First experience of botulinum toxin type A therapy in Russia in comprehensive therapy for Wilson–Konovalov disease diagnosed at the age of 12 years
The main forms of orphan Wilson–Konovalov disease – cerebral, abdominal and mixed forms, with copper accumulation in the central nervous system, patients debut spastic and dystonic attacks. The authors demonstrate the clinical example of the efficiency and safety of botulinum toxin type A for the re...
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| Language: | Russian |
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ABV-press
2024-11-01
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| Series: | Русский журнал детской неврологии |
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| Online Access: | https://rjdn.abvpress.ru/jour/article/view/489 |
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| author | D. A. Krasavina D. O. Ivanov O. R. Orlova V. K. Valiev O. N. Vasilyeva |
| author_facet | D. A. Krasavina D. O. Ivanov O. R. Orlova V. K. Valiev O. N. Vasilyeva |
| author_sort | D. A. Krasavina |
| collection | DOAJ |
| description | The main forms of orphan Wilson–Konovalov disease – cerebral, abdominal and mixed forms, with copper accumulation in the central nervous system, patients debut spastic and dystonic attacks. The authors demonstrate the clinical example of the efficiency and safety of botulinum toxin type A for the relief of spastic and dystonic attacks and pain syndrome in a 12-year-old child with severe multiple contractures of the upper and lower limbs with a resistant Wilson–Konovalov disease’s form. A 12-year-old patient (male) with primary diagnosed resistant form of Wilson–Konovalov disease with deformities of pyramidal-extropyramidal type was diagnosed with contractures of the tongue, deviation of the mandible; contractures of the right shoulder joint, flexor contracture of the right elbow joint, flexion-ulnar contracture of the right wrist joint, flexion contracture of the fingers and severe extensor contracture of the left elbow joint, extensor-ulnar contracture of the left wrist joint, equinovarus contracture of the ankle joints. The patient received two-stage botulinum therapy according to the spasticity and dystonia protocol. Carrying out the first stage of botulinum therapy according to the spasticity protocol gave positive dynamics. Less frequency of the dystonic attacks, the relief of pain syndrome. The extensor contracture was preserved, and a second stage of botulinum therapy was performed to preserve the joint for the soft tissue surgery. Botulinum neuroprotein therapy is fundamental in the treatment of resistant forms of Wilson–Konovalov disease in order to relief dystonic attacks, spasticity and pain syndrome, that can keep joints healthy. |
| format | Article |
| id | doaj-art-d68d6d5d09ee47cab4779673a41b4b60 |
| institution | Kabale University |
| issn | 2073-8803 2412-9178 |
| language | Russian |
| publishDate | 2024-11-01 |
| publisher | ABV-press |
| record_format | Article |
| series | Русский журнал детской неврологии |
| spelling | doaj-art-d68d6d5d09ee47cab4779673a41b4b602025-08-20T04:00:01ZrusABV-pressРусский журнал детской неврологии2073-88032412-91782024-11-01193788810.17650/2073-8803-2024-19-3-78-88330First experience of botulinum toxin type A therapy in Russia in comprehensive therapy for Wilson–Konovalov disease diagnosed at the age of 12 yearsD. A. Krasavina0D. O. Ivanov1O. R. Orlova2V. K. Valiev3O. N. Vasilyeva4Saint Petersburg State Pediatric Medical University, Ministry of Health of RussiaSaint Petersburg State Pediatric Medical University, Ministry of Health of RussiaI.M. Sechenov First Moscow State Medical University, Ministry of Health of RussiaODA Professorial Clinic LLCSaint Petersburg State Pediatric Medical University, Ministry of Health of RussiaThe main forms of orphan Wilson–Konovalov disease – cerebral, abdominal and mixed forms, with copper accumulation in the central nervous system, patients debut spastic and dystonic attacks. The authors demonstrate the clinical example of the efficiency and safety of botulinum toxin type A for the relief of spastic and dystonic attacks and pain syndrome in a 12-year-old child with severe multiple contractures of the upper and lower limbs with a resistant Wilson–Konovalov disease’s form. A 12-year-old patient (male) with primary diagnosed resistant form of Wilson–Konovalov disease with deformities of pyramidal-extropyramidal type was diagnosed with contractures of the tongue, deviation of the mandible; contractures of the right shoulder joint, flexor contracture of the right elbow joint, flexion-ulnar contracture of the right wrist joint, flexion contracture of the fingers and severe extensor contracture of the left elbow joint, extensor-ulnar contracture of the left wrist joint, equinovarus contracture of the ankle joints. The patient received two-stage botulinum therapy according to the spasticity and dystonia protocol. Carrying out the first stage of botulinum therapy according to the spasticity protocol gave positive dynamics. Less frequency of the dystonic attacks, the relief of pain syndrome. The extensor contracture was preserved, and a second stage of botulinum therapy was performed to preserve the joint for the soft tissue surgery. Botulinum neuroprotein therapy is fundamental in the treatment of resistant forms of Wilson–Konovalov disease in order to relief dystonic attacks, spasticity and pain syndrome, that can keep joints healthy.https://rjdn.abvpress.ru/jour/article/view/489wilson–konovalov diseasebotulinum toxin type aspasticitydystoniacontracture |
| spellingShingle | D. A. Krasavina D. O. Ivanov O. R. Orlova V. K. Valiev O. N. Vasilyeva First experience of botulinum toxin type A therapy in Russia in comprehensive therapy for Wilson–Konovalov disease diagnosed at the age of 12 years Русский журнал детской неврологии wilson–konovalov disease botulinum toxin type a spasticity dystonia contracture |
| title | First experience of botulinum toxin type A therapy in Russia in comprehensive therapy for Wilson–Konovalov disease diagnosed at the age of 12 years |
| title_full | First experience of botulinum toxin type A therapy in Russia in comprehensive therapy for Wilson–Konovalov disease diagnosed at the age of 12 years |
| title_fullStr | First experience of botulinum toxin type A therapy in Russia in comprehensive therapy for Wilson–Konovalov disease diagnosed at the age of 12 years |
| title_full_unstemmed | First experience of botulinum toxin type A therapy in Russia in comprehensive therapy for Wilson–Konovalov disease diagnosed at the age of 12 years |
| title_short | First experience of botulinum toxin type A therapy in Russia in comprehensive therapy for Wilson–Konovalov disease diagnosed at the age of 12 years |
| title_sort | first experience of botulinum toxin type a therapy in russia in comprehensive therapy for wilson konovalov disease diagnosed at the age of 12 years |
| topic | wilson–konovalov disease botulinum toxin type a spasticity dystonia contracture |
| url | https://rjdn.abvpress.ru/jour/article/view/489 |
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