Epilepsy surgery has been established as a successful treatment in patients with drug-resistant focal epilepsies. Different standardized surgical approaches were developed for resective or functional treatment. Successful surgical treatment, however, necessitates a careful presurgical evaluation. Although there are international standards for non invasive and invasive presurgical procedures most centers differ in their diagnostic strategy. This article gives a survey of the Bonn Epilepsy surgery program within which more than 1300 patients have been treated. After the method of patient selection different we describe tools of non invasive and invasive evaluation. This article serves only as a description of current practice in one center with a relatively large volume of cases. It presents just one opinion of a specific group within a wide spectrum of possible algorithms for presurgical evaluation that have briefly been outlined for 33 centers in Engels book [4].
SUMMARY We report a case of congenital isolated malabsorption of folic acid, the first in a boy. Only seven previous cases have been reported, and we discuss two aspects-namely, the tendency to infection, with evidence of impairment of both cellular and humoral immunity, and the absence of neurological disturbances.Congenital isolated malabsorption of folate is a rare and specific disorder, causing megaloblastic anaemia in early childhood, which can be corrected by administering very high doses of folic acid orally or by parenteral administration of physiological doses. The case reported here is the first in a boy.Two additional aspects will be emphasised: our patient (a) showed no neurologic disturbances and (b) suffered from a tendency to infections apparently due to partial immunodeficiency. The most striking clinical disturbance was severe and chronic anaemia during the fifth and sixth months of life, with haemoglobin concentrations ranging from 4*5 to 9*2 g/dl and packed cell volume between 15% and 29% and with mean corpuscular volume of 100 u3. The peripheral blood smears showed macrocytosis and hypersegmentation of polymorphonuclear cells; the leucocyte count was in the range of 7000-12 000 mm3 and thrombocytes 100 000-200 000/mm3, with reticulocytes 0*2-1-0%. Coombs tests yielded negative results, and both a Motulsky test and electrophoresis of haemoglobin showed normal results. These data together with the macrocytosis found in the peripheral blood smear were compatible with a diagnosis of megaloblastic anaemia, which was confirmed by the findings of megaloblastic changes in the bone marrow.The megaloblastic anaemia was found to be due to folic acid deficiency, as indicated by the low concentrations of folic acid in red blood cells (15-50 ng/ml, while the normal value is above 250 ng/ml), serum (<1 ng/ml, while normal is above 5 ng/ml), and cerebrospinal fluid (<1 ng/ml, while normal is 15 ng/ml), Blood concentrations of vitamin B12 were 700-800 pg/ml and urine orotic acid concentration was normal too. The folates were determined by the microbiologic assay of Grossowicz et al,' using Lactobacillus casei as test organism. The diet of the mother during lactation and the child's diet were found to be normal; the possibility of dietary folate
Familial occurrence of membranous subaortic stenosis (MSS) is described in three families. The defect was found in 2 siblings in two of these families, and in 3 siblings of the third family. The importance of early diagnosis and treatment of MSS is emphasized. We suggest early evaluation of first-degree relatives of patients with MSS for the possibility of this defect.
Clinicians should apply different CRP cut-off values depending on whether they are trying to rule in or rule out bacterial infection, but also depending on fever duration at the time of CRP testing.
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