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The neurological phenotype in MJD, which can range widely, has been classified into at least three subtypes with characteristic features: cerebellar ataxia with ophthalmoparesis and pyramidal tract signs (type II), with additional extrapyramidal signs (type I), or with distal muscular atrophy (type III) [31].
From admission to the day of MRI, SOFA score, vital signs, type and dose of catecholamine infused, sedatives, analgesics and antibiotics, use of steroids, days of mechanical ventilation, and standard laboratory tests were recorded daily.
The clinical phenotypes of SCA3 were classically classified into three categories: type 1 with early onset pyramidal and extrapyramidal signs, type 2 with cerebellar and pyramidal symptoms, and type 3 with cerebellar involvement and anterior horn cell degeneration [ 62].
The following variables were collected: age, sex, body mass index, and admission simplified acute physiology score (SAPS) II [ 10], sequential organ failure assessment (SOFA) score [ 11], vital signs, type of shock, type of ventilation, drugs needed for the treatment of shock states, biochemical variables, blood cell count and coagulation variables.
Demographic and comorbidity data, relevant time points and dates, laboratory variables, triage categories and vital signs, type of antibiotics, amount and type of fluids (L), administered oxygen (L/min), disposition, and outcome variables were prospectively registered in the digital hospital information system Chipsoft Ezis (Chipsoft, Amsterdam, Netherlands) of each participating hospital.
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