Hyperglycaemia-related complications at the time of diagnosis can cause permanent neurological disability in children with neonatal diabetes.

BACKGROUND: Children with neonatal diabetes often present with diabetic ketoacidosis and hence are at risk of cerebral oedema and subsequent long-term neurological deficits. These complications are difficult to identify because neurological features can also occur as a result of the specific genetic aetiology causing neonatal diabetes. CASE REPORTS: We report two cases of neonatal diabetes where ketoacidosis-related cerebral oedema was the major cause of their permanent neurological disability. Case 1 (male, 18 years, compound heterozygous ABCC8 mutation) and case 2 (female, 29 years, heterozygous KCNJ11 mutation) presented with severe diabetic ketoacidosis at 6 and 16 weeks of age. Both had reduced consciousness, seizures and required intensive care for cerebral oedema. They subsequently developed spastic tetraplegia. Neurological examination in adulthood confirmed spastic tetraplegia and severe disability. Case 1 is wheelchair-bound and needs assistance for transfers, washing and dressing, whereas case 2 requires institutional care for all activities of daily living. Both cases have first-degree relatives with the same mutation with diabetes, who did not have ketoacidosis at diagnosis and do not have neurological disability. DISCUSSION: Ketoacidosis-related cerebral oedema at diagnosis in neonatal diabetes can cause long-term severe neurological disability. This will give additional neurological features to those directly caused by the genetic aetiology of the neonatal diabetes. Our cases highlight the need for increased awareness of neonatal diabetes and earlier and better initial treatment of the severe hyperglycaemia and ketoacidosis often seen at diagnosis of these children.

Assessing mobile food vendors (a.k.a. street food vendors)--methods, challenges, and lessons learned for future food-environment research.

OBJECTIVES: Mobile food vendors (also known as street food vendors) may be important sources of food, particularly in minority and low-income communities. Unfortunately, there are no good data sources on where, when, or what vendors sell. The lack of a published assessment method may contribute to the relative exclusion of mobile food vendors from existing food-environment research. A goal of this study was to develop, pilot, and refine a method to assess mobile food vendors. STUDY DESIGN: Cross-sectional assessment of mobile food vendors through direct observations and brief interviews. METHODS: Using printed maps, investigators canvassed all streets in Bronx County, NY (excluding highways but including entrance and exit ramps) in 2010, looking for mobile food vendors. For each vendor identified, researchers recorded a unique identifier, the vendor's location, and direct observations. Investigators also recorded vendors answers to where, when, and what they sold. RESULTS: Of 372 identified vendors, 38% did not answer brief-interview questions (19% were 'in transit', 15% refused; others were absent from their carts/trucks/stands or with customers). About 7% of vendors who ultimately answered questions were reluctant to engage with researchers. Some vendors expressed concerns about regulatory authority; only 34% of vendors had visible permits or licenses and many vendors had improvised illegitimate-appearing set-ups. The majority of vendors (75% of those responding) felt most comfortable speaking Spanish; 5% preferred other non-English languages. Nearly a third of vendors changed selling locations (streets, neighbourhoods, boroughs) day-to-day or even within a given day. There was considerable variability in times (hours, days, months) in which vendors reported doing business; for 86% of vendors, weather was a deciding factor. CONCLUSIONS: Mobile food vendors have a variable and fluid presence in an urban environment. Variability in hours and locations, having most comfort with languages other than English, and reluctance to interact with individuals gathering data are principal challenges to assessment. Strategies to address assessment challenges that emerged form this project may help make mobile-vendor assessments more routine in food-environment research.

Cognitive function and cerebral emboli after primary hip arthroplasty.

Transcranial Doppler ultrasound has been used to detect cerebral emboli after hip arthroplasty. The cognitive effects of these embolic events are unclear. The aim of this study was to assess cognitive change after primary cemented hip arthroplasty using a range of neuropsychological tests and to measure intraoperative cerebral embolic load. Twenty primary cemented total hip arthroplasties underwent a series of cognitive tests before and at four days after surgery. A range of validated tests assessed: global cognitive function; verbal fluency and speed; immediate and delayed memory recall; attention and mental processing speeds. Intra-operative transcranial Doppler ultrasound monitoring of the middle cerebral artery for embolic signals was also performed. A one-sample Wilcoxon signed-rank test compared median cognitive results post-pre surgery and a Mann-Whitney U test established if there was a cognitive difference between those patients who had detectable cerebral emboli and those who did not. Scatter plot graphs were also used to establish any correlation between the embolic load and clinical cognitive dysfunction. A significant (p<0.01) difference was noted in specific tests assessing mental processing speed, visual searching and sustained and divided attention following surgery. Intra-operative cerebral embolic signals were detected in 11 out of 20 patients and the majority occurred with femoral component cementation and hip reduction. There was no difference in cognitive dysfunction between those patients who had detectable cerebral embolic signals and those who did not and there appeared to be no direct correlation between the size of the embolic load and the level of cognitive dysfunction.

Spinocerebellar ataxia type 8 in Scotland: frequency, neurological, neuropsychological and neuropsychiatric findings.

OBJECTIVES: The objectives of this study were to: (i) establish whether the spinocerebellar ataxia type 8 (SCA 8) expansion is associated with ataxia in Scotland; (ii) test the hypothesis that SCA 8 is associated with neuropsychological impairment; and (iii) review neuroradiological findings in SCA 8. METHODS: The methods included: (i) measurement of SCA 8 expansion frequencies in ataxic patients and healthy controls; (ii) comprehensive neuropsychological assessment of patients with SCA 8 and matched controls, neuropsychiatric interview; and (iii) comparison of patient and matched control magnetic resonance imaging (MRI) scans. RESULTS: (i) 10/694 (1.4%) unrelated individuals with ataxia had combined CTA/CTG repeat expansions >100 compared to 1/1190 (0.08%) healthy controls (P < 0.0005); (ii) neuropsychological assessment revealed a dysexecutive syndrome among SCA 8 patients, not readily explained by motor or mood disturbance; neuropsychiatric symptoms occurred commonly; (iii) cerebellar atrophy was the only salient MRI abnormality in the patient group. CONCLUSIONS: The SCA 8 expansion is associated with ataxia in Scotland. The disorder is associated with a dysexecutive syndrome.

Efficacy of the enteric pathogen screen card in the presumptive identification of Salmonella enteritidis.

The efficacy of the Enteric Pathogen Screen (EPS) card as a rapid method for identification of Salmonella spp. isolated in stool cultures was studied. Of the 120 lactose-negative, sucrose-negative, H2S-producing colonies tested, 74 were identified as Salmonella enteritidis by conventional methods. The reading of the EPS card after 2 h of incubation yielded a sensitivity of 47.2% and a specificity and positive predictive value of 100%, with a negative predictive value of 54.1%. In the final reading of 4 h, a sensitivity of 97.2% and a specificity of 86.9% were obtained, with a positive predictive value of 92.3% and a negative predictive value of 95.2%.