Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review

Abstract

Neuroarthropathy (neuropathic osteoarthropathy), also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot) neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis). We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI) of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine.

Cervical spondylotic myelopathy with syringomyelia presenting as hip Charcot neuroarthropathy: A case report and review of literature

“…However, syringomyelia-associated CN accounts for only approximately 5% of CN cases and easily causes upper limb CN[ 4 , 11 ], which most frequently involves the shoulder and elbow joints[ 3 , 12 ]. Only a few studies have reported that CN of the hip and knee joint is a rare complication of lumbar syringomyelia caused by epidural anesthesia[ 13 , 14 ].…”

Neuroartropatia de Charcot secundária à siringomielia com acometimento isolado de ombro: relato de caso

“…Acredita-se que a teoria neurovascular exerça o papel inicial na etiopatogenia, seguida da teoria neurotraumática que resulta em luxação, fratura e instabilidade (9,10). Dessa forma, é provável que uma combinação destes dois processos seja responsável pelo desenvolvimento da doença (11). A artropatia neuropática deve ser considerada em casos de inchaço articular inexplicável, desproporcional à dor, com uma amplitude limitada de movimentos ativos e passivos da articulação afetada (9).…”

“…A evolução da doença pode ser insidiosa com o paciente por anos assintomático ou com poucos sintomas. Entretanto, o padrão de progressão pode mudar rapidamente e gerar uma importante destruição articular, como no caso relatado (11). A RM da coluna vertebral é o padrão ouro para a identificação da siringe e, consequentemente, para o diagnóstico de siringomielia (8).…”

Treatment of syringomyelia associated with Chiari 1 malformation

“…Развитие пареза в нижних конечностях встречается при далекозашедших стадиях заболевания. Описаны [21] случаи нейропатического поражения суставов (суставы Шарко) у пациента с АК1 и протяженной СМ шейного и грудного отдела позвоночника.…”