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Biomedical subjects

F Andermann

Publications and source records attributed to F Andermann.

At least 289 records · Page 16Linked to original sources

False lateralization by surface EEG of seizure onset in patients with temporal lobe epilepsy and gross focal cerebral lesions.

Medically intractable temporal lobe seizures developed in 3 patients with radiological and clinical evidence of a gross focal cerebral lesion acquired early in life. All had bilateral independent epileptogenic discharges from the lateral and inferomesial regions of both hemispheres. Scalp and sphenoidal electroencephalographic (EEG) recordings suggested that the seizures originated from the side contralateral to the known cerebral lesion. Because the lateralizing evidence presented by the ictal EEG conflicted with the clinical data, depth electrodes were implanted stereotaxically to determine the side of onset of the seizures. These studies showed that the seizures originated from the limbic structures of the damaged hemisphere in all 3 patients. At operation the mesial temporal lobe structures showed gliotic changes in all. The patients have remained seizure-free for 3 to 13 years postoperatively. These findings suggest that depth electrode recordings may be required to clarify the lateralization of seizure onset in such cases. Extracranial EEG findings must be interpreted with caution in epileptic patients who have gross focal lesions.

Adult↗

Effect of generalized spike-and-wave discharge on glucose metabolism measured by positron emission tomography.

Positron emission tomography was used to study the cerebral metabolic rate for glucose (CMRGlc) in 7 adult patients with generalized spike-and-wave activity in the electroencephalogram. No consistent changes were seen in the CMRGlc. There was a slight trend toward an increased CMRGlc in 2 patients with primary generalized epilepsy, while in the 5 other patients with minor deviations from this condition or with secondary generalized epilepsy, the CMRGlc was unaffected by spike-and-wave activity or was below the normal range. Neither the amount of spike-and-wave activity in the EEG nor the presence of clinically evident absence seizures appeared to influence the CMRGlc. Therefore, we conclude that neuronal activity underlying spike-and-wave discharge does not seem to require increased glucose utilization.

Adolescent↗

Magnetic resonance imaging in temporal lobe epilepsy: pathological correlations.

A retrospective single-blind study assessing the value of magnetic resonance imaging (MRI) in 48 patients treated surgically for temporal lobe epilepsy was carried out. The imaging findings were correlated with the surgical findings in all cases. Abnormal MRI signals were detected in 34 of 48 (71%) epileptic patients and in 3 of 48 (6.2%) normal or disease control subjects. Twelve patients had structural foreign-tissue lesions, all detected by MRI. Of 14 patients with severe gliosis of the neocortex and/or mesial temporal structures, 11 had abnormal MRI scans. In patients with mild or moderate gliosis of mesial temporal structures, 6 of 12 had abnormal MRI scans. These results indicate that MRI is a sensitive technique for localizing foreign-tissue lesions, mesial temporal sclerosis, and gliosis in patients with intractable temporal lobe seizures.

Adolescent↗

Concepts of absence epilepsies: discrete syndromes or biological continuum?

There are two current approaches to the clinical conceptualization of the generalized epilepsies. The syndromic approach attempts to subdivide the patient population into relatively homogeneous groups, largely on the basis of clinical and EEG criteria. In contrast, the neurobiological approach aims to formulate a unique profile for each patient by incorporating particulars of the patient onto the background of knowledge regarding the etiologic factors important in generalized epilepsy. The value of these two approaches is discussed with regard to the dual aims of, first, improving the understanding of generalized epilepsy, and second, providing a precise diagnosis, an accurate prognosis, and optimal treatment for the patient.

Adolescent↗

Stability of alpha-1 adrenoceptors in surgically excised human brain.

Alpha-1 adrenoceptor sites were measured in membranes prepared from nonepileptic superficial cortex following temporal lobectomy for lesions in deep medial structures. There was no significant change in receptor density (Bmax) or affinity (Kd) when paired samples were either frozen immediately or kept at room temperature for 24 hours before freezing and storage at -70 degrees C. Regional variability in the Bmax or Kd of alpha-1 adrenoceptor binding was not observed in serial samples from lateral temporal cortex. We previously reported a localized decrease in alpha-1 adrenoceptors in epileptic foci when compared to adjacent nonepileptic tissue obtained from the same patient. As nonepileptic control tissue from an adjacent gyrus is frequently not available in the same specimen, the stability of alpha-1 adrenoceptors justifies the use of postmortem brain for comparative studies.

Adolescent↗

Urinary sediment dolichols in the diagnosis of neuronal ceroid-lipofuscinosis.

Long-chain polyisoprenol alcohol (dolichols) levels are significantly increased in the urinary sediment of patients with infantile, late-infantile, and juvenile forms of neuronal ceroid-lipofuscinosis (NCL). The values in obligate heterozygotes for these diseases are similar to those in patients with other neurological diseases and in healthy controls. Antioxidant treatment of patients with juvenile NCL has no effect on dolichol values. The rate of false-negative results is 13.9% in infantile, 7.5% in late-infantile, and 15.0% in juvenile NCL. False-positive results were found in 8.2 to 14.3% of patients with other neurological diseases and in 15.4% of healthy controls. The test is of considerable value in the diagnosis of NCL and in decisions on whether to perform a biopsy. It is not useful in the screening of random samples, however.

