Recurrence risk of ictal asystole in epilepsy
Conclusion: Our data suggest that in case of clinically suspected IA, the recording of 1 or 2 seizures is not sufficient to rule out IA. Furthermore, the high short-term recurrence risk favors aggressive treatment, including pacemaker implantation if seizure freedom cannot be achieved. (Source: Neurology)
Source: Neurology - August 21, 2017 Category: Neurology Authors: Hampel, K. G., Thijs, R. D., Elger, C. E., Surges, R. Tags: All Epilepsy/Seizures, Epilepsy monitoring ARTICLE Source Type: research

Loss of cutaneous large and small fibers in naive and L-dopa-treated PD patients
Objective: To study small and large fiber pathology in drug-naive and l-dopa–treated patients affected by Parkinson disease (PD) in early phases, before the occurrence of neuropathic electrophysiologic abnormalities. Methods: We enrolled 85 patients with idiopathic PD (male/female 49/36, age 61.3 ± 9.7 years) without electrophysiologic signs of neuropathy, including 48 participants naive to l-dopa treatment. All patients underwent clinical, functional, and morphologic assessment of sensory and autonomic nerves through dedicated questionnaires, quantitative sensory testing, sympathetic skin response, dynamic s...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Nolano, M., Provitera, V., Manganelli, F., Iodice, R., Stancanelli, A., Caporaso, G., Saltalamacchia, A., Califano, F., Lanzillo, B., Picillo, M., Barone, P., Santoro, L. Tags: Parkinson's disease/Parkinsonism, Neuropathic pain ARTICLE Source Type: research

MRI-guided focused ultrasound thalamotomy in non-ET tremor syndromes
Conclusions: Vim MRgFUS is a promising, incision-free, but nevertheless invasive technique to effectively treat tremors other than essential tremor. Future studies on larger samples and longer follow-up will further define its effectiveness and safety. Clinicaltrials.gov identifier: NCT02252380. Classification of evidence: This study provides Class IV evidence that for patients with tremor not caused by essential tremor, MRgFUS of the Vim improves the tremor of the contralateral hemibody at 6 months. (Source: Neurology)
Source: Neurology - August 21, 2017 Category: Neurology Authors: Fasano, A., Llinas, M., Munhoz, R. P., Hlasny, E., Kucharczyk, W., Lozano, A. M. Tags: Dystonia, Parkinson's disease/Parkinsonism, Tremor, Clinical trials Observational study (Cohort, Case control), Surgery/Stimulation ARTICLE Source Type: research

Movement disorder in GNAO1 encephalopathy associated with gain-of-function mutations
Objective: To define molecular mechanisms underlying the clinical spectrum of epilepsy and movement disorder in individuals with de novo mutations in the GNAO1 gene. Methods: We identified all GNAO1 mutations reported in individuals with epilepsy (early infantile epileptiform encephalopathy 17) or movement disorders through April 2016; 15 de novo mutant alleles from 25 individuals were introduced into the Gαo subunit by site-directed mutagenesis in a mammalian expression plasmid. We assessed protein expression and function in vitro in HEK-293T cells by Western blot and determined functional Gαo-dependent cycli...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Feng, H., Sjögren, B., Karaj, B., Shaw, V., Gezer, A., Neubig, R. R. Tags: Chorea, Gene expression studies, Infantile spasms ARTICLE Source Type: research

Caroline M. Klein, MD, PhD (1960-2017)
Caroline Marie Klein, MD, PhD, of Chapel Hill, North Carolina, died on April 27, 2017, at age 56, following a courageous 6-year battle with cancer. Dr. Klein was a noted expert in disorders of the autonomic nervous system and of the peripheral nervous system. (Source: Neurology)
Source: Neurology - August 21, 2017 Category: Neurology Authors: Dyck, P. J. B., Howard, J. F., Chahin, N. Tags: IN MEMORIAM Source Type: research

DNA methylation predicts stroke outcome better: The epigenetic clock is ticking
As a leading cause of disability worldwide, stroke renders people disabled through several mechanisms. Over half of patients have sensorimotor and cognitive deficits, one-third experience depression and social disability, and half never return to work. Our ability to predict recurrent stroke and disability poststroke remains poor despite modern medical advances.1 The uncertainty surrounding the recovery process creates critical knowledge gaps for clinicians deciding on the most appropriate care plan, and leads to substantial anxiety for patients and their families. Identification of factors that affect prognosis after stro...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Wang, L., Pare, G., Rundek, T. Tags: EDITORIALS Source Type: research

