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Dysembryoplastic neuroepithelial tumors: MR findings.

OBJECTIVE: We report MR findings in three patients with dysembryoplastic neuroepithelial tumor (DNT), which is a recently described benign tumor in childhood or young adolescents. MATERIALS AND METHODS: The MR studies of three patients with surgically proven DNT were reviewed. All cases presented with partial complex seizures. Axial and coronal T1- and T2-weighted MR imaging of the head was performed using a 1.5 T MR unit. T1-weighted imaging after Gd-DTPA was also performed. RESULTS: The tumors were located mainly in the cortical to subcortical area with very little perifocal mass effects on MR imaging. They were essentially hypointense on T1-weighted imaging and hyperintense well-demarcated mass on T2-weighted imaging and retained a thick gyrus-like configuration within the lesions. None of them exhibited contrast enhancement after administration of Gd-DTPA. CONCLUSION: Differentiation of DNT from gangliogliomas or other low grade gliomas is possible using these MR features and is important because DNT does not recur after epilepsy surgery, therefore postoperative radiation and chemotherapy are not needed.

Adult↗

Positron emission tomographic measurement of cerebral blood flow and temporal lobectomy.

We used positron emission tomography (PET) and bolus injection of H2(15O) to measure interictal cerebral blood flow (CBF) in 32 patients who had temporal lobectomy for uncontrolled complex partial seizures. Seizure focus localization was confirmed by ictal video-electroencephalographic (video-EEG) telemetry, and patients who had imaging findings suggesting the presence of a tumor were excluded. PET-CBF studies were interpreted using a standard template by raters blinded to EEG and clinical data, and were not used in surgical planning. After surgery, patients were followed for 32 +/- 18 months. Twenty-six patients were seizure free and 6 had persistent seizures. Mean lateral temporal hypoperfusion was 8 +/- 14% in patients who became seizure free and 9 +/- 6% in patients with persistent seizures. Fourteen patients had at least 15%, and 11 at least 20% hypoperfusion, but were not more likely to be seizure free. Three additional patients had 15 to 20% hypoperfusion in the temporal lobe contralateral to their EEG focus. PET measurement of CBF using bolus H2(15O) should not be used to help select patients for temporal lobectomy.

Brain↗

Transient encephalopathy following a single exposure of high-dose methotrexate in a child with acute lymphoblastic leukemia.

An episode of transient encephalopathy after the first course of intravenous high-dose methotrexate (HD-MTX; 1000 mg/m2) was observed in a 4-year-old girl with acute lymphoblastic leukemia. The neurological abnormalities took place 5 days after HD-MTX therapy. She experienced complex partial seizure and left hemiparesis, which resolved spontaneously in 5 days. Cranial computed tomographic scan and magnetic resonance imaging showed multiple low-density lesions in bilateral hemispheres. It is well appreciated that neurotoxicity from MTX follows prolonged exposures, often accompanying or following radiation therapy. To our knowledge, however, there have been no reports that such neurological complications developed following a single exposure of HD-MTX in patients with ALL. Follow-up electroencephalograms showed that she had periodic lateralized epileptiform discharges (PLEDS), suggesting functional deafferentation of cortical neurons following HD-MTX. Moreover, the serum and CSF MTX levels following a second low-dose course and her clinical course suggested that she had presumably central nervous system leukemia at the time of HD-MTX therapy, which might have been related to neurological complications. The pathogenesis of MTX-induced neurotoxicity is discussed.

Antineoplastic Combined Chemotherapy Protocols↗

The concept of an organic dissociative syndrome: what is the evidence?

The question of whether organic conditions, such as complex partial seizures, can cause dissociative symptoms is controversial. Although a diagnostic category for organic dissociation is included in the tenth edition of International Classification of Disease, it has never been identified in the Diagnostic and Statistical Manual. Its inclusion in the upcoming DSM-IV is currently under debate. This article surveys representative literature regarding the role of organic factors in the causation of dissociative symptoms and considers the differential diagnosis of organic dissociation from current and historic perspectives. Dissociative symptoms and disorders (including amnesia, fugue, depersonalization, multiple personality, automatisms, and certain furors) can be induced by a variety of medications, drugs of abuse, and medical illnesses or conditions affecting cerebral function. Organic dissociation can be distinguished from intoxication, amnestic disorder, and delirium. Psychiatric nosology and our conceptualization of altered mental states and functions would benefit from use of the concept of an organic dissociative syndrome, which has clinical, neurophysiologic, and medicolegal significance. Such a category should be included in DSM-IV.

Diagnosis, Differential↗

Corpora amylacea and heat shock protein 27 in Ammon's horn sclerosis.

Increased numbers of corpora amylacea have been observed in the resected mesial temporal lobe of many patients with complex partial seizures (CPS) and Ammon's horn sclerosis (AHS). Several heat shock proteins (HSPs) are induced by seizures and have been suggested as an etiologic factor in the formation corpora amylacea. We quantified corpora amylacea and HSP27-immunoreactive astrocytes in temporal lobe specimens from patients with CPS (28 AHS; 10 non-AHS) and in 5 autopsy controls. Corpora amylacea were increased in each sector of Ammon's horn in the AHS group, significantly so in CA1 and CA3 (p < 0.0001 and p = 0.0097, respectively), compared with the non-AHS group, although there was considerable variability among the specimens. We found HSP27 to be significantly but nonspecifically increased in the resected temporal lobe specimens from all patients with CPS, regardless of the underlying pathology. HSP27 was not, however, expressed within the corpora amylacea, and did not correlate with the number of corpora amylacea in any of the 9 mesial and lateral temporal lobe areas examined.

Adult↗

Temporal lobe CO2 vasoreactivity in patients with complex partial seizures.

The topography of CO2 vasoreactivity during hyperventilation in 8 patients with complex partial seizure (CPS) was visualized using the regional cerebral blood flow (rCBF) as measured by H(2)15O-PET (positron emission tomography) and compared with that of 10 normal volunteers. In the normal volunteers, the vascular response to CO2 (VrCO2 = delta CBF%/delta PaCO2) in the temporal lobe was 2.46 +/- 0.56 (%/mmHg). In the patients with CPS, VrCO2 in the temporal lobe of the affected side was 2.08 +/- 0.40 (%/mmHg), while VrCO2 on the contralateral side was 2.30 +/- 0.46 (%/mmHg). There was a significant difference in VrCO2 between the affected side of the temporal lobes and the temporal lobes of the normal volunteers. Furthermore, there was a tendency for VrCO2 to be lower in the affected than in the contralateral side of the temporal lobe in patients with CPS. As CO2 is the main regulator of CBF, this impaired vasoreactivity may reflect the brain dysfunction in the seizure focus and adjacent areas.

Adolescent↗

Fatality from hepatitis A in a child taking valproate.

We report an 8-year-old boy with complex partial seizures due to congenital stroke, treated with valproate for more than 3 years (the last 2 years were on monotherapy) with no complications during that period except for transient thrombocytopenia. His sister had uncomplicated hepatitis A. One month later, the patient became jaundiced, went into fulminant hepatic failure, and quickly became encephalopathic despite discontinuation of valproate, aggressive supportive therapy, and treatment with carmitine. He then died. He had positive hepatitis A IgM; other causes for acute hepatitis were ruled out. Liver pathology revealed distended hepatocytes with cholestasis and microvesicular changes. We could find in the literature two other articles on four cases who developed liver failure with hepatitis A while on valproate. All those cases, however, recovered. In our patient a usually benign disease became deadly, probably because of the concomitant use of a hepatotoxic medication. Immunizing, with hepatitis A vaccine, all children on valproate therapy who are living in, or traveling to, endemic areas should be considered and is probably advisable.

Acute Disease↗

Characterization of epileptic seizure dynamics using Gabor atom density.

