Editors' Note
: Dr. Del Brutto points out that the "Teaching NeuroImages: Giant neurocysticercosis with unusual imaging manifestations" is actually a cystic hydatid disease, caused by infection with Echinococcus spp. Guo et al., authors of the NeuroImage, agree and blame a translation error for their mistake. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Karam, C., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Correlating microscopic findings with B-mode ultrasound in cervical artery dissection
In a previous study, microscopic findings in superficial temporal artery specimens in patients with cervical artery dissections suggest that the pathophysiologic mechanism is an expanding hematoma in the medial–adventitial border arising from the vasa vasorum.1 The hematoma is half-moon-shaped and can be visualized by T1-weighted fat-saturated MRI.2 (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Dittrich, R. Tags: Ultrasound, All Clinical Neurology, Carotid artery dissection NEUROIMAGES Source Type: research

Pathologic confirmation of retinal ganglion cell loss in multiple system atrophy
Multiple system atrophy (MSA) is a rare adult-onset rapidly progressive fatal neurodegenerative disorder characterized by the abnormal aggregation of misfolded α-synuclein primarily in oligodendrocytes.1 (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Mendoza-Santiesteban, C. E., Palma, J.-A., Ortuno-Lizaran, I., Cuenca, N., Kaufmann, H. Tags: Autonomic diseases, Multiple system atrophy, Optic nerve, Retina CLINICAL/SCIENTIFIC NOTES Source Type: research

Effects of MCI subtype and reversion on progression to dementia in a community sample
Objective: We sought to understand the trajectory of mild cognitive impairment (MCI) better by examining longitudinally different MCI subtypes and progression to dementia and reversion to normal cognition in a community sample. Methods: We evaluated the stability of MCI subtypes and risk of dementia over 4 biennial assessments as part of an ongoing prospective cohort study, the Sydney Memory and Ageing Study. Results: While prevalence of MCI and different MCI subtypes remains relatively stable across all assessments, reversion from MCI and transitions between different MCI subtypes were common. Up to 46.5% of participant...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Aerts, L., Heffernan, M., Kochan, N. A., Crawford, J. D., Draper, B., Trollor, J. N., Sachdev, P. S., Brodaty, H. Tags: All Cognitive Disorders/Dementia, Assessment of cognitive disorders/dementia, MCI (mild cognitive impairment) ARTICLE Source Type: research

Placebo and nocebo responses in restless legs syndrome: A systematic review and meta-analysis
Conclusions: The magnitude of the placebo response in RLS is above the threshold of minimal clinical important difference, and the frequency of adverse events is also considerable. These results are relevant to inform the design and interpretation of future clinical trials. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Silva, M. A., Duarte, G. S., Camara, R., Rodrigues, F. B., Fernandes, R. M., Abreu, D., Mestre, T., Costa, J., Trenkwalder, C., Ferreira, J. J. Tags: Clinical trials Systematic review/meta analysis, Restless legs syndrome ARTICLE Source Type: research

Role of habenula and amygdala dysfunction in Parkinson disease patients with punding
Conclusions: A breakdown of the connectivity among the crucial nodes of the reward circuit (i.e., habenula, amygdala, basal ganglia, frontal cortex) might be a contributory factor to punding in PD. This study provides potential instruments to detect and monitor punding in patients with PD. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Markovic, V., Agosta, F., Canu, E., Inuggi, A., Petrovic, I., Stankovic, I., Imperiale, F., Stojkovic, T., Kostic, V. S., Filippi, M. Tags: MRI, fMRI, Parkinson's disease/Parkinsonism, All Psychiatric disorders ARTICLE Source Type: research

Randomized trial of preladenant, given as monotherapy, in patients with early Parkinson disease
Objective: To evaluate the adenosine 2a receptor antagonist preladenant as a nondopaminergic drug for the treatment of Parkinson disease (PD) when given as monotherapy. Methods: This was a randomized, 26-week, placebo- and active-controlled, parallel-group, multicenter, double-blind trial conducted in adults diagnosed with PD for
Source: Neurology - June 5, 2017 Category: Neurology Authors: Stocchi, F., Rascol, O., Hauser, R. A., Huyck, S., Tzontcheva, A., Capece, R., Ho, T. W., Sklar, P., Lines, C., Michelson, D., Hewitt, D. J., On behalf of the Preladenant Early Parkinson Disease Study Group Tags: Parkinson's disease/Parkinsonism, Clinical trials Randomized controlled (CONSORT agreement), Class I ARTICLE Source Type: research

