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Título

Neuroplasticity on Dyke Davidoff Masson syndrome: a case report

RESUMO

Case presentation:A 57-year-old woman diagnosed with Dyke Davidoff Masson Syndrome presented with cognitive impairment and secondary epilepsy since her childhood. Orphan of both parents as a young infant, her prenatal history is therefore unknown.The patient lives in a public psychiatry institution, where therapy, medical assistance, artistic and ludic activities are offered. The radiological findings evidenced an important unilateral atrophy of the left cerebral hemisphere and an ipsilateral skull hypertrophy. Despite significant brain damage, the patient maintained only cognitive impairment and controlled seizures by the regular use of antiepileptic drugs, being independent for daily living activities. Her neurological examination showed Wartenberg’s sign and hipotrophy in the right upper limb and reduction of motion in the same limb during gait.This favorable clinical outcome may be explained by the innate capacity of neuroplasticity.Discussion:The Dyke-Davidoff-Masson Syndrome is defined as an atrophy or hypoplasia of one cerebral hemisphere following a prior fetal or childhood insult (1). The etiopathogenesis is not clear, but it could be either a vascular insult during intrauterine life or acquired causes like trauma, infection, vascular abnormalities and intracranial hemorrhage in the perinatal period or shortly thereafter (2). Clinical features depend on the extent of brain injury and include hemiparesis or hemiplegia, seizures, mental retardation or learning disability, speech or language disorders and facial asymmetry (2). Diagnosis is settled by clinical history, neurological examination and radiologic findings. Treatment is symptomatic and aimed at controlling seizures and improving quality of life (3). Some patients evolve with minimum neurologic impairment considering the extension of the brain damage. It could be explained by the nervous system's ability to change its activity in response to stimuli by reorganizing its structure, functions, or connections after injuries (4). The basis of neural plasticity derives from synaptic plasticity that refers to changes in the strength of neurotransmission induced by activity experienced by the synapse in the past (5). Final comments:This report illustrates a rare syndrome that has a wide spectrum of cognitive disabilities affecting multiple domains. We defend that neuroplasticity has a key role on its prognost,making it possible for one part of the brain to take up the functions of the disabled part.

Palavras Chave

Cerebral hemiatrophy; Dyke–Davidoff–Masson syndrome; epilepsy; cognitive impairment; neuroplasticity

Área

Miscelânea

Autores

Bruna Andrade de Oliveira, Carolina Rocha da Silveira Brügger Cardoso, Natália da Costa Chiote Pinheiro