Background and purpose: Diagnosis of brain death requires confirmation of the clinical diagnosis by appropriate tests, generally electroencephalography (EEG) and angiography. The diagnostic ...limitations or logistical problems inherent to these tests indicate the need to develop other more appropriate methods. The results obtained with transcranial Doppler (TCD) led us to conduct this prospective study of TCD recordings in brain dead patients. Methods: 130 patients, aged 2–88 years were diagnosed as brain dead between July 1987 and June 1993. Clinical criteria were confirmed in all cases by EEG (n=88) and or angiography (n=64). Intracranial anterior circulation was insonated via temporal windows or, when impossible, via a transorbital approach. The posterior circulation was studied only in more recent patients. Examinations were made as soon as possible after brain death diagnosis and repeated for about 30 min. Vital parameters and treatments were taken into account. Results: There was only one false negative result, in a patient with an extended skull defect, who retained TCD and angiographic intracranial circulation despite confirmed irreversible brain death. All other patients displayed typical ultrasonic patterns of cerebral circulation arrest: an oscillating signal (n=190, 73%), a systolic spike (n=62, 24%) or a unilateral absence of signal (n=5). Despite a total correlation for positive diagnosis, TCD and angiography may differ as to the level of circulation arrest. TCD is useful for patients under sedative drugs. No false positive result was encountered but we were unable to insonate any intracranial artery in 5 patients. Conclusion: Data from previous studies and the results of this study indicate that TCD is a very sensitive and safe method for diagnosing cerebral circulatory arrest. TCD may be used as a confirmatory test alongside EEG and angiography. TCD is more widely applicable than EEG and may be earlier and safer than angiography.
Objective
The currently published works regarding the multiple sclerosis (MS) natural history report data were collected most often on population of patients recruited in MS centers. The aim was to ...compare the natural history of a population of patients followed in a MS centre (MSC) with patients followed outside a MS centre (NMSC).
Methods
Cases were identified through the LORSEP cohort, a network of neurologists (private ambulatory practice, hospitals, and MS centers) in France.
Results
A total of 3602 patients had been analyzed: 1036 MSC patients and 2566 NMSC patients. No difference was observed regarding gender and initial symptoms. Conversely, MSC patients were younger at MS onset and were more likely to have a primary progressive initial form. Median times (years) to the EDSS scores of 3, 4, and 6 were 5.8 (5.0–6.8), 8.4 (7.9–9.0), 16.0 (14.8–18.1) in the MSC group, respectively, whereas corresponding times were 8.4 (7.9–9.0), 12.3 (11.4–13.4), 19.1 (18.0–20.2) in the NMSC group. These differences according to the type of MS supervision were statistically significant for EDSS3 (P < 0.0001), EDSS4 (P < 0.0001), and EDSS6 (P = 0.01), respectively. These findings were confirmed in Cox multivariate models.
Conclusions
The patients followed in a MS centre had earlier disability than patients managed otherwise. Analyses exclusively conducted in patients with MS supervised in specialized centers may falsely misestimate the times needed to reach major disability landmarks. Before using registries to study the natural history of MS, efforts should be performed to verify in how far data are exhaustive and to understand the local health care system.
Lafora disease and Unverricht-Lundborg disease are two forms of progressive myoclonus epilepsies (PME). Recently the gene for Unverricht-Lundborg disease (EPM1) was mapped to chromosome 21q22.3. ...Using three highly polymorphic DNA markers (D21S212, PFKL, and D21S171) which flank the EPM1 locus, we performed linkage analysis to investigate whether or not the EPM1 gene is also implicated in Lafora disease. Linkage was excluded in three North-African pedigrees each comprising at least two affected individuals. This result suggests that differential diagnosis of Lafora disease and Unverricht-Lundborg disease may be facilitated by molecular genetic analysis.
The authors show that therapeutic education of the epileptic patient and his proxies is necessary but not yet organized for drug-resistant epilepsy; the objectives of this education are detailed ...after looking at the current status and the needs. The authors insist on the necessity of a global consideration of the patient: subjective and organic as well, taking into account the psychological and social dimensions and the experiences of the patient. The need of a specific formation for the physician and other health workers is emphasized, awaiting a status for therapeutic education. The authors propose a project of therapeutic education of the patient suffering from drug-resistant epilepsy.
