[Striatopallidodentate calcinosis, hypoparathyroidism and neurological features: a case series study].

Abstract

INTRODUCTION:

The respective roles of hypocalcemia and intracerebral calcifications in the occurrence of various neurological manifestations in hypoparathyroidism is not entirely clear. Nevertheless, therapeutic and prognostic implications are important.

OBJECTIVES:

We analyze the neurological clinical aspects observed in hypoparathyroidism and correlate them to the biological calcium abnormality and radiological CT scan findings. We also compare these results with data reported in the idiopathic form of striatopallidodentate calcinosis.

PATIENTS:

The neurological clinical, CT scan findings and outcome have been retrospectively studied in patients recruited during 13 years (2000-2012) for neurological features associated with hypoparathyroidism or pseudohypoparathyroidism.

RESULTS:

Twelve patients with primary hypoparathyroidism (n=5), secondary to thyroidectomy (n=4) and pseudohypoparathyroidism (n=3) were studied. The sex-ratio was 1 and mean age was 39 years. All patients had a tetany, 60% had epilepsy, associated in one patient with "benign" intracranial hypertension; 50% had behavioral changes. Response to calcium therapy was excellent for all these events. Moderate cognitive deficit was noted in three patients (25%), parkinsonism in two patients and hyperkinetic movement disorders in one other. These events were not responsive to calcium therapy and were more common in cases of extensive brain calcifications and in patients who had pseudohypoparathroidism.

COMMENTS:

This study suggests that, in patients with hypoparathyroidism, epilepsy and psychiatric disorders are induced by hypocalcemia and reversible after its correction. Cognitive and extrapyramidal impairment seem to be related to the progressive extension of intracerebral calcification, particularly in patients with a late diagnosis. In patients with pseudohypoparathyroidism, this finding is different because of the contribution of other factors, specific to this disease.

• Authors: H El Otmani, I Lahlou, L Raji, S Omari, Y Belmansour, F Moutaouakil, F-Z Boulaajaj, M Mouden, I Gam, K Hakim, B El Moutawakil, M-A Rafai, H Fadel, I Slassi
• Journal: Revue neurologique (Rev Neurol (Paris)) 2013 Jun-Jul Vol. 169 Issue 6-7 Pg. 495-501 ISSN: 0035-3787 [Print] France
• Vernacular Title: Calcinose striatopallidodentelée, hypoparathyroïdie et atteinte neurologique: étude de série.
• PMID: 23523016 (Publication Type: Journal Article)
• Copyright: Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Topics

• Adolescent
• Adult
• Aged
• Brain Diseases (diagnostic imaging, epidemiology, etiology)
• Calcinosis (diagnostic imaging, epidemiology, etiology)
• Cohort Studies
• Corpus Striatum (diagnostic imaging)
• Female
• Humans
• Hypoparathyroidism (complications, diagnostic imaging, epidemiology)
• Male
• Middle Aged
• Neuroimaging (methods)
• Retrospective Studies
• Tomography, X-Ray Computed
• Young Adult