OBJECTIVE: The study of epileptic electroencephalograph (EEG) dynamics can potentially provide insights into seizure onset, evolution and termination. We propose a new synthetic measure based on time-frequency decomposition to provide detailed characterization of these dynamic changes. METHODS: The matching pursuit (MP) method allows for continuous time-frequency decomposition. We have developed a derivative of the MP method, the Gabor atom density method (GAD) that facilitates interpretation during the dynamic ictal period. The GAD analysis was applied to intracranial recordings of complex partial seizures (n = 43) of mesial temporal origin in 7 patients. RESULTS: Complex partial seizure occurrence is systematically associated with a GAD increase of 400 +/- 150%. The GAD increase coincides with the electrographical evidence of seizure onset. The similarity between seizures in a given patient is very high with uniform onset slope, maximum level and termination pattern. Global GAD responses over all channels can reveal detailed seizure propagation patterns including secondary independent foci and secondary generalization. CONCLUSIONS: The GAD measure based on the MP decomposition is a reliable tool to detect seizure occurrence in long-term recordings, to differentiate seizures from artifacts on a multi-channel basis and to examine patterns of seizure propagation. The reproducible GAD pattern suggests consistent changes in signal inner structure and may provide new clues about seizure dynamics and evolution. SIGNIFICANCE: The GAD method can provide information about seizure dynamics that can contribute to methods of seizure detection. These analyses may lead to better understanding of seizure termination and help facilitate application of responsive seizure control devices in humans.

Algorithms↗

Reverse crossed cerebellar diaschisis in partial complex seizures related to herpes simplex encephalitis.

Tc-99m HMPAO brain SPECT was performed in a patient who had partial complex seizures for 1 year after successful acyclovir treatment of biopsy-proven herpes simplex encephalitis 2 years earlier. In spite of antiepileptic medications, her seizures were intractable and occurred daily. Tc-99m HMPAO was administered intravenously while she was having subclinical seizures, and brain SPECT demonstrated an area of hyperperfusion in the right temporal lobe medially and in the contralateral cerebellum. This reverse of the crossed cerebellar diaschisis phenomenon in epileptic disorders has not previously been documented.

Adult↗

Cognitive abilities and adjustment with gabapentin: results of a multisite study.

The cognitive and quality of life effects of gabapentin are not yet well explored. While preliminary work in the area has provided positive findings, a large double-blinded study has been needed to explore this area more thoroughly. From 24 sites in North America, 201 adults were studied who had uncontrolled complex partial seizures with or without secondary generalization. Attempts were made to convert each patient from one or two marketed antiepileptic drugs (AEDs) taken in baseline to gabapentin monotherapy (600, 1200, or 2400 mg/day). Tests of cognitive abilities and adjustment were administered at the end of the 8-week baseline period and at the end of the 26-week double-blind treatment period. Analyses of baseline to treatment period changes were conducted for each dose group in comparison with a reference group of placebo-treated patients from another study. In the area of cognitive functioning, no changes in any of the gabapentin groups were found in comparison with the reference group. In the area of adjustment and mood, however, improvement with gabapentin administration was noted on several variables pertaining to emotional and interpersonal adjustment. These results are consistent with findings from previous studies.

Acetates↗

Brain homocarnosine and seizure control of patients taking gabapentin or topiramate.

PURPOSE: To assess the relation between seizure control and brain homocarnosine and gamma-aminobutyric acid (GABA) levels of patients with complex partial seizures taking gabapentin (GBP) or topiramate (TPM) as adjunctive therapy. METHODS: In vivo measurements of GABA and homocarnosine were made of a 14-cc volume in the occipital cortex by using (1)H spectroscopy with a 2.1-Tesla magnetic resonance spectrometer and an 8-cm surface coil. Poor seizure control was defined as more recent seizures than the median for the two groups of patients studied. RESULTS: Homocarnosine levels were higher in patients with better seizure control than in those with poor control. No differences were found in the intracellular GABA levels between the patients who responded to GBP or TPM compared with those who did not. CONCLUSIONS: In the visual neocortex, which is remote from the presumed seizure-onset zone, higher homocarnosine levels were associated with better seizure control in the patients taking GBP or TPM as adjunctive therapy; elevated intracellular GABA levels appeared to offer no additional protection.

Adult↗

Elevated dopa decarboxylase activity in living brain of patients with psychosis.

