Objectives-Functional cerebral imaging PET and SPECT have shown hypometabolism and hypoperfusion in the area of vascular malformation in children with epilepsy due to Sturge-Weber syndrome. However, data are scarce in infants and do not exist in patients with Sturge-Weber disease without epilepsy. The pattern of perfusion during the first two years of life was studied including patients before the onset of seizures. Methods-Twenty two infants with later confirmed Sturge-Weber disease underwent SPECT examination using TOMO-MATIC 564 (Medimatic) and xenon-133 at ages ranging from 8 days to 25 months. Twelve had never had seizures before SPECT and seven underwent a second SPECT a mean seven months later. Cerebral blood flow (CBF) was measured in the whole hemisphere and in the part of the cortex involved in the vascular malformation on both sides as well as a "pathological to normal" index for the hemisphere and vascular malformation. These values were compared with normal age paired values. Results-Compared with controls, CBF and the indices in the hemisphere and vascular malformation were significantly decreased in patients who already had had seizures before SPECT, whereas they were significantly increased in 75% of the patients who had never had any seizures. On second SPECT, the indices were decreased in all patients, including the four who still remained non-epileptic. Conclusions-SPECT therefore detects CBF asymmetry in infants with SturgeWeber disease, which tends to shift with age. The cortex involved in the vascular malformation is hyperperfused during the first year of life before first seizures. The classic hypoperfusion appears after one year of age, even in non-epileptic patients.

Aim  We observed a dramatic response to the ketogenic diet in several patients with highly refractory epilepsy whose seizure frequency had recently worsened. This study aimed to identify whether this characteristic was a useful indication for the ketogenic diet. Method  From the 70 patients who received the ketogenic diet during a 3‐year period at our institution, we retrospectively selected patients with focal epilepsy. There were 22 children, 13 females and nine males, aged from 5 months to 18 years 6 months (mean 6y 9mo, SD 5y 11mo). Fifteen had symptomatic and seven had cryptogenic focal epilepsy. Seizure frequency 1 week before initiating the ketogenic diet was compared with that at 1 month and at the last visit on the diet. Results  Eleven patients were responders (defined as reduction of seizures by more than 50%) at 1 month. Responders were higher (p=0.046) in the group with a recent worsening of seizures than in those with stable seizure frequency. Seven patients were still seizure‐free at 6 months on the diet. Tolerability was excellent in 10 patients. Five patients stopped the diet because of early side effects. Interpretation  The ketogenic diet may be a valuable therapeutic option for children with pharmacoresistant focal epilepsy, particularly those with a recent deterioration of seizure control and neurological status. Because of its rapid effect, the ketogenic diet may be a useful support to intravenous emergency drugs in such a situation.

The prognosis of Sturge-Weber syndrome (SWS) is partly related to early occurrence of seizures but the diagnosis of this phakomatosis may be difficult during the 1st year of life. We have performed a retrospective study of seven patients with confirmed SWS (age 7 days to 3 months). None of the patients was asymptomatic at the time of the study. They all underwent MRI (T1 and T2 sequences) and single photon emission computed tomography (SPECT) at the same time. Regional cerebral blood flow was measured using xenon-133. In all cases, myelination appeared to be accelerated in the areas underlying the leptomeningeal angioma on both MRI sequences. In five cases, SPECT showed hyperperfusion in the damaged hemisphere. In one case, the SPECT was symmetrical and in another it showed hypoperfusion in the damaged hemisphere which was already atrophied. These data suggest that the accelerated myelination is not related to ischemia but to transient hyperperfusion. This MRI pattern can be helpful for the early diagnosis of SWS, which is of utmost importance for preventive antiepileptic treatment.

S ince Rio 92, the struggle against the erosion of biodiversity has been a part of many environmental policies, even though the ways to pursue this goal are not agreed on by all parties. There are many controversies in both northern and southern countries regarding nature areas to be protected and the participation of 'traditional' or local populations in natural resource management. The purpose of the Habitats directive, based explicitly on scientific knowledge, is to preserve biological diversity on a European scale through the conservation of natural habitats and wild fauna and flora. It also advocates that the choice of sites for conservation should take into consideration economic, political and local social constraints. It differs from the standard environmental protection policy approaches in the principles that it espouses -maintaining biodiversity and sustainable development -which played a significant role in revitalizing the decision-making process. These new referentials affect the way in which environmental issues are viewed and add complexity to the way of dealing with them. The transfer of the directive to France (see Alphandery and Fortier 2001) and its local applications initially favoured the scientific dimension at the expense of social issues, thereby provoking resistance, demands, and innovations. The challenge to the site identification process from spokesmen for the rural milieu leads us first of all to ask questions regarding the scientific basis of this nature policy. As this basis is uncertain, incomplete and controversial, these challenges continue. We then consider the text of the directive as it was published in the official journal to highlight the foundations, the theoretically innovative aspects, but also its limitations and contradictions. Our hypothesis is that biodiversity, as conceived in the directive and the proposed framework for action, contains many contradictions which account for the outbursts in political and scientific circles, which its application caused in France. From Rio 92 to Brussels 92: the foundations of the directive A new referentialDuring its writing, the Habitats directive was marked by the scientific, economic and political context of the last twenty years, which can be summarized in the following manner: criticism of the paradigm of development and spreading of the