NEURO

Stroke-like episodes (SLEs) are the hallmark of mitochondrial encephalopathy with lactic acidosis and stroke-like episode (MELAS) syndrome but rarely occur also in other specific or nonspecific mitochondrial disorders. Pathophysiologically, SLLs are most likely due to a regional disruption of the blood-brain barrier triggered by the underlying metabolic defect, epileptic activity, drugs, or other factors. SLEs manifest clinically with a plethora of cerebral manifestations, which not only include features typically seen in ischemic stroke, but also headache, epilepsy, ataxia, visual impairment, vomiting, and psychiatric abnormalities. from Journal of the Neurological Sciences https://ift.tt/2FnRRjF

Hirayama disease is a rare neurological disease affecting primarily men in the second to third decades. To date there are only few reports from Italy.We report the case of three young basketball players who presented with clinical, electrophysiological and MRI findings suggestive for Hirayama disease.Although the pathophysiology of the disease is still unknown, several hypotheses have been suggested and two of these are the disproportionate growth of cervical spine and cervical cord/roots during adolescence and the chronic traumatism. from Journal of the Neurological Sciences https://ift.tt/2CupaAS

There are virtually no population-based data on tremor in normal individuals. Using a population-based sample of 1158 normal adults ages 40–98 years in the Faroe Islands, we characterized the extent of normal action tremor across age and gender strata. Participants drew two Archimedes spirals with each hand, and tremor was systematically quantified by a senior movement disorder neurologist using a reliable and valid ordinal rating scale (ratings = 0–3). Tremor was nearly universal - 1145 (98.9%) participants had a total mean spiral score > 0. from Journal of the Neurological Sciences https://ift.tt/2UMDBY5

Anticoagulant therapy often requires temporary interruption. Nevertheless, the frequency and clinical characteristics of stroke patients who develop stroke during anticoagulant interruption are not fully known. from Journal of the Neurological Sciences https://ift.tt/2OiuCeT

Both conventional and quantitative electromyography (Q-EMG) studies have been conducted in relatively large samples in common myopathies such as muscle dystrophies, inflammatory myopathies and mitochondrial myopathies [1,2]. These studies report a rather modest diagnostic sensitivity, with lowest sensitivity values found in mitochondrial myopathies [3]. In contrast, motor unit action potential (MUP) analysis appears very sensitive in sporadic inclusion body myositis, where a decreased mean duration is observed in 94% of the patients [4]. from Journal of the Neurological Sciences https://ift.tt/2Hwfxpt

The Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS) is a common cognitive screening tool. However, administration and scoring can be time-consuming, and its use of proprietary subtests like the California Verbal Learning Test – II (CVLT-II) is financially limiting. Use of the non-proprietary Rey Auditory Verbal Learning Test (RAVLT) may be provide a valid alternative. from Journal of the Neurological Sciences https://ift.tt/2TLhk0q

from Neurology recent issues https://ift.tt/2HGYel4

Stroke is a leading cause of adult disability. Understanding the role of genetics in stroke outcome could provide novel strategies for improving stroke prognosis and identify new targets to reduce disability caused by stroke. A large number of factors contribute to outcome in patients with stroke, including prior functional and cognitive status, comorbid disease, initial severity of stroke, treatments, complications, brain plasticity, and rehabilitation. 1 How genes influence stroke outcome and interact with these factors remains unclear and needs investigation. from Neurology recent issues https://ift.tt/2HtHRZF

Cerebrovascular disease is increasingly recognized to have a substantial influence on cognition in aging, Alzheimer dementia (AD), and other dementias. 1,2 Biomarkers are urgently needed for the diagnosis, prevention, and treatment of vascular contributions to cognitive impairment. Several magnetic resonance vascular imaging markers have been proposed for cerebrovascular disease, including white matter hyperintensities (WMH), microbleeds, and lacunes, but these have variable sensitivity and specificity. Enlarged perivascular spaces (EPVS), a marker long recognized in radiologic and pathologic studies in association with cerebrovascular disease, have received less attention. Perivascular spaces are pia-lined channels that follow arterioles deep within the gray and white matter. Perivascular and related cells and fluid are implicated in the new and expanding field of glymphatics, which is the brain transport system involved in waste clearance, perivascular transport, and other physiolog

Delirium is a state of acute brain dysfunction, affecting as many as 50% of older patients in hospital, and is associated with prolonged hospitalization, high health care costs, long-term cognitive decline and dementia, and a substantially increased risk of mortality. 1–3 One postulated mechanism is that delirium results from the breakdown of brain network dynamics triggered by a stressor (e.g., major surgery, general anesthesia, infections, or psychoactive drugs) in individuals with preexisting low brain resilience due to deficits in connectivity or plasticity. 2 Multiple lines of evidence support a strong relationship between delirium and dementia and that these conditions share some pathophysiologic mechanisms, including acetylcholine deficiency, inflammation, and reduced cerebral oxidative metabolism. 3 from Neurology recent issues https://ift.tt/2HuBwgD

In addition to the primary damage, traumatic spinal cord injury (SCI) involves complex pathophysiologic processes at distant cord areas from the injury. The study by David et al. 1 assessed the interaction between degenerative processes at and caudal to a cervical SCI lesion using diffusion tensor imaging (DTI)–derived metrics including fractional anisotropy, axial diffusivity, radial diffusivity, and mean diffusivity, and their relation to electrophysiologic and clinical measures of the lower extremity. DTI metrics are sensitive biomarkers of SCI between acute and chronic stages, and has the potential to provide insight into axonal integrity within and adjacent to the SCI lesion noninvasively. 2 from Neurology recent issues https://ift.tt/2HEPyLS

