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Biomedical subjects

M Reuber

Publications and source records attributed to M Reuber.

At least 19 recordsLinked to original sources

Functional symptoms in neurology: questions and answers.

Between 10 and 30% of patients seen by neurologists have symptoms for which there is no current pathophysiological explanation. The objective of this review is to answer questions many neurologists have about disorders characterised by unexplained symptoms (functional disorders) by conducting a multidisciplinary review based on published reports and clinical experience. Current concepts explain functional symptoms as resulting from auto-suggestion, innate coping styles, disorders of volition or attention. Predisposing, precipitating, and perpetuating aetiological factors can be identified and contribute to a therapeutic formulation. The sympathetic communication of the diagnosis by the neurologist is important and all patients should be screened for psychiatric or psychological symptoms because up to two thirds have symptomatic psychiatric comorbidity. Treatment programmes are likely to be most successful if there is close collaboration between neurologists, (liaison) psychiatrists, psychologists, and general practitioners. Long term, symptoms persist in over 50% of patients and many patients remain dependent on financial help from the government. Neurologists can acquire the skills needed to engage patients in psychological treatment but would benefit from closer working relationships with liaison psychiatry or psychology.

Humans↗

Multidimensional assessment of personality in patients with psychogenic non-epileptic seizures.

OBJECTIVES: To determine whether patients with psychogenic non-epileptic seizures (PNES) have evidence of maladaptive personality, and whether they have a single or several different typical pathological personality profiles. METHODS: Patients were recruited from the department of epileptology, Bonn, Germany. In all, 85 patients with PNES and 63 with epilepsy completed a postal questionnaire including the dimensional assessment of personality pathology - basic questionnaire (DAPP-BQ). The DAPP-BQ was also completed by 100 healthy volunteers. The groups were compared and the PNES group was subjected to cluster analysis. RESULTS: Patients with PNES had a greater degree of personality abnormality than clinical and non-clinical controls. There were several clusters of personality pathology. The profile of the largest cluster (n = 43) resembled that found in borderline personality disorder, that of the second largest (n = 37) was characterised by an overly controlled personality, that of the third (n = 4) was similar to the profile in avoidant personality disorder. Outcome differed between clusters. CONCLUSIONS: Maladaptive personality is common in patients with PNES. PNES are associated with several distinct profiles of pathological personality. This is relevant because outcome differed between profiles.

Adult↗

New drugs for the treatment of epilepsy: a practical approach.

The availability of new antiepileptic drugs has broadened the spectrum of medical treatment options in epilepsy. The new agents, together with established drugs, offer substantial choice for doctors treating patients with focal or generalised epilepsy. The newer antiepileptic drugs are not necessarily more effective but usually better tolerated than the traditional agents, mainly because of favourable pharmacokinetic profiles and fewer interactions. Because treatment options have increased, drug therapy can now be tailored to the requirements of individual patients. Nevertheless, significant safety and efficacy issues continue to exist and there is a need for the development of even better agents. This review describes the clinical use of the new antiepileptic drugs, but focuses in particular on monotherapy, the treatment of generalised seizures, teratogenicity, and the cognitive side effect profile of the newer compounds.

Abnormalities, Drug-Induced↗

Intrasubject reproducibility of presurgical language lateralization and mapping using fMRI.

BACKGROUND: fMRI is becoming a standard tool for the presurgical lateralization and mapping of brain areas involved in language processing. However, its within-subject reproducibility has yet to be fully explored. OBJECTIVE: To evaluate within-test and test-retest reliability of language fMRI in consecutive patients undergoing evaluation for epilepsy surgery. METHODS: Thirty-four unselected patients were investigated once (within-test reliability) and 12 patients twice (test-retest reliability). The imaging series consisted of an alternating 25-second synonym judgment condition with a 25-second letter-matching condition repeated 15 times. Reproducibility of activation maps of the first and second half of session 1 or activation maps of sessions 1 and 2 was evaluated by comparing one global and three regional lateralization indexes (Broca's area, remaining prefrontal cortex, temporoparietal area) and on a voxel-by-voxel basis (intraclass correlation coefficient, percentage overlap, correlation of t-values). RESULTS: Global and regional language lateralization was achieved with high reliability within and across sessions. Reproducibility was evenly distributed across both hemispheres but not within each hemisphere. Frontal activations were more reliable than temporoparietal ones. Depending on the statistical threshold chosen, the voxel-by-voxel analysis revealed a mean overlap of activations derived from the first and second investigation of up to 48.9%. CONCLUSION: Language fMRI proved sufficiently reliable for the determination of global and regional lateralization of language representation in individual unselected patients with epilepsy.