Recurrent ictal asystole: Are we doing enough to prevent and treat it?
Mortality rates are considerably higher in people with epilepsy.1,2 Sudden unexpected death in epilepsy (SUDEP) is the most frightening consequence of uncontrolled epileptic seizures.3 The underlying pathophysiology of SUDEP is unclear; however, the best-documented mechanism is the effect of seizures on the heart, either directly or secondary to apnea.3 Since increased frequency of generalized tonic-clonic seizures is the most important risk factor for SUDEP, the current strategy to prevent SUDEP is to achieve seizure control.4 Although cardiac rhythm abnormalities occur in people with epilepsy, they are underrecognized an...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Morita, M. E., Cendes, F. Tags: All Epilepsy/Seizures, Epilepsy monitoring EDITORIALS Source Type: research

Epileptic encephalopathy, movement disorder, and the yin and yang of GNAO1 function
GNAO1 encephalopathy comprises a spectrum of neurologic phenotypes that result from de novo heterozygous mutations in GNAO1, a gene coding for the subunit of a G protein that is highly expressed in the CNS and is involved in second messenger signaling. De novo heterozygous mutations in the gene were first described in patients with a severe, infantile-onset epileptic encephalopathy known as Ohtahara syndrome.1 However, patients with a predominant motor disorder, characterized by infantile hypotonia developing into severe chorea and dystonia, have also been identified.2,3 While it is not unusual for novel neurogenetic disor...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Pearson, T. S., Helbig, I. Tags: Chorea, Gene expression studies, Infantile spasms EDITORIALS Source Type: research

Spotlight on the August 22 issue
(Source: Neurology)
Source: Neurology - August 21, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

2017 Emerging Science Abstracts
The Science Committee is committed to presenting the best neuroscientific research at the Annual Meeting and is pleased to share the following Emerging Science abstracts, which qualified by having key aspects of research conducted after the October 24, 2016, abstract submission deadline. They are new and of sufficient scientific importance to warrant expedited presentation and publication. (Source: Neurology)
Source: Neurology - August 21, 2017 Category: Neurology Tags: AAN ANNUAL MEETING ABSTRACTS Source Type: research

Teaching Video NeuroImages: Missing toe: The relevance of the Brissaud reflex
A 70-year-old man who was admitted to neurointensive care with spastic tetraparesis and altered level of consciousness due to acute subdural hematoma and who had a history of left forefoot amputation presented right Babinski sign and left Brissaud reflex (video at Neurology.org). The Brissaud reflex is characterized by a contraction of the tensor fasciae latae due to stimulus over the plantar aspect of the foot, which is better visualized on the lateral aspect of the thigh.1,2 This is a useful neurologic sign in patients with suspected upper motor neuron disease and absent hallux. It was named after Édouard Brissaud...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Nobrega, F. R., Macedo, P. J. O. M., Vasconcellos, L. F. R. Tags: Clinical neurology examination, Intracerebral hemorrhage RESIDENT AND FELLOW SECTION Source Type: research

