[Pallido-dentate calcifications (apropos of 7 anatomo-clinical case reports)].
"Fahr's Disease" is characterized by bilateral and symmetrical calcifications of the Globus Pallidus (systematically extending to the Commisura Anterior and the Capsula Interna, and less commonly to the Putamen, the Centrum Semi-Ovale and the Cerebral Cortex), and of the Cerebellar Nucleus Dentalus (with spreading to the White Matter and the Cortical Lamellae). Lesions or absence of Parathyroids are frequently related, with subsequent metabolic disorders of Phosphorus and Calcium, but idiopathic cases without hypoparathyroid disturbances are also found. A Morgagni-Morel Hyperostosis Frontalis Interna is often associated with "Fahr's Disease", and there could be a relationship between these two affections. We found in three cases the association between "Fahr's Disease" and Morel's Nodular Dysgenesis of the Frontal Cortex. Most of the cases are sporadic, but observations with a clear familial incidence are also found. Clinically, various Neurological Disorders (cerebellar, extrapyramidal, pyramidal, dysarthria, epileptic seizures) are often but not always observed; the Psychiatric Disorders found in some cases could be fortuitious associations (psychoses), connected to hypothyroidism (oligophrenia), and in aged patients, to unrelated cerebral vascular or degenerative lesions; very seldom, a dementing state could be connected to the spreading of calcifications to the Cerebral Cortex.
D000368:Aged; D002114:Calcinosis / Q000473:pathology*; D002529:Cerebellar Nuclei / Q000473:pathology*; D002561:Cerebrovascular Disorders / Q000473:pathology*; D005260:Female; D005917:Globus Pallidus / Q000473:pathology*; D006801:Humans; D006934:Hypercalcemia / Q000473:pathology; D006957:Hyperostosis Frontalis Interna / Q000473:pathology; D007037:Hypothyroidism / Q000473:pathology; D008297:Male; D010280:Parathyroid Glands / Q000002:abnormalities
Contribution à l'étude des calcifications pallido-dentelées (à propos de sept observations anatomo-cliniques).