Rafael Glikstein

Neurologic impairment due to vitamin E and copper deficiencies in celiac disease.

Celiac disease is an immune-mediated intolerance to gluten, which is found in grains such as wheat and barley. Its estimated prevalence is ∼1% and it remains underdiagnosed in many countries. It causes an enteropathy leading to macro- and/or micronutrient malabsorption, most commonly of iron, vitamin B12, vitamin D, and calcium. Deficiencies of only one or a few nutrients are the most common manifestation of non-diarrheal, “silent” celiac disease, which accounts for 50% of initial presentations.1 Immune- and nonimmune mechanisms provoke diverse neurologic complications in celiac disease including peripheral neuropathy and ataxia.2 ### Case report. We investigated a 58-year-old French-Canadian man with 1 year of progressive leg stiffness and gait unsteadiness. He had mild, longstanding, biopsy-proven celiac disease presenting with dermatitis herpetiformis in 1980. He had received replacement therapy for iron-deficiency anemia and vitamin B12 deficiency during the last decade. There were no other symptoms of celiac disease and he did not adhere to a gluten-free diet. Two of five siblings have celiac disease without neurologic symptoms. CSF and urine examinations were normal. Blood studies revealed a mild microcytic anemia (131 g/L) with acanthocytes; mildly elevated transaminases; serum α-tocopherol (vitamin E) 2.22 × 10−3); serum ceruloplasmin 200 mg/L); …

Imaging of the brain in patients with human immunodeficiency virus infection.

Abstract Neurologic disease in acquired immunodeficiency syndrome (AIDS) patients is related either to opportunistic pathogens or to direct central nervous system (CNS) invasion by the human immunodeficiency virus. Despite the increasing availability of antiretroviral therapy, opportunistic infections continue to afflict patients in the developing world and in other populations with limited access to appropriate treatment. Classic CNS infections in the setting of AIDS include toxoplasmosis, cryptococcosis, progressive multifocal leukoencephalopathy, and cytomegalovirus encephalitis. Additionally, AIDS patients are far more susceptible to acquiring CNS tuberculosis and neurosyphilis, both of which exhibit altered disease characteristics in the setting of immunosuppression. Neuroimaging is a crucial component of the diagnostic work-up of these conditions, and findings include, but are not limited to, intracranial mass lesions, white matter disease, meningoencephalitis, vascular complications, and hydrocephalus. Though various disease processes can produce imaging findings that overlap with one another, certain characteristic patterns may suggest a particular pathogen, and advanced imaging techniques and laboratory tests allow for definitive diagnosis. Knowledge of the imaging patterns seen in the setting of AIDS-related CNS disease is vital to the neuroradiologist, whose interpretation may guide decisions related to treatment and further work-up.

Flow diversion in the treatment of carotid injury and carotid-cavernous fistula after transsphenoidal surgery:

We describe a case of iatrogenic carotid injury with secondary carotid-cavernous fistula (CCF) treated with a silk flow diverter stent placed within the injured internal carotid artery and coils placed within the cavernous sinus. Flow diverters may offer a simple and potentially safe vessel-sparing option in this rare complication of transsphenoidal surgery. The management options are discussed and the relevant literature is reviewed.

Imaging diagnosis and the role of endovascular embolization treatment for vascular intraspinal tumors

Intraspinal tumors comprise a large spectrum of neoplasms, including hemangioblastomas, paragangliomas, and meningiomas. These tumors have several common characteristic imaging features, such as highly vascular mass appearance in angiography, hypointense rim and serpentine flow voids in MRI, and intense enhancement after intravenous contrast administration. Due to their rich vascularity, these tumors represent a special challenge for surgical treatment. More recently, the surgical treatment of intraspinal vascular tumors has benefited from the combination of endovascular techniques used to better delineate these lesions and to promote preoperative reduction of volume and tissue blood flow. Endovascular embolization has been proven to be a safe procedure that facilitates the resection of these tumors; hence, it has been proposed as part of the standard of care in their management.

