Neurolymphomatosis of the thoracic sympathetic chain
Neurolymphomatosis (NL) is defined as lymphomatous invasion of cranial or peripheral nerves by non-Hodgkin lymphoma (NHL). While most cases of NL are due to secondary dissemination from systemic or CNS sites, in rare cases it can be the primary manifestation of the malignancy.1–3 Early recognition of the disease and its precise neuroanatomic localization is critical for successful treatment. (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: Kaulen, L. D., Foss, F. M., Fulbright, R. K., Huttner, A., Baehring, J. M. Tags: PET, All Clinical Neurology, Hematologic, Nerve tumor, All Pain CLINICAL/SCIENTIFIC NOTES Source Type: research

Traumatic brain injury may not increase the risk of Alzheimer disease
Traumatic brain injury (TBI) commonly occurs in civilian and military populations. Some epidemiologic studies previously have associated TBI with an increased risk of Alzheimer disease (AD). Recent clinicopathologic and biomarker studies have failed to confirm the relationship of TBI to the development of AD dementia or pathologic changes, and suggest that other neurodegenerative processes might be linked to TBI. Additional studies are required to determine the long-term consequences of TBI. (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: Weiner, M. W., Crane, P. K., Montine, T. J., Bennett, D. A., Veitch, D. P. Tags: MRI, PET, Alzheimer's disease, Brain trauma, Assessment of cognitive disorders/dementia VIEWS & amp;amp; REVIEWS Source Type: research

Meta-analysis of pharmacogenetic interactions in amyotrophic lateral sclerosis clinical trials
Conclusions: This study incorporated genetic data into past ALS trials to determine treatment effects in a genetic post hoc analysis. Our results suggest that we should reorient our strategies toward finding treatments for ALS, start focusing on genotype-targeted treatments, and standardize genotyping in order to optimize randomization and analysis for future clinical trials. (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: van Eijk, R. P. A., Jones, A. R., Sproviero, W., Shatunov, A., Shaw, P. J., Leigh, P. N., Young, C. A., Shaw, C. E., Mora, G., Mandrioli, J., Borghero, G., Volanti, P., Diekstra, F. P., van Rheenen, W., Verstraete, E., Eijkemans, M. J. C., Veldink, J. H., Tags: Amyotrophic lateral sclerosis, Clinical trials Systematic review/meta analysis, All Genetics ARTICLE Source Type: research

Prediction of work resumption and sustainability up to 1 year after mild traumatic brain injury
Conclusions: RTW after mTBI is a gradual process, with varying levels of RTW throughout the first year after injury. Different predictors were relevant for short- vs long-term work resumption, with occupational factors influencing short-term RTW. However, for both short- and long-term RTW, posttraumatic complaints and signs of psychological distress early after injury were relevant predictors, allowing early identification of patients at risk for problematic work resumption. (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: de Koning, M. E., Scheenen, M. E., van der Horn, H. J., Timmerman, M. E., Hageman, G., Roks, G., Spikman, J. M., van der Naalt, J. Tags: Prognosis, Clinical trials Observational study (Cohort, Case control), Brain trauma ARTICLE Source Type: research

Effects of acute intermittent hypoxia on hand use after spinal cord trauma: A preliminary study
Conclusions: This report suggests the need for further study of AIH as a plasticity "primer" for task-specific training in spinal cord injury rehabilitation. Important clinical questions remain concerning optimal AIH dosage, patient screening, safety, and effect persistence. ClinicalTrials.gov identifier: NCT01272336. (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: Trumbower, R. D., Hayes, H. B., Mitchell, G. S., Wolf, S. L., Stahl, V. A. Tags: Clinical trials Observational study (Cohort, Case control), All Rehabilitation, Spinal cord trauma, EMG, Motor Control ARTICLE Source Type: research

Comment: Is virtual reality a useful adjunct to rehabilitation after spinal cord injury?
Spinal cord injury (SCI) has devastating effects on the CNS, leading to disruption of various neuromuscular, sensory, and autonomic pathways. However, the changes induced in the nervous system extend well beyond the tracts and neurons directly disrupted starting soon after the injury.1 There is substantial neural plasticity that takes place throughout the CNS, including the cortex.2 Trying to improve function and pain after a peripheral injury by intervening at the cortical level has been explored since the mid-1990s with classic mirror box therapy for phantom limb pain.3 (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: Rammo, R., Schwalb, J. M. Tags: ARTICLE Source Type: research

Virtual reality improves embodiment and neuropathic pain caused by spinal cord injury
Conclusions: The present findings show that VR exposure using multisensory stimulation differently affected leg vs body ownership, and is associated with mild analgesia with potential for SCI neurorehabilitation protocols. (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: Pozeg, P., Palluel, E., Ronchi, R., Solca, M., Al-Khodairy, A.-W., Jordan, X., Kassouha, A., Blanke, O. Tags: Neuropathic pain, All Spinal Cord ARTICLE Source Type: research

Female sex, early-onset hypertension, and risk of dementia
Conclusions: Though midlife hypertension was more common in men, it was only associated with dementia risk in women. Sex differences in the timing of dementia risk factors have important implications for brain health and hypertension management. (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: Gilsanz, P., Mayeda, E. R., Glymour, M. M., Quesenberry, C. P., Mungas, D. M., DeCarli, C., Dean, A., Whitmer, R. A. Tags: All Cognitive Disorders/Dementia, Cohort studies, Risk factors in epidemiology ARTICLE Source Type: research

