As part of the workup of polyneuropathy, neurologists often encounter patients with paraproteinemias, also called monoclonal gammopathies. Given the relatively rising prevalence of both polyneuropathy and paraproteinemias in our aging population, this coexistence is frequently due to chance. Still, certain types of paraproteinemias have a well-defined causal role in the development of polyneuropathy, and therefore has implications in their subsequent work-up and management. Once a monoclonal gammopathy has been deemed as “benign” or of undetermined significance, looking at the heavy chain subtype is paramount in deciding whether it is related to a polyneuropathy. Conversely, polyneuropathies associated with monoclonal gammopathies tend to have a distinct clinical, serological, and/or electrodiagnostic phenotype that helps in establishing an association. Recognizing this association will determine approp riate workup and management.
Artículo de Revisión Invitado
Estado Epiléptico Refractario de Aparición Reciente (NORSE): Revisión y actualización. New-Onset Refractory Status Epilepticus (NORSE): Review and Update
The New-Onset Refractory Status Epilepticus (NORSE) is a rare entity that is defined as a clinical presentation, in a patient without active epilepsy or other relevant neurological disorder, who presents a refractory status epilepticus (RSE) without an acute or active structural, toxic, or metabolic cause. NORSE constitutes a diagnostic challenge that implies high morbidity and mortality in the short and long term. Clinically, NORSE is characterized by a prodromal phase, an acute phase, where seizures with diverse semiology that evolve to an RSE occur, and a chronic phase, with neurological deterioration and drug-resistant epilepsy. After an extensive work-up, the cause of NORSE is identified in only half of the cases. The initial treatment is based on the use of conventional anti-seizure drugs, requiring most of patients the use of anesthetics and mechanical ventilation. Better results have been observed with immunotherapy, more recently with anti-cytokine drugs, such as tocilizumab and anakinra, consistent with the immunological/inflammatory mechanisms proposed for the pathophysiology of NORSE.
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Aterosclerosis Carotidea: Una Causa Frecuente Pero Prevenible de Ictus Isquémico. Carotid Atherosclerosis: A Common But Preventable Cause Of Ischemic Stroke
Carotid atherosclerosis is responsible for 10-20% of all ischemic strokes and is characterized by a high risk of stroke recurrence, as well as by its preventable nature through the control of vascular risk factors, intensive medical management, and surgical revascularization in cases of severe stenosis. Pivotal studies in the 90’s showed significant benefit of carotid endarterectomy in patients with severe symptomatic carotid stenosis (>70%), whereas the benefit in patients with moderate stenosis (50-69%) and those with asymptomatic disease was modest. Active areas of research in the field include the identification of novel non-traditional risk factors directed to the early diagnosis and primary prevention of carotid atherosclerosis, the efficacy of intensive medical management to decrease to the risk of stroke among patients with asymptomatic disease, and the development of new surgical revascularization techniques with lower complication rates. Through this revision manuscript we aim to summarize the current knowledge on carotid atherosclerosis and expose a practical approach for its diagnosis and treatment.
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Parálisis Periódica Hipopotasémica Tirotóxica, Una Emergencia Neuroendocrina: Artículo de Revisión. Thyrotoxic Hypokalemic Periodic Paralysis A Neuroendocrine Emergency: Review Article
Thyrotoxic Periodic Paralysis (TPP) is a neuroendocrine emergency and although rare should be part of the differential diagnosis of weakness in a patient presenting with hyperthyroidism. It is usually triggered by high carbohydrate diet, trauma, exposure to cold or strenuous exercise. Most of the cases occur in 20 – 30 year-old Asian males. Diagnosis is clinical with the triad of flaccid paralysis, signs of thyrotoxicosis and hypokalemia. Treatment consist on electrolyte repletion, thionamides and beta blockers. The importance of recognizing the association between hyperthyroidism and hypokalemic paralysis is vital as with successful treatment of hyperthyroidism paralysis resolves.
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Bacterias, Endotoxinas y Neuroinflamación Crónica: ¿Una Etiopatogenia Para las Enfermedad de Alzheimer? Bacteria, Endotoxins And Chronic Neuroinflammation: An Etiopathogenesis For Alzheimer’s Disease?
Alzheimer’s disease represents one of the main health problems in advanced countries. Actually, we do not have an effective therapy for disease and its cause remains unknown. For several decades, research has focused on amyloidogenesis as the primary cause of disease. However, clearly satisfactory results have not been obtained in this line of research. In recent years there has been growing evidence about the role of neuroinflammation in AD and other neurodegenerative diseases. The role of ß-Amyloid as an element of the innate immune response places it in a new position in the pathophysiology of the disease. Alterations of intestinal and oral microbiota could have a role in the generation of neuroinflammatory changes, either directly by pathogens or by bacterial endotoxins. Endotoxins are polysaccharides of gram-negative bacteria that produce a potent immune reaction. Recently, there is evidence that gingipains have a role in production of neurotoxicity and amyloidogenesis. Gingipains are endotoxins produced by a pathogen associated with chronic periodontitis, Porphyromonas gingivalis. Gingipains generate direct neurotoxicity and its effect could be reversed with various molecules that are currently under development.
