Dyke-Davidoff-Masson Syndrome: Main clinical and radiological findings- systematic literature review.

Autor: Rondão MBA; Bachelor Academic, Medical School of ABC. 2000, Lauro Gomes Avenue, Santo André SP 09060-870, Brazil. Electronic address: mabi_as@hotmail.com., Hsu BRRHS; Bachelor Academic, Medical School of ABC. 2000, Lauro Gomes Avenue, Santo André SP 09060-870, Brazil., Centeno RS; Adjunct Professor of Neurosurgery Department of Neurology and Neurosurgery, Federal University of São Paulo. 1500, Sena Madureira Street, São Paulo-SP 04021-001, Brazil; Neurosurgeon in charge of Epilepsy Surgery Program, Federal University of São Paulo. 1500, Sena Madureira Street, São Paulo-SP 04021-001, Brazil; Postgraduate Division of Department of Neurology and Neurosurgery, Federal University of São Paulo. 1500, Sena Madureira Street, São Paulo-SP 04021-001, Brazil., de Aguiar PHP; Postgraduate Division of Department of Neurology and Neurosurgery, Federal University of São Paulo. 1500, Sena Madureira Street, São Paulo-SP 04021-001, Brazil; Research Professor of Research and Innovation Department and Laboratory of Cellular and Molecular Biology, Medical School of ABC. 2000, Lauro Gomes Avenue, Santo André SP 09060-870, Brazil.
Jazyk: angličtina
Zdroj: Seizure [Seizure] 2023 Aug; Vol. 110, pp. 58-68. Date of Electronic Publication: 2023 Jun 03.
DOI: 10.1016/j.seizure.2023.04.020
Abstrakt: Introduction: Dyke-Davidoff-Masson syndrome (DDMS), or cerebral hemiatrophy, was first described in 1933. It is characterised by cerebral injury that causes hypoplasia in one of the cerebral hemispheres. The disease has different clinical degrees and two aetiologies: congenital and acquired. Radiological findings depend on the degree of injury and the patient's age at the time.
Objective: To provide information on the main clinical and radiological characteristics of this disease.
Methods: A systematic review of the PubMed, MEDLINE, and LILACS databases was conducted using only one keyword. Dyke-Davidoff-Masson syndrome. A total of 223 studies were identified, and the results are presented in tables and graphics.
Results: The mean age of the patients was 19.44 (0-83 years), and the majority were male (55.32%). The most common types of epileptic seizures were generalised tonic-clonic seizures (31 cases), focal impaired awareness seizures (20 cases), focal motor seizures (13 cases), focal to bilateral tonic-clonic seizures (nine cases), and focal myoclonic seizures (one case). The main features of the disease were rapid deep tendon reflexes and extensor cutaneous-plantar tendon reflexes (30 cases - 16%), contralateral hemiparesis or hemiplegia (132 cases - 70%), gait alterations (16 cases - 9%), facial paralysis (nine cases - 5%), facial asymmetry (58 cases - 31%), limb asymmetry (20 cases - 11%), delayed developmental milestones (39 cases - 21%), intellectual disability (87 cases - 46%), and language/speech disorders (29 cases - 15%). Left hemisphere atrophy was the most prevalent.
Conclusion: DDMS is a rare syndrome, and several questions regarding this disease remain unanswered. This systematic review aims to elucidate the most common clinical and radiological aspects of the disease and emphasises the need for further investigation.
Competing Interests: Declaration of Competing Interest none
(Copyright © 2023 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)
Databáze: MEDLINE
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