Alemdar Murat
Department of Neurology, Sakarya University Training and Reserach Hospital, Sakarya, Turkey.
Agri. 2018 Oct;30(4):206-208. doi: 10.5505/agri.2017.62582.
Fahr disease is an idiopathic disorder characterized with deposition of calcium and a few other minerals in basal ganglia, cerebellum and subcortical brain area. A 51 years old female with the complaints of pain, numbness, tingling and weakness in both upper extremities for six months was referred to our electromyography laboratory with a suspicion of carpal tunnel syndrome. She got the diagnosis of Fahr disease upon the investigations for the convulsions that she experienced ten years ago. Beside, she had a generalized anxiety disoder. Neurological examination revealed mild to moderate weakness in flexion and extension of forearm, and extension of hand on both sides. She described dysesthesia on C6 & C7 dermatomes, bilaterally. Symmetric calsifications on both cerebellar hemispheres and basala ganglia were present on cranial CT. Median and ulnar nerve conduction studies were normal on both sides. Concentric needle electromyography revealed chronic neurogenic changes on the morphology of motor unit potentials recorded from the muscles of C6 & C7, bilaterally. Cervical magnetic resonance imaging revealed discopathies on C4-5, C5-6 and C6-7 levels causing myelomalacia. Neuropathic pain, paresthesia or muscle weakness on upper extremities are rare in Fahr disease. Presented case got the diagnosis of cervical discopathies in late as those findings were supposed to be related with Fahr disease. Therefore, clinicians should be aware of common findings occured during the course of this disease, and consider the possible coincidental pathologies when the atypical neurological deficits are observed in these patients.
Fahr病是一种特发性疾病,其特征是钙和其他一些矿物质沉积于基底神经节、小脑和皮质下脑区。一名51岁女性,因双上肢疼痛、麻木、刺痛和无力6个月就诊于我们的肌电图实验室,怀疑患有腕管综合征。她在对10年前经历的惊厥进行检查后被诊断为Fahr病。此外,她还患有广泛性焦虑障碍。神经系统检查发现双侧前臂屈伸及手部伸展轻度至中度无力。她描述双侧C6和C7皮节感觉异常。头颅CT显示双侧小脑半球和基底神经节有对称性钙化。双侧正中神经和尺神经传导研究正常。同心针电极肌电图显示双侧从C6和C7肌肉记录的运动单位电位形态有慢性神经源性改变。颈椎磁共振成像显示C4-5、C5-6和C6-7水平存在椎间盘病变导致脊髓软化。在Fahr病中,上肢出现神经性疼痛、感觉异常或肌肉无力较为罕见。该病例因这些发现被认为与Fahr病有关而较晚才诊断出颈椎间盘病变。因此,临床医生应了解该病过程中出现的常见表现,并在这些患者出现非典型神经功能缺损时考虑可能同时存在的病理情况。