The hypofrontality theory of the pathogenesis of schizophrenia predicts that cortical lesions cause psychosis. During a search for abnormalities of catecholaminergic neurotransmission in patients with complex partial seizures of the mesial temporal lobe, we discovered an increase of the rate of metabolism of an exogenous dopa tracer (6-[18F]fluoro-L-dopa) in the neostriatum of a subgroup of patients with a history of psychosis. When specifically assayed for this abnormality, patients with schizophrenia revealed the same significant increase of the rate of metabolism in the striatum. The finding is consistent with the theory that a state of psychosis arises when episodic dopamine excess is superimposed on a trait of basic dopamine deficiency in the striatum. The finding is explained by the hypothesis that cortical insufficiency, a proposed pathogenetic mechanism of both disorders, causes an up-regulation of the enzymes responsible for dopa turnover in the neostriatum as well as the receptors mediating dopaminergic neurotransmission.

Adult↗

Increased contralateral cerebellar uptake of technetium-99m-HMPAO on ictal brain SPECT.

UNLABELLED: Crossed cerebellar diaschisis (CCD) is a well-known brain SPECT finding in stroke patients. Two reports, however, have described supratentorial and contralateral cerebellar hyperperfusion (crossed cerebellar hyperperfusion) on ictal brain SPECT in epileptic patients. The purpose of this study was to assess the usefulness of crossed cerebellar hyperperfusion (CCH) for the detection of epileptic foci on ictal scan. METHODS: Twelve patients with complex partial seizures having characteristic clinical, electroencephalographic (EEG) and brain SPECT findings were included. Fifteen to 20 mCi 99mTc-HMPAO were injected intravenously during the seizure period or the aura for the ictal SPECT study. The SPECT findings were visually assessed to determine whether the finding of CCH was valuable in the localization of ictal foci. RESULTS: Epileptic foci were found in the right temporal (n = 6), left temporal (n = 4), right occipital (n = 1) and left frontal (n = 1) areas. CCH was observed in 8 (75%) of the 12 patients. In two patients, contralateral cerebellar uptake was more obvious than that in the epileptic foci. In the interictal scans, cerebellar activity, which was increased in ictal period, was equalized in seven of eight patients, while perfusion was diminished in the remaining patient. CONCLUSION: CCH is a frequent finding of ictal brain SPECT and may aid in the lateralization of epileptic foci.

Adolescent↗

Persistent unihemispheric perceptual impairments in humans following focal seizures.

Perception has been linked to a highly coordinated activation of cortical regions whose functional organization and performance is subject to plastic changes. We tested whether chronic repetitive disturbances of the brain by focal epileptic activity have a long-standing detrimental effect on the perceptual performance in the affected hemisphere. Nine patients were examined who had a history of complex partial seizures but no structural cerebral damage on magnetic resonance imaging and no evidence of ongoing epileptic activity on scalp electroencephalography and who had clinically been without seizures for at least 3 days. The side of primary epileptic involvement was determined by seizure semiology (n = 2), focal electroencephalographic slowing (n = 3) or focal abnormality during single photon emission topography (SPECT) (n = 4). The computer controlled psychometric assessment of the somesthetic frequency discrimination revealed that the perception in the hand corresponding to the affected hemisphere was impaired relative to the contralateral hand (P < 0.01), and to the performance of a group of normal controls (P < 0.01). We conclude that mechanisms related to focal epileptic activity can result in regional perceptual decrements even when there is no clinical or surface-electroencephalographic evidence of epileptic discharges. This in turn suggests that somatosensory testing may be of help in localizing, or at least lateralizing an epileptic focus.

Adult↗

Dysphasia accompanied by periodic lateralized epileptiform discharges.

We encountered a 67-year-old man who presented with repetitious dysphasia accompanied by periodic lateralized epileptiform discharges (PLEDs) on the electroencephalogram. A good correlation was established between the dysphasia and the PLEDs. A persistent partial seizure accompanied by PLEDs originating in the left hemisphere presented with dysphasia clinically. None of the previously reported patients with epileptic dysphasia had accompanying PLEDs. The administration of carbamazepine was successful to terminate the seizure, however valproate was not. This case indicates that PLEDs may produce significant, however reversible, functional damage, and may advance our understanding of the pathophysiology of epileptic dysphasia.

