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Immunoglobulin G4-related disease is an increasingly recognized, idiopathic systemic disorder that might be associated with elevated serum IgG4 level and tissue infiltration by IgG4-positive plasma cells. We describe the clinical features and biopsy findings in a patient who presented with features suggestive of pachymeningitis and multiple cranial neuropathies. Meningeal biopsy and other laboratory studies established the diagnosis of IgG4-related hypertrophic pachymeningitis. Despite treatment with corticosteroids and mycophenolate mofetil, the patient exhibited a fluctuating progressive course, which stabilized with rituximab, although the radiological findings persisted over 2½ years of follow-up. Our case highlights many important evolving concepts in the disorder, including unusual pathologic features, lack of correlation between serum IgG4 levels and the clinical course, and posttreatment clinicoradiological discordance. We provide potential explanations for this discrepancy, highlight the validity of novel cerebrospinal fluid studies and progressive systemic involvement despite use of immune-suppressive treatments, and emphasize the usefulness of rituximab as a disease-stabilizing agent.

The etiology of spinal cord infarcts (SCIs), besides being related to aortic perioperative events, in large subset of SCIs, remains cryptogenic. We present a first case of SCI in a patient with hereditary spherocytosis and discuss the potential pathophysiologic considerations for vascular compromise. A 43-year-old woman with a history of hereditary spherocytosis, post splenectomy status, presented with chest, back, and shoulder pain with subsequent myelopathic picture; SCI extending from C4-T2 was confirmed by MRI. Despite aggressive treatment her stroke progressed leading to her demise. Her autopsy confirmed the SCI and revealed some incidental findings, but the cause of SCI remained unidentified. Exclusion of the known etiologies of SCI by extensive negative workup including autopsy evaluation suggested that SCI in our case was related to her history of hereditary spherocytosis. Both venous and arterial adverse vascular events, at a higher rate, have been associated in patients with hereditary spherocytosis who had their spleens removed compared to nonsplenectomized patients. Postsplenectomy increases in the platelet, red blood cell count, leukocyte count, and cholesterol concentrations are postulated to contribute to increased thrombotic risk. Additional prothrombotic factors include continuous platelet activation and adhesion as well as abnormalities of the red blood cell membrane.

A 37-year-old man with a known history of neurofibromatosis 1 (NF1) presented within 2 days of diarrhoeal illness followed by encephalopathy, facial twitching, hypoglycaemia, hypotension, tachycardia and low-grade fever. Examination showed multiple cafe-au-lait spots and neurofibromas over the trunk, arms and legs and receptive aphasia with right homonymous hemianopia, which resolved. Workup for cardiac, inflammatory and infectious aetiologies was unrevealing. A brain MRI showed gyral swelling with increased T2 fluid-attenuated inversion recovery signal and diffusion restriction in the left cerebral cortex. Neuroendocrine findings suggested panhypopituitarism with centrally derived adrenal insufficiency. Supportive treatment, hormone supplementation, antibiotics, antivirals and levetiracetam yielded clinical improvement. A follow-up brain MRI showed focal left parieto-occipital atrophy with findings of cortical laminar necrosis. In conclusion, we describe a case of NF1-associated panhypopituitarism presenting as hypoglycaemic seizures and stroke-like findings, hitherto unreported manifestations of NF1. Prompt recognition and treatment of these associated conditions can prevent devastating complications.

Purpose: Evaluate the reliability of the change from the flat, posterolaterally oriented facets of the thoracic segments to the curved, posteromedially oriented facets of the lumbar spine, the “facet transition”, as a marker for the T12 segment and determine if variations in rib number are associated with lumbosacral transitional segments. Materials and methods: 244 patients underwent whole spine CT examinations and the positions of the thoracolumbar facet transition, type of thoracolumbar facet transition (gradual or abrupt), position of the lowest thoracic ribs, and presence or absence of lumbosacral transitional anatomy were recorded. A Fisher Exact Test was used to determine if there was an association between a variant number of ribs and transitional anatomy at the lumbosacral junction. Results: The thoracolumbar facet transition was located at the eighteenth segment in 50/244 (20%), nineteenth segment in 184/244 (75%), and twentieth segment in 10/244 (4%) of cases. The thoracolumbar facet transition was abrupt in 227/244 (93%). The lowest set of ribs was observed at the nineteenth segment in 225/244 (92%), twentieth segment in 11/244 (5%), and eighteenth segment in 8/244 (3%). The lowest fully-formed intervertebral disc was located between the twenty-third and twenty-fourth segments in 9/244 (4%), twenty-fourth and twenty-fifth segments in 216/244 (88%), and twenty-fifth and twenty-sixth segments in 19/244 (8%). Coexistent lumbosacral transitional anatomy was seen in 5/7 (71%) with eleven, 8/11 (73%) with thirteen, and 24/234 (10%) with twelve ribs. There was an association between variant numbers of ribs and coexistent lumbosacral transitional anatomy (p

Intra-arterial thrombolysis is increasingly being used in the treatment of acute ischemic stroke with a failure rate of recanalization as high as 66%. We describe a case of acute ischemic stroke secondary to occlusion of the middle cerebral artery that failed intra-arterial thrombolytic therapy but responded to rescue balloon angioplasty.