Teaching Video NeuroImages: Nine syndrome in inferior paramedian pontine infarction: More than meets the eye
A 54-year-old man with a medical history notable for diabetes mellitus and coronary artery bypass surgery presented to the emergency department with acute-onset vertigo, diplopia, hemiparesis, and hemihypesthesia of his left arm and leg. On cranial nerve examination, he displayed a conjugate right horizontal gaze palsy, diminished right eye adduction, horizontal left-beating nystagmus on leftward gaze, paralytic pontine exotropia of the left eye, gaze-evoked vertical nystagmus, and peripheral right facial palsy (figure 1; video at Neurology.org). These features were consistent with an ischemic pontine lesion affecting the ...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Maas, R. P. P. W. M., Verrips, A. Tags: DWI, All Neuro-ophthalmology, Ocular motility, Nystagmus, Infarction RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Huge carotid artery aneurysm in TSC2/PKD1 contiguous gene syndrome
A 1-day-old infant developed heart murmur with tachypnea. Echocardiography showed multiple biventricular cardiac rhabdomyomas (figure, A). Brain sonography showed multiple cerebral cortical tubers (figure, B) and subependymal nodules (figure, D and E), confirming tuberous sclerosis. Bilateral renal cysts were present (figure, C). (Source: Neurology)
Source: Neurology - August 21, 2017 Category: Neurology Authors: Wang, B., Tu, Y.-F., Tsai, Y.-S. Tags: All Imaging, All Pediatric, All Education, All Genetics RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Extensive cortical involvement in Creutzfeldt-Jakob disease
A 53-year-old alcoholic man presented with a 2-day history of worsening confusion. Initial examination showed appendicular hypertonia with multifocal arrhythmic asynchronous myoclonic jerks suggestive of cortical myoclonus occurring spontaneously intermittently and stimulus sensitive, with exaggerated deep tendon reflexes and extensor plantars. MRI brain demonstrated low-grade restricted diffusion affecting the entire cerebral cortex (figure 1), sparing the subcortical gray matter and cerebellum.1 T1-weighted imaging and fluid-attenuated inversion recovery sequences showed mild cerebral atrophy. 14-3-3 protein testing was ...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Hettige, S., Badve, M., Narasimhan, M., Masters, L., Wang, S. Tags: DWI, Prion, Prion disease; see Infections/prion RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A 57-year-old man with unilateral anosmia, papilledema, and meningismus
A 57-year-old previously healthy man was referred to our clinic for bilateral vision loss. (Source: Neurology)
Source: Neurology - August 21, 2017 Category: Neurology Authors: Bradshaw, M. J., Bloch, K. C., Davis, L. T., Craig-Owens, L. D., Ely, K., Longmuir, R. Tags: Meningitis, Fungal infections, All Neuro-ophthalmology, Metastatic tumor RESIDENT AND FELLOW SECTION Source Type: research

Response to pain
She is lying there behind a glass wall too covered in white: air too white, people too white crowded around her funeral chorus of ridiculous moves: her arm is raised and left to fall her eyes are opened and left to close her blood is food to a machine that turns it white, its sins forgiven. White coats, birds of prey! where have you taken her? my faith in you has vanished like a hunted animal you seek to give your prisoner the gift of pain so she would rise in screams above the whiteness of that stiffening bed. In vain! you absurd creatures! what can you hope to save in violence? A soul? not possible. The body alone? not p...
Source: Neurology - August 21, 2017 Category: Neurology Authors: Parvan, A. Tags: All Cerebrovascular disease/Stroke REFLECTIONS: NEUROLOGY AND THE HUMANITIES Source Type: research