Adolescent↗

Diagnostic delay in psychogenic nonepileptic seizures.

Delay to diagnosis was studied in 313 consecutive patients with psychogenic nonepileptic seizures (PNES). On average, patients with PNES were diagnosed 7.2 years after manifestation (SD 9.3 years). Younger age, interictal epileptiform potentials in the EEG, and anticonvulsant treatment were associated with longer delays. Other patient factors did not explain the great variability of the time to diagnosis, suggesting that physician factors contributed to delays.

Adolescent↗

Time-efficient T2 relaxometry of the entire hippocampus is feasible in temporal lobe epilepsy.

OBJECTIVE: To test the clinical usefulness and reliability of a new dual-echo turbo-spin-echo (TSE) sequence for rapid and regional hippocampal T2 relaxometry. METHODS: Hippocampal T2 relaxation time (HRT) was determined by a TSE sequence on three to four consecutive coronal images in 16 control subjects and 12 patients with mesial temporal lobe epilepsy. HRT was related to neuropathology findings in hippocampal specimens including neuronal cell density (ND), results of visual analysis of MR images, clinical outcome after epilepsy surgery, and hippocampal volumetry. RESULTS: Rapid HRT differentiated patients from control subjects; all cases of hippocampal sclerosis (HS; n = 10) were correctly diagnosed. HRT showed a strong correlation with ND in CA1 (p < 0.02) and CA3 (p < 0.05). Diagnoses based on rapid relaxometry concurred fully with results of visual inspection. Mean HRT was prolonged ipsilaterally in all patients with excellent postoperative seizure outcome and bilaterally prolonged or normal in patients with poorer outcome. Rapid HRT was concordant with hippocampal volumetry in 10 of 12 patients. Regional HRT of control subjects revealed significantly higher values in the anterior than posterior hippocampus. In patients with unilateral HS, this gradient was absent. The gradient was also absent contralaterally to HS, although surgical outcome was excellent. CONCLUSIONS: Hippocampal dual-echo TSE-relaxometry can be regarded a reliable technique to detect and quantify HS. With a scan time of 3.31 minutes and immediate off-line analysis lasting a few minutes only, TSE-T2 relaxometry is easy to integrate in the routine diagnostic assessment of hippocampal morphology in large numbers of patients.

Adolescent↗

[Tolerance of high dosage carbamazepine monotherapy in treatment of epilepsy].

We examined whether high dose carbamazepine (CBZ) monotherapy (CBZ serum levels > 9 mg/l) is well tolerated. Forty-one patients with localisation-related epilepsy were enrolled. They received high dose monotherapy either from the time of recruitment (30/41), or their CBZ treatment was pushed into the high dose range (11/41) during the observation period (mean 145 days). CBZ and CBZ metabolite serum levels, full blood counts, liver function tests, electrolytes were measured in addition to clinical assessments. The mean daily CBZ dose was 1515 mg (median 1500, range 600-3600 mg). Mean CBZ serum level was 11.1 mg/l (median 11.1, range 5.3-16.4 mg/l). Eleven of 41 patients (26.8%) reported side effects (mean serum level 11.4, median 11.6, range 8.7-13.7 mg/l), but side effects were intolerable in only five patients so that the CBZ dose had to be reduced (mean CBZ serum level 11.4, median 11.6, range 8.7-13.7 mg/l). The total daily CBZ dose but not CBZ or CBZ metabolite levels were correlated with side effects. No treatment changes had to be undertaken because of abnormal laboratory parameters. High dose CBZ anticonvulsant monotherapy is well tolerated.

