Rasmussen encephalitis with ipsilateral brain stem involvement in an adult patient.
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Adult↗
Biomedical subjects
C G Bien
Publications and source records attributed to C G Bien.
Transient splenium lesions in presurgical epilepsy patients: incidence and pathogenesis.
INTRODUCTION: Transient splenium corporis callosi (SCC) lesions are related to rapid reduction of antiepileptic drugs (AEDs). The range of substances with predilection for SCC changes, their pathophysiology and their occurrence are still unknown. METHODS: In a prospective 2-year study an epilepsy-dedicated MRI protocol supplemented by DWI and ADC maps was performed after AED withdrawal for diagnostic seizure provocation in all patients with pharmacoresistant seizures locally admitted to the Department of Epileptology. RESULTS: Of 891 presurgical epilepsy patients, 6 (0.7%) had SCC lesions with cytotoxic edema on DWI. Carbamazepine combined with other AEDs was administered in five of those patients. In the study period we observed identical lesions in a schizophrenic patient treated with olanzapine and citalopram, in a patient with oropharyngeal carcinoma treated with alkylating agents, and in a hypernatremic patient following neurohypophyseal granular cell tumor surgery. CONCLUSION: Transient SCC lesions are related to rapid AED reduction but may occur in similar conditions with fluid balance alterations. We contribute further clinical data in this field to better classify the pharmaceuticals that are prone to the described cerebral cytotoxic side effects in the SCC and to clarify their incidence among presurgical epilepsy patients.
Adult↗
MRI brain volumetry in Rasmussen encephalitis: the fate of affected and "unaffected" hemispheres.
Rasmussen encephalitis (RE) is regarded as a unihemispheric disease. Serial three-dimensional MRIs of 18 patients under immunotherapy were analyzed volumetrically and planimetrically. Median volume loss was significantly higher in the affected than in the unaffected hemispheres (29.9 cm3/y vs 6.8 cm3/y). Correlation of the planimetrically and volumetrically assessed hemispheric ratios (HRs) was significant. The results support the concept of RE as a basically unilateral disease. Planimetric HR assessment is valid and time efficient.
Adolescent↗
Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement.
Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patients.
Adolescent↗
[VGKC antibodies associated with limbic encephalitis].
Since the initial description of limbic encephalitis (LE) in 1960/1968, several subforms of this clinico-neuropathological syndrome have been identified. The best known is paraneoplastic LE. However, non-paraneoplastic forms have been reported, too. Very recently, autoantibodies against voltage-gated potassium channels have been described in association with LE. The diagnostic workup of such a case and the apparently typical good response to long-term immunotherapy of this LE subform are described.
Antibodies↗
[Out-of-body experience as possible seizure symptom in a patient with a right parietal lesion].
Out-of-body experiences are reported in a variety of diseases and physiologic conditions. We report a 44-year-old patient with epigastric auras, psychomotor and grand mal seizures, and paroxysmal experiences during which he believed to have left his body and seen himself from the outside. Electroencephalography showed a right temporal and right parietal seizure pattern on several occasions, and a lesion in the right parietal lobe was detected by cranial magnetic resonance imaging. Histological examination showed a pleomorphic xanthoastrocytoma. Following extirpation of the lesion, the patient has been seizure-free for 5 years.
Adult↗
[Recent insights into Rasmussen encephalitis].
Rasmussen encephalitis (RE) is a severe and at the same time pathophysiologically fascinating condition. The chronic inflammation affects one of the two cerebral hemispheres and destroys it during the disease process that lasts from months to years. The patients -- mostly children -- suffer from frequent pharmacoresistant seizures, often in the form of epilepsia partialis continua. In parallel to the atrophy of the affected hemisphere, the neurological functions associated with it decline continuously. This results in a final stage with a usually high-grade sensorimotor hemisyndrome, hemianopia, cognitive impairment and -- if the language-dominant hemisphere is affected -- aphasia. Research results in the last 5 years have contributed to a deeper understanding of the pathogenesis of this condition. Formal diagnostic criteria have been proposed, and new therapeutic options have emerged by which the disease progression can be slowed or stopped. This article summarizes the current research results on the background of older data and gives recommendations regarding diagnostic and therapeutic procedures in RE patients.
Clinical Trials as Topic↗
An open study of tacrolimus therapy in Rasmussen encephalitis.