Aged↗

Dipole modelling and intracranial EEG recording: correlation between dipole and ictal onset zone.

This study includes 11 patients (3 males, 8 females) with mean age of 29 years (range: 15-42 years) who underwent a presurgical evaluation for refractory complex partial seizures (CPS). In all patients, neuroimaging (1.5 T optimum-MR) demonstrated intracranial structural abnormalities (space-occupying: n = 2; atrophic: n = 8; dysplastic: n = 1) and video-EEG monitoring showed CPS, because of discrepancies in the non-invasive examinations, all underwent additional intracranial EEG monitoring. After tailored resective procedures, all but one patient became seizure free. Mean follow-up was 30 months (range: 12-52 months). Results of intracranial EEG recording were compared with spatiotemporal dipole mapping of interictal and ictal epileptic discharges. Interictal dipole modelling revealed two distinct dipole patterns. Patients with lesions located in the medial temporal lobe uniformly presented a combined dipole that consisted of a radial and a tangential component with a high degree of elevation relative to the axial plane. Patients with extrahippocampal lesions had a less stable dipole with a predominant radial component. Dipole modelling of early ictal discharges revealed a striking correspondence with the interictal findings in individual patients. Elevation of ictal dipoles was always congruent with localisation based on intracranial EEG recordings. Interictal and ictal dipole mapping of medial temporal lobe sources may limit the number of surgical candidates for refractory CPS that need intracranial EEG recording. Whether ictal dipole modelling can be equally useful in extratemporal epilepsy remains to be proven.

Adolescent↗

Contribution to the quantification of two dimensional spaces in endocranial CT and MR in patients with epilepsy.

OBJECTIVE: The aim of this study is to show the connection between the morphometrical analysis of the width of the lateral ventricles measured in CT and MR images and the power of the frequency spectra of EEG signals in a total of 120 patients with epilepsy suffering from complex partial seizures without the clear localization and without the visible lesion. More than 800 CT and MR images of 120 patients with epilepsy were analyzed by the two-dimensional morphometrical analysis according to the methods presented in this study. Indices of the relation between the width of the right and left lateral ventricle were normally distributed. EEG frequency analysis of 8 channels, each of 32 seconds duration, was performed using 128 Hz sampling rate and FFT (Fast Fourie Transform) in 2 groups of patients consisting of 12 patients each: the first group with the significantly greater dimensions of the left lateral ventricle, and the second with the significantly greater size of the right one. Preliminary results of the analysis have shown that the absolute power of the theta frequency band was significantly increased in all brain regions in both groups of patients, and the relative ones in the parietocentral region bilaterally and in the left temporooccipital regions in patients with the wide lateral ventricle in the left hemisphere.

Cerebral Ventricles↗

Serum levels of carbamazepine and cortical excitability by magnetic brain stimulation.

We investigated the correlation between serum levels of carbamazepine (CBZ) and motor excitability studied by different parameters of transcranial magnetic stimulation (TMS) in patients at the beginning of antiepileptic treatment. A total of 10 patients with complex partial seizures following stroke were treated with loading doses of CBZ. Motor evoked potential (MEP) was recorded from the thenar eminence (TE) muscles of the unaffected arm. In all patients, we studied rest and active motor threshold (rMT, aMT), MEP amplitude and cortical silent period (CSP). In three patients, intracortical inhibition (ICI) and intracortical facilitation (ICF) were measured using paired TMS at short interstimulus intervals (1-25 ms). The recording sessions were performed before treatment and after 7, 15 and 60 days (SD=16 days). Serum level of CBZ were monitored at each recording session. We observed a progressive increase in rMT and aMT until the serum levels of CBZ reached a steady state condition. No significant changes were observed in MEP amplitude, CSP, ICI and ICF. This study documents the increase of both motor threshold and drug serum levels in patients treated with loading doses of CBZ, suggesting a relationship between drug metabolism and the effect on motor cortical excitability.

Adult↗