Tethered cord syndrome resembling Charcot-Marie-Tooth disease in closed spinal dysraphism.

نویسندگان

  • Paulo Victor Sgobbi de Souza
  • José Luiz Pedroso
  • Wladimir Bocca Vieira de Rezende Pinto
  • Orlando G P Barsottini
  • Acary Souza B Oliveira
چکیده

A 23-year-old man presented with progressive difficulty walking since childhood. Examination showed pes cavus, hammer toes, and peroneal atrophy (Figure 1). Electroneuromyography revealed chronic denervation with an axonal pattern, and Charcot-Marie-Tooth disease was suspected. Urinary retention had started 3 years previously. Spinal cord magnetic resonance imaging (MRI) showed a lipomeningomyelocele at the lumbosacral region with spinal cord tethering (Figure 2). The skin of the lumbosacral region was normal. Surgery was performed without immediate improvement. Tethered cord syndrome due to occult spinal cord dysraphism is a diverse clinical entity, and cutaneous manifestations may be absent. Pes cavus and hammer toes are rare but can be present and strongly resemble Charcot-MarieTooth disease1-3. The emergence of urinary symptoms is a hallmark for the diagnosis of cauda equina syndrome instead of neuropathy.

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عنوان ژورنال:
  • Arquivos de neuro-psiquiatria

دوره 72 2  شماره 

صفحات  -

تاریخ انتشار 2014