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Split posterior tibial-tendon transfers in children with cerebral spastic paralysis and equinovarus deformity.

In the treatment of equinovarus deformity of the foot in children with cerebral spastic paralysis, to supplement heel-cord lengthening, we split the posterior tibial tendon and transferred its posterior half laterally into the peroneus brevis tendon. Thirty-seven operations were performed on thirty-one hemiplegic, quadriplegic, and diplegic children who were followed for a mean of eight years postoperatively (range, four to fourteen years), at which time twenty-six of the thirty-one children were skeletally mature. There were thirty excellent, four good, and three poor results. The results did not deteriorate with time. Thirty-two of thirty-four children with good or excellent results had functioning dorsiflexors of the foot and were brace-free postoperatively. The children who had a poor result had recurrent deformity, but none had development of a calcaneal or calcaneovalgus deformity.

Adolescent↗

Surgical treatment of congenital kyphosis associated with progressive spastic paralysis in an adult patient.

A 38-year-old man presented with untreated congenital kyphosis associated with progressive spastic gait. To prevent progression of the spastic paralysis, rigid correction of the severe spinal deformity arising from the congenital kyphosis was performed by one-stage posterior closing-wedge osteotomy, without occurrence of neurological complications. Progression of the paralysis has not been identified for 30 months after the operation and a slight improvement in gait was recognized. The current case is categorized as type I deformity of congenital kyphosis in the upper thoracic spine, which is normally treated surgically before the adolescent growth phase begins.

Adult↗

Novel missense mutation in ALS2 gene results in infantile ascending hereditary spastic paralysis.

OBJECTIVE: Recessive mutations in ALS2 (juvenile amyotrophic lateral sclerosis) are causative for early-onset upper motor neuron diseases, including infantile ascending hereditary spastic paralysis (IAHSP). The goal of this study is to identify novel disease-causing ALS2 mutations. METHODS: Mutations in ALS2 were screened by direct sequencing of complementary DNA obtained from patients' lymphoblasts. RESULTS: We report a novel ALS2 missense mutation in patients affected by IAHSP. This homozygous G669A mutation in exon 4 is predicted to result in a tyrosine substitution at cysteine 156 of the RCC1 (regulator of chromatin condensation)-like domain, encoding a putative guanine exchange factor for Ran guanosine triphosphatase, leading to a loss of ALS2 function due to instability of mutant protein. INTERPRETATION: These results highlight the important role of the RCC1-like domain in ALS2 stability and function that is essential for upper motor neuron maintenance.

Adult↗

[Palliative surgical treatment of spastic paralysis in the lower extremity].

AIMS: The purpose of this paper is to describe our experience with and to review the best results in the surgical treatment of patients suffering from spastic paralysis of the lower limbs. DEVELOPMENT: To enable a correct indication of the techniques to be employed the authors recommend a thorough examination of the types of deformity (fixed, dynamic or mixed) and the use of specific tests for exploring the different deformities. These are necessary steps to be able to interpret the different disorders in a global manner and thus reach diagnostics that provide us with a proper surgical therapeutic orientation about the spastic hip, knee, ankle and foot. Due to the importance of the overall problem, it is becoming increasingly more frequent to advise multidisciplinary work involving the collaboration of different specialists (neurologists, rehabilitators, physiotherapists, psychologists, paediatricians, neurophysiologists and orthopaedic surgeons). Spasticity is as heterogeneous as the results of the different treatment projects. The techniques used must allow the rehabilitation therapy to be continued. Surgical intervention is recommended when the damage to the CNS has stabilised and the patient is over 4 years old. The psychic state of the patient and the family must also be evaluated. CONCLUSIONS: The objective of the treatment in patients who can walk is to improve motor functioning, the type of gait and to prevent fixed deformities from developing. In patients who do not walk, the aim is to improve their hygiene and their capacity to sit and to walk. These indications are indispensable to be able to successfully perform a little-known area of orthopaedic surgery which does not respond to the techniques used in flaccid paralysis surgery.

Humans↗

Classification and surgical treatment of the thumb-in-palm deformity in cerebral palsy and spastic paralysis.

Over a 20-year period, 59 children with spasticity mainly due to cerebral palsy underwent surgery for correction of a thumb-in-palm deformity. A classification of the deformity based on the functional anatomy of the thumb divided the problem into four different types. A retrospective analysis of the results has shown the classification to be helpful in selecting a surgical option for treatment. In addition, the classification helps to keep accurate records, predict patient progress, and coordinate postoperative treatment.

Cerebral Palsy↗