The role of genetics in ethanol-induced cell death in the forebrain and brainstem
(Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Escalation therapy for multiple sclerosis in Austria: Analysis of gender differences in the nationwide registry
(Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Author response: Autopsy validation of 123I-FP-CIT dopaminergic neuroimaging for the diagnosis of DLB
Professor Abe raises the important issue of the involvement of Lewy body disease in the substantia nigra (SN) when making the diagnosis of dementia with Lewy bodies (DLB). It has long been known that, even later on in their illness, about 25% of patients with DLB do not show features of parkinsonism. This is recognized in the consensus criteria for DLB, which do not require parkinsonism for diagnosis. Patients with DLB can have normal dopaminergic imaging because, as with our 3 patients who also had no parkinsonism,1 there is no significant involvement of the SN by Lewy body disease. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Thomas, A. J., Attems, J., Colloby, S. J., O'Brien, J. T., McKeith, I., Walker, R., Lee, L., Burn, D., Lett, D. J., Walker, Z. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Autopsy validation of 123I-FP-CIT dopaminergic neuroimaging for the diagnosis of DLB
The article by Thomas et al.1 validated 123I-FP-CIT dopaminergic neuroimaging for the diagnosis of autopsy-proven dementia with Lewy bodies (DLB). The authors depicted 3 patients with DLB who met pathologic criteria for Lewy body disease but had normal 123I-FP-CIT imaging.1 Although further description concerning severity of parkinsonism remains unclear, these patients may not have severe parkinsonism. Thus, the patients' dopaminergic neuroimaging may not be included in scans without evidence of dopaminergic deficit. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Abe, K. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Hyperbaric oxygen: B-Level evidence in mild traumatic brain injury clinical trials
We thank Drs. Hampson and Holm for commenting on our Views & Reviews article.1 It is true that PaO2 was not measured in the published studies and assumptions were made regarding normal metabolism and pulmonary function of the study participants. This was a valid assumption, as all participants were cleared to be placed inside a pressure vessel and not excluded due to pulmonary or metabolic dysfunctions. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Figueroa, X. A., Wright, J. K. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Hyperbaric oxygen: B-Level evidence in mild traumatic brain injury clinical trials
In their article, Drs. Figueroa and Wright1 reported a reanalysis of hyperbaric oxygen's effect on mild traumatic brain injury and claimed that oxygen content of arterial blood plasma (oxygen dissolved in plasma) during hyperbaric exposure correlates with treatment response. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Hampson, N. B., Holm, J. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In "Hyperbaric oxygen: B-level evidence in mild traumatic brain injury clinical trials," Drs. Figueroa and Wright reviewed published clinical trials on the effect of hyperbaric oxygen therapy (HBOT) on mild to moderate traumatic brain injury/persistent postconcussion syndrome (mTBI/PPCS) and concluded that HBOT has therapeutic effects on mTBI/PPCS symptoms and can alleviate posttraumatic stress disorder symptoms secondary to a brain injury. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Karam, C., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Supranuclear gaze palsy and horizontal ocular oscillations in Creutzfeldt-Jakob disease
A 59-year-old patient presented with marked cognitive impairment. Examination revealed supranuclear gaze palsy and ocular oscillations (video at Neurology.org). Extremity and axial muscle tone was increased, accompanied by full body tremulousness suggestive of polymyoclonus. Motor abnormalities had progressed over 3 months but cognitive problems developed in 1–2 weeks. MRI (figure) and CSF (positive real-time quaking-induced conversion) were diagnostic of Creutzfeldt-Jacob disease (CJD). (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Wallach, A. I., Park, H., Rucker, J. C., Kaufmann, H. Tags: Prognosis, All Neuro-ophthalmology, Nystagmus, Prion disease; see Infections/prion, Assessment of cognitive disorders/dementia VIDEO NEUROIMAGES Source Type: research

VCP-related multisystem proteinopathy presenting as early-onset Parkinson disease
The valosin-containing protein (VCP) is involved in a plethora of cellular processes including membrane dynamics, DNA damage response, and protein quality control.1 Its essential role in humans is highlighted by diverse clinical phenotypes linked to VCP mutations: (1) inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD); (2) amyotrophic lateral sclerosis; (3) Charcot-Marie-Tooth disease type 2; and (4) hereditary spastic paraplegia (reviewed by Evangelista et al.2). Moreover, mutant VCP has been implicated in the pathogenesis of Parkinson disease (PD).3 (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Regensburger, M., Türk, M., Pagenstecher, A., Schröder, R., Winkler, J. Tags: All Medical/Systemic disease, Parkinson's disease/Parkinsonism, Peripheral neuropathy, Muscle disease CLINICAL/SCIENTIFIC NOTES Source Type: research

Effects of exercise on fitness and health of adults with spinal cord injury: A systematic review
Conclusions: Exercise improves fitness and cardiometabolic health of adults with chronic SCI. The evidence on effective exercise types, frequencies, intensities, and durations should be used to formulate exercise guidelines for adults with SCI. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: van der Scheer, J. W., Martin Ginis, K. A., Ditor, D. S., Goosey-Tolfrey, V. L., Hicks, A. L., West, C. R., Wolfe, D. L. Tags: Clinical trials Systematic review/meta analysis, All Rehabilitation, All Spinal Cord VIEWS & amp;amp; REVIEWS Source Type: research

