Calcificações dos gânglios da base na infância

Abstract

We report the study of four children with bilateral basal ganglia calcifications (BGC) visualized on CT scan. Epilepsy was the clinical manifestation of three patients whose laboratory investigation revealed abnormal calcium metabolism. The first aim of this paper is to call attention to a treatable entity that can cause epileptic syndromes in infancy and childhood. The second purpose is to review the literature comparing with our fourth child who presented encephalopathy with BGC.

Computerized tomography findings in Fahr's syndrome

“…This kind of abnormality is related with many etiologies that can be classified as inflammatory (CMV infection, neurocysticercosis, toxoplasmosis, neurobrucellosis, tuberculosis, HIV infection), tumoral (astrocytomas), hypoxic and vascular (arteriovenous malformations calcified infarct, ischemic encephalophaty), endocrine (hypoparathyroidsm, pseuso and pseudohypoparathyroidism, hyperparathyroidism), toxic (CO and Pb intoxication, hypervitaminosis D, radiotherapy), metabolic and degenerative (senility, mithocondrial encephalopaties, leukodistrophic diseases, idiopathic familial, motor neuron disease, myotonic muscular dystrophy, carbonic anidrase deficit, biopterin deficit) and other (malabsorption, Down syndrome, lupus, tuberous sclerosis, arthrogriposis) [8][9][10][11][12][13] .…”

Fahr’s Disease Presenting with Manic Symptoms

“…3,7 Fahr's disease, however, needs to be distinguished from Fahr's syndrome in which basal ganglia calcification is secondary to other disorders such as hypoparathyroidism. 8,9 Basal ganglia calcification can also be seen in other conditions like endocrinopathies, systemic diseases, infections, primary and secondary calcified brain tumours and various other diseases.…”

Fahr's disease: a rare neurological presentation in a tropical setting

“…This will help differentiate idiopathic Fahr's disease (unremarkable laboratory test results) from secondary cases especially due to hypoparathyroidism. Some authors have used the term Fahr's syndrome to describe the basal ganglia calcification, which is secondary to some other disorders such as hypoparathyroidism 11 , 12 .…”