Ｃｏｎｇｅｎｉｔａｌ　ｓｅｎｓｏｒｙ　ｎｅｕｒｏｐａｔｈｙの長期Ｘ線像の追跡

• 佐浦 隆一

  神戸大学整形外科

• 石川 斉

  神戸大学医療技術短期大学部

• 岡田 幸也

  六甲病院

書誌事項

タイトル別名

• Long-term follow-up of the radiological features of a case with congenital sensory neuropathy.

抄録

A case of congenital sensory neuropathy with developing neurotrophic bone and joint disease in the right foot is reported. The patient was a 17-year-old girl who, at the age of 4, had swelling of the soft tissue in the tarsal area of the right foot and insensitivity to pain of the hands and feet. Severe neurotrophic bone and joint disease of the tarsal bones developed in her right foot despite preventative therapy with bracing.<BR>The disease has been characterized in the literature as follows; (1) onset in infancy, (2) nonprogression, (3) normal intelligence, (4) absence of superficial sensory input, (5) muscular hypotonia in affected limbs, (6) absence of deep tendon reflexes, (7) normal automonic function and (8) decreased sensory nerve conduction velocity. Our patient showed most of these features.<BR>The differential diagnoses of the disease include congenital indifference to pain, congenital insensitivity to pain, hereditary radicular neuropathy and other peripheral neuropathy caused by organic involvement such as myelomeningocele, by metabolic disease and by infectious disease.<BR>Good management in this disease is based on proper appreciation of the disease and the prevention of complications. The nature and limitations of the disease must be explained to both patient, especially in childhood, and parents. Patients must, above all, learn to prevent the possibility of mechanical and thermal trauma. Early recognition of the disease is important to prevent or minimize joint instability and neuropathic bone and joint disease.

収録刊行物

• 日本リウマチ・関節外科学会雑誌

  日本リウマチ・関節外科学会雑誌 8 (1), 157-164, 1989

  日本関節病学会

キーワード

• congenital sensory neuropathy
• differential diagnosis
• radiographic features
• neuropathic joint

詳細情報

• CRID: 1390282679738307840
• NII論文ID: 130004003232
• DOI: 10.11551/jsjd1982.8.157
• ISSN: 18849059
• ISSN: 02873214
• 本文言語コード: ja
• データソース種別:
  • JaLC
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可