Browsing by Author "Salgado, V"
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- DOK7 myasthenic syndrome with subacute adult onset during pregnancy and partial response to fluoxetine.Publication . Santos, M; Cruz, S; Peres, J; Santos, L; Tavares, P; Basto, JP; Salgado, V; Herrero Valverde, ADOK7 congenital myasthenic syndrome (DOK7-CMS) generally presents early in life and is treated with salbutamol or ephedrine. This report describes an atypical case of a 39-year-old woman who presented with proximal upper limb weakness in the third trimester of pregnancy and was initially diagnosed with seronegative myasthenia gravis. Dramatic clinical worsening under pyridostigmine and further inefficacy of steroids, intravenous human immunoglobulin (IVIG) and plasma exchange (PLEX) led to the presumptive diagnosis of a CMS. Initially, a slow-channel CMS was regarded as more probable due to prominent finger extension weakness. Accordingly, fluoxetine was started and a lengthy improvement was seen. Clinical deterioration occurred after fluoxetine withdrawal, when a c.1124_1127dup homozygous mutation was detected in DOK7 gene. Afterwards, salbutamol was started and the patient became asymptomatic. This case highlights the importance of considering CMS before an adult-onset myasthenic syndrome and suggests a benefit from fluoxetine not previously reported in DOK7-CMS.
- In-Hospital and Long-Term Prognosis after Spontaneous Intracerebral Hemorrhage among Young Adults Aged 18-65 YearsPublication . Bernardo, F; Rebordão, L; Machado, S; Salgado, V; Pinto, ABACKGROUND: Spontaneous intracerebral hemorrhage (ICH) accounts for 10%-15% of all strokes and has an estimated annual incidence of 5/100,000 in young adults. Limited data on prognosis after ICH in young adults are available. We aimed to identify prognostic predictors after ICH among adults aged 18-65 years. METHODS: We retrospectively selected all patients with ICH from a prospective single-center registry of adults with first stroke before 65 years between 1997 and 2002. We recorded in-hospital mortality as well as mortality and recurrent stroke after discharge until December 1, 2018. For in-hospital analysis, we compared patients that died in-hospital versus patients discharged alive. For long-term analysis, we compared patients that died in follow-up versus patients still alive. Independent prognostic predictors were identified using multivariate analyses. RESULTS: Among 161 patients included, 24 (14.9%) died in-hospital. Among in-hospital survivors, 5-year survival was 92.0%, 10-year survival 78.1%, and 15-year survival 62.0%. After median follow-up of 17 years, 47.4% of patients died, 18 patients had ischemic stroke, and 6 recurrent ICH. Regarding in-hospital prognosis, coma at admission (OR .02 [.00-.11]) was independent predictor for mortality whereas alcoholic habits (OR 12.32 [1.82-83.30]) was independent predictor for survival. An increasing age (OR 1.08 [1.03-1.12]), higher blood glucose levels (OR 1.01 [1.00-1.01]), and hypertension (OR 2.21 [1.22-4.00]) were independent predictors of long-term mortality after ICH. CONCLUSIONS: Alcoholic habits may influence in-hospital survival after ICH in young adults. Long-term mortality in young adults seems to be lower than in elderly and was predicted by higher blood glucose levels and hypertension.