Early skin denervation in hereditary and iatrogenic transthyretin amyloid neuropathy
Objective: To elucidate early skin denervation in hereditary transthyretin (TTR) amyloidosis and iatrogenic TTR amyloidosis. Methods: We investigated intraepidermal nerve fiber density (IENFD) and clinical findings in 32 patients with hereditary TTR amyloidosis, 11 asymptomatic mutation carriers, 6 patients with iatrogenic TTR amyloidosis, and 23 healthy volunteers. Results: IENFD values were reduced in patients with the V30M mutation (1.9 ± 2.1 per 1 mm), patients with non-V30M mutations (5.8 ± 3.2 per 1 mm), and patients with iatrogenic TTR amyloidosis (3.5 ± 1.8 per 1 mm) compared with healthy vol...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Masuda, T., Ueda, M., Suenaga, G., Misumi, Y., Tasaki, M., Izaki, A., Yanagisawa, Y., Inoue, Y., Motokawa, H., Matsumoto, S., Mizukami, M., Arimura, A., Deguchi, T., Nishio, Y., Yamashita, T., Inomata, Y., Obayashi, K., Ando, Y. Tags: All Clinical Neurology, All Medical/Systemic disease, Peripheral neuropathy, All Genetics ARTICLE Source Type: research

HLA-A*24:02 as a common risk factor for antiepileptic drug-induced cutaneous adverse reactions
Conclusions: HLA-A*24:02 is a common genetic risk factor for cutaneous adverse reactions induced by aromatic antiepileptic drugs in the southern Han Chinese and possibly other ethnic populations. Pretreatment screening is recommended for people in southern China. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Shi, Y.-W., Min, F.-L., Zhou, D., Qin, B., Wang, J., Hu, F.-Y., Cheung, Y.-K., Zhou, J.-H., Hu, X.-S., Zhou, J.-Q., Zhou, L.-M., Zheng, Z.-z., Pan, J., He, N., Liu, Z.-S., Hou, Y.-Q., Lim, K.-S., Ou, Y.-m., Hui-Ping Khor, A., Ng, C.-C., Mao, B.-J., Liu, X Tags: All Toxicology, Patient safety, Case control studies, Antiepileptic drugs, All Genetics ARTICLE Source Type: research

Prospective association between {beta}2-microglobulin levels and ischemic stroke risk among women
Conclusion: High levels of B2M were associated with an increased risk of ischemic stroke among women. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Rist, P. M., Jimenez, M. C., Rexrode, K. M. Tags: All Cerebrovascular disease/Stroke, Case control studies, Risk factors in epidemiology ARTICLE Source Type: research

Poststroke epilepsy in long-term survivors of primary intracerebral hemorrhage
Objective: To identify the incidence and predisposing factors for development of poststroke epilepsy (PSE) after primary intracerebral hemorrhage (PICH) during a long-term follow-up. Methods: We performed a retrospective study of patients who had had their first-ever PICH between January 1993 and January 2008 in Northern Ostrobothnia, Finland, and who survived for at least 3 months. These patients were followed up for PSE. The associations between PSE occurrence and sex, age, Glasgow Coma Scale (GCS) score on admission, hematoma location and volume, early seizures, and other possible risk factors for PSE were assessed usi...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Lahti, A.-M., Saloheimo, P., Huhtakangas, J., Salminen, H., Juvela, S., Bode, M. K., Hillbom, M., Tetri, S. Tags: Incidence studies, Risk factors in epidemiology, All Epilepsy/Seizures, Intracerebral hemorrhage, Cortical localization ARTICLE Source Type: research

