Left vagal nerve stimulation in six patients with hypothalamic hamartomas.

Six patients with medically refractory epilepsy secondary to hypothalamic hamartomas were treated with intermittent stimulation of the left vagal nerve. Three of the patients had remarkable improvements in seizure control. Four of these six patients had severe autistic behaviors.

Prospective long-term study of vagus nerve stimulation for the treatment of refractory seizures.

Purpose: To determine the long-term efficacy of vagus nerve stimulation (VNS) for refractory seizures. VNS is a new treatment for refractory epilepsy. Two short-term double-blind trials have demonstrated its safety and efficacy, and one long-term study in 114 patients has demonstrated a cumulative improvement in efficacy at 1 year.

Brain mechanisms for reading: the role of the superior temporal gyrus in word and pseudoword naming.

The purpose of this study was to test the neurological validity of a dual-route model of reading by asking patients, who were undergoing electrocortical stimulation mapping, to read words with irregular print-to-sound correspondences and pseudowords. Brain activation profiles were also obtained from these patients during an auditory and a visual word recognition task using whole-head magnetic source imaging. We demonstrated that reading is subserved by at least two brain mechanisms that are anatomically dissociable.

Insights into brain function and neural plasticity using magnetic source imaging.

This review outlines the rationale for the use of magnetoencephalography (MEG) or magnetic source imaging (MSI), a noninvasive functional imaging technique, and the features that any imaging method should display to make a substantial contribution to cognitive neuroscience. After a brief discussion of the basic experimental approach used in the authors' studies, the use of early sensory components of brain magnetic responses is reviewed to address issues of the functional organization of the primary sensory cortices, followed by a comment on the clinical use of these components. Second, normative studies focusing on the late components of magnetic responses for establishing the validity and reliability of MSI maps of the language-specific cortex in normal subjects are reviewed.

Genetic and neuroradiological heterogeneity of double cortex syndrome.

Mutations in the X-linked doublecortin gene appear in many sporadic cases of double cortex (DC; also known as subcortical band heterotopia), a neuronal migration disorder causing epilepsy and mental retardation. The purpose of this study was to examine why a significant percentage of sporadic DC patients had been found not to harbor doublecortin mutations and to determine whether clinical features or magnetic resonance imaging scan appearance could distinguish between patients with and without doublecortin mutations. Magnetic resonance imaging scan analysis differentiated patients into the following four groups: anterior biased/global DC with doublecortin mutation (16 of 30; 53%), anterior biased/global DC without mutation (8 of 30; 27%), posterior biased DC without mutation (3 of 30; 10%), and limited/unilateral DC without mutation (3 of 30; 10%).

Localization of language-specific cortex by using magnetic source imaging and electrical stimulation mapping.

Object: In this paper the authors demonstrate the concordance between magnetic source (MS) imaging and direct cortical stimulation for mapping receptive language cortex.

Methods: In 13 consecutive surgical patients, cortex specialized for receptive language functions was identified noninvasively by obtaining activation maps aided by MS imaging in the context of visual and auditory word-recognition tasks. Surgery was then performed for treatment of medically intractable seizure disorder (eight patients), and for resection of tumor (four), or angioma (one).

Language dominance determined by magnetic source imaging: a comparison with the Wada procedure.

Objective: To evaluate the validity of data derived from magnetic source imaging (MSI) regarding cerebral dominance for language in patients with intractable seizure disorder.

Method: The authors performed functional imaging of the receptive language cortex using a whole-head neuromagnetometer in 26 consecutive epilepsy patients who also underwent the intracarotid amobarbital (Wada) procedure. During MSI recordings, patients engaged in a word recognition task.

Magnetoencephalographic mapping of the language-specific cortex.

Object: In this paper the authors introduce a novel use of magnetoencephalography (MEG) for noninvasive mapping of language-specific cortex in individual patients and in healthy volunteers.

Methods: The authors describe a series of six experiments in which normative MEG data were collected and the reliability, validity, and topographical accuracy of the data were assessed in patients who had also undergone the Wada procedure or language mapping through intraoperative cortical stimulation.

