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Latest developments in clinical neuroscience

Gérard Said
European Neurological Society

The many different fields of neurology have made significant progress recently. For example, advances in thrombolytic therapy have occurred alongside those in sleep disorders and new treatments of Parkinson’s and Alzheimer’s diseases. Coverage of these issues were made available at the European Neurological Society (ENS) meeting in Lausanne in May 2006. The 700 high-quality scientific papers selected for platform or poster presentation during the meeting, out of the 950 papers submitted, reflect the intense activity of clinical research among academic centres in Europe.

Neuroimaging is another growth area. Developing noninvasive methods of investigation that can be repeated without any inconvenience have major consequences for patient management. Magnetic resonance imaging (MRI) and diffusion-weighted imaging have revolutionised stroke imaging by allowing morphologic brain changes to be shown only one hour after the acute event. This is especially helpful in depicting small acute ischaemic lesions and in separating them from old cerebral damage. New techniques can serve to extend the time window of successful acute thrombolysis and for guiding more complex treatment decisions.

Work on the genetics of dementia have shown that fronto-temporal lobe dementia was both pathologically and genetically heterogeneous. Key papers on genetic aspects of progressive supranuclear palsy, Parkinson’s disease and Wilson’s disease were also presented at the meeting. Progress has been made also in the reliability of biochemical screening tests for dementia and mild cognitive impairment.

Cerebrovascular disorders, epilepsy and narcolepsy
In the field of cerebrovascular disorders, a large German study showed that telemedicine with telephone consultation can reliably link rural areas to stroke centres and improve the quality of care of stroke patients. Progress has also been made by a Swiss team in understanding the relationship between humour and cataplexy in narcoleptic patients thanks to PET scan studies. A multinational group reported on an in-vivo PET study of the mutant a4b2 nicotinic receptors in patients with autosomal dominant nocturnal frontal lobe epilepsy. They showed that the regional decrease of nAChR density in prefrontal cortex is concordant with a focal epilepsy.

Multiple sclerosis
Much is also going on in multiple sclerosis (MS). For example, a large epidemiologic Italian study found that patients with progressive MS from onset develop the disease at a later age and are more often males. Also, MRI allows reliable measurement of cord atrophy, which seems to be a clinically relevant outcome measure in experimental treatment trials in primary progressive MS. The morphologic range and significance of idiopathic inflammatory demyelinating lesions as compared to MS using MRI have not yet been systematically explored. For this purpose a multinational team identified four types of demyelinating lesions that will be useful for classification  and comparison of idiopathic demyelination with MS. The new drug, natalizumab, which had been briefly introduced in the US and withdrawn after development of progressive multifocal leukoencephalothy in a few patients treated for MS, is about to be introduced in Europe. A symposium has been devoted to this promising drug, effects and prevention of side-effects.

Peripheral nerve disorders
Peripheral nerve disorders are traditionally strongly represented at ENS meetings. A novel mutation of the myelin protein zero gene causing late-onset axonal Charcot-Marie-Tooth disease was reported by an Italian team. Papers from a French team reported significant progress in the management and understanding of familial amyloid polyneuropathy. One study stressed the role of transthyretin released by choroid plexus in amyloid deposits after liver transplantation – the only treatment currently available.  Phenotypic variations of familial amyloid polyneuropathy and misdiagnosis of patients were underlined and an international group reported on the different expression of the same mutation in large groups of patients from France, Portugal, Sweden and Brazil.

The forum for clinical neuroscience
The wealth of new data presented at the ENS meeting this year confirms it as the main forum for clinical neuroscience in Europe. The ENS is committed to continuous medical education and high-level clinical neuroscience; free registration and housing have been provided to 350 neurologists in training from all over Europe and will be for at least for the next two years. Also, in order to promote exchanges between European departments of neurology, stipends are available to young neurologists who wish to undertake a research project in a foreign department. Internationally, we have started a joint membership with the American Academy of Neurology and plan to hold joint teaching courses in 2007.