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Background and Purpose: Brain AVM, cerebral abscess, and ischemic stroke are among the well known neurologic manifestations of hereditary hemorrhagic telangiectasia. However, recently reported data suggest an additional association with malformations of cortical development. The purpose of this study was to determine the prevalence of malformations of cortical development in a population of pediatric patients with hereditary hemorrhagic telangiectasia., Materials and Methods: A retrospective review of brain MRIs from 116 pediatric patients was performed. Each patient was referred from our institution's Hereditary Hemorrhagic Telangiectasia Clinic. Each MRI included a 3D sequence, most frequently MPRAGE. The 3D sequence was evaluated by a neuroradiology fellow, with specific attention to the presence or absence of malformations of cortical development. Positive studies were subsequently reviewed by 2 attending neuroradiologists, who rendered a final diagnosis., Results: Fourteen of 116 (12.1%) patients were found to have a malformation of cortical development. Among these 14, there were 12 cases of polymicrogyria and 2 cases of bifrontal periventricular nodular heterotopia., Conclusions: Pediatric patients with hereditary hemorrhagic telangiectasia have a relatively high prevalence of malformations of cortical development, typically perisylvian polymicrogyria., (© 2017 by American Journal of Neuroradiology.)

Accurate and timely diagnosis of intracranial vasculopathies is important due to significant risk of morbidity with delayed and/or incorrect diagnosis both from the disease process as well as inappropriate therapies. Conventional vascular imaging techniques for analysis of intracranial vascular disease provide limited information since they only identify changes to the vessel lumen. New advanced MR intracranial vessel wall imaging (IVW) techniques can allow direct characterisation of the vessel wall. These techniques can advance diagnostic accuracy and may potentially improve patient outcomes by better guided treatment decisions in comparison to previously available invasive and non-invasive techniques. While neuroradiological expertise is invaluable in accurate examination interpretation, clinician familiarity with the application and findings of the various vasculopathies on IVW can help guide diagnostic and therapeutic decision-making. This review article provides a brief overview of the technical aspects of IVW and discusses the IVW findings of various intracranial vasculopathies, differentiating characteristics and indications for when this technique can be beneficial in patient management., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Background: Myelopathy secondary to fibrocartilaginous emboli is rarely reported and has been documented antemortem only once. The pathophysiology of fibrocartilaginous emboli lacks consensus, although the role of trauma has been previously entertained., Methods: We reviewed the reported cases of myelopathy secondary to fibrocartilaginous emboli. We extensively evaluated two cases of myelopathy in otherwise healthy individuals using myelography and serial magnetic resonance imaging (MRI). Evaluation for underlying systemic disease was conducted in both cases., Results: No evidence of infectious, autoimmune, inflammatory, or neoplastic disease was found in either patient. Furthermore, no other lesions were found in the neuroaxis. Findings on serial MRIs were consistent with vascular lesions thought to be venous strokes., Conclusions: We report two cases of acute myelopathies associated with heavy lifting, in otherwise healthy young men. Serial MRI studies in each case demonstrated lesions consistent with venous infarcts. We postulate that migration of disc material into the perivertebral venous system was the result of increased intervertebral disc pressure and simultaneous excessive Valsalva associated with heavy lifting. We further postulate that this may be a more prevalent cause of acute myelopathy than previously recognized.

A chart analysis for a 5-year period through December 1992 identified four full-term neonates with lobar hemorrhage. Prenatal and obstetrical histories were uncomplicated; all infants presented with seizures within 48 h following birth. Perinatal asphyxia and isoimmune thrombocytopenia were associated with lobar hemorrhage in two children. Although computed tomography was sufficient for diagnosis, magnetic resonance evaluation provided assessment of specific cortical injury and the age of the hemorrhage. No infant underwent surgical intervention; three children reached developmental milestones at a mean follow-up of 3.3 years.