroencephalogram findings support the diagnosis of temporal lobe onset seizures.

Adolescent↗

Hypothalamic hamartoma and seizures: a treatable epileptic encephalopathy.

Hypothalamic hamartomas may be associated with gelastic seizures, focal seizures, and a generalized epileptic encephalopathy, with severe seizures and cognitive and behavior decline. Despite earlier views to the contrary, good evidence now exists that all these clinical features are caused, directly or indirectly, by the hamartoma. Resection of these lesions was long regarded as too hazardous and unlikely to benefit seizure control. It is now clear that hypothalamic hamartomas can be effectively treated with a variety of surgical approaches with sustained seizure control and often seizure freedom. Qualitative observations suggest that behavior and cognition also improve with treatment, but quantitative validation is required. The specific approach should be tailored according to the surgical anatomy of the lesion and the experience of the surgeon. Choices include a transcallosal approach (good for intraventricular lesions), a pterional approach (useful for interpeduncular lesions), a transventricular endoscopic approach, or destruction of the lesion with radiofrequency probes or gamma knife radiosurgery. The previously dismal outlook for children with severe seizures associated with this lesion has now dramatically changed. These insights may have implications for other epileptic encephalopathies of childhood.

Adolescent↗

Modulation of cerebral GABA by topiramate, lamotrigine, and gabapentin in healthy adults.

BACKGROUND: Anticonvulsant drugs have multiple mechanisms of action. Recent in vivo MRS studies suggest that cerebral gamma-aminobutyric acid (GABA) increases occur with the administration of certain anticonvulsants in humans. OBJECTIVE: To investigate the effect of topiramate, gabapentin, and lamotrigine on cerebral GABA concentrations in healthy volunteers and correlate the GABA concentrations with serum drug levels. METHODS: Seventeen healthy adults were randomly assigned to receive topiramate, gabapentin, and lamotrigine and underwent GABA measurements using a 4.1-T magnet from a 13.5-mL volume over the occipital region. GABA concentrations and serum levels were measured at 3 and 6 hours following administration of an acute single dose of one of the drugs. Thereafter, drugs were titrated over 4 weeks to target doses, with GABA measurements performed at 2 and 4 weeks. RESULTS: Cerebral GABA concentrations rose 70% in the acute phase compared with baseline for topiramate. GABA rose 48% at 6 hours with gabapentin but not with lamotrigine. With long-term dosing and once target doses were achieved at 4 weeks, significant elevations in GABA were observed compared with baseline for all three drugs (topiramate 46%, gabapentin 25%, lamotrigine 25%). CONCLUSION: This study demonstrates that single doses of topiramate and gabapentin increase cerebral GABA concentrations acutely (hours) in healthy individuals, but all drugs at clinically utilized doses increase cerebral GABA at 4 weeks. These results suggest that the mechanisms of action of anticonvulsant drugs are more complex and are likely to be multiple in nature.

Acetates↗

Risk to verbal memory following anterior temporal lobectomy in patients with severe left-sided hippocampal sclerosis.

BACKGROUND: Previous investigations indicate low risk for memory loss following anterior temporal lobectomy (ATL) in patients with severe hippocampal sclerosis (HS) compared with patients with mild HS. However, these conclusions have been established primarily with group-level analyses. OBJECTIVE: To investigate individual base rate risk for verbal memory loss following ATL in patients who have pathologically verified mild, moderate, or severe HS. PATIENTS AND METHODS: One hundred fifteen patients with unilateral temporal lobe epilepsy (68 with left-sided and 47 with right-sided epilepsy) were included. Acquisition, retrieval, and recognition components of verbal memory, as measured by the California Verbal Learning Test, were assessed before and after ATL. Postoperatively, the degree of neuronal loss and reactive gliosis of the hippocampus was assessed via a 3-tiered rating system establishing mild, moderate, and severe pathologic features. Patients with preoperative magnetic resonance imaging-based evidence of lesions outside the mesial temporal area (side of surgical resection) were excluded. RESULTS: Neither seizure laterality nor severity of HS was associated with preoperative verbal memory performance. Postoperatively, the left-sided ATL group demonstrated significant decline across the acquisition (P<.01), retrival (P<.001), and recognition (P<.001) verbal memory components compared with the right-sided ATL group. Patients who underwent left-sided ATL and had mild HS displayed the largest magnitude and percentage proportion of postoperative decline across all verbal memory components. However, 28 (48%) of the 58 patients who underwent left-sided ATL and who had moderate and severe HS displayed statistically reliable declines on retrieval aspects of verbal memory. Most patients undergoing right-sided ATL, regardless of the extent of hippocampal pathologic features, displayed no postoperative memory change. CONCLUSIONS: Substantial individual heterogeneity of memory outcome exists across groups of patients undergoing ATL, with various degrees of pathologically verified HS. Patients undergoing left-sided ATL who have mild HS seem at greatest risk for broad-spectrum verbal memory decline. However, when examining outcome on a patient-by-patient basis, many patients undergoing left-sided ATL who have moderate to severe HS were also vulnerable to verbal memory loss. This risk seems selective to a retrieval-based aspect of verbal memory.

Adolescent↗

Rasmussen's Encephalitis.

When the diagnosis of Rasmussen's encephalitis is being considered, it is important to rapidly exclude other causes of epilepsia partialis continua and hemiparesis. Although there is no good data from randomized trials of different immune-related therapies, treatment with immunoglobulin G (IgG), steroids, or plasmapheresis is advocated as first-line therapy. It is not unreasonable to institute at least two treatment options (eg, IgG followed by plasmapheresis) if response to the first treatment is poor. Functional hemispherectomy and its variants are associated with a lower long-term complication rate.

Journal Article↗

Gender, Memory, and Hippocampal Volumes: Relationships in Temporal Lobe Epilepsy.

Previous research has suggested bilateral hippocampal support for verbal memory in women with early left-hemisphere injury and that women experience better verbal memory outcome following anterior temporal lobectomy (ATL). The present study investigated two issues: (1) Do women have better verbal memory outcome following ATL compared with men? (2) Are verbal memory abilities differentially supported by the right and left hippocampus in males and females? Verbal memory performance [Wechsler Memory Scale: Logical Memory (LM) savings score] was assessed in 70 patients who underwent ATL. MRI volumetric measurements of the left and right hippocampus were performed. No LM savings score difference was found between groups preoperatively although a statistically significant gender effect (P < 0.04) was found for postoperative LM savings scores. Females displayed better postoperative memory performance, regardless of side of surgery. Preoperative verbal memory performance was not associated with right or left hippocampal volumes in either left or right ATL females, although the right hippocampus was positively associated with memory performance for left ATL males. Hippocampal volumes were not associated with postoperative LM savings scores for any group. Results suggest that prose recall was only modestly influenced by gender and that bilateral hippocampal support for prose recall was not present in our female patients.

Journal Article↗