Dados do Trabalho


Título

Fahr's syndrome: a case report

RESUMO

Case presentation: M. M. V, male, 76 years old, presented symptoms consistent with parkinsonian syndrome and convulsive symptoms associated with fever for two years. Later he progressed with worsening of motor symptoms, presenting chorea and neuropsychiatric alterations such as irritability, excessive anxiety, and mental confusion. One year ago, after a seizure event, the patient presented decreased level of consciousness, was admitted to the hospital, and requested a CT scan. It revealed calcifications affecting the basal ganglia, dentate nuclei of the cerebellum, and white matter bilaterally, suggestive of Fahr's disease. Laboratory tests were requested and demonstrated hypocalcemia, vitamin B12 deficiency, and low PTH levels. The patient started his therapy during hospitalization with supplementation of calcitriol, calcium carbonate, and cholecalciferol. After two months of hospitalization, clinical treatment was supplemented with quetiapine, phenytoin, levodopa, biperiden, and zolpidem, demonstrating significant improvement in neuropsychiatric and motor symptoms.
Discussion: Fahr's disease is a rare neurodegenerative disorder characterized by idiopathic, symmetrical, and bilateral calcification of the basal ganglia. Symptoms include movement disorders, with parkinsonian syndrome being the most common, in addition to seizures, severe headache, and neuropsychiatric changes. Treatment aims to control symptoms and improve quality of life.
Final comments: The literature is still outdated when it comes to how to manage these patients, and studies are needed to control the symptoms and improve the patient's quality of life since it is an irreversible condition and there is no definitive treatment according to the guidelines.

Palavras Chave

Fahr's syndrome; neuropsychiatry; neurodegenerative disease

Área

Neurologia Cognitiva E Do Envelhecimento

Autores

YGOR ROBERTO RAMOS GONÇALVES SOARES, EMANUELLY NOGUEIRA GOMES DE ARRUDA, MARIA EDUARDA DE OLIVEIRA FERNANDES, GLORIA JEANE DE OLIVEIRA FERNANDES