Jornal de Neurologia Clínica e Neurocirurgia

Fahr’s Syndrome with Hypoparathyroidism Presenting with Acute Ischemic Stroke

Abstract

Krishnendu Choudhury*, Biswajit Paul, Koushik Ray, and Shibendu Ghosh

Fahr’s syndrome comprises of calcification in basal ganglia, thalamus and cerebellar dentate nucleus with or without neurodegenerative or psychiatric manifestations. It may be idiopathic or secondary to hypoparathyroidism. It can be genetically transmitted to families in an autosomal dominant manner, or can be sporadic. Common neurological manifestations of fahr’s syndrome are movement disorders including Parkinsonism, chorea, dystonia or spasticity. Dystonia and paratonia also manifest as abnormalities of muscle tone, but arise more due to the network dysfunction between the basal ganglia and the Cerebello-thalamo-cortical connections. Ischemic cerebrovascular disease may be associated, and incidence of ischemic or hemorrhagic stroke in fahr’s syndrome has been rarely reported.

We report here an elderly woman who had presented with acute ischemic stroke and found to have fahr’s syndrome in brain imaging. She was also detected to have primary hypoparathyroidism with decreased serum vitamin D3 and parathormone deficiency.