Hashimoto's encephalopathy mimicking Creutzfeldt-Jakob disease: brain biopsy findings.
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Biopsy↗
Biomedical subjects
E Bromfield
Publications and source records attributed to E Bromfield.
Mutations in the X-linked filamin 1 gene cause periventricular nodular heterotopia in males as well as in females.
Periventricular heterotopia (PH) is a human neuronal migration disorder in which many neurons destined for the cerebral cortex fail to migrate. Previous analysis showed heterozygous mutations in the X-linked gene filamin 1 (FLN1), but examined only the first six (of 48) coding exons of the gene and hence did not assess the incidence and functional consequences of FLN1 mutations. Here we perform single-strand conformation polymorphism (SSCP) analysis of FLN1 throughout its entire coding region in six PH pedigrees, 31 sporadic female PH patients and 24 sporadic male PH patients. We detected FLN1 mutations by SSCP in 83% of PH pedigrees and 19% of sporadic females with PH. Moreover, no PH females (0/7 tested) with atypical radiographic features showed FLN1 mutations, suggesting that other genes may cause atypical PH. Surprisingly, 2/24 males analyzed with PH (9%) also carried FLN1 mutations. Whereas FLN1 mutations in PH pedigrees caused severe predicted loss of FLN1 protein function, both male FLN1 mutations were consistent with partial loss of function of the protein. Moreover, sporadic female FLN1 mutations associated with PH appear to cause either severe or partial loss of function. Neither male could be shown to be mosaic for the FLN1 mutation in peripheral blood lymphocytes, suggesting that some neurons in the intact cortex of PH males may be mutant for FLN1 but migrate adequately. These results demonstrate the sensitivity and specificity of DNA testing for FLN1 mutations and have important functional implications for models of FLN1 protein function in neuronal migration.
Abnormalities, Multiple↗
Treatment of paroxysmal sympathetic storm with labetalol.
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Adult↗
Consumer acceptance of vegetarian sweet potato products intended for space missions.
Sweet potato is one of the crops selected for NASA's Advanced Life Support Program for potential long-duration lunar/Mars missions. This article presents recipes of products made from sweet potato and determines the consumer acceptability of products containing from 6% to 20% sweet potato on a dry weight basis. These products were developed for use in nutritious and palatable meals for future space explorers. Sensory evaluation (appearance/color, aroma, texture, flavor/taste, and overall acceptability) studies were conducted to determine the consumer acceptability of vegetarian products made with sweet potato using panelists at NASA/Johnson Space Center in Houston, TX. None of these products including the controls, contained any ingredient of animal origin with the exception of sweet potato pie. A 9-point hedonic scale (9 being like extremely and 1 being dislike extremely) was used to evaluate 10 products and compare them to similar commercially available products used as controls. The products tested were pancakes, waffles, tortillas, bread, pie, pound cake, pasta, vegetable patties, doughnuts, and pretzels. All of the products were either liked moderately or liked slightly with the exception of the sweet potato vegetable patties, which were neither liked nor disliked. Mean comparisons of sensory scores of sweet potato recipes and their controls were accomplished by using the Student t-test. Because of their nutritional adequacy and consumer acceptability, these products are being recommended to NASA's Advanced Life Support Program for inclusion in a vegetarian menu plan designed for lunar/Mars space missions.
Diet, Vegetarian↗
Sweet potato in a vegetarian menu plan for NASA's Advanced Life Support Program.
Sweet potato has been selected as one of the crops for NASA's Advanced Life Support Program. Sweet potato primarily provides carbohydrate--an important energy source, beta-carotene, and ascorbic acid to a space diet. This study focuses on menus incorporating two sets of sweet potato recipes developed at Tuskegee University. One set includes recipes for 10 vegetarian products containing fom 6% to 20% sweet potato on a dry weight basis (pancakes, waffles, tortillas, bread, pie, pound cake, pasta, vegetable patties, doughnuts, and pretzels) that have been formulated, subjected to sensory evaluation, and determined to be acceptable. These recipes and the other set of recipes, not tested organoleptically, were substituted in a 10-day vegetarian menu plan developed by the American Institute of Biological Sciences (AIBS) Kennedy Space Center Biomass Processing Technical Panel. At least one recipe containing sweet potato was included in each meal. An analysis of the nutritional quality of this menu compared to the original AIBS menu found improved beta-carotene content (p<0.05). All other nutrients, except vitamin B6, and calories were equal and in some instances greater than those listed for NASA's Controlled Ecological Life Support Systems RDA. These results suggest that sweet potato products can be used successfully in menus developed for space with the added benefit of increased nutrient value and dietary variety.
