We have found numerous case reports, but no systematic study of the megadolichovertebrobasilar anomaly ( MDVBA ). The purpose of this paper is to evaluate the relationships between arterial shifts of the vertebro-basilar system and neurological findings in the posterior fossa in our series of 132 cases. We found a high percentage (77.3%) of angiographic-clinical correlations having evaluated the arterial shifts, measured in mm, of the vertebro-basilar system in a frontal and a sagittal plane and concluded that the greater the degree of dislocation, the greater the number of positive cases. Nevertheless it is not possible to predetermine the presence of particular neurosymptomatology related to arterial dislocation degrees.

Background Thrombotic microangiopathy (TMA) is a clinical syndrome encompassing a large group of rare but severe disorders including thrombotic thrombocytopenic purpura (TTP) and both typical and atypical forms of hemolytic uremic syndrome (HUS). The key role of the complement system is well known in TTP and atypical HUS, but recent reports describe its involvement in the pathogenesis of HUS secondary to gastrointestinal infections due to Shiga toxin-producing Escherichia coli (STEC). Methods TMA mainly affects the kidney, but extra-renal complications are frequently described. The involvement of the central nervous system (CNS) represents often a life-threatening condition and it can result in serious long-term disability in HUS patients who overcome the acute phase of illness. In the present study, we retrospectively analyzed a pediatric cohort of a single tertiary pediatric hospital in Southern Italy, in which this complication occurred in 12/54 children (22% of cases), of whom five with severe neurological involvement had been successfully treated with eculizumab. Results The great clinical variability of brain injury in our cohort has led us to retrospectively build a Bneurological score^useful to assess the clinical severity of neurologic involvement. Subjects with higher neurologic score due to the most severe CNS involvement resulted in the group of patients early treated with eculizumab, obtaining a good clinical response (four out five patients). In conclusion, the early treatment with eculizumab in children with severe neurological involvement during STEC-HUS was associated with complete regression of both acute kidney injury (AKI) and neurological lesions observed at magnetic resonance imaging (MRI).

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CSF NF-L tracks mainly grey matter damage in patients with CIS suggestive of MS.

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