Gowda Vykuntaraju K, Srinivasan Varunvenkat M, Muthane Yasha, Narayanappa Gayathri
Department of Pediatric Neurology, Indira Gandhi institute of Child Health, Bangalore, Karnataka, India.
Department of Neuropathology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India.
J Pediatr Neurosci. 2020 Jul-Sep;15(3):279-282. doi: 10.4103/jpn.JPN_141_19. Epub 2020 Nov 6.
Macrophagic myofasciitis is a rare inflammatory myopathy characterized by peri-fascicular macrophage infiltration without muscle necrosis. Here we report two children presented in the early infancy. Case 1: a 5-month-old girl presented with lack of neck control and floppiness. On examination, generalized hypotonia, absent deep tendon reflexes, and motor power of 2/5 (Medical Research Council grade) were observed. Case 2: a 17-day-old boy presented with poor feeding, tachypnea, and floppiness. On examination, decreased tone in all limbs and power of <2/5 in all limbs with absent reflexes were observed. Routine investigations including serum Creatine phosphokinase of both babies were normal. Muscle biopsy showed features of macrophagic myofasciitis in both infants. Any floppy infant of lower motor neuron type macrophagic myofasciitis should be considered in addition to inherited causes.
巨噬细胞性肌炎是一种罕见的炎症性肌病，其特征为束周巨噬细胞浸润而无肌肉坏死。在此我们报告两例在婴儿早期发病的患儿。病例1：一名5个月大的女孩，表现为颈部控制能力差和身体松软。检查发现，全身肌张力减退、深腱反射消失，肌力为2/5（医学研究委员会分级）。病例2：一名17天大的男孩，表现为喂养困难、呼吸急促和身体松软。检查发现，四肢肌张力降低，四肢肌力均<2/5，反射消失。两名婴儿的常规检查包括血清肌酸磷酸激酶均正常。肌肉活检显示两名婴儿均有巨噬细胞性肌炎的特征。除遗传性病因外，对于任何下运动神经元型巨噬细胞性肌炎导致的身体松软婴儿都应予以考虑。