Diet, Vegetarian↗
Effect of seizures on cerebral blood flow measured with 15O-H2O and positron emission tomography.
To study quantitative alterations in regional cerebral blood flow (rCBF) accompanying seizures, and to assess the utility of ictal activation PET scanning as a noninvasive clinical tool for localization of epileptogenic foci, we used pentylenetetrazole (PTZ) to induce seizures during 15O-water positron emission tomography (PET) CBF measurement in 15 patients with uncontrolled complex partial seizures (CPS) who had been referred for surgical evaluation. Continuous EEG monitoring was performed during the PET scans. After baseline scans were obtained, each patient was injected with 150-300 mg PTZ. Two patients had generalized tonic-clonic seizures (GTCs). CBF increases were asymmetrical. Two patients (in 1 the seizure occurred spontaneously, without PTZ injection) who had CPS had bitemporal 70-80% increases in CBF. Thalamic CBF increased during both CPS and GTCS. Five patients had an increase in focal EEG interictal abnormality, accompanied by focal flow decreases in 3. PTZ injection not accompanied by clinical seizures did not increase CBF. Partial seizures may be associated with bilateral increases in CBF, and subcortical gray regions are involved in ictal activation.
Adult↗
The dynamics of metabolic change following seizures as measured by positron emission tomography with fludeoxyglucose F 18.
OBJECTIVE: To examine the time course of alterations in glucose metabolism in relation to the interval from the last seizure, focus laterality, seizure frequency, and seizure type. DESIGN: Metabolic study with the use of positron emission tomography with fludeoxyglucose F 18. Blinded scan evaluation with use of a standard template. Multivariate regression analysis of positron emission tomographic data. SETTING: National Institutes of Health Clinical Center, Bethesda, Md. PATIENTS: Thirty-two adults with intractable partial epilepsy and lateralized seizure onset documented by video-electroencephalographic monitoring. MAIN OUTCOME MEASURE: Normalized metabolic rate for glucose ipsilateral and contralateral to the epileptic focus. RESULTS: The most dramatic changes occurred in inferior temporal regions; the midtemporal region was affected as well. Effects lasting 48 hours were found after both simple and complex partial seizures. The time course was different for the two types of seizures. The inferior temporal metabolic rate ipsilateral to the focus increased compared with the interictal rate during the 24-hour period following simple partial seizures; a nadir occurred in the second 24 hours. The rate then rose to an intermediate level after 48 hours. The relative to an intermediate level after 48 hours. The relative regional increase in ipsilateral metabolism following complex partial seizures persisted for 48 hours before falling. CONCLUSION: The brain may take longer than 24 hours after a partial seizure to return to its baseline state.
Adolescent↗
Cardiovascular causes of loss of consciousness in patients with presumed epilepsy: a cause of the increased sudden death rate in people with epilepsy?
UNLABELLED: BACKGROUND, METHODS, AND RESULTS: Syncope and seizures are often indistinguishable clinically. We present a series of 12 patients diagnosed as having epilepsy. Despite normal or nonspecific electroencephalographic findings, 11 of 12 patients were treated or offered treatment with long-term anticonvulsant agents. Subsequently, diagnoses of arrhythmic or neurally mediated syncope were made in all patients using Holter monitoring, long-term ambulatory loop electrocardiographic recording, or tilt-table studies. Arrhythmias included torsades de pointes (four patients), atrioventricular nodal reentrant supraventricular tachycardia (one patient), and sinus arrest (two patients). The remaining five patients had neurally mediated syncope with hypotension and bradycardia, including asystole in two patients. Treatment for the documented cardiovascular abnormalities resulted in the alleviation of syncopal symptoms. CONCLUSIONS: Because the observed cardiovascular abnormalities are potentially fatal, this series suggests that undiagnosed cardiac syncope may contribute to the documented increased sudden death rate in patients with presumed epilepsy. Cardiac causes of loss of consciousness should be considered in patients with presumed epilepsy, atypical premonitory symptoms (such as nausea, lightheadedness, or palpitations), nondiagnostic electroencephalograms, and failure to respond to anticonvulsant therapy.
