Sallì Marcello, D'Arienzo Antonio, Bonanno Mariella, Morello Salvatore, Sanfilippo Antonino, Mauro Giulia Letizia, D'Arienzo Michele
Physical Medicine and Rehabilitation Department, University of Palermo, Palermo, Italy.
Orthopaedics and Traumatology Department, University of Palermo, Palermo, Italy.
Clin Cases Miner Bone Metab. 2016 Sep-Dec;13(3):265-267. doi: 10.11138/ccmbm/2016.13.3.265. Epub 2017 Feb 10.
Fahr's syndrome, also known as "Bilateral Striopallidodentate Calcinosis" (BSPDC) primitive, is a rare neurological disease characterized by the presence of idiopathic, bilateral, symmetrical and abnormal deposition of calcium in areas of the brain that control movements including the basal ganglia, dentate nuclei of the cerebellum, nuclei of thalamus and semi-oval center. We describe a case of a 76-year-old male patient underwent reduction and fixation of a subtrochanteric fracture with intramedullary nail. During post-operative rehabilitation therapists's patient management was difficult due to obvious extrapyramidal symptoms characterized by dysarthria, rigidity, bradykinesia, postural instability. A CT scan, performed for the onset of stiffness and confusion before the operation, showed: IV ventricle eumorphic and in axis; expansion in atrophic sense of supratentorial ventricular system; bilateral, diffuse and coarse calcifications of the basal ganglia in the cerebellar and occipital cortex, elements compatible with Fahr's syndrome. The patient presented repeated postural instability episodes in the upright position, with loss balance tendency and recurrent falls. Fahr's syndrome patient is a "weak" patient, which requires a multi-disciplinary approach in order to prevent the mobility reduction, to improve the condition of postural instability, thus reducing the risk of fractures using preventive measures in domestic environment.
Fahr综合征，也称为原发性“双侧纹状体苍白球齿状核钙化症”（BSPDC），是一种罕见的神经系统疾病，其特征是在控制运动的脑区，包括基底神经节、小脑齿状核、丘脑核和半卵圆中心，出现特发性、双侧、对称且异常的钙沉积。我们描述了一例76岁男性患者，该患者接受了股骨转子下骨折的髓内钉复位固定术。术后康复期间，治疗师对患者的管理很困难，因为患者出现了明显的锥体外系症状，表现为构音障碍、僵硬、运动迟缓、姿势不稳。术前因出现僵硬和意识模糊而进行的CT扫描显示：第四脑室形态正常且位于正中线上；幕上脑室系统呈萎缩性扩张；小脑和枕叶皮质的基底神经节出现双侧、弥漫性和粗大钙化，这些表现符合Fahr综合征。该患者在直立位时反复出现姿势不稳发作，有失去平衡的倾向且反复跌倒。Fahr综合征患者是“脆弱”患者，需要多学科方法来防止活动能力下降，改善姿势不稳状况，从而通过在家庭环境中采取预防措施降低骨折风险。
