Biomedical subjects
Publications and source records attributed to M Fohlen.
Rhombencephalosynapsis is an unfrequent malformation of the posterior fossa essentially characterized by vermian agenesis or hypogenesis, fusion of the cerebellar hemispheres and fusion of the dentate nuclei. Supratentorial abnormalities are usually associated. No specific clinical syndrome can be described in relation with this disorder. We report a case diagnosed by MRI in a living patient.
BACKGROUND: Hot water epilepsy belongs to the group of reflex epilepsies. Seizures are provoked by hot water, due to the association of both cutaneous and heat stimuli. Described mainly in India and Japan, it seems to be rare in Europe where it occurs in young children. CASE REPORTS: Five infants aged between 6 months to 2 years had seizures during bathing with activity arrest, hypotonia and vasoactive modification. Sometimes clonic movements could be observed. The diagnosis was confirmed by EEG recorded during bath in the fives cases, with video for two of them. The course of the seizures and of the psychomotor development were favorable. CONCLUSION: Hot water epilepsy is a benign epilepsy. Its incidence could be underestimated because seizures can be confused with febrile convulsions or vagal fits.
BACKGROUND: The classification of cerebral cortical dysplasia is difficult and there are histological similarities between focal cortical dysplasia (FCD) and hemimegalencephaly. Objectives. To correlate the MR features and histological data of cortical dysplasias. MATERIALS AND METHODS: The MR appearances of 17 brains were examined. According to the signal intensity within the pathological area on T2-weighted (T2-W) sequences we selected two groups. RESULTS: Group 1 comprised ten patients with high signal in the dysplastic area on T2-W images. This group included five hemimegalencephalies, three frontal quadramegalencephalies, and one gyral dysplasia. The pathological hemisphere was reduced in size in one case. The cortex was thickened in all cases on T1-weighted (T1-W) images. There was loss of delineation between white matter (WM) and grey matter (GM) in all cases on both T1-W and T2-W sequences. The differential diagnosis with tumour, neoplastic-like malformation or polymicrogyria was questionable. Group 2 comprised seven patients presenting without increased signal within the dysplastic area on T2-W images. WM and GM were of similar signal intensity in six cases, and delineation between white and grey matter was absent in all cases. There were mild abnormalities on T1-W sequences in all cases. The dysplasias were limited to a lobe in five cases and a gyrus in two cases. In all cases, depiction of the malformation was a greater diagnostic problem than the differential diagnosis. CONCLUSIONS: A constant MR sign in our series was the loss of delineation between WM and GM in the dysplastic area. This correlated well with the observed histological disorganisation. Markedly high signal within the dysplastic area seems to be related to myelin abnormalities rather than glial cell abnormalities.
Corpus callosotmy was introduced in 1940 as a palliative treatment for generalized epilepsies. The improvement of the surgical technique, and the simplification of the initial "total commissurotomy" made that procedure proposed in order to decrease the frequency and the severity of the seizures occurring in the secondary geralzed epilepsies. However the indication criteria remain unclear, due to the difficulty for analysing the results and the feterogenity of the series. A careful selection requiring a comprehensive epilepsy team remains mandatory despite the relative simplicity of the procedure.
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