Scola Rosana Herminia, Lin Kátia, Iwamoto Fãbio Massaiti, Arruda Walter Oleschko, Werneck Lineu Cesar
Divisions of Neurology and Neuromuscular Disorders, Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paran Brazil, Curitiba PR, Brazil.
Arq Neuropsiquiatr. 2003 Sep;61(3A):687-90. doi: 10.1590/s0004-282x2003000400031. Epub 2003 Sep 16.
Ankylosing spondylitis (AS) is an inflammatory disorder of unknown cause that primarily affects the axial skeleton. Neurological manifestations of AS are usually related to spinal deformities. Previous studies of the paraspinal muscles of AS patients showed muscle fiber atrophy, and core fibers. On the other hand, central core disease (CCD) is a genetic condition that primarily involves the skeletal muscles, but can present articular deformities secondary to muscular weakness. We report the case of a 45-year-old man with clinical and radiological diagnosis of AS and proximal muscular weakness in the lower limbs. Needle electromyography showed myopathic features and nerve conduction study was normal. Muscle biopsy disclosed almost complete predominance of type-1 fibers, and fibers with central cores. This is the first report of AS and CCD. Whether central core myopathy is coincidental or a new association with AS is discussed.
强直性脊柱炎（AS）是一种病因不明的炎症性疾病，主要影响中轴骨骼。AS的神经学表现通常与脊柱畸形有关。先前对AS患者椎旁肌的研究显示肌纤维萎缩以及核心纤维。另一方面，中央核心病（CCD）是一种主要累及骨骼肌的遗传性疾病，但可因肌肉无力而出现关节畸形。我们报告一例45岁男性患者，临床及影像学诊断为AS，伴有下肢近端肌肉无力。针极肌电图显示肌病特征，神经传导研究正常。肌肉活检显示几乎完全以1型纤维为主，以及有中央核心的纤维。这是AS与CCD并存的首例报告。文中讨论了中央核心肌病是巧合还是与AS的一种新关联。
