Compte-rendu de congrès • Neuroméditerranée VIII
© 2007. Elsevier Masson SAS. Tous droits réservés
for familial epilepsy, which may elucidate the pathophysiology of the epilepsies in general.
● Epilepsy surgery in childhood J. Helen Cross Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust, London, UK.
Epilepsy surgery is not new; it has been documented for over one hundred years. With advances in neurosurgical and neuroanaesthetic techniques and relative low morbidity from planned neurosurgical procedures, earlier surgery has been advocated in children. However the majority of adults coming to an epilepsy surgery programme have had a history of seizures since childhood so why have they not been evaluated earlier? There is no doubt that the range of individual presenting for surgery in childhood remains wider than that in adulthood. Whilst temporal lobectomy is the most common procedure in adults, this procedure makes up 20-50p.cent of paediatric surgical series. Developmental tumours remain the commonest pathology, although hippocampal sclerosis makes up a significant number. In addition the clinical spectrum of HS may be wider than previously thought, with a high incidence of behaviour disorder amongst those children coming for surgical evaluation. In particular, as with developmental pathologies, there appears to be a subgroup who are within the autistic spectrum range in whom benefits are clear from surgical resection with or without complete seizure freedom. Hemispherectomy makes up a further one third of procedures performed. This is where the clinical spectrum remains the most wide, ranging from the early onset catastrophic syndromes seen with some developmental lesions to those with near normal cognition but a progressive pathology such as Rasmussens encephalitis. Whatever the underlying cause of the hemisyndrome associated with epilepsy, early evaluation is warranted to determine the best timing of surgery if appropriate. Extratemporal resections make up a further third of procedures, although suitable candidates may be more difficult to determine. Although detailed imaging techniques, both structural and functional, have allowed non invasive evaluation of an increasing number of children, invasive EEG recording may be required either where the exact limits of the epileptogenic area are not defined, or where a structural abnormality may be in close proximity to functional cortex. Functional procedures (corpus callosotomy, multiple subpial transection) are considered in a few highly selected cases with specific goals for outcome. The role of vagal nerve stimulation is still being evaluated. The outcome from surgery is most commonly reported with regard to seizure freedom although of course there are other considerations of particular relevance in childhood. Where a focal resection has been performed, the degree of epileptogenic tissue removed is likely to be a major determinant of seizure freedom although the degree to which
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this can be achieved is also related to the underlying pathology. There is some evidence that seizure outcome following surgery for developmental lesions may deteriorate with time, that is the likelihood of seizure freedom is less in the longer as opposed to the short term but that outcome with such lesions may be better with earlier surgery. The lesser likelihood of seizure control however does not preclude consideration, providing the aims of surgery are realistic and clearly identified preoperatively. Many children are also likely to achieve a substantial reduction in seizure frequency with a reduction in anticonvulsant requirement. Developmental and behavioural outcome have been reported as improved following surgery in many studies but have been difficult to quantify, particularly in the very young. As a consequence it is important to obtain as much information as possible about the nature of the epilepsy and the procedure planned prior to any surgery, with clear outcome aims clarified with the family.
● Monotherapy versus polytherapy of anti-epileptic drugs: why and when H.S. Hosny Prof of Neurology, Cairo University, Cairo Egypt.
Antiepileptic drug treatment of epilepsy to prevent or minimize recurrent epileptic seizures begins with the use of a single agent as monotherapy. The primary reason why two or more drugs are used together is the failure of monotherapy to control the seizures. Depending on the seizure type or syndrome, control may be complete or very poor. In adult onset seizures control varies between 35p.cent and 60 p.cent for partial seizures and 80p.cent for idiopathic generalized tonic-clonic seizures. When seizures continue despite increasing doses of AEDs to the maximum that can be tolerated, a second AED is usually added to the first in an effort to to achieve complete seizure control. The aim of this study is to assess the efficacy of AEDs in monotherapy in partial and generalized epilepsy and whether it is needed to reach the maximal tolerated dose in uncontrolled patients at a moderate dose or early switch to polytherapy. A total of 600 patients were classified (according to the ILAE) and evaluated, 225 who had an idiopathic generalized epilepsy and the rest with partial epilepsy. The results and recommendations will be discussed in the presentation.
● Management of affective disorders in epilepsy eylert E. Brodtkorb Senior Consultant, Professor, Department of Neurology and Clinical Neurophysiology St. Olav’s Hospital, Trondheim University Hospital, Norway.