Distribution of lacunes in cerebral amyloid angiopathy and hypertensive small vessel disease
Objective: To evaluate whether the burden of deep and lobar lacunes differs between patients with intracerebral hemorrhage (ICH) with definite/probable cerebral amyloid angiopathy (CAA) per the Boston criteria and hypertensive small vessel disease (HTN-SVD; ICH in basal ganglia, thalami, brainstem). Methods: We defined lobar and deep lacunes similar to the topographic distribution used for ICH and cerebral microbleeds (CMBs). We then compared their distribution between patients with CAA-ICH and those with strictly deep CMB and ICH (HTN-ICH). The independent associations of lacune location with the diagnosis of CAA-ICH and...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Pasi, M., Boulouis, G., Fotiadis, P., Auriel, E., Charidimou, A., Haley, K., Ayres, A., Schwab, K. M., Goldstein, J. N., Rosand, J., Viswanathan, A., Pantoni, L., Greenberg, S. M., Gurol, M. E. Tags: Infarction, Intracerebral hemorrhage ARTICLE Source Type: research

Restless legs syndrome: Losing sleep over the placebo response
The placebo response is a vexing problem for clinical trial design and drug development. Defined as a clinical improvement that occurs in participants treated with a physiologically inert substance, the placebo response has been largely ascribed to "expectation of clinical improvement and Pavlovian conditioning."1 Other factors certainly play a role, however, including strict application of inclusion and exclusion criteria at study entry with subsequent reversion to the mean; that is, participants and investigators, knowing entry criteria for a trial, may subconsciously (or intentionally) overrate signs and sympt...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Feigin, A. Tags: Clinical trials Systematic review/meta analysis, Restless legs syndrome EDITORIALS Source Type: research

Lacunes: Black holes in our understanding of cerebral amyloid angiopathy
The origin of lacunes, small cavities in the brain, remained a matter of great controversy until Fisher1 used serial neuropathologic sections to identify thrombotic occlusion of small cerebral arteries in association with most, but not all, lacunes. Lacunes appear on MRI as black holes on sequences that are T1-weighted or T2-weighted with CSF suppression. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Smith, E. E., Lee, J.-M. Tags: Infarction, Intracerebral hemorrhage EDITORIALS Source Type: research

Spotlight on the June 6 issue
(Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching NeuroImages: Giant cystic echinococcosis with unusual imaging manifestations
A 6-year-old girl presented with a 3-month history of progressive left-eye strabismus and vision loss. MRI scan of the brain showed a single hypointense lesion in the left cerebral hemisphere with no perilesional edema or contrast enhancement (figure 1, A–C). At surgery, the lesion was shown to be a single large parasitic cyst measuring approximately 7.0 x 6.5 x 6.0 cm (figure 2, A–C). The patient underwent a complete resection and then was given antihelminthic therapy. This resulted in an uneventful recovery. Pathology confirmed a diagnosis of brain cyst with scolex. This imaging feature of giant cystic echino...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Nie, D., Xia, L., Chen, J., Shi, W., Sun, G., Guo, J. Tags: Medical care, MRI, Parasitic infections, Post-infectious, Central pain RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Fahr syndrome caused by hypoparathyroidism
A 57-year-old woman with a remote history of thyroidectomy but no prior neurologic symptoms developed seizures, fever, and coma. Limited evaluation at her local hospital in rural Haiti demonstrated serum hypocalcemia (4 mg/dL) and extensive bilateral subcortical calcification in the cerebral and cerebellar hemispheres on CT (figure). This pattern of calcification can occur in inherited conditions such as familial idiopathic basal ganglia calcification (Fahr disease) and acquired conditions (referred to as Fahr syndrome) including parathyroid dysfunction and intrauterine infection.1,2 Given our patient's severe hypocalcemia...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Dade, E., Saint-Joy, V., Haynes, N. A., Berkowitz, A. L. Tags: CT, All Clinical Neurology, Endocrine, Basal ganglia RESIDENT AND FELLOW SECTION Source Type: research

