G Model
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Images in rheumatology
Ataxic dysarthria revealing Fahr’s syndrome and hypoparathyroidism Hanen Loukil , Faten Frikha ∗ , Mouna Snoussi , Raida Ben Salah , Sahar El Aoud , Zouhir Bahloul Department of Internal Medicine, Hedi Chaker Hospital, 3029 Sfax, Tunisia
Fig. 1.
A 39-year-old man was admitted with the complaint of physical and psychological asthenia, dysarthria and ataxia. Computed tomography (CT) scan of the brain revealed bilateral and symmetrical intracerebral calcifications of the central gray nuclei, the dentate nuclei of cerebellum and the periventricular white matter (Fig. 1). Laboratory tests revealed hypocalcemia at 1.55 mmol/L and hyperphosphataemia at 2.1 mmol/L. Serum intact parathyroid hormone level was 5 pg/mL (normal range 15–56 pg/mL).The diagnosis of Fahr’s syndrome revealing hypoparathyroidism was made. Subsequently, a treatment with calcium and vitamin D was started. Fahr’s syndrome is an uncommon clinicopathological entity characterized by symmetrical and bilateral intracerebral calcifications, located in the basal ganglia [1]. Hypoparathyroidism is most commonly associated with Fahr’s syndrome. This includes idiopathic or post-surgical hypoparathyroidism, and explains the frequency of phosphocalcic metabolism disorder. Hypocalcemia may explain most clinical features: cataract, neuromuscular
excitability, neurological signs and various neuropsychiatric symptoms. . . Computed tomography is the preferable method of localizing and assessing the intracerebral calcifications [2]. The outcome of this syndrome is favorable and the treatment of phosphocalcic abnormalities leads to improvement of clinical involvement. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Morgane L, Trimarchi F, Benvenga S. Fahr’s disease. Lancet 2002;359: 759. [2] Rafai MA, Oumari S, Lytim S, et al. Le syndrome de Fahr : aspects cliniques, radiologiques et étiologiques. Feuillets de Radiologie 2014;54:2–8.
∗ Corresponding author. Tel.: +216 98 657 098. E-mail address: [email protected] (F. Frikha). http://dx.doi.org/10.1016/j.jbspin.2014.06.009 1297-319X/© 2014 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Loukil H, et al. Ataxic dysarthria revealing Fahr’s syndrome and hypoparathyroidism. Joint Bone Spine (2014), http://dx.doi.org/10.1016/j.jbspin.2014.06.009
ARTICLE IN PRESS
BONSOI-4058; No. of Pages 1
Joint Bone Spine xxx (2014) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Images in rheumatology
Ataxic dysarthria revealing Fahr’s syndrome and hypoparathyroidism Hanen Loukil , Faten Frikha ∗ , Mouna Snoussi , Raida Ben Salah , Sahar El Aoud , Zouhir Bahloul Department of Internal Medicine, Hedi Chaker Hospital, 3029 Sfax, Tunisia
Fig. 1.
A 39-year-old man was admitted with the complaint of physical and psychological asthenia, dysarthria and ataxia. Computed tomography (CT) scan of the brain revealed bilateral and symmetrical intracerebral calcifications of the central gray nuclei, the dentate nuclei of cerebellum and the periventricular white matter (Fig. 1). Laboratory tests revealed hypocalcemia at 1.55 mmol/L and hyperphosphataemia at 2.1 mmol/L. Serum intact parathyroid hormone level was 5 pg/mL (normal range 15–56 pg/mL).The diagnosis of Fahr’s syndrome revealing hypoparathyroidism was made. Subsequently, a treatment with calcium and vitamin D was started. Fahr’s syndrome is an uncommon clinicopathological entity characterized by symmetrical and bilateral intracerebral calcifications, located in the basal ganglia [1]. Hypoparathyroidism is most commonly associated with Fahr’s syndrome. This includes idiopathic or post-surgical hypoparathyroidism, and explains the frequency of phosphocalcic metabolism disorder. Hypocalcemia may explain most clinical features: cataract, neuromuscular
excitability, neurological signs and various neuropsychiatric symptoms. . . Computed tomography is the preferable method of localizing and assessing the intracerebral calcifications [2]. The outcome of this syndrome is favorable and the treatment of phosphocalcic abnormalities leads to improvement of clinical involvement. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Morgane L, Trimarchi F, Benvenga S. Fahr’s disease. Lancet 2002;359: 759. [2] Rafai MA, Oumari S, Lytim S, et al. Le syndrome de Fahr : aspects cliniques, radiologiques et étiologiques. Feuillets de Radiologie 2014;54:2–8.
∗ Corresponding author. Tel.: +216 98 657 098. E-mail address: [email protected] (F. Frikha). http://dx.doi.org/10.1016/j.jbspin.2014.06.009 1297-319X/© 2014 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Loukil H, et al. Ataxic dysarthria revealing Fahr’s syndrome and hypoparathyroidism. Joint Bone Spine (2014), http://dx.doi.org/10.1016/j.jbspin.2014.06.009