Metastatic renal cell carcinoma mimicking a schwannoma in a dorsal root ganglion: case report

Peripheral nerve tumors are soft-tissue tumors that can occur in any nerve throughout the body. The majority of peripheral nerve tumors arise from elements of the nerve sheath with the two most common being neurofibromas and schwannomas. More than 90% of all peripheral nerve tumors are benign. When there is peripheral nerve involvement in metastatic carcinoma, it is often via contiguous spread from the primary mass; hematogenous seeding to a peripheral nerve is seldom seen. In this report the authors describe the even rarer case of metastatic renal cell carcinoma mimicking a schwannoma in a dorsal root ganglion. Cases from the literature show the rarity of this finding and its late clinical appearance. Given that survival in patients with metastatic carcinoma continues to increase, dorsal root ganglion metastasis may become more common over time.

Imaging Findings in Sensorineural Hearing Loss: A Pictorial Essay

Sensorineural hearing loss (SNHL) is a common presenting complaint, particularly in the aging population [1] and can be caused by a wide range of pathologies affecting the cochlea, cranial nerve VIII, and the central auditory pathway. The central auditory pathway includes the bony and membranous labyrinth, the internal auditory canal (IAC), the cerebellopontine angle (CPA), the brain stem, the thalamus, and the temporal lobe. New diagnosis of SNHL is a common reason for requesting cross sectional imaging. Given the wide range of potential pathologies (Table 1), both high resolution computed tomography (CT) and gadolinium enhanced magnetic resonance imaging (MRI) are used in the initial assessment of SNHL although, where available, MRI is commonly used as first-line imaging. Causes of hearing loss that primarily present with conductive or mixed hearing loss such as otosclerosis, cholesteatoma, and temporal bone fracture are not included in the scope of this review. The most common cause of SNHL is presbycusis, affecting more than one-third of adults older than 75 years of age. Presbycusis is caused by age related degenerative changes involving the organ of Corti, which are beyond the resolution of current imaging techniques. Imaging is carried out to assess for alternative, potentially treatable, causes of SNHL. The purpose of this pictorial essay is to review important radiologic features of common and

Psychological comorbidities and compliance to interventional treatment of patients with cutaneous vascular malformations

Purpose The aim of this study was to assess qualitatively the psychological stressors affecting patients with cutaneous vascular malformations and hemangiomas (CVM-H) and their impact on compliance to interventional treatment. Methods A retrospective chart review was conducted of all patients with CVM-H treated by interventional neuroradiology at a single academic institution during a five-year period (2009–2014). Psychological complaints were documented during each clinic visit by a neuroradiologist. Compliance to interventional treatment was defined by adherence to the scheduled treatment sessions. Fisher’s exact test was used to assess for associations between psychological complaints and compliance. Results Seventy-five patients were assessed, of whom 49 (65.3%) were female, with an age range of 2–78 years (mean age 30.2 years). All except one patient older than seven years of age (n = 71; 94.6%) had a psychological complaint, including fear of negative appearance (n = 53; 70.6%), dissatisfaction with appearance (n = 46; 61.3%), low self-esteem (n = 35; 46.6%), anxiety (n = 16; 21.3%), stress (n = 13; 17.3%), bullying (n = 5; 6.6%), and low mood (n = 4; 5.3%). Twenty-three (31%) patients were non-compliant. Low self-esteem was significantly associated with non-compliance (p = 0.0381). Conclusion There is a high prevalence of psychological comorbidities among patients treated for CVM-H. This has potential implications for interventional treatment, as it was found that low self-esteem is significantly associated with non-compliance. These results suggest the need for early psychological support in these patients in order to maximize compliance to interventional treatment.

