Acta neurologica Belgica
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Acta neurologica Belgica · Jun 2021
Case ReportsAre patients with Down syndrome vulnerable to life-threatening COVID-19?
Patients with Down syndrome are at increased risk of respiratory syncytial virus- and H1N1-related death. Literature on COVID-19 in Down syndrome patients is unavailable thus far. We describe the clinical course of 4 patients with Down syndrome during an outbreak of COVID-19. ⋯ Another patient receives supportive care in our institution. Our case series is the first report on probable increased risk of life-threatening disease course of COVID-19 in patients with Down syndrome. Proper surveillance, the adherence of social distancing, and the use of personal protective equipment will be essential in reducing morbidity and mortality in our patients.
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Acta neurologica Belgica · Jun 2021
ReviewDopa-responsive dystonia, DRD-plus and DRD look-alike: a pragmatic review.
Dopa-responsive dystonia (DRD) and DRD plus are diseases of the dopamine pathway with sizeable genetic diversity and myriad presentations. DRD has onset in childhood or adolescence with focal dystonia, commonly affecting lower limb, diurnal fluctuations with evening worsening of symptoms and a demonstrable sleep benefit. DRD "plus" has "atypical features" which include infantile onset, psychomotor delay, cognitive abnormalities, oculogyric crisis, seizures, irritability, spasticity, hypotonia, ptosis, hyperthermia and cerebellar dysfunction. ⋯ Although levodopa is the mainstay of therapy, response to this drug can be unsatisfactory in DRD plus and DRD look-alike and other drugs are tried. Simultaneous management of HPA leads to remarkable improvement in both motor and cognitive functions. The aim of this review is to help neurology practitioners in treating patients with DRD, DRD-plus and DRD look-alike as many of them have excellent outcome with appropriate therapy.