Seven patients with Rasmussen encephalitis (RE) were treated with the immunosuppressant tacrolimus and followed for a median of 22.4 months. They were compared with 12 historical untreated RE patients (median follow-up 13.9 months). The tacrolimus-treated patients had a superior outcome regarding neurologic function and progression rate of cerebral hemiatrophy but no better seizure outcome. No treated patient, but 7 of 12 control patients, became eligible for hemispherectomy. Tacrolimus did not have any major side effects.
Adolescent↗
Rasmussen encephalitis with active inflammation and delayed seizures onset.
Severe focal epilepsy is regarded as a clinical hallmark of Rasmussen encephalitis (RE). The authors report two children with progressive hemiparesis, contralateral hemispheric atrophy, and pathologic features characteristic for RE. At histologic diagnosis and over several months, neither patient experienced seizures. The report enlarges the clinical spectrum of RE and suggests that seizures are not an obligatory presenting symptom of the disorder.
Age of Onset↗
Diagnosis and staging of Rasmussen's encephalitis by serial MRI and histopathology.
OBJECTIVE: To correlate MRI and histopathologic findings in patients with Rasmussen's encephalitis (RE). PATIENTS AND METHODS: MRI features of 10 patients with RE were studied on serial scans. In surgical specimens from these patients, densities of T lymphocytes, microglial cells and nodules, and reactive astrocytes were evaluated. RESULTS: Densities of T cells, microglial nodules, and astrocytes were inversely correlated to disease duration. MRI abnormalities had a focal onset and spread across one hemisphere. The following course of MRI abnormalities in a given brain region was observed: on very early scans, the cortex was swollen and showed a hyperintense T2/fluid-attenuated inversion recovery signal. Consecutively, progressive atrophy of the affected hemisphere occurred. Correlation of MRI features with quantitative histopathology revealed that there was a higher number of T cells and reactive astrocytes in the earlier MRI stages compared with the late (merely atrophic) stage. CONCLUSION: These data suggest a disease course in RE with the highest inflammatory intensity in the early stages and a subsequent decrease in inflammation. The MRI abnormalities and their characteristic sequence may help to identify patients with RE and to obtain informative biopsies.
Adolescent↗
GluR3 antibodies: prevalence in focal epilepsy but no specificity for Rasmussen's encephalitis.
Eight patients with Rasmussen's encephalitis, 40 patients with noninflammatory focal epilepsy, 104 patients with various neurologic diseases, and 16 healthy donors were tested for the prevalence of antibodies against the GluR3 receptor in serum and CSF. Reactivities against different peptides derived from various portions of this glutamate receptor subtype were detectable in a significantly higher number of patients with focal epilepsy than in those with other neurologic diseases, but they were not specific for the diagnosis of Rasmussen's encephalitis.
Adolescent↗
Monotherapy trials in antiepileptic drugs: are modified "presurgical studies" a way out of the dilemma?
Monotherapy trials in epilepsy are confronted with a dilemma: either they are in conflict with ethical requirements, or they are scientifically not meaningful. Monotherapy trials, which are performed as controlled studies randomizing patients to ineffective (pseudo)placebo treatment, are incompatible with the Helsinki Declaration. On the other hand, equivalence or noninferiority studies using an active-control design do not permit valid conclusions on the efficacy of the test drug. Therefore, they do not fulfill scientific requirements for trials on new drugs. As an alternative approach, a monotherapy trial design for epilepsy patients undergoing presurgical evaluation was outlined. During presurgical evaluation, antiepileptic drugs are routinely tapered down for seizure recording. This situation is used for a placebo-controlled short-term monotherapy trial. Four trials according to this design have been completed so far. Recently, several points of concern have been raised against this design, especially for matters of ethics and external validity. In the present article, these objections are outlined and discussed. In the proposed modification the randomization and the titration of the test drug or control begins prior to the presurgical investigations. The advantages are: the test drug does not have to be titrated quickly, pure monotherapy conditions are achieved, and the subjects do not have to experience more seizures than are required for the presurgical evaluation.
Anticonvulsants↗
Long-term seizure outcome and antiepileptic drug treatment in surgically treated temporal lobe epilepsy patients: a controlled study.
