SUMMARYNeurosyphilis results from infection of the central nervous system (CNS) by Treponema pallidum. Neurosyphilis can occur at any time after initial infection, particularly in immunocompromised patients. We describe a case of a 47-year-old man who presented with mixed dysphasia, right upper motor neuron facial palsy and right hemiparesis. Collateral history from the family revealed slowly progressive cognitive impairment and behavioural changes. Neuroimaging was unremarkable, suggesting a non-vascular aetiology. As there was no clear cause for the presentation, a lumbar puncture was performed and serum samples were sent for syphilis serology. The diagnosis of neurosyphilis was made on the basis of a positive RPR test in cerebrospinal fluid. The patient was treated with benzylpenicillin and made a full recovery, with restoration of cognitive function to baseline. BACKGROUND
Schizencephaly is a rare central nervous system (CNS) malformation secondary to neuronal migration defects. The pathogenesis is complex and is secondary to environmental and genetic factors. Clinically, the majority of patients present with varying degrees of motor and psychological disturbances. Imaging plays a cornerstone in the diagnosis by identifying the characteristic lesional features and recognising other associated abnormalities such as an absent septum pellucidum and corpus callosum dysgenesis. Here, we present a male paediatric case who presented with an interestingly asymptomatic unilateral right closed-lip schizencephaly and review the aetiology, clinical presentation and imaging characteristics of the disease and associated literature.
Background: Although the arteriovenous fistula (AVF) confers superior benefits over central venous catheters (CVC), utilisation rates remain low among prevalent haemodialysis (HD) patients. The goal of this study was to determine the evolution of vascular access type in the first year of dialysis and identify factors associated with conversion from CVC to a functioning AVF. Methods: We studied adult patients (n=610) who began HD between the 01/01/2015 and 31/12/2016 and were treated for at least 90 days using data from the National Kidney Disease Clinical Patient Management System in the Irish Health System. Prevalence of vascular access type was determined at day 90 and 360 following dialysis initiation and at 30-day intervals. Multivariable logistic regression explored factors association with CVC at day 90, and Cox regression evaluated predictors of conversion from CVC to AVF on day 360. Results: CVC use was present in 77% of incident patients at day 90 with significant variation across HD centres (from 63 % to 91%, P<0.001) that persisted following case-mix adjustment. From day 90 to day 360, AVF use increased modestly from 23% to 41%. Conversion from CVC to AVF increased over time but the likelihood was lower for older patients [adjusted hazard ratio (HR) 0.43, 95% CI 0.19-0.96 for age >77yrs vs referent], for patients with lower BMI [HR 0.95, (0.93- 0.98) per unit decrease in BMI], and varied significantly across HD centres [from HR 0.25, (0.08-0.74) to 2.09 (1.04-4.18)]. Conclusion: CVC are the predominant type of vascular access observed during the first year of dialysis with low conversion rates from CVC to AVF. Substantial centre variation exists in the Irish health system that is not explained by patient-related factors alone.
An otherwise healthy young man presented with gradual progressive fatigue for the past 12 months disturbing his daily activities. Clinical examination revealed marked generalised muscular hypertrophy including the temporalis muscles bilaterally. Investigation revealed that the patient was grossly hypothyroid due to Hashimoto's thyroiditis with rhabdomyolysis and acute kidney injury. The finding of muscle weakness and pseudohypertrophy in association with hypothyroidism is called Hoffman's syndrome. The patient was hydrated and thyroxine replacement initiated. On follow-up, the patient showed clinical as well as biochemical improvement.
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