Conclusions: Findings include: 1) receptive language-specific areas can be reliably activated by simple language tasks and this activation can be readily recorded in short MEG sessions; 2) MEG-derived maps of each individual are reliable because they remain stable over time and are independent of whether auditory or visual stimuli are used to activate the brain; and 3) these maps are also valid because they concur with results of the Wada procedure in assessing hemispheric dominance for language and with the results of cortical stimulation in identifying the precise topography of receptive language regions within the dominant hemisphere.

A comparison of magnetoencephalography, MRI, and V-EEG in patients evaluated for epilepsy surgery.

Purpose: To determine the efficacy and relative contribution of several diagnostic methods [ictal and interictal scalp and intracranial EEG, magnetic resonance imaging (MRI), and magnetoencephalography (MEG)] in identifying the epileptogenic zone for resection.

Methods: This was a prospective study using a masked comparison-to-criterion standard. Fifty-eight consecutive patients with refractory partial epilepsy from two university comprehensive epilepsy programs were studied.

Safety of rapid intravenous infusion of valproate loading doses in epilepsy patients.

IV valproate may be given to patients requiring rapid elevation of serum valproate or for patients unable to take oral medication. Previously we established the loading dose of IV valproate needed to achieve serum concentrations of > 100 ug/ml. This study evaluated the safety of rapid infusion of valproate to achieve high therapeutic levels.

Magnetic resonance angiography of primary varicella vasculitis: report of two cases.

Two patients with onset of hemiparesis 3 weeks following primary varicella infection demonstrated contralateral temporal lobe and basal ganglia infarctions on magnetic resonance imaging. In both cases, magnetic resonance angiography (MRA) was performed and demonstrated flow abnormalities ipsilateral to the infarcts. Digital subtraction angiography was performed in one case; however, the findings were significantly less conspicuous than those of the MRA.

Physiology of perception: cortical stimulation and recording in humans.

Objectives: 1) To determine the effect of stimulus train duration (TD) on sensory perception using direct stimulation of somatosensory and visual cortices. 2) To investigate the occurrence of evoked potentials in response to stimulation that is subthreshold for perception.

Background: Studies of the mechanisms of conscious perception using direct cortical stimulation and recording techniques are rare.

Atypical temporal lobe language representation: MEG and intraoperative stimulation mapping correlation.

Functional brain imaging techniques hold many promises as the methods of choice for identifying areas involved in the execution of language functions. The success of any of these techniques in fulfilling this goal depends upon their ability to produce maps of activated areas that overlap with those obtained through standard invasive procedures such as electrocortical stimulation. This need is particularly acute in cases where active areas are found outside of traditionally defined language areas.

Characterization of mutations in the gene doublecortin in patients with double cortex syndrome.

Mutations in the X-linked gene doublecortin, which encodes a protein with no dear structural homologues, are found in pedigrees in which affected females show "double cortex" syndrome (DC; also known as subcortical band heterotopia or laminar heterotopia) and affected males show X-linked lissencephaly. Mutations in doublecortin also cause sporadic DC in females. To determine the incidence of doublecortin mutations in DC, we investigated a cohort of eight pedigrees and 47 sporadic patients with DC for mutations in the doublecortin open reading frame as assessed by single-stranded conformational polymorphism analysis.

A multicenter study of the efficacy of the ketogenic diet.

Objective: To determine the efficacy of the ketogenic diet in multiple centers.

Design: A prospective study of the change in frequency of seizures in 51 children with intractable seizures who were treated with the ketogenic diet.

Setting: Patients were enrolled from the clinical practices of 7 sites.

Pediatric use of intravenous and intramuscular phenytoin: lessons learned.

First developed in 1950, parenterally administered phenytoin offered substantial advantages over parenterally administered phenobarbital and paraldehyde, which were the treatments for status epilepticus until the 1960s. During the 1950s, clinical research established the pediatric dosage of parenteral phenytoin for the treatment of seizures, which was based on the adult dosage adjusted to each child's weight. Studies in the late 1970s and early 1980s established more appropriate dosing for neonates and children on a milligram-per-kilogram basis.

