J Clin Neurol.  2007 Mar;3(1):57-61. 10.3988/jcn.2007.3.1.57.

Striopallidodentate Calcification and Progressive Supranuclear Palsy-Like Phenotype in a Patient with Idiopathic Hypoparathyroidism

Affiliations
  • 1Department of Neurology, The Catholic University of Korea, Seoul, Korea. neuronet@catholic.ac.kr

Abstract

We present a 77-year-old woman with levodopa-nonresponsive parkinsonism, dementia, and supranuclear gaze palsy on vertical and horizontal gaze. Laboratory findings were consistent with idiopathic hypoparathyroidism, and brain computed tomography showed extensive bilateral calcifications of the basal ganglia, centrum semiovale, dentate nuclei, and cerebellar white matter. These results illustrate that striopallidodentate calcification due to hypoparathyroidism may present with symptoms mimicking progressive supranuclear palsy.

Keyword

Striopallidodentate calcification; Idiopathic hypoparathyroidism; Progressive supranuclear palsy

MeSH Terms

Aged
Basal Ganglia
Brain
Dementia
Female
Humans
Hypoparathyroidism*
Paralysis
Parkinsonian Disorders
Phenotype*
Supranuclear Palsy, Progressive

Figure

  • Figure 1 Computed tomography (CT) of the brain demonstrated extensive, bilateral calcification of the basal ganglia, centrum semiovale, and bilateral dentate nuclei of the cerebellum (A). Magnetic resonance imaging revealed mixed high- and low-intensity signals compatible with the distribution of calcification on CT. The red nucleus and substantia nigra appeared normal (B).


Reference

1. Scotti G, Scialfa G, Tampieri D, Landoni L. MR imaging in Fahr disease. J Comput Assist Tomogr. 1985. 9:790–792.
Article
2. Manyam BV, Bhatt MH, Moore WD, Devleschoward AB, Anderson DR, Calne DB. Bilateral striopallidodentate calcinosis: cerebrospinal fluid, imaging, and electrophysiological studies. Ann Neurol. 1992. 31:379–384.
Article
3. Kobari M, Nogawa S, Sugimoto Y, Fukuuchi Y. Familial idiopathic brain calcification with autosomal dominant inheritance. Neurology. 1997. 48:645–649.
Article
4. Geschwind DH, Loginov M, Stern JM. Identification of a locus on chromosome 14q for idiopathic basal ganglia calcification (Fahr disease). Am J Human Genet. 1999. 65:764–772.
Article
5. Kim SH, Yoo BG, Kim KS, Yoo KM, Joh YD. Familial idiopathic striopallidodentate calcifications. J Korean Neurol Assoc. 2003. 21:191–194.
6. Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry. Mov Disord. 2001. 16:258–264.
Article
7. Trautner RJ, Cummings JL, Read SL, Benson DF. Idiopathic basal ganglia calcification and organic mood disorder. Am J Psychiatry. 1988. 145:350–353.
Article
8. Lopez-Villegas D, Kulisevsky J, Deus J, Junque C, Pujol J, Guardia E, et al. Neuropsychological alterations in patients with computed tomography-detected basal ganglia calcification. Arch Neurol. 1996. 53:251–256.
Article
9. Benke T, Karner E, Seppi K, Delazer M, Marksteiner J, Donnemiller E. Subacute dementia and imaging correlates in a case of Fahr's disease. J Neurol Neurosurg Psychiatry. 2004. 75:1163–1165.
Article
10. Saver JL, Liu GT, Charness ME. Idiopathic striopallidodentate calcification with prominent supranuclear abnormality of eye movement. J Neuroophthalmol. 1994. 14:29–33.
Article
11. Galvez-Jimenez 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. 15:1273–1276.
Article
12. Calabrese VP, Hadfield MG. Parkinsonism and extraocular motor abnormalities with unusual neuropathological findings. Mov Disord. 1991. 6:257–260.
Article
13. Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996. 47:1–9.
Article
14. Lowe J, Lennox G, Leigh PN. Graham DI, Lantos PL, editors. Disorders of movement disorders and system degenerations. Green-field's Neuropathology. 1997. Vol II:6th edn. London: Arnold;281–366.
15. Bronsky D, Kushner DS, Dubin A, Snapper I. Idiopathic hypoparathyroidism and pseudohypoparathyroidism: case reports and review of the literature. Medicine. 1958. 37:317–352.
Article
16. Nath U, Ben-Shlomo Y, Thomson RG, Morris HR, Wood NW, Lees AJ, et al. The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK. Brain. 2001. 124:1438–1449.
Article
17. Vaamonde J, Legarda I, Jimenez-Jimenez J, Zubieta JL, Obeso JA. Levodopa-responsive parkinsonism associated with basal ganglia calcification and primary hypoparathyroidism. Mov Disord. 1993. 8:398–400.
Article
18. Hempel A, Henze M, Berghoff C, Garcia N, Ody R, Schroder J. PET findings and neuropsychological deficits in a case of Fahr's disease. Psychiatry Res. 2001. 108:133–140.
Article
19. Hikosaka O, Takikawa Y, Kawagoe R. Role of the basal ganglia in the control of purposive saccadic eye movements. Physiol Rev. 2000. 80:953–978.
Article
Full Text Links
  • JCN
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr