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 Video 1 Complex movement disorders in early onset hypoparathyroidism. Online content including video sequences viewable at: https://www.thieme-connect.com/products/ejournals/html/10.1055/s-0044-1788778. ). Brain magnetic resonance imaging (Figure 1) and brain CT (Figure 2) revealed extensive bilateral calcification.
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.
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.11 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...
,22 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...
Notably, stereotypic-like movements were only previously described by Galvez-Jimenez et al.11 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...
Central nervous system calcifications are present in up to 74% of patients with hypoparathyroidism.33 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...
Despite its prevalence, there is no clear relationship between the location and extent of the calcifications and the clinical phenotype.33 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...
References
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1Gálvez-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 -
2Manyam 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 -
3Mannstadt 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
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Publication in this collection
23 Sept 2024 -
Date of issue
2024
History
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Received
24 May 2024 -
Accepted
29 June 2024