Vagus nerve stimulation therapy for partial-onset seizures: a randomized active-control trial.

Objective: The purpose of this multicenter, add-on, double-blind, randomized, active-control study was to compare the efficacy and safety of presumably therapeutic (high) vagus nerve stimulation with less (low) stimulation.

Background: Chronic intermittent left vagus nerve stimulation has been shown in animal models and in preliminary clinical trials to suppress the occurrence of seizures.

Methods: Patients had at least six partial-onset seizures over 30 days involving complex partial or secondarily generalized seizures.

On the surgical treatment of refractory epilepsy in tuberous sclerosis complex.

The role of surgery in the treatment of refractory epilepsy (RE) in tuberous sclerosis complex (TSC) is poorly defined. Four patients with RE and TSC were evaluated for epilepsy surgery from 1994 to 1996. Three of four patients developed infantile spasms within 5 months of birth.

Corpus callosotomy for medically intractable seizures.

To identify factors influencing outcome and morbidity in patients selected for corpus callosotomy, we retrospectively reviewed 23 patients with intractable generalized seizures who underwent corpus callosotomy between 1991 and 1994. Three patients had a complete corpus callosotomy, while 20 had an anterior callosotomy. Three of those patients subsequently had completion of the anterior callosotomy.

Quality of life perception in patients with intractable epilepsy or pseudoseizures.

Objectives: To contrast and compare self-reported quality of life in patients with intractable epilepsy and pseudoseizures and to examine the relationship between self-reports and objective measures of cognitive functioning in both of these groups.

Design: Case series using profile analysis and analysis of covariance.

Setting: University epilepsy surgery program.

Asymmetries in the effect of side of seizure onset on recognition memory following intracarotid amobarbital injection.

Purpose: To assess interhemispheric differences in recognition memory for objects during the intracarotid amobarbital sodium procedure (IAP).

Methods: The recognition memory for real objects of patients with either right (RTLE; n = 28) or left (LTLE; n = 22) temporal lobe epilepsy was assessed at baseline, and after left and right intracarotid amobarbital sodium injection.

Results: There were no differences between groups on baseline performance.

Lennox-Gastaut syndrome.

Lennox-Gastaut syndrome (LGS) is one of the intractable epilepsies of childhood that is associated with an epileptic encephalopathy. Although LGS has been accepted as a distinct epilepsy syndrome for the last 30 years, understanding of its pathogenesis is still incomplete. Because this heterogenous entity has many diverse etiologies, some with specific therapy, a complete evaluation is necessary.

Identification of side of seizure onset in temporal lobe epilepsy using memory tests in the context of reading deficits.

Sixty patients with temporal lobe epilepsy were classified into reading deficient (RD; n = 21) and non-reading deficient (non-RD; n = 39) groups. Selective deficits in verbal or nonverbal memory, consistent with side of seizure onset, were evident in the non-RD patients. Both verbal and nonverbal memory performance were reduced equivalently in individuals with RD, regardless of side of seizure onset.

Effects of duration of epilepsy on the uncoupling of metabolism and blood flow in complex partial seizures.

We derived interhemispheric asymmetry indices (AIs) in interictal glucose uptake and blood flow in the temporal lobes of patients with intractable complex partial seizures from 18F and 15O positron emission tomograms. All patients subsequently underwent either left (n = 16) and right (n = 18) temporal lobectomy. We determined the effects on AIs of clinical seizure variables, including duration of seizure disorder, age at seizure onset, frequency of complex partial seizures, history of secondary generalization, history of febrile seizures, and magnetic resonance imaging evidence for mesial temporal sclerosis.

Effects of temporal lobe epilepsy on spatial and figural aspects of memory for a complex geometric figure.

The preoperative delayed memory performance on the Rey-Osterrieth Complex Figure (Lezak, 1983) of 54 patients with complex partial seizures of temporal lobe origin was analyzed using 3 different indices. One index (composite) was derived using a common scoring method that included both spatial and figural aspects of memory in its score. The other two indices were derived emphasizing either spatial or figural aspects of memory for the elements of the figure separately.