[Proceedings: Intrasellar cholesteatoma: pre- and post-operative investigation (author's transl)].
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Biomedical subjects
Publications and source records attributed to R Khalil.
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The authors report 31 cases of "vascular epilepsy" among 280 cerebral strokes confirmed by cranial computerized tomography. A high incidence of ischemia (28 cases : 90%) is noted. Epileptic seizures are initial (14 cases) or sequellar (17 cases) manifestations of cerebral stroke. Partial seizures are the most frequent (58%), particularly "Jacksonian" motor fits, which, when initial, often lead to status epilepticus. Frequency and bad prognosis of initial status epilepticus are pointed out.
The authors report two computed tomographic observations of Fahr's disease without disturbances in calcium-phosphorus metabolism; the first case is a by chance discovery and the second one is characterized by a cerebellar tremor. From the literature data they discuss successively clinical, radiological and computed tomographic, neuropathological, aetiopathogenetic and therapeutic aspects actually presented by strio-pallido-dentate calcifications (SPD) or Fahr's disease.
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The authors report clinical and scanographic findings in 26 cases of capsulo-thalamic hemorrhage. They stress the frequency of arterial hypertension as a pre-disposing factor. The clinical picture is represented by the association of disorders of consciousness (usually obnubilation), contralateral flaccid hemiplegia with hemianesthesia, oculomotor disorders (disturbance of vertical gaze, miosis) and disorders of linguistic and motor behaviour. The paraclinical examination of choice is scanography which enables the site, volume and extent of the hemorrhage to be estimated, together with the mass effect and intraventricular rupture. Clinical and scanographic follow-up indicates a favourable prognosis for capsulo-thalamic hemorrhage (77% survival). Follow-up findings point to several unfavourable prognostic factors: immediate coma, persistent and massive flaccid hemiplegia, hemorrhage size exceeding 3 cm, and severe intraventricular rupture. In the light of the data in the literature, the authors discuss the clinical and scanographic signs of capsulo-thalamic hemorrhage.
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A 49 year-old women with no medical history suddenly presented bilateral striato-capsular infarct causing frontal-like behavioural disturbances associating inertia with loss of drive, interest and affect, and preservation of intellectual function ("athymhormic syndrome" or "loss of psychic self-activation"). Ischaemic lesions mainly affected right globus pallidus and left lentiform nucleus with no involvement of the caput of the nuclei caudati. Such changes were close to anoxic lesions known to cause the same symptomatology. Infarction was attributed to a Moyamoya disease on angiographic data. Bilateral involvement of basal ganglia, arterial borderzones or both, may explain the frequent occurrence of neuropsychological disturbances in Moyamoya disease.
Primary progressive aphasia is defined as a gradually appeared and gradually worsening disorder of speech without any major alteration of the other cognitive functions. It is regarded by some authors as a syndrome which may be due to various degenerative diseases of the cerebral cortex (notably Alzheimer's disease, owing to its frequency), while others see in it an autonomous disease related to a neuropathological process that is distinct from the main degenerative dementias. The principal clinical particularity of primary progressive aphasia is that it spares the patient's autonomy for a long time, but ultimately turns into global dementia. Despite the diversity of aphasic aspects accompanied or not, in neuroimaging, by morphological and metabolic asymmetry to the expense of the left hemisphere, a review of the autopsy cases published shows that the vast majority corresponded to a neuropathological pattern devoid of the characteristic features of Alzheimer's disease and nearer to Pick's disease. In practice, the diagnosis of Alzheimer's disease can be excluded in all patients whose clinical presentation and cerebral neuroimaging results are compatible with primary progressive aphasia. Moreover, the fact that this new clinicoanatomical entity has been individualized constitutes a definite step towards a better comprehension of degenerative dementias.
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