Pearls & Oy-sters: Symptomatic cerebral vasospasm on conventional angiography following temporal lobe epilepsy surgery
Cerebral vasospasm is a complication of temporal lobe epilepsy (TLE) surgery. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Rao, S., Narayanan, S., Nanjireddy, R., Mittal, S., Basha, M. Tags: Infarction, Epilepsy surgery RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A 16-year-old girl with subacute weakness and sensory loss
A previously healthy 16-year-old girl developed numbness and tingling in the lower extremities that progressed over a 10-week period to the upper limbs. Two weeks prior to admission, she also developed weakness in her arms and legs, to the point of needing help getting dressed and support for walking. She denied sphincter or systemic symptoms. During the previous year, she had complained of intermittent shooting pains and tingling down her arms. Her psychiatric history was relevant for obsessive-compulsive traits and depression, and family history included autoimmune diseases in 2 sisters, an uncle, and 2 grandparents. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Quintanilla-Bordas, C., Nourbakhsh, B., Strober, J., Raffel, C., Waubant, E. Tags: MRI, Clinical neurology history, Tics, Spinal cord trauma; see Trauma/spinal cord trauma, Transverse myelitis RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A 10-year-old boy with bilateral vision loss
A 10-year-old previously healthy Caucasian boy was referred for evaluation of bilateral loss of vision with abnormal appearing optic nerves. Two months before the referral, the patient started to exhibit a decrease in vision both at near and at distance with difficulty reading books and difficulty viewing the chalkboard at school. One month prior to the referral, the patient was seen by his primary care physician with a complaint of intermittent headaches, which varied in severity, occurring as frequently as daily. The patient had been taking ibuprofen regularly to alleviate the pain. Vital signs were within normal limits....
Source: Neurology - June 5, 2017 Category: Neurology Authors: Bulwa, Z., Nichols, J., Gupta, N. Tags: All Neuro-ophthalmology, Optic nerve, Visual loss, All Pediatric, Gene therapy RESIDENT AND FELLOW SECTION Source Type: research

Author response: Safety of domperidone in treating nausea associated with dihydroergotamine infusion and headache
We appreciate Dr. Braillon's comments on our recent article.1 (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Robbins, N. M., Goadsby, P. J. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Safety of domperidone in treating nausea associated with dihydroergotamine infusion and headache
The retrospective study by Robbins et al.1 warrants comment. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Braillon, A. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Formal faculty observation and assessment of bedside skills for 3rd-year neurology clerks
I appreciate Dr. Sethi's comments on our article,1 and his acknowledgement of the importance of bedside teaching. Measures to combat neurophobia are critical to improve neurologic diagnosis and care across health care providers. I agree that improved formal assessment and feedback of neurologic skills is an important step at the medical student level. When not accomplished formally during medical school, the future non-neurologist is less likely to have an adequate level of confidence in his or her own history and examination skills. Consequently, he or she may feel less confidant making diagnostic and therapeutic decision...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Thompson Stone, R. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Formal faculty observation and assessment of bedside skills for 3rd-year neurology clerks
I read with interest the study by Thompson Stone et al.,1 in which a formalized bedside skills evaluation (BSE) was instituted for 3rd-year medical students on the neurology clerkship. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Sethi, N. K. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In reference to "Formal faculty observation and assessment of bedside skills for 3rd-year neurology clerks," Dr. Sethi and author Dr. Thompson Stone agree that evaluating medical students' neurologic history-taking and examination skills using real patients and then giving feedback is a critical step in combating neurophobia. In reference to "Safety of domperidone in treating nausea associated with dihydroergotamine infusion and headache," Dr. Braillon expands on the safety data available for dihydroergotamine and questions several methodologic points of the study. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Alcauskas, M., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Alveolar echinococcosis presenting with simultaneous cerebral and spinal involvement
A 37-year-old Tibetan shepherdess presented with headache and weakness of the lower limbs for 10 months. Examination disclosed a positive straight-leg-raising test. Neuroimaging showed a heterogeneous mass in the left temporal lobe (figure 1) and an intradural mass in the lumbosacral canal with L4 vertebral body destruction (figure 2). Chest and abdominal CT scans were negative. Two operations yielded a tissue diagnosis of alveolar echinococcosis and postoperative albendazole therapy was started. Simultaneous involvement of brain and the spinal cord in primary extrahepatic alveolar echinococcosis is rare. Early diagnosis a...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Cheng, J., Meng, J., He, W., Hui, X. Tags: CT, MRI, Parasitic infections, Spinal cord infection NEUROIMAGES Source Type: research

