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Sneddon¿s syndrome: a comprehensive review of the literature
Sneddon’s syndrome (SS) is a rare non-inflammatory thrombotic vasculopathy characterized by the combination of cerebrovascular disease with livedo racemosa(LR). The Orpha number for SS is ORPHA820. It has been estimated that the incidence of SS is 4 per 1 million per annum in general population and generally occurs in women between the ages of 20 and 42 years. LR may precede the onset of stroke by years and the trunk and/or buttocks are involved in nearly all patients. The cerebrovascular manifestations are mostly secondary to ischemia (transient ischemic attacks and cerebral infarct). Other neurological symptoms range ...
Source: Orphanet Journal of Rare Diseases - December 31, 2014 Category: Internal Medicine Authors: Shengjun WuZiqi XuHui Liang Source Type: research

Recurrent status epilepticus as the primary neurological manifestation of CADASIL: A case report
Publication date: 2015 Source:Epilepsy & Behavior Case Reports, Volume 3 Author(s): Naim Haddad , Catherine Ikard , Kim Hiatt , Vignesh Shanmugam , James Schmidley Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) often presents with a history of migraine with aura and eventual manifestations of dementia with unrelenting, repeated cerebral vascular insults. Only 6–10% of patients with CADASIL have been reported to develop seizures, and status epilepticus (SE) is exceedingly rare. Here, we describe a patient who presented with recurrent SE, with eventual biopsy ...
Source: Epilepsy and Behavior Case Reports - April 2, 2015 Category: Neurology Source Type: research

Brachial Plexus Neuritis as Presenting Manifestation of Disseminated Coccidioidomycosis. (P6.323)
CONCLUSIONS:This is the first reported case of coccidioidomycosis presenting with PNS involvement. Both remote inflammatory changes and direct local fungal invasion noted in this case highlight the wide spectrum of clinico-pathological presentations seen in coccidioidomycosis infection. As this fungal disease is identified with increasing frequency, clinicians should be aware of its atypical manifestations. Study Supported by: Disclosure: Dr. Shah has nothing to disclose. Dr. Liao has nothing to disclose. Dr. Shanina has nothing to disclose. Dr. Smith has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Shah, R., Liao, B., Shanina, E., Smith, G. Tags: Fungi, Parasites, and Other Infectious Disorders Source Type: research

Cardiac manifestations of Sneddon´s syndrome
Sneddon's syndrome (SS) is rare, non-inflammatory arteriopathy characterized by cerebrovascular disease and livedo reticularis (LR). It generally affects women between 20 and 42years of age [1], so this entity is increasingly recognised as a cause of stroke in young adults [2]. The pathophysiology of SS is not fully understood, but the presence of antiphospholipid antibodies in 50% of patients [3], as well as the skin and brain biopsy findings, suggest a thrombotic vasculopathy of medium and small arteries.
Source: International Journal of Cardiology - April 21, 2015 Category: Cardiology Authors: Fernando Dominguez, Burkert Pieske, Sebastian Kelle Tags: Letter to the Editor Source Type: research

Cardiac manifestations of Sneddon's syndrome
Sneddon's syndrome (SS) is rare, non-inflammatory arteriopathy characterized by cerebrovascular disease and livedo reticularis (LR). It generally affects women between 20 and 42years of age [1], so this entity is increasingly recognized as a cause of stroke in young adults [2]. The pathophysiology of SS is not fully understood, but the presence of antiphospholipid antibodies in 50% of patients [3], as well as the skin and brain biopsy findings, suggests a thrombotic vasculopathy of medium and small arteries.
Source: International Journal of Cardiology - April 21, 2015 Category: Cardiology Authors: Fernando Dominguez, Burkert Pieske, Sebastian Kelle Tags: Letter to the editor Source Type: research

Small‐nerve‐fiber pathology in critical illness documented by serial skin biopsies
Conclusion: Serial IENFD measurements confirmed the development of small‐fiber sensory involvement in the acute phase of critical illness. Muscle Nerve, 2015
Source: Muscle and Nerve - May 29, 2015 Category: Internal Medicine Authors: Miroslav Skorna, Roman Kopacik, Eva Vlckova, Blanka Adamova, Milena Kostalova, Josef Bednarik Tags: Research Article Source Type: research

Malignant Middle Cerebral Artery Infarction Resulting from Dissection of Middle Cerebral Artery
A 30-year-old man presented with headache, left hemiparesis, and uncal herniation. Magnetic resonance imaging showed acute infarction in the right middle cerebral artery (MCA). Lifesaving craniotomy proceeded because of the uncal herniation. Assessment of brain tissue led to a diagnosis of MCA dissection. His skin biopsy showed ultrastructural abnormalities of dermal connective tissue. This is the unique case that dissection was diagnosed from both a biopsy specimen of the MCA and the dermal connective tissue.
Source: Journal of Stroke and Cerebrovascular Diseases - August 13, 2015 Category: Neurology Authors: Kenichi Sakuta, Renpei Sengoku, Hidetaka Mitsumura, Yu Kono, Issei Kan, Shogo Kaku, Yasuyuki Iguchi Tags: Case Report Source Type: research

Summary: International Kidney Cancer Symposium
Conclusions:  Ideal ischemia time is 20-25 minutes or less improves short and long term renal function.  >25 minutes carried 5 year risk of new onset stage 4 CKD No differences on GFR for cold vs. warm ischemia times Preoperative GFR and the percent of kidney preserved was a better predictor of post op GFR.  No ischemia preserves renal function better than warm. Longer cold ischemia times were equivalent to shorter warm ischemia times. Quality and quantity of the remaining kidney is associated with ultimate renal function. Robotics in RCC Surgery Gennady Bratslavsky, MD The...
Source: Kidney Cancer Association - December 15, 2011 Category: Urology & Nephrology Source Type: news