Quality improvement in neurology: Inpatient and emergency care quality measure set: Executive summary
The emergence of inpatient neurologic specialists including neurohospitalists and neurointensivists has led to an increased focus on the quality of neurologic care delivered in the hospital. Each year, there are over 1.5 million nonsurgical neurologic hospital discharges in the United States, making neurology a large inpatient specialty even without accounting for nonadmitted emergency patients.1 National quality metrics in other non-neurologic specialties have focused primarily on inpatient care since hospitals, insurers, professional organizations, and the general public have turned their attention to outcomes achieved d...
Source: Neurology - August 14, 2017 Category: Neurology Authors: Josephson, S. A., Ferro, J., Cohen, A., Webb, A., Lee, E., Vespa, P. M. Tags: All Health Services Research, Critical care, Delirium, All Practice Management SPECIAL ARTICLE Source Type: research

Anoctamins (TMEM16 proteins): Functions and involvement in neurologic disease
Anoctamins (anion channels with 8 transmembrane domains [ANO]), also known as transmembrane proteins with 16 domains (TMEM16), are highly conserved intracellular calcium (Ca2+)–activated proteins with multiple cellular functions. The ANO/TMEM16 family includes 10 members (ANO1/TMEM16A to ANO10/TMEM16K), with distinct distributions in tissues. Some of these proteins, such as ANO1 and ANO2, are prototypical Ca2+-activated chloride (Cl–) channels. Others, including ANO6, mediate Ca2+-dependent exposure of phospholipids that are normally expressed in the inner leaflet of the membrane to the extracellular surface; t...
Source: Neurology - August 14, 2017 Category: Neurology Authors: Benarroch, E. E. Tags: CLINICAL IMPLICATIONS OF NEUROSCIENCE RESEARCH Source Type: research

MRI evidence of acute inflammation in leukocortical lesions of patients with early multiple sclerosis
Conclusions: The presence of enhancing lesions affecting the cortex and adjacent white matter, although transient and not frequent, suggests that at least some cortical lesions are related to blood–brain barrier disruption. Our data support the concept that there may be an acute inflammatory phase in the development of leukocortical MS lesions. Clinicaltrials.gov identifier: NCT00176592. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Maranzano, J., Rudko, D. A., Nakamura, K., Cook, S., Cadavid, D., Wolansky, L., Arnold, D. L., Narayanan, S. Tags: MRI, Multiple sclerosis ARTICLE Source Type: research

Open-label trial of ranolazine for the treatment of myotonia congenita
Objective: To determine open-label, pilot study whether ranolazine could improve signs and symptoms of myotonia and muscle stiffness in patients with myotonia congenita (MC). Methods: Thirteen participants were assessed at baseline and 2, 4, and 5 weeks. Ranolazine was started after baseline assessment (500 mg twice daily), increased as tolerated after week 2 (1,000 mg twice daily), and maintained until week 4. Outcomes included change from baseline to week 4 in self-reported severity of symptoms (stiffness, weakness, and pain), Timed Up and Go (TUG), hand grip and eyelid myotonia, and myotonia on EMG. Results: Self-repo...
Source: Neurology - August 14, 2017 Category: Neurology Authors: Arnold, W. D., Kline, D., Sanderson, A., Hawash, A. A., Bartlett, A., Novak, K. R., Rich, M. M., Kissel, J. T. Tags: All Neuromuscular Disease, Muscle disease, EMG, Ion channel gene defects ARTICLE Source Type: research

Effect of gluten-free diet on cerebellar MR spectroscopy in gluten ataxia
Conclusions: The demonstration of increased NAA/Cr ratio on repeat scanning following strict GFD strengthens previous findings of clinical improvement of the ataxia in patients with GA. The presence of enteropathy is not a prerequisite for such improvement; therefore patients with positive serology and negative duodenal biopsy should still be treated with strict GFD. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Hadjivassiliou, M., Grünewald, R. A., Sanders, D. S., Shanmugarajah, P., Hoggard, N. Tags: MRS, Autoimmune diseases, All Clinical Neurology, Gait disorders/ataxia ARTICLE Source Type: research

Florbetapir imaging in cerebral amyloid angiopathy-related hemorrhages
Conclusions: Cortical florbetapir uptake is increased in patients with CAA-related ICH relative to those with deep ICH. Although 18F-florbetapir PET can label vascular β-amyloid and might serve as an outcome marker in future clinical trials, its diagnostic value in acute CAA-related ICH seems limited in clinical practice. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Raposo, N., Planton, M., Peran, P., Payoux, P., Bonneville, F., Lyoubi, A., Albucher, J. F., Acket, B., Salabert, A. S., Olivot, J. M., Hitzel, A., Chollet, F., Pariente, J. Tags: PET, Intracerebral hemorrhage ARTICLE Source Type: research