PURPOSE: To evaluate the long-term impact of surgical treatment on seizure outcome and antiepileptic drug (AED) use in patients with pharmacoresistant temporal lobe epilepsy (TLE). METHODS: Comparison of seizure outcome and AED us in operated-on TLE patients (n=148) and nonsurgically treated TLE patients (n=94) at a baseline visit and a follow-up visit after a mean period of 4.8 years. RESULTS: At follow-up, 44.6% of the surgical patients and 4.3% of the nonsurgical patients had been continuously seizure- free since the baseline visit (including the immediate postoperative period). A further 17.6% of the operated-on and 3.2% of the not operated-on patients had been seizure-free for at least the previous year; 37.8% of the surgical and 92.5% of the nonsurgical patients had had seizures during the previous 12 months (p < 0.001). Of the surgical patients, 8.8% versus none of the nonsurgical patients were AED free at follow-up; 55.4% versus 20.2% were receiving monotherapy, and 35.8% versus 79.8% were receiving polytherapy (p < 0.001). Mean number of AEDs and mean change in number of AEDs were significantly more favorable in operated-on than in non-operated-on patients. Further subgroup analysis revealed that not only the continuously seizure-free surgical patients, but also the operated-on patients with ongoing seizures took fewer AEDs than their respective non-operated-on counterparts. CONCLUSIONS: This controlled study for the first time provides comprehensive information on long-term seizure outcome and AED use in surgical TLE patients. It shows a more favorable seizure outcome and AED use in the surgically treated patients. The latter holds true even for the not seizure-free patient subgroup.
Adult↗
Is it really Alzheimer's disease?
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Alzheimer Disease↗
Limbic encephalitis not associated with neoplasm as a cause of temporal lobe epilepsy.
OBJECTIVE: To describe four patients with temporal lobe epilepsy with limbic encephalitis unrelated to neoplasm. METHODS: The authors performed a retrospective evaluation of patient data obtained during presurgical evaluation, with additional CSF analyses, serum analyses, and histopathologic investigations. RESULTS: The patients shared the following clinical features: onset of the disease in young adulthood with subacute onset or exacerbation of frequent intractable temporal lobe seizures, verbal and visual memory deficits, and affective abnormalities. MRI showed variably extended areas of increased T2 signal in limbic structures and adjacent areas. In the histopathologic investigation, chronic inflammation was observed without evidence of a viral origin. There was no evidence of an underlying malignancy. CONCLUSIONS: Nonparaneoplastic limbic encephalitis should be included in the differential diagnosis of adult patients with temporal lobe epilepsy.
Adult↗
Localizing value of epileptic visual auras.
It is difficult to differentiate between seizures of occipital or temporal lobe origin in patients with focal epileptic seizures associated with visual aura. These are often suspected to originate from the visual cortex, which causes reluctance to propose resection as treatment for the affected patient. To determine the value of localizing different types of visual auras, we report on 20 patients experiencing visual aura from a series of 878 surgically treated patients suffering from intractable focal seizures. In all of these patients, a morphological abnormality was identified on MRI (n = 18) or cranial CT (n = 2). These abnormalities were shown to represent the morphological correlate of the epileptogenic zone in each case, as demonstrated by intracranial ictal EEG recordings and/or seizure freedom after focal resective surgery. Elementary hallucinations, illusions and visual loss were reported not only by all patients with occipital lobe epilepsy, but also by patients with occipitotemporal and anteromedial temporal seizure onset. Complex hallucinations never occurred in occipital lobe seizures but were present in the two other groups. The same correlation was found for concentric changes of visual field (tunnel vision), a newly described ictal phenomenon. We conclude that elementary hallucinations, illusions and visual loss, although typical for occipital lobe epilepsy, can also occur in anteromedial temporal or occipitotemporal seizures and are therefore not a discordant feature in presurgical evaluation of patients with suspected temporal lobe epilepsy. Complex hallucinations and tunnel vision, however, should be considered concordant only with the assumption of an anteromedial temporal or occipitotemporal seizure onset.
Adolescent↗
[Galen on the topic of abnormalities].
Galen on deformities--Galen's treatment of problems of deformities has its primary source in his twofold teleological conviction that nature acts purposefully and that the human organism and his parts serve their purposes in an optimal way. Thus he defines deformities as functionally deviant body parts. Operative therapy therefore is indicated only if an abnormal part of the body hinders its normal function. On the basis of scientific physiological concepts of his time Galen blames avoidable diatetic failures of the parents as causes of deformities. Galen's engagement on this point reveals the influence of his own experiences as a physician. Galen's statements on deformities show that his teleological concept of nature, his practical medical experience and scientific theories of his time enable him to draw fruitful theoretical and practical conclusions for matters of definition, indication for treatment and prophylaxis of deformities.
Congenital Abnormalities↗
[The "break" in Aristotle's presentation of genetic contributions of man and woman. Or: must he, who says a, always say b?].
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Female↗