Adolescent↗

New-onset psychogenic seizures after intracranial neurosurgery.

BACKGROUND: Patients with physical brain abnormalities have an increased risk of developing psychogenic nonepileptic seizures (PNES). Here we describe patients who developed PNES after intracranial neurosurgery for indications other than the control of refractory epileptic seizures and explore whether neurosurgical intervention is at risk factor for PNES. METHOD: We searched the database of 372 patients diagnosed with PNES at our department over the last 10 years and identified 17 patients (4.6%) in whom PNES first started after intracranial neurosurgery. Surgical procedures included the complete or partial resection of a meningioma, AV malformation, cavernoma, plexus papilloma, neurinoma, astrocytoma, oligodendroglioma, dysontogenetic cyst, the drainage of a brain abscess and removal of a subdural hematoma. PNES were documented by ictal video-EEG, ictal EEG, or ictal observation and examination in all cases. The diagnosis of additional epileptic seizures were confirmed by ictal EEG/video-EEG, or made on the basis of a clinical assessment by an experienced epileptologist. FINDINGS: Five patients had purely psychogenic postoperative seizure disorders, twelve had epileptic and psychogenic attacks. Median age at neurosurgery was 32 years (range 5-54), median latency between surgery and onset of PNES was 1 year (range 0-17 years). INTERPRETATION: PNES may develop after intracranial neurosurgery undertaken for other indications than the control of refractory epileptic seizures. Younger patients with a history of pre-operative psychiatric problems or epileptic seizures and surgical complications may be at higher risk. A diagnosis of PNES should be considered in patients who develop refractory seizures after neurosurgery.

Adaptation, Psychological↗

Evidence of brain abnormality in patients with psychogenic nonepileptic seizures.

Markers of brain abnormalities in patients with psychogenic nonepileptic seizures (PNES) were studied to explore whether physical brain disorder is associated with an increased risk of PNES. Evidence of epileptiform EEG changes, MRI abnormalities, and neuropsychological (NPS) deficits was obtained from the records of 329 consecutive patients in whom the diagnosis of PNES was established at our center between 1991 and 2001. Two hundred six patients had PNES alone, and 123 PNES and epilepsy (PNES+E). In the PNES-only group, at least one marker of brain disorder was detected in 22.3% of patients (epileptiform potentials in 8.7%, MRI changes in 9.7%, NPS deficits in 9.7%). The actual prevalence of abnormalities is likely to be higher because 54.9% of the patients with only psychogenic seizures did not undergo MRI or neuropsychological testing. Evidence of brain abnormality was found more frequently in the PNES+E group (epileptiform potentials in 70.7%, MRI changes in 60.2%, NPS deficits in 52.8%). Although markers of brain abnormality were detected much less commonly in the PNES than in the PNES+E group, they were still found in a substantial proportion of patients with PNES alone. This suggests that physical brain disease plays a role in the development of PNES. Markers of physical abnormality were not more common in the right hemisphere.

Journal Article↗

Stroke after internal jugular venous cannulation.

OBJECTIVE: To alert clinicians to the stroke risk associated with carotid artery injury secondary to attempted internal jugular venous (IJV) cannulation. METHODS: Case reports and review of the literature. RESULTS: Four patients developed a stroke following carotid artery (CA) injury during attempted IJV cannulation using the landmark technique. In all cases the arterial puncture was detected immediately and firm pressure applied for several minutes. In three cases there was evidence of intimal injury and thrombus formation. Two strokes were delayed by more than 24 h. One patient died. A review of studies describing 4487 IJV line insertion attempts using the landmark technique reveals that 5.9% of attempts are associated with CA injury. CONCLUSION: Cannulation of the IJV using visible and palpable landmarks is associated with a risk of stroke. Arterial injury and stroke should be mentioned when consent is obtained for cannulation. Consideration should be given to a reduction of the arterial injury risk by using ultrasound guidance during line insertion.