Bevacizumab, temozolomide, and radiotherapy for newly diagnosed glioblastoma: comprehensive safety results during and after first-line therapy
Conclusion The safety profile was consistent with that expected from radiotherapy/temozolomide plus bevacizumab. The increased AE incidence with bevacizumab did not impact patients' ability to receive standard-of-care treatment or to undergo further surgery.
Source: Neuro-Oncology - June 6, 2016 Category: Cancer & Oncology Authors: Saran, F., Chinot, O. L., Henriksson, R., Mason, W., Wick, W., Cloughesy, T., Dhar, S., Pozzi, E., Garcia, J., Nishikawa, R. Tags: Clinical Investigations Source Type: research

Rare-33. intracerebral hemorrhage as rare complication of intravascular lymphoma
Intravascular lymphoma (IVL) is an aggressive multifocal large cell lymphoma, characterized by neoplastic proliferation and vascular occlusion within the lumina of arterioles, capillaries and venules with a predilection for the central nervous system and skin. The clinical presentation is frequently non-specific and may consist of changes in mental status, focal neurological deficits, seizures, fevers of unknown origin and skin changes. Due to the rarity and absence of specific diagnostic procedures apart from biopsy, diagnosis is difficult and often post-mortem. Neuroimaging findings vary widely, ranging from normal to di...
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Czap, A., Mantica, M. Tags: RARE TUMORS Source Type: research

Recognizing CADASIL: a Secondary Cause of Migraine with Aura
AbstractPurpose of ReviewCADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an important disease to consider in the differential diagnosis of migraine with aura. This review examines current literature regarding migraine in CADASIL, as well as diagnostic and treatment modalities.Recent FindingsRecent studies have shown that smoking is a modifiable risk factor for progression of CADASIL (Chabriat et al. in Stroke 47:4 –11,2015). Specific imaging changes and findings on clinical exam can predict disease progression. However, migraine symptoms often precede MRI changes (G...
Source: Current Pain and Headache Reports - March 8, 2017 Category: Neurology Source Type: research

A father ’s hope for his son’s life
Juan and Fredy in 2017. Juan was looking forward to having his son, Fredy, 14, finally come home to live with him. The teenager had been living under the care of his grandmother since he was a toddler. But on that long-awaited homecoming day, Juan was quickly jarred from feeling great joy to grave concern. “When I saw his face, one side looked very different from the other and his lip was swollen,” says Juan. “He admitted right away that his face had been hurting.” Juan remembered that the last time he’d seen his son — more than one year ago — Fredy’s face had looked slightly different then too. But whateve...
Source: Thrive, Children's Hospital Boston - April 12, 2017 Category: Pediatrics Authors: Kat J. McAlpine Tags: Diseases & Conditions Our Patients’ Stories Dr. Cameron Trenor Dr. Carolyn Rogers Dr. Darren Orbach Dr. Reza Rahbar Dr. Salim Afshar interventional radiology juvenile nasopharyngeal angiofibroma tumor Source Type: news

Endocrine Disruptors and Health Effects in Africa: A Call for Action
Conclusion: To address the many challenges posed by EDCs, we argue that Africans should take the lead in prioritization and evaluation of environmental hazards, including EDCs. We recommend the institution of education and training programs for chemical users, adoption of the precautionary principle, establishment of biomonitoring programs, and funding of community-based epidemiology and wildlife research programs led and funded by African institutes and private companies. https://doi.org/10.1289/EHP1774 Received: 16 February 2017 Revised: 22 May 2017 Accepted: 24 May 2017 Published: 22 August 2017 Address correspond...
Source: EHP Research - August 23, 2017 Category: Environmental Health Authors: Daniil Lyalko Tags: Commentary Source Type: research

Neurologic attack and dynamic perfusion abnormality in neuronal intranuclear inclusion disease
Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in cells in the central, peripheral, and autonomic nervous system and visceral organs. Although clinical diagnosis was difficult until recently, an increasing number of adult cases of leukoencephalopathy have been diagnosed antemortem with NIID based on the characteristic hyperintensity in the corticomedullary junction on diffusion-weighted imaging (DWI) and confirmatory skin biopsy.1 While adult-onset NIID is characterized by slowly progressive dementia, it may also present with acute...
Source: Neurology Clinical Practice - December 11, 2017 Category: Neurology Authors: Fujita, K., Osaki, Y., Miyamoto, R., Shimatani, Y., Abe, T., Sumikura, H., Murayama, S., Izumi, Y., Kaji, R. Tags: MRI, SPECT, DWI, All Cognitive Disorders/Dementia Case Source Type: research

Summary: International Kidney Cancer Symposium
Conclusions:  Ideal ischemia time is 20-25 minutes or less improves short and long term renal function. >25 minutes carried 5 year risk of new onset stage 4 CKD No differences on GFR for cold vs. warm ischemia times Preoperative GFR and the percent of kidney preserved was a better predictor of post op GFR.  No ischemia preserves renal function better than warm. Longer cold ischemia times were equivalent to shorter warm ischemia times. Quality and quantity of the remaining kidney is associated with ultimate renal function. Robotics in RCC Surgery Gennady Bratslavsky, MD The opening question ...
Source: Kidney Cancer Association - December 15, 2011 Category: Urology & Nephrology Source Type: news