A cerebral, with copper accumulation in the central nervous system, debut with spastic and dystonic attacks.

Our clinical example demonstrates the efficacy and safety of botulinum toxin type A for stopping attacks and pain syndrome in a 12-year-old child with severe multiple contractures of the upper and lower extremities in the resistant form of Wilson-Konovalov disease.

A patient with a primarily diagnosed resistant form of Wilson-Konovalov disease with pyramidal-extrapyramidal deformities was diagnosed with tongue contractures, dislocation of the lower jaw; contractures of the right shoulder joint, elbow joint, wrist joint, flexion contracture of the fingers and pronounced extension contracture of the left elbow joint, left wrist joint, equinovarus contracture of the ankle joints. The patient received a two-stage session of botulinum therapy according to the protocol for treating spasticity and dystonia.

The first stage of botulinum therapy according to the protocol for treating spasticity gave positive dynamics. Reduction in the frequency of dystonic attacks, relief of pain syndrome.

The extension contracture was preserved, and the second stage of botulinum therapy was carried out to preserve the joint for soft tissue surgery.

The drugs were re-administered 8 months later during the rehabilitation stage. The extension contracture of the left upper limb and equinovarus deformity of the ankle joints of both limbs remained. Thanks to botulinum therapy, the contracture had a dynamic nature, in connection with which the child underwent soft tissue surgery with tendon lengthening, isolation and transplantation of a number of tendons to create a mid-physiological position of the limbs. Botulinum neuroprotein therapy is the fundamental method of treating resistant forms of Wilson-Konovalov disease with the aim of stopping attacks of dystonia, spasticity and pain syndrome, which helps maintain joint health, as well as lifelong pathogenetic therapy.