Structural signature of classical versus late-onset friedreich's ataxia by Multimodality brain MRI.

INTRODUCTION: Friedreich's ataxia (FRDA) is the most common autosomal-recessive ataxia worldwide. It is characterized by early onset, sensory abnormalities, and slowly progressive ataxia. However, some individuals manifest the disease after the age of 25 years and are classified as late-onset FRDA (LOFA). Therefore, we propose a transversal multimodal MRI-based study to investigate which anatomical substrates are involved in classical (cFRDA) and LOFA. METHODS: We enrolled 36 patients (13 with LOFA) and 29 healthy controls. All subjects underwent magnetic resonance imaging in a 3 T device; three-dimensional high-resolution T1-weighted images and diffusion tensor images were used to assess gray and white matter, respectively. We used T1 multiatlas approach to assess deep gray matter and cortical thickness measures to evaluate cerebral cortex and DTI multiatlas approach to assess white matter. All analyses were corrected for multiple comparisons. RESULTS: Group comparison showed that both groups presented gray matter atrophy mostly in the motor cortex. Regarding white matter, we found abnormalities in the cerebellar peduncles, pyramidal tracts, midbrain, pons, and medulla oblongata for both groups, but the microstructural abnormalities in the cFRDA group were more widespread. In addition, we found that the corticospinal tract presented more severe microstructural damage in the LOFA group. Finally, the midbrain volume of the cFRDA, but not of the LOFA group, correlated with disease duration (R = -0.552, P = 0.012) and severity (R = -0.783, P < 0.001). CONCLUSION: The cFRDA and LOFA groups have similar, but not identical neuroimaging damage pattern. These structural differences might help to explain the phenotypic variability observed in FRDA. Hum Brain Mapp 38:4157-4168, 2017. © 2017 Wiley Periodicals, Inc.

Rapid response to meningococcal disease cluster in Foya district, Lofa County, Liberia January to February 2018.

INTRODUCTION: Early detection of disease outbreaks is paramount to averting associated morbidity and mortality. In January 2018, nine cases including four deaths associated with meningococcal disease were reported in three communities of Foya district, Lofa County, Liberia. Due to the porous borders between Lofa County and communities in neighboring Sierra Leone and Guinea, the possibility of epidemic spread of meningococcal disease could not be underestimated. METHODS: The county incidence management system (IMS) was activated that coordinated the response activities. Daily meetings were conducted to review response activities progress and challenges. The district rapid response team (DRRT) was the frontline responders. The case based investigation form; case line list and contacts list were used for data collection. A data base was established and analysed daily for action. Tablets Ciprofloxacin were given for chemoprophylaxis. RESULTS: Sixty-seven percent (67%) of the cases were males and also 67% of the affected age range was 3 to 14 years and attending primary school. The attack rate was 7/1,000 population and case fatality rate was 44.4 % with majority of the deaths occurring within 24-48 hours of symptoms onset. Three of the cases tested positive for Neisseria Meningitidis sero-type W while six cases were Epi-linked. None of the cases had recent meningococcal vaccination and no health-worker infections were registered. CONCLUSION: This cluster of cases of meningococcal disease during the meningitis season in a country that is not traditionally part of the meningitis belt emphasized the need for strengthening surveillance, preparedness and response capacity to meningitis.