SciCombinator

Discover the most talked about and latest scientific content & concepts.

Journal: Current neurology and neuroscience reports

31

The neurocutaneous disorders (NCDs) embrace an extensive group of developmental disorders associated with involvement of the skin, central nervous system (CNS), and/or the peripheral nervous system (PNS). The neurocutaneous manifestations relate to the common ectodermal origin of these organs. This review intended for the practicing clinical neurologist focuses on selected aspects of the NCDs primarily those associated with cerebrovascular disease. Our emphasis is primarily on those NCDs with genetic heterogeneity and their neurological manifestations.

Concepts: Central nervous system, Nervous system, Psychology, Brain, Neurology, Skin, Ectoderm, Peripheral nervous system

28

Sporadic inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy (IIM) after age 50 years. It presents with chronic insidious proximal leg and distal arm asymmetric muscle weakness. Despite similarities with polymyositis (PM), it is likely that IBM is primarily a degenerative disorder rather than inflammatory muscle disease. IBM is associated with a modest degree of creatine kinase (CK) elevation and an electromyogram (EMG) demonstrates a chronic irritative myopathy. Muscle histopathology demonstrates endomysial inflammatory exudates surrounding and invading non-necrotic muscle fibers often times accompanied by rimmed vacuoles. We review IBM with emphasis on recent developments in the field and discuss ongoing clinical trials.

Concepts: Muscle, Muscular system, Creatine kinase, Neuromuscular disease, Inclusion body myositis, Myopathy, Myositis, Inflammatory myopathy

27

The commensal flora that lives in the human gut is a unique ecosystem that has evolved over millennia with human beings. The importance of the microbiota in various bodily functions is gradually becoming more apparent. Besides the gut microbiome playing a role in bowel-related disorders, a role in metabolic and autoimmune disorders is becoming clearer. The gut bacteria play a role in educating the immune system and hence may be a player in the development of multiple sclerosis. We examine the different sources of information linking the gut microbiota to multiple sclerosis and examine the future avenues for utilizing the knowledge of the gut microbiome to potentially treat and prevent multiple sclerosis.

Concepts: Immune system, Antibody, Bacteria, Gut flora, Evolution, Organism, Immunology, Parasitism

26

The special relationship between migraine and epilepsy has been recognized for centuries and was formally acknowledged by Gowers in his 1906 lecture “Borderland of Epilepsy.” The term migralepsy was introduced by Lennox and Lennox in 1960, with multiple cases described in the literature since that time. In the ensuing years, the relationship between migraine and epilepsy has proven complex. The 2 conditions have been found to be comorbid with each other, suggesting a common underlying mechanism or genetic tendency. Specific diseases with both phenotypes provide further evidence of a common pathophysiology, and as the mechanism of migraine has been further elucidated, commonalities with seizure have been recognized. The terms “hemicrania epileptica” and “migraine triggered seizure” were defined by the International Headache Society, formalizing the concept that one can lead to the other. However, case reports and case series in the literature reveal that distinguishing between the 2 entities can be challenging. The concept of migralepsy is likely to evolve as greater understanding of both conditions is gained.

Concepts: Epilepsy, NATO, Headaches, Winston Churchill, Tony Blair, Special Relationship, United Kingdom – United States relations, Lennox International

7

Tumor necrosis factor-α (TNF-α) blockers are a popular therapeutic choice in a number of inflammatory diseases. Thus far, five TNF- α blockers have been approved for clinical use (etanercept, infliximab, adalimumab, golimumab. and certolizumab). Despite being considered relatively safe, serious side effects associated with immune suppression have been reported, including central and peripheral nervous system (CNS) demyelinating disorders. It is still elusive whether these events are mere coincidence or a side effect of anti-TNF-α use. In this paper, we review the published case reports of CNS demyelination associated with anti-TNF-α therapy and present the follow-up of our 4 previously reported patients who developed neurologic symptoms suggestive of CNS demyelination after having received anti-TNF-α treatment. We also discuss the possible role of TNF-α blockers in demyelination.

