• Title/Summary/Keyword: Charcot-Marie-Tooth disease

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Combined First Metatarsal and Calcaneal Osteotomy for Fixed Cavovarus Deformity of The Foot (내반 요족 변형에서 시행한 제1중족골 및 종골에 대한 절골술)

  • Chu, In-Tak;Park, Jong-Min;Yoo, Jong-Min;Chung, Jin-Wha
    • Journal of Korean Foot and Ankle Society
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    • v.14 no.2
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    • pp.130-134
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    • 2010
  • Purpose: The aim of this study was to evaluate the result of combined first metatarsal and calcaneal osteotomy for static cavovarus deformity of the foot. Materials and Methods: We performed a dorsal closing wedge $1^{st}$ metatarsal osteotomy and a lateral and upward displacement calcaneal osteotomy for 9 patients, 12 feet (6 male and 3 female). The mean age at the time of operation was 37 years and the mean followup period was 27 months. The causes of deformity were 2 poliomyelitis, 1 cerebral palsy, 1 Charcot-Marie-Tooth disease and 5 idiopathic type. Five lateral ligament reconstructions of the ankle and six percutaneous Achilles tendon lengthenings were added. The surgical results in terms of pain, function and alignment of the foot were evaluated by means of AOFAS ankle-hindfoot score and talo-$1^{st}$ metatarsal, calcaneus-$1^{st}$ metatarsal and calcaneal pitch angles were checked with weight bearing radiographs in lateral projection. Results: Talo-$1^{st}$ metatarsal and calcaneal pitch angles were reduced from the mean preoperative values of $21^{\circ}$ and $25^{\circ}$ to $12^{\circ}$ and $19^{\circ}$, respectively, at last followup. Also, calcaneus-$1^{st}$ metatarsal angle was increased from the mean $114^{\circ}$ to $114^{\circ}$. The mean AOFAS score was improved from 44.5 points preoperatively to 89.2 points at followup. There were 1 metatarso-cueiform joint nonunion, 1 sural nerve injury and 3 remaining symptomatic claw toes. Conclusion: Combined first metatarsal and calcaneal osteotomy appears to be an effective procedure for the treatment of adult static cavovarus foot.

Quantitative Analysis of Electrophysiological Characteristics of CIDP and CMT Type 1: Sensory Nerve Research (CIDP와 CMT 1형의 전기생리학적 특성에 대한 정량 분석: 감각신경연구)

  • Kang, Ji-Hyuk
    • Korean Journal of Clinical Laboratory Science
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    • v.53 no.2
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    • pp.151-157
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    • 2021
  • Charcot-Marie-Tooth disease (CMT) is a slowly progressive hereditary degenerative disease and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits. The purpose of this study was to analyze and compare the electrophysiological characteristics observed in sensory nerve conduction studies (SNCS) of both diseases. A retrospective study of 65 patients with a diagnosis of CIDP (N=35) and CMT type I (N=30) was performed. This study analyzed No potentials ratio, distal compound nerve action potential (dCNAP) of various nerve types, and a correlation coefficient analysis of the sensory nerve conduction velocity (SNCV). As a result, I found that CMT 1 was more severe systemic demyelinating and axonal polyneuropathy better than CIDP (P<0.05). In a quantitative analysis of dCNAP and SNCV, especially sural nerve was the most severe nerve injury observed in both diseases. In correlation and scatter plot analysis, CMT 1 showed relatively high correlations compared to CIDP based on the correlation coefficient analysis (Fisher's Z test) of SNCV. The results of this study suggested that CMT 1 showed the slowness in SNCV, one of the characteristics of demyelinating polyneuropathy, and this slowing had a uniform pattern. In conclusion, electrophysiological characteristic of SNCS may be useful in the diagnosis and research between patients with CMT 1 and CIDP.

Analysis of Clinical and Radiographic Outcome of the Reconstructive Surgery for the Cavovarus Foot Deformity (요내반족 변형에 대한 재건수술의 임상적 및 방사선학적 결과 분석)

  • Jung, Hong-Geun;Park, Jae-Yong;Lee, Dong-Oh;Eom, Joon-Sang;Chung, Seung-Hee
    • Journal of Korean Foot and Ankle Society
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    • v.18 no.2
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    • pp.62-67
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    • 2014
  • Purpose: Reconstructive surgeries for equinocavovarus foot deformities are quite variable, including hind-midfoot osteotomy or arthrodesis, soft tissue procedure, tendon transfers, etc. Comprehensive evaluation of the deformity and its etiology is mandatory for achievement of successful deformity correction. Few studies in this field have been reported. We report on the clinical and radiographic outcome of reconstruction for cavovarus foot deformities. Materials and Methods: The study is based on 16 feet with cavovarus foot deformities that underwent bony and soft tissue reconstructive surgery from 2004 to 2008. We evaluated the etiologies, varieties of surgical procedures performed, pain score, functional scores, and patient satisfaction and measured the radiographic parameters. Results: The average age at the time of surgery was 39.4 years old, with a male/female ratio of 9/4 and an average follow-up period of 23.9 months (range, 12~49 months). The etiologies of the cavovarus deformity were idiopathic 7 feet, residual poliomyelitis 5 feet, Charcot-Marie-Tooth disease 2 feet, and Guillain-Barre syndrome and hemiplegia due to cerebrovascular accident sequela 1 foot each. Lateral sliding calcaneal osteotomies were performed in 12 feet (75%), followed by Achilles tendon lengthening and plantar fascia release in 11 feet (69%), and first metatarsal dorsiflexion osteotomy/arthrodesis and tendon transfer in 10 feet (63%). Visual analogue scale pain score showed improvement, from an average of 4.2 to 0.5 points. American Orthopaedic Foot and Ankle Society ankle-hindfoot score showed significant improvement, from 47.8 to 90.0 points (p<0.05). All patients were satisfied. Ankle range of motion improved from $27.5^{\circ}$ to $46.7^{\circ}$. In radiographic measurements, calcaneal pitch angle improved from $19.1^{\circ}$ to $15.8^{\circ}$, Meary angle from $13.0^{\circ}$ to $9.3^{\circ}$, Hibb's angle from $44.3^{\circ}$ to $37.0^{\circ}$, and tibio-calcaneal axis angle from varus $17.5^{\circ}$ to varus $1.5^{\circ}$ Conclusion: We achieved successful correction of cavovarus foot deformities by performing appropriate comprehensive reconstructive procedures with improved functional, radiographic measures and high patient satisfaction.