Idiopathic hypoparathyroidism presenting as chorea

Osteomalacia presenting as chorea.

“…Only four of the nine cases showed evidence of basal ganglia calcification; there is a poor correlation between this feature and the presence of neurological symptoms4 and the ganglia dysfunction probably reflected the metabolic consequences of hypocalcaemia.' In our patient chorea seemed to be related to the degree of hypocalcaemia in that abnormal movements were seen only when the serum calcium fell below [1][2][3][4][5][6] mmol/l (6-3 mg/100 ml).…”

Corticobasal degeneration syndrome with basal ganglia calcification: Fahr's disease as a corticobasal look‐alike?

“…The factors that influence the development of the clinical phenotype of basal ganglia calcification remain uncertain. 32 Proposed mechanisms of extrapyramidal dysfunction include altered vessel permeability to calcium, tissue ischaemia, 21,36 abnormal synaptic excitability and ephaptic transmission, 21,36 or local shifts in calcium concentration, possibly mediated by altered activity of the cerebral isoform of parathyroid hormoneresponsive adenyl cyclase. 31,37 Positron emission tomography evidence suggests that nigrostriatal dopaminergic pathways remain intact in familial striopallidodentate calcinosis.…”

Extrapyramidal Symptoms and Advanced Calcification of the Basal Ganglia in a Patient with Autosomal Dominant Hypocalcemia

“…In a report describing 17 hypoparathyroidism patients with extrapyramidal symptoms, McKinney (19) demonstrated that the most common extrapyramidal symptom was chorea, observed in six cases. The proposed causes of extrapyramidal symptoms associated with hypoparathyroidism are tissue ischemia, resulting from hyalinization and erosion caused by calcium deposits in the small vascular walls and dysfunction of the basal ganglia induced by enhanced excitability of the nerve fibers and synapses associated with hypocalcemia (19).…”

“…The proposed causes of extrapyramidal symptoms associated with hypoparathyroidism are tissue ischemia, resulting from hyalinization and erosion caused by calcium deposits in the small vascular walls and dysfunction of the basal ganglia induced by enhanced excitability of the nerve fibers and synapses associated with hypocalcemia (19). Because extrapyramidal symptoms are also noted in cases of idiopathic hypoparathyroidism without hypocalcemia, some scientists believe that the extrapyramidal symptoms observed in patients with hypoparathyroidism are caused by calcification of the basal ganglia.…”