Extrapyramidal symptoms and advanced calcification of the basal ganglia in a patient with autosomal dominant hypocalcemia

Intern Med. 2013;52(18):2077-81. doi: 10.2169/internalmedicine.52.8375.

Abstract

Most cases of hypoparathyroidism with decreased parathyroid hormone (PTH) secretion, excluding secondary hypoparathyroidism, are considered to be idiopathic. We herein report a relatively rare case of hypoparathyroidism with extrapyramidal symptoms, including brachybasia and a frozen gait, caused by advanced basal ganglia calcification in a 64-year-old man with hypoparathyroidism. A DNA (deoxyribonucleic acid) analysis of blood samples obtained from the patient and his eldest daughter revealed autosomal dominant hypocalcemia (ADH) with mutations in the calcium-sensing receptor (CaSR) gene. In cases of chronic hypoparathyroidism, calcification of the basal ganglia is observed if the patient is not treated for a long period. However, extrapyramidal symptoms as a complication of hypoparathyroidism are relatively rare.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Basal Ganglia Diseases / etiology
  • Basal Ganglia Diseases / pathology*
  • Basal Ganglia Diseases / physiopathology
  • Base Sequence
  • Calcinosis / etiology
  • Calcinosis / pathology*
  • Calcinosis / physiopathology
  • DNA Mutational Analysis
  • Female
  • Genes, Dominant
  • Heterozygote
  • Humans
  • Hypercalciuria / complications
  • Hypercalciuria / genetics*
  • Hypercalciuria / physiopathology*
  • Hypocalcemia / complications
  • Hypocalcemia / genetics*
  • Hypocalcemia / physiopathology*
  • Hypoparathyroidism / complications
  • Hypoparathyroidism / congenital*
  • Hypoparathyroidism / genetics
  • Hypoparathyroidism / physiopathology
  • Male
  • Middle Aged
  • Mutation
  • Receptors, Calcium-Sensing / genetics
  • Tomography, X-Ray Computed

Substances

  • CASR protein, human
  • Receptors, Calcium-Sensing

Supplementary concepts

  • Hypercalciuric Hypocalcemia, Familial