Biological and psychosocial factors may interact in a complex way in affective and seizure disorders. This fact is important when considering management of mood dysfunction in patients with epilepsy. Psychological difficulties and
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social/vocational disability may be a greater problem than the seizures and may be related to the perceived social stigma associated with the diagnosis. Insufficient information about the disorder and inadequate professional support is common. Supportive cognitive therapy, specialist epilepsy nursing, and structured educational programmes are essential parts of an operative comprehensive epilepsy service. Intervention of this kind has demonstrated a beneficial effect on the quality of life in patients with active epilepsy. During recent years, it has been increasingly recognized that AEDs have différent pharmacodynamic properties and multiple applications. Some may have CNS side effects which may negatively influence affective disorders, whereas others have mood stabilizing or even thymoleptic or anxiolytic effects. It is important to be familiar with the various potential effects of the different treatment modalities, including vagal nerve stimulation. The therapy should be carefully tailored to the clinical profile of each patient. Both benefits and risks should be weighed when treating epileptic patients with antidepressants. Mutual pharmacokinetic and pharmacodynamic interactions should be considered. Compounds without known association with seizures should be preferred. SSRIs are usually safe and effective, but further research is needed to define optimal strategies. In mood disorders, attempts to analyze intrinsic neurobiological factors and the pharmacological profiles of prescribed AEDs, as well as individual psychosocial aspects should be performed. All these considerations have to be taken into account for the individual patient.
● Instructive cases from the neurology service S.I. Harik Professor and Chairman of Neurology, University of Arkansas for Medical Sciences, USA.
Several relatively difficult, yet instructive actual cases in clinical neurology were be presented. There are lessons to be learned from each case. Audience interaction is encouraged. The give and take that I hope will materialize will help focus on many important issues in clinical neurology that are illustrated by these cases. The subject matter of the cases include: stroke, central nervous system infections, brain tumors, IVIG treatment for Guillian-Barré Syndrome, and seizure disorders.
● Diagnosis of sensory neuropathy J.-M. Leger Reference Center for Neuromuscular Diseases, Bâtiment Babinski, Salpêtrière hospital, Paris.
A number of disorders of the Peripheral Nervous System (PNS) can present with purely or predominant sensory symptoms and signs, mainly a subacute or chronic sensory ataxia. They mainly encompass the so-called sensory neuronopathy (ganglionopathy), which is associated with the involvement of dorsal root ganglion (DRG) sensory neurons, but also distal axonal polyneuropathy in its predomi-
nantly sensory presentation (small fiber sensory polyneuropathy), and less often demyelinating polyneuropathy, which may present with purely or predominantly sensory features. The 2 first types of sensory neuropathy have a relatively clear-cut clinical and electrophysiological profile. In the last one, the diagnosis may be easy when electrodiagnostic studies show typical abnormalities of motor nerve conduction velocities (MNCV), but be very difficult when these abnormaliies are lacking. Sensory neuronopathies are frequently associated with dysimmune or neoplastic diseases, and with the use of toxic agents. Small fiber sensory polyneuropathies are classically found in association with diabetes mellitus, alcohol abuse and nutritional deficiency, HIV infection and amyloidosis. In a number of cases, however, no etiology is found despite repeated investigations, which leads to define the specific subgroup of chronic idiopathic axonal polyneuropathy (CIAP). Lastly, sensory demyelinating polyneuropathies are mainly those associated with an anti-MAG IgM monoclonal gammopathy, but may be chronic inflammatory demyelinating polyneuropathies (CIDP). This review will focuse on recent advances in defining the clinical features, pathophysiological basis and management of these different sensory neuropathies.
● Sensory ataxic neuropathy J. Pouget, J.P. Azulay, S. Attarian, A. Verschueren, D. Uzenot Department of Neuromuscular Diseases, La Timone Hospital, 13005 Marseille.
Sensory ataxic neuropathies are uncommon disorders characterised by a predominant loss of large myelinated fibers which impairs kinaesthesia and leads to sensory ataxia and loss of tendon reflexes. Lesions concern peripheral nerves or dorsal root ganglions or dorsal roots. Clinical presentation of proprioceptive deafferentation concerns mainly motor than sensory symptoms. Sensory ataxia of gait must be distinguished from cerebellar gait ataxia and from the unsteadiness caused by weakness. Sensory ganglionopathy usually includes sensory ataxia of upper and lower limbs, a painful dysesthetic syndrome, widespread impairment of cutaneous sensation and some autonomic involvement. Causes of sensory ataxic neuropathy are multiple. Toxic, metabolic, infectious, dysimmune and hereditary etiologies have been reported. Toxics concern vitamin B6, cis-platinum and taxol and are easily recognised. Metabolic causes are vitamin B12 and vitamin E deficiency. Sensory ataxia has been described in association with celiac disease. Interest has recently developed concerning dysimmune origin with several newly described clinico-biological syndromes. Among IgM paraproteonaemic neuropathies, anti-MAG neuropathy is the best defined and most frequent. It has distinctive clinical, electrophysiological and pathological features that may distinguish it from CIDP and other IgM-related neuropathy. Several therapies have been tried with most often no effect
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