RESUMO
Abstract Hypoparathyroidism (HP) is a rare metabolic disorder and causes hypocalcemia because parathyroid hormone secretion is inadequate to mobilize calcium from bone and reabsorb calcium from kidney and gut. Anterior neck surgery is the most common cause of acquired HP and autoimmune HP is the next most common form in adults. The duration, severity, and rate of development of hypocalcemia determine the clinical presentation. A variety of organs can be affected by calcification, more frequently kidneys, but also joints, eyes, skin, vasculature, and other organ systems and, although rarely seen, intracerebral calcifications. We report four cases of bilateral basal ganglia calcifications (BGC) also known as Fahr's syndrome related to hypoparathyroidism. Fahr's syndrome is characterized by bilateral symmetrical calcification of areas of the brain that control movements including basal ganglia, thalamus, and others; it is a rare inherited or sporadic neurological disorder with a prevalence of less than 1/1.000.000. Main symptoms related to bilateral BGC include extra-pyramidal and cerebellar disorders, cognitive impairment, epileptic seizures, and psychiatric changes. BGC has been established as a possible outcome of HP. Its prevalence, demonstrated in the HP cohorts, varied significantly from 12 up to 74%. Currently, computed tomography (CT) is the most valuable method for diagnosis. The treatment include symptomatic support and identification of causes, but there is no specific treatment limiting the progression of calcification in the basal ganglia. Especially in HP, an early treatment can prevent calcification and neurophysiological disorders.
Resumo O hipoparatireoidismo (HP) é um distúrbio metabólico raro e causa hipocalcemia, pois a secreção do paratormônio é inadequada para mobilizar o cálcio dos ossos e reabsorver o cálcio dos rins e intestino. A cirurgia cervical anterior é a causa mais comum de HP adquirido. O HP autoimune é a segunda causa mais comum em adultos. A duração, gravidade e taxa de desenvolvimento da hipocalcemia determinam a apresentação clínica. Diversos órgãos podem ser afetados pela calcificação, mais frequentemente os rins, mas também articulações, olhos, pele, vasculatura e outros órgãos e, embora raramente vista, calcificações intracerebrais. Relatamos quatro casos de calcificações dos gânglios da base (CGB) bilaterais, também conhecidas como síndrome de Fahr relacionadas ao hipoparatireoidismo. A síndrome de Fahr é caracterizada pela calcificação bilateral simétrica de áreas do cérebro que controlam os movimentos incluindo os gânglios da base, tálamo e outros; é um distúrbio neurológico raro, hereditário ou esporádico, com uma prevalência 1/1.000.000. Os principais sintomas relacionados à CGB bilateral incluem distúrbios extrapiramidais e cerebelares, comprometimento cognitivo, convulsões e alterações psiquiátricas. A CGB foi estabelecida como uma possível consequência do HP. Sua prevalência, demonstrada nas coortes de HP, variou significativamente de 12 a 74%. Atualmente, a tomografia computadorizada (TC) é o método mais valioso para o diagnóstico. O tratamento inclui suporte sintomático e identificação de causas, mas não há tratamento específico limitando a progressão da calcificação nos gânglios da base. Especialmente no HP, um tratamento precoce pode prevenir calcificações e distúrbios neurofisiológicos.
RESUMO
Hypoparathyroidism (HP) is a rare metabolic disorder and causes hypocalcemia because parathyroid hormone secretion is inadequate to mobilize calcium from bone and reabsorb calcium from kidney and gut. Anterior neck surgery is the most common cause of acquired HP and autoimmune HP is the next most common form in adults. The duration, severity, and rate of development of hypocalcemia determine the clinical presentation. A variety of organs can be affected by calcification, more frequently kidneys, but also joints, eyes, skin, vasculature, and other organ systems and, although rarely seen, intracerebral calcifications. We report four cases of bilateral basal ganglia calcifications (BGC) also known as Fahr's syndrome related to hypoparathyroidism. Fahr's syndrome is characterized by bilateral symmetrical calcification of areas of the brain that control movements including basal ganglia, thalamus, and others; it is a rare inherited or sporadic neurological disorder with a prevalence of less than 1/1.000.000. Main symptoms related to bilateral BGC include extra-pyramidal and cerebellar disorders, cognitive impairment, epileptic seizures, and psychiatric changes. BGC has been established as a possible outcome of HP. Its prevalence, demonstrated in the HP cohorts, varied significantly from 12 up to 74%. Currently, computed tomography (CT) is the most valuable method for diagnosis. The treatment include symptomatic support and identification of causes, but there is no specific treatment limiting the progression of calcification in the basal ganglia. Especially in HP, an early treatment can prevent calcification and neurophysiological disorders.