Long-term antithrombotic treatment in intracranial hemorrhage survivors with atrial fibrillation
Conclusions: In observational studies, anticoagulation with VKA is associated with a lower rate of IS than APA or no-ATM without increasing ICH recurrence significantly. A randomized controlled trial is needed to determine the net clinical benefit of anticoagulation in ICH survivors with AF. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Korompoki, E., Filippidis, F. T., Nielsen, P. B., Del Giudice, A., Lip, G. Y. H., Kuramatsu, J. B., Huttner, H. B., Fang, J., Schulman, S., Marti-Fabregas, J., Gathier, C. S., Viswanathan, A., Biffi, A., Poli, D., Weimar, C., Malzahn, U., Heuschmann, P., Tags: Stroke prevention, All Cerebrovascular disease/Stroke, Cardiac, Embolism, Intracerebral hemorrhage ARTICLE Source Type: research

Cardiovascular health in young adulthood and structural brain MRI in midlife: The CARDIA study
Objective: To examine the association between the American Heart Association (AHA) Life's Simple 7 (LS7) metric and brain structure. Methods: We determined cardiovascular health (CVH) according to the AHA LS7, assigning 0, 1, or 2 points for meeting poor, intermediate, or ideal criteria for the 7 components (range 0–14) at baseline (aged 18–30 years in 1985–1986) and year 25 follow-up examination for 518 participants of the Coronary Artery Risk Development in Young Adults (CARDIA) brain MRI substudy. Visit-based CVH score and average score was assessed in relation to percent of intracranial volume of nor...
Source: Neurology - August 14, 2017 Category: Neurology Authors: Bancks, M. P., Allen, N. B., Dubey, P., Launer, L. J., Lloyd-Jones, D. M., Reis, J. P., Sidney, S., Yano, Y., Schreiner, P. J. Tags: MRI, All epidemiology, Risk factors in epidemiology ARTICLE Source Type: research

Pretreatment seizure semiology in childhood absence epilepsy
Conclusions: Almost all absence seizures are characterized by a pause in activity or staring, but rarely is this the only feature. Semiologic features tend to cluster, resulting in identifiable absence seizure subtypes with significant intraparticipant seizure phenomenologic heterogeneity. One seizure subtype, pause/stare and eye involvement but no motor automatisms, is specifically associated with a worse treatment outcome. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Kessler, S. K., Shinnar, S., Cnaan, A., Dlugos, D., Conry, J., Hirtz, D. G., Hu, F., Liu, C., Mizrahi, E. M., Moshe, S. L., Clark, P., Glauser, T. A., For the Childhood Absence Epilepsy Study Group Tags: Antiepileptic drugs, Epilepsy semiology, Video/ EEG use in epilepsy, Generalized seizures, Absence seizures ARTICLE Source Type: research

Transcranial magnetic stimulation distinguishes Alzheimer disease from frontotemporal dementia
Conclusions: TMS is a noninvasive procedure that reliably distinguishes AD from FTD and HC and, if these findings are replicated in larger studies, could represent a useful additional diagnostic tool for clinical practice. Classification of evidence: This study provides Class III evidence that TMS measures can distinguish patients with AD from those with FTD. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Benussi, A., Di Lorenzo, F., Dell'Era, V., Cosseddu, M., Alberici, A., Caratozzolo, S., Cotelli, M. S., Micheli, A., Rozzini, L., Depari, A., Flammini, A., Ponzo, V., Martorana, A., Caltagirone, C., Padovani, A., Koch, G., Borroni, B. Tags: Diagnostic test assessment, Alzheimer's disease, TMS, Frontotemporal dementia, Assessment of cognitive disorders/dementia ARTICLE Source Type: research