Adult↗

The localizing value of ictal consciousness and its constituent functions: a video-EEG study in patients with focal epilepsy.

Using ictal neuropsychological testing in pre-surgical patients with focal epilepsies, we examined the localizing value of the constituent functions of consciousness as opposed to 'conscious behaviour' as a unitary variable. 'Conscious behaviour' was defined in terms of awareness and responsiveness. The constituent functions of consciousness examined included the orientation to the examiner, intentional behaviour demonstrated by expressive or receptive speech, and postictal memory. Frequency and patterns of impairment of constituent functions and 'conscious behaviour' were assessed. To achieve this, pre-surgical video-EEG (n = 40) or video-electrocorticography recordings (n = 76) of ictal neuropsychological assessments were reviewed retrospectively. Patients were divided into groups with frontal (n = 29), right temporal (n = 21), left temporal (n = 38) and bitemporal (n = 28) seizure activity. Consciousness was most commonly impaired in patients with bitemporal and left temporal seizure activity. There were different patterns of impairment of the assessed constituent functions in the four groups: patients with frontal seizure activity showed loss of orientation behaviour and expressive speech whereas patients with left temporal seizure activity had impairments of memory, expressive and receptive speech. Patients with seizure activity limited to the right temporal lobe rarely exhibited ictal impairment of any of the assessed functions. In contrast, patients with bitemporal seizure activity showed impairment of all examined functions. Hence, normal functioning of the left temporal lobe or both temporal lobes is necessary for the preservation of all constituent aspects of consciousness. The localizing value of patterns of impairment of constituent functions is superior to that of 'consciousness' as a whole.

Adolescent↗

Reproductive dysfunction in women with epilepsy: recommendations for evaluation and management.

BACKGROUND: Epilepsy is commonly associated with reproductive endocrine disorders. These include polycystic ovary syndrome (PCOS), isolated components of this syndrome such as polycystic ovaries, hyperandrogenaemia, hypothalamic amenorrhoea, and functional hyperprolactinaemia. OBJECTIVE: To summarise the currently known relations between epilepsy and reproductive endocrine disorders. METHODS: A review of clinical experience and published reports. RESULTS: The most likely explanations for endocrine disorders related to epilepsy or antiepileptic drugs are: (1) a direct influence of the epileptogenic lesion, epilepsy, or antiepileptic drugs on the endocrine control centres in the brain; (2) the effects of antiepileptic drugs on peripheral endocrine glands; (3) the effects of antiepileptic drugs on the metabolism of hormones and binding proteins; and (4) secondary endocrine complications of antiepileptic drug related weight changes or changes of insulin sensitivity. Regular monitoring of reproductive function at visits is recommended, including questioning about menstrual disorders, fertility, weight, hirsutism, and galactorrhoea. Particular attention should be paid to patients on valproate and obese patients or those experiencing significant weight gain. Single abnormal laboratory or imaging findings without symptoms may not constitute a clinically relevant endocrine disorder. However, patients with these kinds of abnormalities should be monitored to detect the possible development of a symptomatic disorder associated with, for example, menstrual disorders or fertility problems. CONCLUSIONS: If a reproductive endocrine disorder is found, antiepileptic drug treatment should be reviewed to ensure that it is correct for the particular seizure type and that it is not contributing to the endocrine problem. The possible benefits of a change in treatment must be balanced against seizure control and the cumulative side effect of alternative agents.

Anticonvulsants↗

Standard magnetic resonance imaging is inadequate for patients with refractory focal epilepsy.