Patients with Purkinje cell cytoplasmic autoantibody type 2 (PCA-2) and collapsin response-mediator protein-5 (CRMP-5) autoantibody can present with multifocal elements of encephalomyeloneuropathy. Except for an anecdotal report, case descriptions of paraneoplastic small fibre neuropathy are lacking. We report paraneoplastic small fibre neuropathy followed by chorea associated with small cell lung cancer. A man aged 57 years with a 35 pack-year smoking history presented with painless subacute paresthesia and weight fluctuation. A non-length-dependent small fibre neuropathy was confirmed by skin biopsy. Further testing revealed positive serum PCA-2 and CRMP-5 autoantibodies, which after positron emission tomography-CT led to histological confirmation of a small cell lung cancer. Initially, abnormal MRI and cerebrospinal fluid studies suggested central nervous system (CNS) involvement which was subclinical; however, 6 months later during antitumour therapy, the patient became symptomatic with choreoathetosis. After combined chemoradiation as well as immunosuppressive and symptomatic therapies, the clinical course stabilised, although residual neurological deficits remained at follow-up a year later. Coexistent PCA-2 and CRMP-5 autoantibodies may occur in the setting of small fibre peripheral neuropathy and choreoathetosis and predict cancer type. Two paraneoplastic syndromes can present successively over months; subclinical CNS involvement with evolving basal ganglia abnormalities can be a paraneoplastic manifestation. In the appropriate clinical setting, paraneoplastic testing should be considered in patients presenting with small fibre neuropathy.

Faced with a single neuroradiological image of an unknown patient, how confident would you be to make a differential diagnosis? Despite advanced imaging techniques, a confident diagnosis also requires knowledge of the patient's age, clinical data and the lesion location. Pattern Recognition Neuroradiology provides the tools you will need to arrive at the correct diagnosis or a reasonable differential diagnosis. This user-friendly book includes basic information often omitted from other texts: a practical method of image analysis, sample dictation templates and didactic information regarding lesions/diseases in a concise outline form. Image galleries show more than 700 high quality representative examples of the diseases discussed. Whether you are a trainee encountering some of these conditions for the first time or a resident trying to develop a reliable system of image analysis, Pattern Recognition Neuroradiology is an invaluable diagnostic resource.

No atlas of neurovascular anatomy would be complete without a description of the circle of Willis. The circle of Willis is known to almost everyone who has an interest in or who has studied the intracranial vascular system. The circle of Willis is a polygonal-shaped collection of arteries at the base of the brain that provides a pathway of communication between the anterior (carotid) and the posterior (vertebral-basilar) circulations. Images demonstrating the components of the circle of Willis are seen throughout this text. This collection of arteries is especially important in providing a pathway for collateral circulation in cases of intracranial vascular occlusions. Unfortunately a complete circle of Willis is present in only approximately 18% of the population. The “classical” circle of Willis includes both internal carotid arteries, both A1 anterior cerebral artery segments, both posterior communicating arteries, both P1 posterior cerebral artery segments, and the anterior communicating artery. In most cases one or multiple portions of the circle of Willis are developmentally/congenitally hypoplastic or absent. Common congenital variations include hypoplasia or absence of the A1 anterior cerebral artery segment (25%), hypoplasia or absence of a posterior communicating artery (32%), or hypoplasia or absence of the P1 posterior cerebral artery segment with fetal/direct origin from the ipsilateral supraclinoid segment of the internal carotid artery (15–22%). Additionally, the anterior communicating artery may be fenestrated or absent (9%). An incomplete circle of Willis is the norm rather than the exception and often results in the isolation of a vascular territory.

The 3D Angiographic Atlas of Neurovascular Anatomy and Pathology is the first atlas to present neurovascular information and images based on catheter 3D rotational angiographic studies. The images in this book are the culmination of work done by Neil M. Borden over several years using one of the first 3D neurovascular angiographic suites in the United States. With the aid of this revolutionary technology, Dr Borden has performed numerous diagnostic neurovascular angiographic studies as well as endovascular neurosurgical procedures. The spectacular 3D images he obtained are extensively labeled and juxtaposed with conventional 2D angiograms for orientation and comparison. Anatomical color drawings and concise descriptions of the major intracranial vascular territories further enhance understanding of the complex cerebral vasculature.

