Abstract
To the Editor,
We appreciate the interest in our article detailing the diagnosis of the Wilson’s disease. In the first section of our article, we discussed the genetic and pathophysiology of copper (Cu) metabolic disorder that affects the organs and the manifestation at different sites through the human body. In our case, the diagnosis of Wilson’s disease was fulfilled with the initial presentation of jaundice and neuromuscular involvement with dysarthria, dystonia, and tremor. 1 Further investigation revealed decreased ceruloplasmin level (<10 mg/dL), Cu deposition in brain (MRI image in Figure 2) and a heterozygous mutation on ATP7B gene 2 –4 (Figure 1). Although there was no Kayser–Fleischer rings under ophthalmological examination, the above findings were able to make the diagnosis of Wilson’s disease. In the beginning, the patient’s family preferred to receive a conservative treatment instead of surgical intervention, and Botox injection was only a trial for this patient. The use of zinc in the medical management is out the scope of this article. Moreover, the aim of our article is discussing about the neuromuscular presentation of the Wilson’s disease and its surgical management.
Lambrinudi procedure is an effective method to correct severe equinocavovarus deformity of the foot. Although the subtalar joint is fused, without a posterior tibialis tendon transfer to lateral column, the varus deformity will be persistent due to the spasticity of the posterior tibialis tendon. In the article, we mentioned about the restoration of function of ankle dorsiflexion, and subtalar eversion is mainly focused on providing a static stabilizer rather than a dynamic one which can keep a plantigrade foot for assisting walking. Although the right lower limb muscle power is 3, it is different from the singe tendon function since neurological spasticity of posterior tibialis tendon and Achilles tendon is the major deformed force of foot malconformation. Transfer of spastic posterior tibialis tendon to the lateral column can simultaneously decrease the contracture force of the posterior tibialis tendon and add an everted and dorsiflexed force for keeping foot alignment. Peroneal longus transfer to peroneal brevis may be an option, but it can only provide everted force and may not adequately counteract the effect of contracture from posterior tibialis tendon.