Balo concentric sclerosis evolving from apparent tumefactive demyelination
We present a patient whose initial MRI was consistent with TD but who later developed MRI characteristics in keeping with BCS, emphasizing the difficulty in distinguishing accurately between different subtypes of demyelination early in their course. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Hardy, T. A., Corboy, J. R., Weinshenker, B. G. Tags: MRI, PET, All Demyelinating disease (CNS) CLINICAL/SCIENTIFIC NOTES Source Type: research

Practice guideline summary: Reducing brain injury following cardiopulmonary resuscitation: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology
Objective: To assess the evidence and make evidence-based recommendations for acute interventions to reduce brain injury in adult patients who are comatose after successful cardiopulmonary resuscitation. Methods: Published literature from 1966 to August 29, 2016, was reviewed with evidence-based classification of relevant articles. Results and recommendations: For patients who are comatose in whom the initial cardiac rhythm is either pulseless ventricular tachycardia (VT) or ventricular fibrillation (VF) after out-of-hospital cardiac arrest (OHCA), therapeutic hypothermia (TH; 32–34°C for 24 hours) is highly li...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Geocadin, R. G., Wijdicks, E., Armstrong, M. J., Damian, M., Mayer, S. A., Ornato, J. P., Rabinstein, A., Suarez, J. I., Torbey, M. T., Dubinsky, R. M., Lazarou, J. Tags: Coma, Brain trauma, Cardiac, Critical care SPECIAL ARTICLE Source Type: research

Novel mutations in dystonin provide clues to the pathomechanisms of HSAN-VI
Conclusions: Unlike the previous HSAN-VI family, our description indicates that DST mutations may be associated with a nonlethal and nonsyndromic phenotype. Neuronal loss affects large and small sensory nerve fibers as well as autonomic ones. Induced-PSC findings suggest that dystonin defect might alter proper development of the peripheral nerves. Dystonin-a2 plays a major role in the HSAN-VI phenotype. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Manganelli, F., Parisi, S., Nolano, M., Tao, F., Paladino, S., Pisciotta, C., Tozza, S., Nesti, C., Rebelo, A. P., Provitera, V., Santorelli, F. M., Shy, M. E., Russo, T., Zuchner, S., Santoro, L. Tags: Peripheral neuropathy, Gene expression studies ARTICLE Source Type: research

Skin nerve phosphorylated {alpha}-synuclein deposits in idiopathic REM sleep behavior disorder
Conclusions: Our preliminary findings suggest that skin biopsy in patients with iRBD might be a safe and sensitive procedure to be further tested in order to detect p-α-syn deposits in the premotor stage of synucleinopathies. Classification of evidence: This study provides Class III evidence that p-α-syn skin deposits identify patients with iRBD. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Antelmi, E., Donadio, V., Incensi, A., Plazzi, G., Liguori, R. Tags: All Movement Disorders, Parkinson's disease/Parkinsonism, All Sleep Disorders, Parasomnias ARTICLE Source Type: research

Endovascular therapy for ischemic stroke: Save a minute--save a week
Conclusions: Small reductions in endovascular delays lead to marked health benefits over patients' lifetimes. Services need to be optimized to reduce delays to endovascular therapy. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Meretoja, A., Keshtkaran, M., Tatlisumak, T., Donnan, G. A., Churilov, L. Tags: Outcome research, Quality of life, All Cerebrovascular disease/Stroke, Models of care, Infarction ARTICLE Source Type: research

Autologous hematopoietic stem cell transplantation in multiple sclerosis: A meta-analysis
Conclusions: The emerging evidence on this therapeutic approach in MS indicates that the largest benefit/risk profile form this therapeutic approach can be obtained in patients with aggressive MS with a relapsing-remitting course and who have not yet accumulated a high level of disability. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Sormani, M. P., Muraro, P. A., Schiavetti, I., Signori, A., Laroni, A., Saccardi, R., Mancardi, G. L. Tags: Clinical trials Systematic review/meta analysis, Multiple sclerosis ARTICLE Source Type: research

