Striatopallidodentate calcinosis, hypoparathyroidism and neurological features: A case series study

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

Référence2851
Année2013
TypeArticle
Lien document
Lien externehttp://www.em-consulte.com/article/816436/alertePM
Disciplines associéesCentre Hospitalier Universitaire Ibn Rochd Casablanca
AuteurH. El Otmani
Auteurs associésLahlou I, Raji L, S. Omari, Belmansour Y, Moutaouakil F, Boulaajaj FZ, Mouden M, Gam I, Hakim K, El Moutawakil B, Rafai MA, Fadel H, Slassi I
DisciplineNeurologie
RevueRev Neurol
Référence Revue169(6-7):495-501.