Physiotherapeutic Intervention in Patients Affected by the Syndrome of Spoan: An Experience Report

Abstract

The SPOAN syndrome is a neurodegenerative disease of autosomal recessive inheritance that causes atrophy of muscles of the upper and lower limbs of the affected individuals. The disease had its initial characterization carried out in individuals originating in the upper western part of the city of Serrinha dos Pintos, State of Rio Grande do Norte, by researchers from the Center for Human Genome Studies and Hospital das Clínicas of the University of São Paulo in the year 2005. Its clinical characteristics include: congenital and atrophic optic atrophy Progressive spastic paraplegia, with early onset of life, axonal neuropathy, with clinical onset after the first decade of life, startle response with unexpected sounds, dysarthria, spinal and foot deformities, and extrapyramidal signs. The present text aims to report the cases of patients with SPOAN under the physiotherapeutic assistance of the Expanded Nucleus of Family Health (NASF) in the city of Serrinha dos Pintos-RN. Experience report:This is an experience report on the care of patients with SPOAN syndrome assisted by the municipality's NASF. In the municipality there were 14 patients with SPOAN, but only 4 (28.5%) of these patients attended the physiotherapeutic care. The treatment lasted an average of 40-50 minutes, with moderate resting pauses between sessions. They consisted of: passive joint mobilization exercises, scapular and pelvic girdle dissociation exercises, intra-articular mobilization exercises, stretching, muscle strengthening , respiratory exercises, myofascial release, Kabat methods, Bobath and Pilates, electrotherapy, and auriculotherapy. Final considerations: Physiotherapic care given to patients with SPOAN syndrome is considered to be extremely important for the preservation of functionality of these patients, since there is a satisfactory clinical evolution, with improvement of muscular strength and flexibility, range of joint movement and respiratory capacity. Therefore, future studies regarding the diagnosis and prognosis of SPOAN are necessary, since early appropriate therapeutic intervention allows the best functional outcome.

Síndrome de SPOAN: uma análise de conceito segundo o método evolucionário de Rodgers

“…De acordo com Queiroz et al, (2019), a síndrome afeta mais de um caminho ou região do centro sistema nervoso em pacientes portadores. Em geral, os sinais que acompanham a paraplegia espástica hereditária complexa são: espasticidade, debilitação, hiper-reflexividade e a Sinal de Babinski, que caracteriza os casos típicos de paraplegia espástica hereditáriapara estes, há sintomas que podem ser adicionados: neuropatia motora e periférica e atrofia óptica, entre outros.…”

“…Ademais, é importante ressaltar que, de acordo com Queiroz et al, (2019), atualmente, no município de Serrinha dos Pintos, há14 pacientes com SPOAN, dos quais apenas 4 (28,5%) frequentam o atendimento fisioterapêutico prestado pelo Núcleo Ampliado de Saúde da Família (NASF). Embora os profissionais encontrem dificuldade em relação à falta de referências na literatura, para orientação em relação ao tratamento, e, ainda, o prognóstico de SPOAN não seja esperançoso, observa-se melhoras nesses pacientes, a exemplo da manutenção da força muscular e flexibilidade, a amplitude de movimento articular e a capacidade respiratória.…”