Adolescent↗
The secondarily generalized tonic-clonic seizure: a videotape analysis.
We studied 120 generalized tonic-clonic seizures (GTCSs) in 47 patients with video-EEG telemetry. GTCSs were preceded by antecedent seizures, including 13 simple partial, 70 complex partial, 17 simple partial leading to complex partial, seven tonic, seven clonic, and one typical absence. We divided GTCSs into the following phases: onset of generalization, pretonic clonic, tonic, tremulousness, and clonic. The mean GTCS duration was 62 seconds. There was a non-significant trend toward longer duration on reduced antiepileptic drug doses. Marked heterogeneity in GTCS phenomenology was present; only 27% of seizures included all five phases. Individual phase duration and clinical expression, including tonic and clonic phases, was highly variable. The clinical phenomena suggest that multiple cortical and subcortical routes of spread may exist. When GTCSs last longer than 2 minutes, intravenous antiepileptic drug treatment should be initiated.
Adolescent↗
Premonitory symptoms in epilepsy.
We studied premonitory symptoms in 128 patients with partial and 20 patients with primary generalized epilepsy. Sixty symptoms were reported by 43 (29%) patients, all of whom had partial seizure disorders (P < .01). Symptoms began at least 30 minutes before seizure onset, lasted 10 minutes to 3 days, and were continuous. Irritability, depression, headache, 'funny feeling', and confusion were the most common symptoms. Premonitory symptoms are a warning system in some patients and may permit therapeutic interventions.
Adolescent↗
Pathology of temporal lobe foci: correlation with CT, MRI, and PET.
Twenty-six patients with medically refractory complex partial seizures had temporal lobectomy after evaluation, which included prolonged scalp EEG recordings, positron emission tomography (PET), MRI, and x-ray CT. PET showed a region of focal interictal temporal hypometabolism corresponding to electrographic localization of seizure onset in 21. Five patients had a region of increased MRI signal intensity on the spin echo image in the region of the EEG focus, 2 had an abnormality ipsilateral to but distinct from the EEG focus, and 1 had bilateral findings. CT was abnormal in 3 cases; 2 had tumors. Three patients had low grade tumors (1 with a normal PET). PET can detect metabolic dysfunction associated with mild pathologic changes in epileptic foci, but increased signal intensity on MRI does not necessarily correlate with the degree of pathologic abnormality. Tumors may be less likely when both CT and MRI are normal.
Adolescent↗
Autoscopic phenomena with seizures.
We report the cases of 10 patients with seizures and autoscopic phenomena, which include seeing one's double and out-of-body experiences, and review 33 additional cases of autoscopic seizures from the literature. Autoscopic phenomena may be symptoms of simple partial, complex partial, or generalized tonoclonic seizures. Autoscopic seizures may be more common than is recognized; we found a 6.3% incidence in the patients we interviewed. The temporal lobe was involved in 18 (86%) of the 21 patients in whom the seizure focus could be identified. There was no clear lateralization of lesions in patients with ictal autoscopy. The response of autoscopic episodes to treatment usually paralleled that of the underlying seizure disorder. Autoscopic phenomena are likely to be discovered only on specific questioning of patients with epilepsy and may be an important, distressing feature of a chronic seizure disorder.
Adult↗
The effect of carbamazepine on cerebral glucose metabolism.
We used positron emission tomography with 18F-2-deoxy-D-glucose to study the effect of carbamazepine on local cerebral metabolic rate for glucose (lCMRGlc) in 9 patients with complex partial seizures. Twenty regions of interest were evaluated. Seven control patients had serial scans without a drug change. Metabolic rates were significantly (p less than 0.05) lower in patients on carbamazepine in 6 of 20 regions of interest (3 left cerebral hemisphere, 3 right). Mean lCMRGlc was 7.4 +/- 2.0 mg/min/100 in patients on carbamazepine and 8.8 +/- 2.5 in patients off carbamazepine (p less than 0.00005; cutoff level for 180 comparisons: 0.00027). The mean (+/- SEM) difference in lCMRGlc between scans was 12 +/- 2%. No significant changes in lCMRGlc on serial scans were detected in any of the 20 regions for the control group. The mean (+/- SEM) variation for control regions of interest was 1 +/- 1%. This study showed that carbamazepine depresses cerebral glucose metabolism as much as phenytoin does, but much less than phenobarbital does. The difference in effect on lCMRGlc may be related to drug mechanisms of action, as well as to effects on memory, learning, mood, and behavior.