Increased connectivity of hub networks and cognitive impairment in multiple sclerosis
Objective: To investigate default-mode network (DMN) and frontoparietal network (FPN) dysfunction in cognitively impaired (CI) patients with multiple sclerosis (MS) because these networks strongly relate to cognition and contain most of the hubs of the brain. Methods: Resting-state fMRI and neuropsychological assessments were performed in 322 patients with MS and 96 healthy controls (HCs). Patients with MS were classified as CI (z score
Source: Neurology - May 29, 2017 Category: Neurology Authors: Meijer, K. A., Eijlers, A. J. C., Douw, L., Uitdehaag, B. M. J., Barkhof, F., Geurts, J. J. G., Schoonheim, M. M. Tags: fMRI, Multiple sclerosis ARTICLE Source Type: research

BDNF Val66Met predicts cognitive decline in the Wisconsin Registry for Alzheimer's Prevention
Conclusions: In a middle-aged cohort with AD risk, carriage of the BDNF Met allele was associated with steeper decline in episodic memory and executive function. This decline was exacerbated by greater Aβ burden. These results suggest that the BDNF Val66Met polymorphism may play an important role in cognitive decline and could be considered as a target for novel AD therapeutics. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Boots, E. A., Schultz, S. A., Clark, L. R., Racine, A. M., Darst, B. F., Koscik, R. L., Carlsson, C. M., Gallagher, C. L., Hogan, K. J., Bendlin, B. B., Asthana, S., Sager, M. A., Hermann, B. P., Christian, B. T., Dubal, D. B., Engelman, C. D., Johnson, S Tags: PET, Alzheimer's disease, Cognitive aging, All Genetics ARTICLE Source Type: research

Space and location of cerebral microbleeds, cognitive decline, and dementia in the community
Conclusions: Mixed or a higher load of CMBs, with some specificity for location, is associated with accelerated cognitive decline in older people. These findings suggest a role for hypertensive vasculopathy and the combined effect of hypertensive and cerebral amyloid angiopathy in the pathogenesis of cognitive deterioration. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Ding, J., Sigurthsson, S., Jonsson, P. V., Eiriksdottir, G., Meirelles, O., Kjartansson, O., Lopez, O. L., van Buchem, M. A., Gudnason, V., Launer, L. J. Tags: MRI, Other cerebrovascular disease/ Stroke, Vascular dementia, Cognitive aging, Cohort studies ARTICLE Source Type: research

White matter microstructure, cognition, and molecular markers in fragile X premutation females
Objective: To examine the interrelationships between fragile X mental retardation 1 (FMR1) mRNA and the FMR1 exon 1/intron 1 boundary methylation, white matter microstructure, and executive function, in women with a FMR1 premutation expansion (PM; 55–199 CGG repeats) and controls (CGG
Source: Neurology - May 29, 2017 Category: Neurology Authors: Shelton, A. L., Cornish, K. M., Godler, D., Bui, Q. M., Kolbe, S., Fielding, J. Tags: DWI, Executive function, Cerebellum, Association studies in genetics ARTICLE Source Type: research

Personal reflections about Lewis P. (Bud) Rowland (1925-2017)
Editor's Note: In the previous issue of Neurology® (May 23, 2017), we lamented the passing of Dr. Rowland with a featured In Memoriam. Bud touched the lives of many, some of whom wrote comments to the AAN and the Neurology Editorial Office upon notification of his death. These comments were included at the end of the In Memoriam, but the remembrances by Dr. Fahn were received too late to include in the last issue. Here we again remember our friend through the memories of Dr. Fahn. —Robert A. Gross, MD, PhD, FAAN (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Fahn, S. Tags: IN MEMORIAM Source Type: research