Vulnerable carotid plaque imaging and histopathology without a dedicated MRI receiver coil

Stroke is associated with vulnerable carotid artery plaques showing specific histopathologic features, namely a lipid-rich necrotic core, intraplaque hemorrhage, ulceration, and thin fibrous cap. While ultrasound and computed tomography (CT) can identify carotid plaques and determine the extent of stenosis, magnetic resonance imaging (MRI) provides further information regarding plaque composition and morphology. In this feasibility study, three patients with symptomatic, moderately stenosed plaques were imaged with CT angiography (CTA) and MRI (3T and 1.5T) without a dedicated receiver coil. The patients subsequently underwent carotid endarterectomy with en-bloc excision of the plaque. The CT and MR images were analyzed independently by three neuroradiologists to identify vulnerable plaque features. The images were correlated with the histopathology to confirm the findings. All three patients had one or more vulnerable plaque features on histopathology. MRI allowed for better characterization of these features when compared to CTA. The pre- and post-contrast T1-weighted (T1W) images were most helpful for identifying the lipid-rich necrotic core and thin fibrous cap, while the time of flight-magnetic resonance angiography (TOF-MRA) and contrast-enhanced (CE)-MRA were excellent for detecting plaque hemorrhage and ulceration, respectively. The 3T images showed superior spatial and contrast resolution compared to the 1.5T images for all sequences. By providing direct correlation between imaging and histopathology, this study demonstrates that 3T MRI without a dedicated surface coil is an excellent tool for assessing plaque vulnerability. In smaller hospitals or those with limited resources, it is reasonable to consider conventional MRI for patient risk stratification. Further studies are needed to determine how MRI and plaque vulnerability can be incorporated into routine clinical practice.

Role of neuroimaging in multidisciplinary approach towards Non-Alzheimer’s dementia

AbstractDementia is defined as chronic deterioration of intellectual function and cognitive skills significant enough to interfere with the ability to perform daily activities. Recent advances in the treatment of dementia have renewed interest in the use of various neuroimaging techniques that can assist in the diagnosis and differentiation of various subtypes. Neuroimaging and computational techniques have helped the radiological community to monitor disease progression of various neurodegenerative conditions presenting with dementia, such as Alzheimer disease, frontotemporal lobe dementia (FTLD), progressive supranuclear palsy (PSP) and multisystem atrophy-cerebellar variant (MSA-C), and their response to newer therapies. Prompt identification of treatable or reversible forms of dementia, such as tumours, subdural haemorrhage and intracranial dAVF, is crucial for the effective management of these conditions. It is also prudent to recognize the imaging spectrum of metabolic, infective and autoimmune diseases with rapidly progressing dementia, such as methanol toxicity, central pontine myelinolysis (CPM), delayed post hypoxic leukoencephalopathy (DPHL), HIV, Creutzfeldt-Jakob Disease (CJD), Sjogrens syndrome, multiple sclerosis (MS), radiation necrosis and Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS), which are difficult to treat and often require palliative care. This pictorial review emphasizes various non-Alzheimer’s dementia entities and discusses their imaging highlights. Teaching Points • Non Alzheimer’s dementia constitutes a broad spectrum of conditions. • Neuroimaging plays an important role in differentiating treatable from irreversible dementia. • Neuroimaging is often non-specific in early stages of neurodegenerative conditions with dementia. • Neuroimaging plays an important role in the multimodal approach towards management of dementia.

Inflammatory Cerebrospinal Fluid in Sporadic Creutzfeldt-Jakob Disease

BACKGROUND Sporadic Creutzfeldt-Jakob disease (CJD) is a fatal, transmissible spongiform encephalopathy characterized by rapidly progressive dementia, myoclonus, ataxia and akinetic mutism. The underlying mechanism is believed to be a conformational change of a native prion protein which characteristically fails to provoke an immune response. Commensurate with the latter, cerebrospinal fluid (CSF) classically exhibits a non-inflammatory profile. CASES We report two patients with pathologically-proven sporadic CJD presenting with a significant CSF pleocytosis. CONCLUSION Although uncommon, the presence of an inflammatory CSF profile should not exclude the diagnosis of sporadic CJD.