Journal of neuroimaging : official journal of the American Society of Neuroimaging
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Review Case Reports
Clinical and Neuroimaging Manifestations of Erdheim-Chester Disease: A Review.
Erdheim-Chester disease (ECD) is a rare disorder characterized by accumulation of non-Langerhans cell histiocytes in multiple organs. The clinical manifestations are protean and vary from asymptomatic focal disease to potentially fatal multisystem disorder. The commonest presentation is symmetric osterosclerotic lesions of lower extremity long bones; other organs, including cardiovascular, nervous, and endocrine system may be affected. ⋯ Rare primary neurological presentation can occur mimicking CNS inflammatory, neoplastic, or demyelinating disorders. We report an unusual case of ECD presenting with progressive encephalopathy and ataxia along with multifocal brainstem and cerebellar lesions. A comprehensive review of clinical and neuroimaging features and immunohistochemical and molecular characteristic of ECD are presented along with review of neuroimaging findings in two previously reported cases.
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Epilepsy is among one of the most common neurologic disorders. The role of magnetic resonance imaging (MRI) in the diagnosis and management of patients with epilepsy is well established, and most patients with epilepsy are likely to undergo at least one or more MRI examinations in the course of their disease. ⋯ Familiarity with normal anatomic variations is essential in the accurate diagnosis and image interpretation, as these variations may be mistaken for epileptogenic foci, leading to unnecessary follow-up imaging, or worse, unnecessary treatment. After a brief overview of normal imaging anatomy of the mesial temporal lobe, this article will review a few important common and uncommon anatomic variations, mimics, and pitfalls that may be encountered in the imaging evaluation of patients with epilepsy.
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Extracranial carotid artery stenosis (ECAS) due to the presence of atherosclerotic plaque is a well-known risk factor for stroke. Several structural imaging studies have investigated the effect of ECAS on the brain, focusing on structural damage (in particular cerebral small vessel disease) and on the rearrangement of the cerebral circulation. For example, it is known from the literature an association between carotid stenosis and cerebral small vessel disease, and it is also noted that a series of compensatory mechanisms are activated by the cerebrovascular system in order to overcome the cerebral hypoperfusion that is induced by the ECAS. ⋯ Although these studies have been performed on small cohorts of patients without standardized protocols, it is plausible that in the future, such studies will help find early stage markers of cognitive impairment. This could permit an extension of indications for revascularization for the treatment of cognitive impairment in selected patients. The aim of this narrative review is to discuss the current knowledge on the effects of ECAS on brain and cognition by analyzing the main evidence from animal and clinical studies, with a special focus on rs-fc MR studies.
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Sickle cell disease is the most common hereditary hemoglobinopathy, which results in abnormally shaped and rigid red blood cells. These sickle-shaped red blood cells cause vaso-occlusion and ischemic phenomena that can affect any organ in the body. As a common cause of disability, the neurological manifestations of sickle cell disease are particularly important. ⋯ Impaired blood supply to bone is associated with osteomyelitis and osteonecrosis. Fat embolization syndrome is a rare complication of osteonecrosis, which may cause devastating neurological impairment. Awareness and early recognition of the diverse manifestations of sickle cell disease on neuroimaging is crucial to ensure optimal treatment in a complex patient cohort.
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Mechanical Thrombectomy (MT) using modern devices has proved to be efficacious in re-establishing intracranial circulation and reduced procedural times. We outline a number of endovascular techniques utilizing the distal access catheter, the circumstances where they are applicable, and the outcomes achieved. ⋯ In this pictorial review, we have demonstrated techniques where distal access catheter when used as a sole suction device, or combined with a stent-retriever, has been able to provide a rapid route to successful reperfusion, with minimal complications related to our endovascular procedures.