Adolescent↗

Giant axonal neuropathy: central abnormalities demonstrated by evoked potentials.

Previous studies of giant axonal neuropathy have reported clinical and pathological findings that indicate involvement of the central nervous system. We studied 3 boys with giant axonal neuropathy, who were 14 to 16 years of age, using auditory, visual, and somatosensory evoked potentials. Absence of waveforms and prolongation of peak and interwave latencies were found. Abnormalities were noted in all modalities. The auditory brainstem evoked response in particular indicated a significant increase in brainstem conduction time. These studies add clinical neurophysiological confirmation of the central nervous system involvement in this disorder and may also provide a means of quantitative evaluation of its progression.

Adolescent↗

Abnormalities of T-lymphocyte subsets in epileptic patients.

This study concerns the distribution of T-cell subsets as determined by specific monoclonal antibodies in 50 individuals with complex partial seizures (31) and primary generalized tonic-clonic seizures (19), and in 30 healthy controls. The epileptic group had significantly fewer circulating T4 "helper" lymphocytes and significantly greater number of T8 "suppressor" lymphocytes than the controls. The T4/T8 ratio was consistently significantly lower in the epileptic group. There was no relation between lymphocyte subsets or T4/T8 ratio and antiepileptic medication. The results suggest a derangement of cell-mediated immunity in individuals with epilepsy.

Adolescent↗

The place of stereotactic depth electrode recording in epilepsy.

A series of 70 patients studied with stereotactically implanted depth electrodes is analyzed. Indications and grouping fall into three main categories: the bitemporal series (52 patients), where there is ambiguity as to the lateralization of the focus; the unilateral series (6 patients), where the main problem is one of localization within one hemisphere, and the generalized and/or multifocal series (12 patients), where a primary focus is suspected. The best indication is represented by the group of 'bitemporal cases' which turned out to have a well-lateralized onset with intracerebral recording.

Electrodes, Implanted↗

Patterns of seizure activation after withdrawal of antiepileptic medication.

Effects of withdrawal of anticonvulsant drugs on the temporal profile of occurrence and the type of seizures were investigated in 40 intractable epileptic patients who were candidates for surgical treatment. EEG and behavior were monitored while drugs were reduced to allow localization of the epileptogenic region. The rapid withdrawal of drugs caused a rebound effect, triggering either generalized seizures during a brief period or a longer-lasting increase in partial seizures. These increases in seizure frequency appeared related to change in dosage rather than to dosage itself, since they remained largely confined to the early period following reduction of an anticonvulsant.

Adult↗

Juvenile progressive dystonia: a new phenotype of GM2 gangliosidosis.

A 10-year-old boy developed progressive dystonia and dementia. His symptoms had begun at age 2 1/2 years, and he had been unable to walk by 8 years. At age 10 he was severely dystonic, unable to use his hands to feed himself, and almost anarthric . He had dysphagia and urinary incontinence, and functioned at a 4-year-old level of mental development. The mean percentages of beta-hexosaminidase A measured in serum, leukocytes, and fibroblasts by the heat denaturation method, each on three separate assays, were 5.9, 9.8, and 13.0%, respectively. These values are higher than in Tay-Sachs disease but are similar to levels seen in late-onset or adult cases of GM2 gangliosidosis. This patient appears to represent a new phenotype of juvenile GM2 gangliosidosis having dystonia as the dominant symptom.

Child↗

The hemi 3 syndrome. Hemihypertrophy, hemihypaesthesia, hemiareflexia and scoliosis.

Three unrelated girls presented with a developmental syndrome of hypertrophy involving half or a quadrant of the body and not involving the face. The appearance was one of inappropriately large size of the affected side rather than contralateral atrophy. On the larger side, there was hypertrophy of muscle and increased power as well as an increase in diameter, but not in length, of long bones. There was areflexia and decreased pain and temperature sensation on that side. The patients also had progressive scoliosis and foot deformities on the enlarged side. One patient had a lumbar myelomeningocoele , and all 3 had a family history of neural tube closure defects. EMG, nerve conduction studies, EEG, skull x-rays, PEG, and cerebral CT scans were normal. Myelography did not demonstrate an enlarged cord, and in particular there was no evidence for syringomyelia. Chromosome studies revealed normal karyotypes. Sex chromatin was female on both sides in one patient. A defect of the dorsal lip of the neural tube or the neural crest is postulated to explain the abnormality. The association with closure defect in one patient and a positive family history of other neural tube defects in all 3 patients suggests that the developmental defect occurs at an early embryonic stage. Recognition of the syndrome is important. It can be distinguished clinically from hemiatrophy of cerebral origin. The neurological abnormalities are static, but the scoliosis is progressive and requires correction. The condition is associated with an increased prevalence of neural tube closure defects in the family, and forms part of a spectrum of genetically and embryologically related CNS malformations with multifactorial inheritance. Probands, parents, siblings and parents' siblings should be counselled that the risks of spina bifida and anencephaly in their offspring are the same as those in relatives of probands with classical neural tube defects, and should be offered prenatal diagnosis.

Adult↗