The illustrations in this chapter are intended to provide the reader with a reference point for the images that follow in subsequent chapters. These illustrations provide the reader with the necessary background anatomy and the most commonly encountered patterns of vascular branching. Variability and inconstancy are the rule rather than the exception in how the vasculature tree branches within the neck and face, as well as how it branches intracranially. It is important to understand that vessels are named for the territory of tissue that they supply. We hope that your understanding of neurovascular anatomy is enhanced by the application of this concept in the atlas. The illustrations that are presented are meant to provide the reader with a basic understanding of the relationship of the vascular tree with the tissues they nourish. During your journey through this three-dimensional (3D) atlas, refer back to these color illustrations when needed.

After passing through the foramen magnum the vertebral artery gives rise to numerous branches before uniting with the opposite vertebral artery at the vertebral-basilar junction. Multiple small perforating arteries arise from the distal vertebral artery which supplies the lateral regions of the medulla. The posterior inferior cerebellar artery (PICA) usually arises from the distal vertebral artery 1-2 cm below the vertebral-basilar junction. These arteries are often asymmetric and their size can vary. There is a reciprocal relationship between the size of the PICA and that of the ipsilateral anterior inferior cerebellar artery (AICA) which arises from the basilar artery. When the AICA is large, the ipsilateral PICA is often small or sometimes can be absent. In this situation a large medial branch of the AICA supplies the PICA vascular territory. The PICA divides into branches supplying the ipsilateral cerebellar tonsil, inferior vermis, and the inferior cerebellar hemisphere. Small perforators from the proximal PICA can supply the lateral and posterior medulla. The anterior spinal artery usually arises from the distal 1 cm of the vertebral artery. This vessel often unites with the anterior spinal artery branch from the contralateral vertebral artery to form the main anterior spinal artery. The posterior meningeal artery which supplies the falx cerebelli arises from the distal vertebral artery near the foramen magnum. This vessel courses both posteriorally and superiorally. The vertebral arteries unite to form the basilar artery ventral to the superior medulla/inferior pons.

Objective To evaluate the contribution of CT angiography (CTA) in predicting clinical outcome in a broad spectrum of patients presenting with acute neurological deficits suggestive of brain ischemia, to assess its strengths and limitations in this setting, and examine its influence on selection of patients for thrombolytic treatment. Patients and methods Prospective, observational outcome study of 54 consecutive patients with acute neurological deficits suggestive of brain ischemia who received immediate CTA. Clinical outcome was compared for patients presenting with and without arterial occlusion on CTA. Treatment decisions made by a vascular neurologist blinded to CTA results were compared to CTA cognizant treatment. Results For patients presenting with slight to moderate neurological deficits, the sensitivity and specificity for predicting good clinical outcome was 0.62 and 0.79, respectively, using the initial NIH Stroke Scale (NIHSS) score alone, and 0.38 and 0.92 if additionally, CTA showed no occlusion. For patients presenting with more severe deficits, the sensitivity and specificity for predicting poor clinical outcome using the NIHSS score alone was 0.79 and 0.60, compared to 0.67 and 0.92 if CTA showed vessel obstruction. CTA correctly identified six stroke mimickers. Selection of patients for thrombolysis made with knowledge of CTA results were more often conservative, and corresponded to CTA blinded decisions in 42/50 cases (84%, r = 0.72). Conclusions Combining CTA results with the neurological exam allows increased specificity for predicting clinical outcome as compared to predictions based on admission NIH Stroke Scale score alone. Awareness of CTA results was occasionally associated with less aggressive treatment and testing decisions.

Background and Purpose — We sought to evaluate the ability of CT angiography (CTA) to determine vessel occlusion before acute stroke treatment and to predict its impact on patient outcome. Methods — Consecutive patients with acute focal neurological deficits received immediate brain CTA. Occlusion on CTA was correlated with other neuroimaging studies and clinical outcome. Results — Diagnostic CTA was obtained in 54 patients: catheter angiography (digital subtraction angiography) confirmed the CTA findings in 12 of 14 patients (86%). CTA results were consistent with at least 1 other neuroimaging study in 40 of 50 patients (80%). Patients with occlusion on CTA had significantly worse discharge National Institutes of Health Stroke Scale (NIHSS) score (mean 14.3 versus 4.5, P =0.0023). In multivariate analysis, both CTA-determined presence of occlusion and admission NIHSS score were independent predictors of clinical outcome. Conclusions — In our study there was good agreement between acute CTA interpretation and subsequent imaging studies. CTA evidence of occlusion correlated strongly and independently with poor clinical outcome. CTA provides relevant data regarding vessel patency in acute stroke, which may be of value in selecting patients for aggressive treatment.