Brain↗
Dissociative states and epilepsy.
Since symptoms of chronic dissociative disorders such as multiple personality disorder (MPD) may be shared by patients with seizure disorders, we investigated the possible relationship between dissociative states and epilepsy. We monitored 6 MPD patients with intensive video-EEG recordings to determine whether epileptic phenomena have any correlation to the dissociative symptoms experienced by these patients. Previously, physicians had diagnosed epilepsy in all 6 patients; however, none proved to have epilepsy. In addition, we studied dissociative symptoms in 71 epileptic patients with the aid of a standardized questionnaire, the Dissociative Experiences Scale, and compared them with age-matched controls. While the group median score of cases with complex partial seizures was higher than that of normal controls, it was significantly lower than that of the psychiatric patients with MPD. Partial seizure patients with dominant hemisphere foci had higher depersonalization subscale scores than those with nondominant foci. Our data suggest that epilepsy is not a primary pathophysiologic mechanism for developing dissociative symptoms.
Adult↗
Anterior temporal (T1/T2) electrodes in the evaluation of patients with seizure disorders.
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Adult↗
Hydrocephalic dementia and spinal cord tumor. Report of a case and review of the literature.
A 68-year-old woman presented with an 18-month history of low back pain followed by leg weakness, dementia, and incontinence. Myelography revealed an intradural, extramedullary block from L-2 to L-4, and cranial computed tomography demonstrated ventriculomegaly. Excision of a benign schwannoma resulted in rapid relief of back pain and more gradual normalization of mental function and hydrocephalus. Five similar cases of dementia and hydrocephalus complicating spinal cord tumor have been reported. A variety of mechanisms have been proposed to explain the association but the pathophysiology is still unclear. Spinal tumor should be considered in the differential diagnosis of dementia and of communicating hydrocephalus.
Aged↗
Sex differences in patterns of EEG asymmetry.
This paper reports three studies showing sex differences in EEG asymmetry during self-generated cognitive and affective tasks. In the first experiment, bilateral EEG, quantified for alpha on-line, was recorded from right-handed subjects while they either whistled, sang or recited lyrics of familiar songs. The results revealed significant asymmetry between the whistle and talk conditions only for subjects with no familial left-handedness and, within this group, only for females and not for males. In the second experiment, bilateral EEG was recorded while right-handed subjects (with no familial left-handedness) self-induced covert affective and non-affective states. Results revealed significantly greater relative right-hemisphere activation during emotion versus non-emotion trials only in females; males showed no significant task-dependent shifts in asymmetry between conditions. The third experiment was designed to test the hypothesis that females show greater percent time asymmetry than males during biofeedback training for symmetrical and asymmetrical EEG patterns. Results confirmed this prediction as well as indicating that females show better control of such asymmetrical cortical patterning. These findings provide new neuropsychological support for the hypothesis of greater bilateral flexibility in females during self-generation tasks.
Affect↗
Felbamate: a clinical trial for complex partial seizures.
We performed a randomized, double-blind, three-period cross-over study of felbamate (FBM, 2-phenyl-1,3-propanediol dicarbamate: Carter-Wallace 554) in patients with complex partial seizures. Patients continued carbamazepine (CBZ) throughout the study and were observed in the hospital for the entire trial period. The entry criteria required at least six seizures in a 3-week baseline period (and no more than 1 week with a single seizure) with CBZ alone. Thirty subjects were randomized. Two left the study after randomization, 1 owing to seizure exacerbation, and 1 owing to hyponatremia, which may have been related to CBZ therapy. The daily dosage of 50 mg/kg (maximum 3,000 mg) FBM per day was well tolerated by all 28 patients who completed the study. Only mild adverse experience were observed during the trial. FBM reduced CBZ level (p less than 0.0001; 95% confidence interval -28%, -20%). There was no significant difference in seizure frequency between placebo and FBM periods (one-sided p = 0.172), but when a correction was made for the lower CBZ level noted during FBM periods, the data suggested a strong antiseizure effect of FBM.
Adult↗