Complex movement disorders in early onset hypoparathyroidism

Distúrbios do movimento complexos em hipoparatireoidismo de início precoce

A 23-year-old female patient with long-standing untreated idiopathic hypoparathyroidism presented with complex involuntary movements. Physical examination revealed generalized dystonia, painful spasms, and stereotypies (^{Video 1}). Brain magnetic resonance imaging (Figure 1) and brain CT (Figure 2) revealed extensive bilateral calcification.

Figure 1
Brain MRI: A/D - T1 hyperintense signal in the basal ganglia and dentate nuclei; B/E - T2 low to isointense signal in the basal ganglia and dentate nuclei; C/F - SWI hyposignal in the basal ganglia, dentate nuclei and subcortical white matter.

Figure 2
Brain CT scan disclosing bilateral and symmetrical calcification in the basal ganglia, thalamus, dentate nuclei, and subcortical white matter.

Hypoparathyroidism can present with a variety of neurological symptoms (Figure 3), including, though less commonly, movement disorders such as chorea, parkinsonism, dystonia, choreoathetosis, and paroxysmal dyskinesia.¹,² Notably, stereotypic-like movements were only previously described by Galvez-Jimenez et al.¹ Central nervous system calcifications are present in up to 74% of patients with hypoparathyroidism.³ Despite its prevalence, there is no clear relationship between the location and extent of the calcifications and the clinical phenotype.³

Figure 3
The figure demonstrates the broad spectrum of neurological manifestations associated with hypoparathyroidism. Additionally, it depicts ion changes in the blood.

References

• ¹
  Gálvez-Jiménez N, Hanson MR, Cabral J. Dopa-resistant parkinsonism, oculomotor disturbances, chorea, mirror movements, dyspraxia, and dementia: the expanding clinical spectrum of hypoparathyroidism. A case report. Mov Disord 2000;15(06): 1273–1276. Doi: 10.1002/1531-8257(200011)15:6<1273::aidmds1038>3.0.co;2-o
  » https://doi.org/10.1002/1531-8257(200011)15:6<1273::aid-mds1038>3.0.co;2-o
• ²
  Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry. Mov Disord 2001;16(02):258–264. Doi: 10.1002/mds.1049
  » https://doi.org/10.1002/mds.1049
• ³
  Mannstadt M, Bilezikian JP, Thakker RV, et al. Hypoparathyroidism. Nat Rev Dis Primers 2017;3:17080. Doi: 10.1038/nrdp.2017.55
  » https://doi.org/10.1038/nrdp.2017.55

Publication Dates

• Publication in this collection
  23 Sept 2024
• Date of issue
  2024

History

• Received
  24 May 2024
• Accepted
  29 June 2024