Early and lethal neurodegeneration with myasthenic and myopathic features: A new ALG14-CDG
Conclusions: We report a genetic syndrome combining myasthenic features and severe neurodegeneration with therapy-refractory epilepsy. The underlying cause is a glycosylation defect due to mutations in ALG14. These cases broaden the phenotypic spectrum associated with ALG14 congenital disorders of glycosylation as previously only isolated myasthenia has been described. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Schorling, D. C., Rost, S., Lefeber, D. J., Brady, L., Müller, C. R., Korinthenberg, R., Tarnopolsky, M., Bönnemann, C. G., Rodenburg, R. J., Bugiani, M., Beytia, M., Krüger, M., van der Knaap, M., Kirschner, J. Tags: EMG, Neonatal, Neonatal seizures ARTICLE Source Type: research

Automatic measurement of prosody in behavioral variant FTD
Conclusions: Reduced f0 range reflects impaired prosody in bvFTD. This is associated with neuroanatomic networks implicated in language production and social disorders centered in the frontal lobe. These findings support the feasibility of automated speech analysis in frontotemporal dementia and other disorders. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Nevler, N., Ash, S., Jester, C., Irwin, D. J., Liberman, M., Grossman, M. Tags: Executive function, Frontotemporal dementia, Dementia aphasia ARTICLE Source Type: research

Inpatient quality metrics in neurology: A grand challenge
The need to ensure quality in medicine has become paramount in the current environment of health care accountability, affordability, and access. There are many tools that are used to ensure quality, including peer review, continuing medical education, maintenance of certification, formal disease-specific guidelines,1 and quality measures.2 The lines between these various tools can sometimes be blurred and lead to confusion for the clinical neurologist. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Vespa, P. M., Ferro, J., Josephson, S. A. Tags: EEG, Video/ EEG use in epilepsy, Neonatal seizures, All Genetics, Ion channel gene defects EDITORIALS Source Type: research

Affective prosody in frontotemporal dementia: The importance of "pitching it right"
"It's not what you said; it's how you said it!" How many times have you said or heard that while interacting with a family member or colleague? It is clear that tone of voice (modulations in pitch, loudness, and rhythm of speech, together called prosody) conveys a great deal of intent and emotion in nearly every exchange. Imagine that your spouse's response to a very special birthday gift is "I've never seen anything like it," spoken in a completely monotone voice. You would not be able to tell if he or she loves it or hates it. On the flip side, suppose you say to your spouse, lovingly, "I can har...
Source: Neurology - August 14, 2017 Category: Neurology Authors: Leyton, C. E., Hillis, A. E. Tags: Executive function, Frontotemporal dementia, Dementia aphasia EDITORIALS Source Type: research

Spotlight on the August 15 issue
(Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching Video NeuroImages: The underrecognized diphasic dyskinesia of Parkinson disease
Dyskinesia is a common motor complication in levodopa-treated Parkinson disease (PD), associated with higher doses, greater disease severity, and longer disease duration.1 Often assumed to be a peak-dose phenomenon, the diphasic (beginning-of-dose or end-of-dose) variant may be ignored, as exemplified by a patient with PD whose dyskinesia was initially interpreted as peak-dose (video at Neurology.org). Rapid improvement with apomorphine, a short-acting levodopa-equipotent dopamine agonist, confirmed its diphasic nature.2 Recognition of dyskinesia subtype based on the relationship with levodopa dose cycles (figure) facilita...
Source: Neurology - August 14, 2017 Category: Neurology Authors: Verhagen Metman, L., Espay, A. J. Tags: Parkinson's disease/Parkinsonism, Chorea RESIDENT AND FELLOW SECTION Source Type: research

Teaching Video NeuroImages: Trapezius muscle hypertrophy in multifocal motor neuropathy
Neurogenic muscle hypertrophy has been reported in spinal muscular atrophy, radiculopathy, postpolio syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, and multifocal motor neuropathy (MMN). (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Bourque, P. R., Al-Hajji, M., Zwicker, J., Warman Chardon, J. Tags: All Neuromuscular Disease, Peripheral neuropathy, Chronic inflammatory demyelinating polyneuropathy, Muscle disease, All clinical neurophysiology RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Classic Ramsay Hunt syndrome and associated MRI findings
An 85-year-old woman with dementia presented with left ear pain, vertigo, and mild left peripheral facial weakness of unclear chronicity. MRI demonstrated contrast enhancement of cranial nerves (CNs) VII and VIII consistent with Ramsay Hunt syndrome (RHS)1 (figure 1). She was treated with steroids and acyclovir. On evaluation 4 days later, she had developed the classic RHS triad of ear pain, ipsilateral facial paralysis, and vesicular rash, plus hyperacusis and persistent vertigo (figure 2).2 (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Labin, E., Tore, H., Alkuwaiti, M., Streib, C. Tags: All Imaging, MRI, All Clinical Neurology, Post-infectious RESIDENT AND FELLOW SECTION Source Type: research