OBJECTIVES: Patients with intractable epilepsy may benefit from epilepsy surgery especially if they have a radiologically demonstrable cerebral lesion. Dedicated magnetic resonance imaging (MRI) protocols as performed at epilepsy surgery centres can detect epileptogenic abnormalities with great sensitivity and specificity. However, many patients with epilepsy are investigated with standard MRI sequences by radiologist outside epilepsy centres ("non-experts"). This study was undertaken to compare standard MRI and epilepsy specific MRI findings in patients with focal epilepsy. METHODS: Comparison of results of standard MRI reported by "non-expert" radiologists, standard MRI evaluated by epilepsy "expert" radiologists, and epilepsy specific MRI read by "expert" radiologists in 123 consecutive patients undergoing epilepsy surgery evaluation between 1996 and 1999. Validation of radiological findings by correlation with postoperative histological examination. RESULTS: Sensitivity of "non-expert" reports of standard MRI reports for focal lesions was 39%, of "expert" reports of standard MRI 50%, and of epilepsy dedicated MRI 91%. Dedicated MRI showed focal lesions in 85% of patients with "non-lesional" standard MRI. The technical quality of standard MRI improved during the study period, but "non-expert" reporting did not. In particular, hippocampal sclerosis was missed in 86% of cases. Neuropathological diagnoses (n=90) were predicted correctly in 22% of "non-expert" standard MRI reports but by 89% of dedicated MRI reports. CONCLUSIONS: Standard MRI failed to detect 57% of focal epileptogenic lesions. Patients without MRI lesion are less likely to be considered candidates for epilepsy surgery. Patients with refractory epilepsy should be referred to an MRI unit with epileptological experience at an early point.

Brain↗

Language mapping in less than 15 minutes: real-time functional MRI during routine clinical investigation.

Neurosurgical interventions often require the presurgical determination of language dominance or mapping of language areas. Results obtained by fMRI are closely correlated with invasive procedures such as electrical stimulation mapping or the intracarotid amobarbital test. However, language fMRI is not used routinely, because postprocessing is time-consuming. We utilized a real-time analysis software installed directly on the MR console computer and SPM99 as reference postprocessing software. We assessed the reliability of the immediate determination of language dominance based on individual activation maps by comparing the results of the visual analysis of images derived from conventional postprocessing with those produced by the real-time tool. All images were rated independently by six senior neurologists blinded to other data. We validated the robustness of the real-time method statistically by comparing global and regional lateralization indices derived from real-time and postprocessing analysis. Functional MRI was performed with a standard 1.5-T whole-body scanner. Brain activity was contrasted between an alternating semantic judgment and letter matching task. Twelve right-handed, healthy control subjects and 12 consecutive patients with drug-resistant, localization-related epilepsy were investigated. The semantic condition induced almost invariably left hemispheric activations in Broca's area, the premotor cortex, the dorsolateral prefrontal cortex, and the temporoparietal region. Although real-time analysis reduced noise less effectively than SPM99, visual ratings and lateralization indices produced highly concordant results with both methods. In conclusion, real-time fMRI, as used here, allowed reliable language lateralization and mapping in less than 15 min during routine clinical MRI investigation with no need for postprocessing.

Adult↗

The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies.

The clinical and laboratory phenotype of a paraproteinaemic neuropathy syndrome termed chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies is described in a series of 18 cases. Previous single case reports have outlined some features of this syndrome. All 18 cases were defined by the presence of serum IgM antibodies which react principally with NeuAc (alpha2-8)NeuAc(alpha2-3)Gal-configured disialosyl epitopes common to many gangliosides including GDlb, GD3, GTlb and GQlb. In 17 out of 18 cases, the serum contained benign IgM paraproteins, and in four of these cases at least two IgM paraproteins were present. The IgM antibodies were also cold agglutinins in 50% of cases. The clinical picture comprised a chronic neuropathy with marked sensory ataxia and areflexia, and with relatively preserved motor function in the limbs. In addition, 16 out of 18 cases had motor weakness affecting oculomotor and bulbar muscles as fixed or as relapsing-remitting features. When present in their entirety, these clinical features have been described previously under the acronym CANOMAD: chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies. This distribution of clinical features is reminiscent of Miller Fisher syndrome, in which acute-phase anti-disialylated ganglioside IgG antibodies are found. Clinical electrophysiology and nerve biopsy show both demyelinating and axonal features. A partial response to intravenous immunoglobulin and other treatments is reported in some cases.

Adult↗