Articles appearing in the December 2018 issue from Neurology recent issues https://ift.tt/2HseC9x

The field of neuro-oncology has recently experienced a renaissance in the understanding of the molecular underpinnings and pathophysiology of glioma. Genetic markers have significant implications regarding treatment responsiveness and prognosis and are now the primary basis for classification. This article gives an updated understanding of the pathogenesis and mechanisms of resistance of glioma via discussion of 4 molecular and genetic markers: MGMT, IDH , 1p/19q, and TERT . from Neurology recent issues https://ift.tt/2OdBTg3

Wilder Penfield's contributions to the structure–function relationships of the brain are well-known. Less well-known is the influence that Ivan Pavlov and the conditioned reflex had on Penfield's understanding of epileptogenesis, and on his concept of the acquisition of memories, language, and perception—what Penfield referred to as the physiology of the mind. Penfield invoked conditioned reflexes to explain responses to electrocortical stimulation of the temporal lobes that encompass memory, perception, and affect. Penfield referred to these responses as experiential phenomena since he considered that they constituted a record of past experiences. Penfield also invoked the conditioned reflex to explain the acquisition of the interpretive aspects of written and spoken language in the dominant temporal cortex. This article describes and discusses these neglected aspects of Penfield's work, and how they contributed to a broader understanding of the functional integration of t

Looking out from atop Uganda National Mosque's minaret, one of the highest points in Kampala, the capital city of Uganda, one sees winding narrow roads turning bright orange with rainfall converging to meet at the mosque in Old Kampala. These roads are congested with a lawless swirl of traffic involving pedestrians, matatus (communal taxis), and boda-bodas (motorbikes). There are women in colorful gomesis (traditional dress in the Buganda kingdom), men in dress shirts, and children selling fruit on the side of the roads, all working under the heat of the equatorial sun to bring life to this beautiful, chaotic city. As a resident in my second year of neurology training, I had the opportunity to visit and participate in care for this Ugandan community. I spent 6 weeks in Kampala on an international elective rotation coordinated through the Yale/Stanford Johnson & Johnson Global Health Scholars Program at Mulago Hospital, an experience that enhanced my training as a neurologist an

Neurologic diseases are the leading cause group of disability-adjusted life-years (DALYs) and the second leading cause group of death, with most DALYs and deaths occurring in low-income countries. 1 The WHO has documented a deficiency of neurologists in low-income countries (0.03–0.13 per 100,000 population), which contrasts with upper-middle income and high-income countries (1.09–4.5 per 100,000 population). 2 Approximately 26 million people live in sub-Saharan African (SSA) nations where there are no neurologists. 3 Furthermore, there are few opportunities to receive clinical training in neurology within Africa, with fewer than 10 countries with neurology training programs. 4 The scarcity of neurologists not only leads to poor patient outcomes but also discourages the young generation of doctors in countries such as Uganda from a career in neurology. 5 from Neurology recent issues https://ift.tt/2Obuii6

Muraro et al. highlighted recent reports of rare complications related to alemtuzumab. While adverse events associated with this highly effective multiple sclerosis therapy have been identified in clinical trials, the importance of postmarketing surveillance cannot be understated. from Neurology recent issues https://ift.tt/2Fk1IYO

We read with interest the range of cases highlighting rare side effects of the monoclonal antibody alemtuzumab. 1 We reported a case involving a 51-year-old woman who presented with breathlessness 1 month postinitiation of alemtuzumab therapy for treatment of multiple sclerosis. 2 Results of imaging, lung function testing, and bronchoscopy were consistent with a noninfectious pneumonitis. She had complete clinical and radiologic response to treatment with oral steroids. The time course of her symptoms, their subsequent resolution, and the lack of alternative precipitant made alemtuzumab the likely causative agent. It was, therefore, prudent not to repeat the course of alemtuzumab and, instead, adopt an alternative disease-modifying therapy. from Neurology recent issues https://ift.tt/2ObHPGb

We thank Drs. Whiteside and Trip for their comment on our editorial. 1 The authors highlight noninfectious pneumonitis as a rare potential complication of alemtuzumab. 2,3 The time course of onset in the 2 reported cases was different, with symptoms developing a few days or 1 month after infusion. Although not proven, this suggestion of immune-mediated mechanisms seem reasonable. 2 This reaction may arise from antibody-dependent cell-mediated or complement-mediated cytolysis, which leads to cytokine release and then type III/IV hypersensitivity reaction and lung-resident T-cell activation. As for the side effects highlighted in our editorial, 1 clinicians should recognize noninfectious pneumonitis as a potential rare complication of alemtuzumab, as it can respond well to corticosteroids. from Neurology recent issues https://ift.tt/2FmcRbL

In one of the largest MRI-based cross-sectional (n = 119) and prospective observational studies (n = 49) of patients with and without migraine, Messina et al. investigated morphometric brain changes associated with episodic migraine and migraine severity. While migraineurs were older than control patients in this investigation, there was no difference in total brain volume or gray matter volume at baseline or after a median of 4 years of follow-up. from Neurology recent issues https://ift.tt/2Obg2FW