Concepts: Immune system, Central nervous system, Nervous system, Rheumatoid arthritis, Myelin, Tumor necrosis factor-alpha, Psoriatic arthritis, TNF inhibitor

6

While over half of women with migraine report improvement during pregnancy, having a history of migraine may increase the chance of negative health outcomes. The state of pregnancy increases the risk of several dangerous secondary headache disorders, especially those associated with hypertensive disorders of pregnancy, and providers need to know the red flags to diagnose and treat emergently. Non-pharmacological migraine treatments can be instituted in advance of pregnancy as many are considered the safest options during pregnancy, but understanding the safety of medications and dietary supplements ensures appropriate care for the refractory migraine patient. New controversy exists over the safety of several historically routine and safe migraine treatment options in pregnancy, such as magnesium, acetaminophen, ondansetron, and butalbital. While it is not clear if breastfeeding decreases the postpartum recurrence of migraine, understanding safe treatment options during lactation can allow women to continue breastfeeding while achieving migraine relief.

Concepts: Hypertension, Prolactin, Breastfeeding, Non-steroidal anti-inflammatory drug, Paracetamol, Ibuprofen, Headache

5

This review aims to help neurologists managing atrial fibrillation (AF) patients who had an ischemic stroke and/or with intracranial hemorrhage (ICH) markers, therefore at high embolic/hemorrhagic risks.

Concepts: Blood, Stroke, Atrial fibrillation, Transient ischemic attack, Bleeding, Subarachnoid hemorrhage, Atrial flutter

3

Amyotrophic lateral sclerosis (ALS) is now recognised to be a heterogeneous neurodegenerative syndrome of the motor system and its frontotemporal cortical connections. The development and application of structural and functional imaging over the last three decades, in particular magnetic resonance imaging (MRI), has allowed traditional post mortem histopathological and emerging molecular findings in ALS to be placed in a clinical context. Cerebral grey and white matter structural MRI changes are increasingly being understood in terms of brain connectivity, providing insights into the advancing degenerative process and producing candidate biomarkers. Such markers may refine the prognostic stratification of patients and the diagnostic pathway, as well as providing an objective assessment of changes in disease activity in response to future therapeutic agents. Studies are being extended to the spinal cord, and the application of neuroimaging to unaffected carriers of highly penetrant genetic mutations linked to the development of ALS offers a unique window to the pre-symptomatic landscape.

Concepts: Brain, Medical imaging, Brain tumor, Nuclear magnetic resonance, Magnetic resonance imaging, Amyotrophic lateral sclerosis, Radiology, Frontotemporal dementia

3

In acute ischemic stroke, critical hypoperfusion is a frequent cause of hypoxic tissue injury: As cerebral blood flow (CBF) falls below the ischemic threshold of 20 mL/100 mL/min, neurological symptoms develop and hypoxic tissue injury evolves within minutes or hours unless the oxygen supply is restored. But is ischemia the only hemodynamic source of hypoxic tissue injury?Reanalyses of the equations we traditionally use to describe the relation between CBF and tissue oxygenation suggest that capillary flow patterns are crucial for the efficient extraction of oxygen: without close capillary flow control, “functional shunts” tend to form and some of the blood’s oxygen content in effect becomes inaccessible to tissue.This phenomenon raises several questions: Are there in fact two hemodynamic causes of tissue hypoxia: Limited blood supply (ischemia) and limited oxygen extraction due to capillary dysfunction? If so, how do we distinguish the two, experimentally and in patients? Do flow-metabolism coupling mechanisms adjust CBF to optimize tissue oxygenation when capillary dysfunction impairs oxygen extraction downstream?Cardiovascular risk factors such as age, hypertension, diabetes, hypercholesterolemia, and smoking increase the risk of both stroke and dementia. The capillary dysfunction phenomenon therefore forces us to consider whether changes in capillary morphology or blood rheology may play a role in the etiology of some stroke subtypes and in Alzheimer’s disease.Here, we discuss whether certain disease characteristics suggest capillary dysfunction rather than primary flow-limiting vascular pathology and how capillary dysfunction may be imaged and managed.

Concepts: Blood, Blood vessel, Stroke, Traumatic brain injury, Neurology, Dementia, Ischemia, Hypoxia

2

Mild cognitive impairment is a common feature of Parkinson’s disease, even at the earliest disease stages, but there is variation in the nature and severity of cognitive involvement and in the risk of conversion to Parkinson’s disease dementia. This review aims to summarise current understanding of mild cognitive impairment in Parkinson’s disease. We consider the presentation, rate of conversion to dementia, underlying pathophysiology and potential biomarkers of mild cognitive impairment in Parkinson’s disease. Finally, we discuss challenges and controversies of mild cognitive impairment in Parkinson’s disease.

Concepts: Alzheimer's disease, Parkinson's disease, Dementia