Journal Club: Long-term functional outcome in patients with acquired infections after acute spinal cord injury
Infections, particularly pneumonia, are the primary cause of mortality in individuals with spinal cord injury (SCI).1 Several factors may contribute to the high rate of infections in the SCI population, including motor paralysis and reduced reflexes resulting in aspiration, invasive procedures, and so-called SCI-induced immune depression syndrome (SCI-IDS).2,3 SCI-IDS is thought to occur when the connection between the CNS and the immune system are disrupted by a lesion in the spinal cord, resulting in a decrease in immune function.3 In addition to increased morbidity and mortality, infections after SCI may affect neurolog...
Source: Neurology - August 14, 2017 Category: Neurology Authors: Warner, F. M., Tong, B., Jutzeler, C. R., Cragg, J. J., Scheuren, P. S., Kramer, J. L. K. Tags: All Infections, Spinal cord trauma; see Trauma/spinal cord trauma RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A demure teenager and her dystonic foot
A 13-year-old girl presented with a 4-year history of abnormal gait. At age 9, her parents noticed that she would run awkwardly "on the balls of her feet" and subsequently, that the rhythm of her running would break down with sustained exercise. There was no diurnal variation in her symptoms. There was no history of perinatal insults and early development was normal. There was no significant medical or psychiatric comorbidity and her family history was unremarkable. Examination of the patient's gait is demonstrated in video 1 at Neurology.org. (Source: Neurology)
Source: Neurology - August 14, 2017 Category: Neurology Authors: Cullinane, P. W., Browne, P., Hennessy, M. J., Counihan, T. J. Tags: Dystonia, Parkinson's disease/Parkinsonism, All Pediatric, All Genetics RESIDENT AND FELLOW SECTION Source Type: research

Child Neurology: Childhood basilar artery occlusion and stroke
Stroke is one of the major causes of childhood mortality. Pediatric arterial ischemic stroke (PAIS) has an annual estimated rate as high as 3.3 cases per 100,000 children (with the vertebrobasilar territory involved in up to 36% of cases); however, the incidence of isolated childhood basilar artery occlusion (BAO) and stroke (BAS) is unknown.1 Adult BAO carries up to a 90% mortality rate, while death or severe neurologic deficits may be seen in 50% of children with BAO/BAS.1,2 The following case report describes a 12-year-old boy with BAO leading to BAS. Clinical symptoms, differential diagnoses, associated comorbidities, ...
Source: Neurology - August 14, 2017 Category: Neurology Authors: Lobbous, M., Williams, S., Rashid, S. Tags: Childhood stroke, Stroke in young adults, All Cerebrovascular disease/Stroke, Pediatric stroke; see Cerebrovascular Disease/ Childhood stroke RESIDENT AND FELLOW SECTION Source Type: research

Optimal management of patients with asymptomatic carotid stenosis
(Source: Neurology)
Source: Neurology - August 7, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Prevalence and incidence of epilepsy: A systematic review and meta-analysis of international studies
(Source: Neurology)
Source: Neurology - August 7, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Author response: Prevalence and incidence of epilepsy: A systematic review and meta-analysis of international studies
We thank Zack and Kobau for their interest in our article.1 We agree there is marked heterogeneity among studies and acknowledged this throughout the article. We attempted to mitigate heterogeneity by using a random effects model and reported medians with 25th and 75th percentiles alongside pooled estimates to provide readers with a more complete picture of data distribution and heterogeneity. In the article, we also identified geographic outliers.1 Despite marked heterogeneity, pooled estimates and medians were similar. Together, they provide a valid overview of this literature and were accompanied in the article by appro...
Source: Neurology - August 7, 2017 Category: Neurology Authors: Jette, N., Fiest, K. M., Sauro, K. M., Wiebe, S., Patten, S. B. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research