Charcot spinal arthropathy in congenital insensitivity to pain.

Abstract

Neuropathic joints can arise secondary to multiple etiologies. Charcot, in 1868, 1 described neuropathic joints due to tabes dorsalis. Later, Morvan 2 noted a series of cases with neuropathic joints and compromised sensation. Most of these patients had syringomyelia. Jordan, in 1936, 3 first recognized the presence of neuropathic joints in diabetic patients. Diabetes mellitus is now recognized as the most common cause of neuropathic arthropathy. Estimates state that one of every 680 diabetic patients develops Charcot arthropathy at some site. 4 The incidence of spinal involvement has ranged from 6% to 21% in reported cases of Charcot arthropathy. 5,6 Mitchell, reporting in 1831, 7 noted a relationship between spinal cord lesions and arthropathies. In 1884, Kronig 8 reported the first case of neuropathic spinal arthropathy. The reported causes of neuropathic spinal arthropathy include tabes dorsalis, 5,9-14 syringomyelia, 15.16 spinal cord tumors, 16-18 traumatic spinal cord injury, 18-22 acute transverse myelitis, 17 and diabetes mellitus. 4,23,24 Congenital insensitivity to pain is a very rare cause of neuropathic arthropathy. In 1932, Dearborn 25 reported a case of congenital general pure analgesia. The report described a carnival worker calling himself the Human Pin Cushion. Since that time, various authors have proposed theories to explain the cause of this disorder. To date, however, no single anatomic, physiologic, or pathologic entity has been identified as the cause of congenital insensitivity to pain. 26-32 In 1953, Petrie 33 described the orthopedic manifestations of congenital insensitivity to pain. Piazza et al, in 1988, 34 reported a case of neuropathic spinal arthropathy in congenital insensitivity to pain. A 28-year-old woman with destruction at the L1-L2 interspace developed kyphosis and instability. She remained neurologically intact and was fused successfully with staged anterior and posterior procedures. 34 Heggeness 35 recently reported the case of a 17-year-old girl who developed truncal asymmetry during pregnancy. A dense paraparesis developed 10 days after delivery. She had developed a Charcot spine at L3 and L4 with significant canal compromise. She underwent anterior decompression and stabilization of the spine after which she made a complete neurologic recovery. Two other cases of spinal arthropathy with congenital insensitivity to pain have appeared in literature. 33,36 We report the case of a 12-year-old girl with congenital insensitivity to pain and a neuropathic spine who suffered a progressive neurologic compromise secondary to her L4-5 lesion.