The American Academy of Neurology affirms the revival of cooling for the revived
It is estimated that a cardiac arrest occurs approximately every minute in the United States.1 Beyond the mere return of spontaneous circulation (ROSC) and gross survival at discharge, good neurologic function with minimal disability is the goal for revived cardiac arrest patients. Partnering with the cardiology and emergency and critical care communities, neurologists helped implement therapeutic hypothermia (TH) and targeted temperature management (TTM), major breakthroughs in post–cardiac arrest care.2 Mitigating secondary brain injury after cardiac arrest (BICA) with TH is supported by 2 landmark randomized contr...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Kapinos, G., Becker, L. B. Tags: Coma, Cardiac, Critical care EDITORIALS Source Type: research

Endovascular thrombectomy: Time is still brain
The "Time is brain" concept should be familiar to anyone who cares for patients with acute ischemic stroke. IV tissue plasminogen activator (tPA) reduces disability after stroke when administered within 4.5 hours of symptom onset, but the benefit of IV tPA is time-dependent.1 Earlier treatment improves functional outcomes. Quantified many ways, studies provide estimates in terms of functional outcome at 90 days, neurons saved, and days of disability-free life gained.1–3 As a result, door-to-needle time, defined as the time from hospital arrival to the initiation of IV tPA, has become an important quality me...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Mullen, M. T., Chaturvedi, S. Tags: Outcome research, Quality of life, All Cerebrovascular disease/Stroke, Models of care, Infarction EDITORIALS Source Type: research

Autologous hematopoietic stem cell transplantation for MS: Safer than previously thought
Multiple sclerosis (MS) is likely initiated by an autoimmune response that targets the CNS. A common conceptualization of the early stages of MS is that immunocompetent cells of the adaptive immune system, recognizing hitherto unknown autoantigens, invade the CNS. These invasions happen in discrete attacks in focal areas of the CNS, giving rise to the well-known features of blood–brain barrier breakdown and lesion formation visible on MRI. This immune process is critically dependent on immunologic memory, and with time a pool of autoreactive lymphocytes is maintained. What if these immunologic memories could be erase...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Burman, J., Fox, R. J. Tags: Clinical trials Systematic review/meta analysis, Multiple sclerosis EDITORIALS Source Type: research

Genetics, white matter, and cognition: The effects of methylation on FMR1
The fragile X-associated tremor/ataxia syndrome (FXTAS) is an X-linked genetic disorder in which the premutation (55–200 CGG repeats) in fragile X mental retardation 1 (FMR1) causes elevated levels of FMR1 mRNA, which in turn causes RNA toxicity through sequestration of critical proteins, dysregulated DNA repair processes, and production of FMRpolyG, a toxic protein produced from repeat-associated non-AUG (RAN) translation.1,2 FXTAS is associated with tremor and ataxia, and it presents in aging carriers of the premutation. However, there is evidence that the premutation causes changes in the brain far earlier than th...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Nyquist, P. A., Hagerman, R. Tags: DWI, Executive function, Cerebellum, Association studies in genetics EDITORIALS Source Type: research

Spotlight on the May 30 issue
(Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

MS and bone marrow transplant: Not for most patients
In the article "Autologous hematopoietic stem cell transplantation in multiple sclerosis: A meta-analysis," Sormani et al.1 found studies that had already been published about autologous hematopoietic stem cell transplantation (aHSCT) for treatment of multiple sclerosis (MS) (see the next page for an introduction to aHSCT in the treatment of MS). (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Frederick, M. C., Bourdette, D. Tags: All Demyelinating disease (CNS), Multiple sclerosis PATIENT PAGES Source Type: research

