[Complete atrioventricular block during temporal lobe epilepsy. Apropos of a case]. / Bloc auriculo-ventriculaire complet au cours d'une épilepsie temporale. A propos d'un cas.

The authors report the case of a 37 years old woman with no previous medical history, admitted to hospital for investigation of unexplained syncope, sometimes associated with generalised fits. After standard non-invasive cardiovascular investigations, no diagnosis could be made. The tilt test induced a minor syncopal episode without reproducing the clinical symptoms. In view of the discordance between the induced and spontaneous symptoms, a neurological opinion was requested. During the recording of an electroencephalogram, syncopal atrioventricular block was observed, preceded by auditory hallucinations, reproducing exactly the clinical symptoms. Analysis of the sequence of events showed the conduction defect to arise after the onset of the epileptic fit, indicating a diagnosis of syncopal complete atrioventricular block complicating cryptogenic temporal epilepsy, and requiring specific treatment. This case illustrates the importance of close collaboration between cardiologists and neurologists in the management of cases of unexplained syncope.

[Bilateral 3rd cranial nerve palsy disclosing oligodendroglioma]. / Paralysie bilatérale du III révélatrice d'un oligodendrogliome.

The patient was a 30 year-old man. He had no previous history. For several months he experienced a slowly progressive horizontal diplopia which was the expression of a bilateral third cranial nerve palsy with an intact intrinsic component. Muscular or neuro-muscular pathology as myasthenia was initially suspected but not confirmed. CT scan and MRI revealed an atypical left temporo-insular lesion which led us to discuss a chronic inflammatory pathology as sarcoidosis or a tumoral process. Finally cerebral biopsy showed a high grade oligodendroglioma. Symptomatology was attributed to infiltration of the peduncles from this tumor. Such a case has never been seen before. Early neuroradiological explorations would be useful in case of clinical suspicion of cranial nerve palsy.

Relevance of the quantification of apolipoprotein E in the cerebrospinal fluid in Alzheimer's disease.

Apolipoprotein E (Apo E), one of the major structural and functional apolipoproteins, has recently been implicated in the pathogenesis of Alzheimer's disease (AD). Several studies revealed that Apo E4 isoform is associated with the pathogenic process in AD. A significant reduction of cerebrospinal fluid (CSF) Apo E level in AD patients has been reported in two studies. To further investigate the physiopathological significance of such a variation of Apo E concentration in the CSF, we performed a quantification of Apo E by an enzyme linked immunosorbent assay (ELISA). There were no significant differences in CSF Apo E level between AD cases and control subjects or patients suffering from other neurological diseases. Gender, age and Apo E phenotype explained none of CSF Apo E concentration variability.

White matter magnetic resonance imaging hyperintensity in Alzheimer's disease: correlations with corpus callosum atrophy.

We have previously demonstrated with MRI that as well as marked white matter involvement in late-onset Alzheimer's disease (AD), atrophy of the corpus callosum may also be present. This finding prompted us to study possible correlations between atrophy of the corpus callosum and white matter hyperintensity (WMH) and between white matter lesions and the severity of the disease. We compared the corpus callosum and white matter lesions on MRI from 15 AD patients and 15 controls. The white matter lesions were scored according to the Scheltens' rating scale. We found a significant reduction of the area of the corpus callosum and more severe white matter lesions in AD patients than in controls. Both atrophy of the corpus callosum and the severity of lesions depended mainly on the diagnosis of senile dementia of the Alzheimer type and on age but not on the diagnosis of presenile AD. We demonstrated a negative correlation between white matter lesions scores and areas of corpus callosum in AD patients and no correlation between the white matter lesions and the severity of the disease. We demonstrated that white matter lesions including WMH and atrophy of the corpus callosum are more frequent in AD than in controls. The predominance of white matter lesions in senile AD may be explained by the combination of aging and disease processes.

[Subcortical dementia of the Neumann type. Contribution of diagnostic imaging]. / Démence sous-corticale type Neumann. Apport de l'imagerie morphologique et fonctionnelle.

A 36 year-old patient presented with a dementia of frontal type, gait disturbances, incontinence and a pseudo-bulbar palsy, which caused death at age 40. Brain biopsy of the frontal lobe showed an extensive deep subcortical gliosis. A high level of GFAP was detected by immunoblotting in the biopsy. Clinical and neuropathological observations are similar to cases described as Neumann Progressive Subcortical Gliosis. Single Photon Emission Computer Tomography showed a bilateral frontotemporal hypoperfusion, and Magnetic Resonance Imaging large periventricular and subcortical hyperintensities in both hemispheres, the brainstem and the cerebellum. The hyperintensities on T2-weighted MR images might be related to the intense gliosis. The contribution of such imaging data to diagnosis must be confirmed by other clinico-pathological cases.