Clinical Reasoning: A 45-year-old woman with immobility and incontinence
A 45-year-old woman presented with immobility and incontinence in July 2016. Her symptoms started 6 months prior to presentation, when she had multiple falls (without major injuries) at home because of weakness in the lower extremities. She experienced urinary incontinence 1 month later. An indwelling catheter was placed and clamped every 2–3 hours in the daytime and kept open during the night. Later, she preferred not to walk for fear of falling. The patient started to feel stiffness and tightness in her lower extremities 3 months later. The condition worsened gradually to the extent that she could barely move her l...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Zhang, B., Liu, G., Chen, Y., Tian, S., Laast, K., Bai, Y., Wu, Y. Tags: All Clinical Neurology, All Spinal Cord RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A 27-year-old man with acute-onset ataxia
A 27-year-old man with a history of diabetes mellitus (DM) and asthma presented to the emergency department 1 month after the onset of dysarthria and ataxia. The symptoms were noted abruptly upon waking. He swayed on standing, fell easily, and noted tremor when manipulating objects. His speech was nearly unintelligible. He also had 1 month of mild distal paresthesias and a 30-pound unintentional weight loss. He denied diplopia, dysphagia, preceding illness, or other systemic symptoms. Over the course of the month, his dysarthria improved but his imbalance remained unchanged. His delay in seeking medical attention was due t...
Source: Neurology - May 29, 2017 Category: Neurology Authors: Risco, J., Weiss, M. Tags: MRI, Gait disorders/ataxia, Peripheral neuropathy, All Demyelinating disease (CNS) RESIDENT AND FELLOW SECTION Source Type: research

Mystery Case: Don't fall for pseudo-INO!
A 31-year-old woman with a 4-day history of diplopia showed slow adducting saccades in the left eye (pseudo–internuclear ophthalmoplegia [P-INO]1,2; see video 1 at Neurology.org), left inferior rectus muscle weakness, and upper limb fatigability. Edrophonium testing with transient resolution of P-INO led to a diagnosis of myasthenia gravis (MG) (see video 2), which was later confirmed by positive acetylcholine receptor antibodies. (Source: Neurology)
Source: Neurology - May 29, 2017 Category: Neurology Authors: Traber, G. L., Valko, Y., Gulik, R., Weber, K. P. Tags: Myasthenia, Ocular motility, Diplopia (double vision) RESIDENT AND FELLOW SECTION Source Type: research

Author response: Early start of DOAC after ischemic stroke: Risk of intracranial hemorrhage and recurrent events
We thank Prof. Kawada for his interest in our article.1 We agree that larger patient cohorts more accurately estimate the risk of intracranial hemorrhage (ICH) from direct, non–vitamin K antagonist oral anticoagulants (DOACs). However, the definition of ICH is crucial. In our study we looked at symptomatic ICH (sICH) only, and observed only 1 patient with sICH.1 In contrast, the cited study of Cappellari et al.2 looked at both sICH and asymptomatic ICH: Of the 7 patients with any ICH after DOAC start, only 1 had sICH. Therefore, the rates of sICH were comparable between the 2 studies. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Seiffge, D. J., De Marchis, G. M. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Early start of DOAC after ischemic stroke: Risk of intracranial hemorrhage and recurrent events
Seiffge et al.1 evaluated the effect of direct, non–vitamin K antagonist oral anticoagulants (DOACs) or vitamin K antagonists on intracranial hemorrhage (ICH) and recurrent ischemic events in patients with acute ischemic stroke or TIA and nonvalvular atrial fibrillation (NVAF). I have a concern about the study. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Kawada, T. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: The contemporary spectrum of multiple sclerosis misdiagnosis: A multicenter study
We appreciate the comments of Dalla Costa et al. on our Contemporary Issues article.1 As discussed, this study was not designed to assess the frequency of multiple sclerosis (MS) misdiagnosis or its specific causes.1 We catalogued the diagnoses ultimately assigned to patients mistaken as having MS and reported the evaluation of the MS specialists who determined that a misdiagnosis had occurred according to their analysis of what led to misdiagnosis. Accepting this limitation, application of MS diagnostic criteria to a neurologic syndrome not typical for MS contributed to misdiagnosis in 65% of cases.1 MS diagnostic criteri...
Source: Neurology - May 22, 2017 Category: Neurology Authors: Solomon, A. J., Weinshenker, B. G. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: The contemporary spectrum of multiple sclerosis misdiagnosis: A multicenter study
We read with interest the multicenter observational study by Solomon et al.,1 which assessed the prevalence and possible causes of multiple sclerosis (MS) misdiagnosis. We are concerned about the interpretation of the results and the assertion of the conclusion that misinterpretation and misapplication of MS criteria are substantial contemporary contributors to misdiagnosis. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Dalla Costa, G., Martinelli, V., Comi, G. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research