Metformin vs sulfonylurea use and risk of dementia in US veterans aged >=65 years with diabetes
Objective: To determine whether metformin is associated with a lower incidence of dementia than sulfonylureas. Methods: This was a retrospective cohort study of US veterans ≥65 years of age with type 2 diabetes who were new users of metformin or a sulfonylurea and had no dementia. Follow-up began after 2 years of therapy. To account for confounding by indication, we developed a propensity score (PS) and used inverse probability of treatment weighting (IPTW) methods. Cox proportional hazards models estimated the hazard ratio (HR) of incident dementia. Results: We identified 17,200 new users of metformin and 11,440 new ...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Orkaby, A. R., Cho, K., Cormack, J., Gagnon, D. R., Driver, J. A. Tags: Endocrine, All Cognitive Disorders/Dementia, Alzheimer's disease, Vascular dementia, All epidemiology ARTICLE Source Type: research

Change in multimodal MRI markers predicts dementia risk in cerebral small vessel disease
Conclusions: This longitudinal prospective study provides evidence that change in MRI measures including DTI, over time durations during which cognitive change is not detectable, predicts cognitive decline and progression to dementia. It supports the use of MRI measures, including DTI, as useful surrogate biomarkers to monitor disease and assess therapeutic interventions. (Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: Zeestraten, E. A., Lawrence, A. J., Lambert, C., Benjamin, P., Brookes, R. L., Mackinnon, A. D., Morris, R. G., Barrick, T. R., Markus, H. S. Tags: MRI, DWI, Other cerebrovascular disease/ Stroke, All Cognitive Disorders/Dementia, Assessment of cognitive disorders/dementia ARTICLE Source Type: research

Thrombolysis in acute ischemic stroke in patients with dementia: A Swedish registry study
We examined access to thrombolysis and its outcomes at 3 months (death, residency, and modified Rankin Scale [mRS] score). Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated with logistic and ordinal logistic regression. Results: The median age at stroke onset was 83 years in both groups. IVT was administered to 94 (7.0%) patients with dementia and 639 (9.5%) patients without dementia. The OR of receiving IVT was 0.68 (95% CI 0.54–0.86) for patients with dementia. When the analysis was repeated exclusively among patients independent in everyday activities, dementia status was no longer significant ...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Zupanic, E., von Euler, M., Kareholt, I., Contreras Escamez, B., Fastbom, J., Norrving, B., Religa, D., Kramberger, M. G., Winblad, B., Johnell, K., Eriksdotter, M., Garcia-Ptacek, S. Tags: Prognosis, All Cognitive Disorders/Dementia, Cohort studies, Infarction ARTICLE Source Type: research

Polycystic kidney disease among 4,436 intracranial aneurysm patients from a defined population
Objective: To define the association of autosomal dominant polycystic kidney disease (ADPKD) with the characteristics of aneurysmal subarachnoid hemorrhage (aSAH) and unruptured intracranial aneurysm (IA) disease. Methods: We fused data from the Kuopio Intracranial Aneurysm database (n = 4,436 IA patients) and Finnish nationwide registries into a population-based series of 53 IA patients with ADPKD to compare the aneurysm- and patient-specific characteristics of IA disease in ADPKD and in the general IA population, and to identify risks for de novo IA formation. Results: In total, there were 33 patients with ADPKD with a...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Nurmonen, H. J., Huttunen, T., Huttunen, J., Kurki, M. I., Helin, K., Koivisto, T., von und zu Fraunberg, M., Jääskeläinen, J. E., Lindgren, A. E. Tags: Stroke in young adults, Cohort studies, Subarachnoid hemorrhage, All Genetics ARTICLE Source Type: research

The beginning of precision medicine in ALS?: Treatment to fit the genes
Amyotrophic lateral sclerosis (ALS) affects approximately 1 in 400 adults of western European ancestry, making it the most common degenerative disease of the motor neuron network. ALS has a mean age at onset of 65 and 85%–90% of cases occur sporadically. Ten to fifteen percent of cases have a recognized genetic contribution, usually in known ALS gene-carrying families.1 In populations of European extraction, the commonest cause of familial ALS, accounting for up to 40% of familial cases, is the C9orf72 hexanucleotide repeat expansion.2 C9orf72 has a broader associated phenotype including frontotemporal dementia and a...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Armon, C., Hardiman, O. Tags: EDITORIALS Source Type: research

Oral antidiabetic drugs and dementia risk: Does treatment matter?
As the population ages, dementia grows as a public health problem. The rising life expectancy and the aging of the so-called baby boomer cohort translate to a substantial number of people reaching ages of high risk for age-related conditions like dementia. As a major cause of disability and dependency in elderly people, dementia puts social and economic burden on patients and their families and affects health care systems worldwide. In the absence of a cure, primary prevention will have the largest effect on the reduction of dementia occurrence.1 Thus, public health research should focus on the identification of modifiable...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Fink, A., Haenisch, B. Tags: All Cognitive Disorders/Dementia, All epidemiology EDITORIALS Source Type: research

Polycystic kidney disease and intracranial aneurysms: Some answers, but many questions remain
Unruptured intracranial aneurysms (UIA) occur in about 2%–3% of the population. Several medical conditions are associated with a UIA presence, including autosomal dominant polycystic kidney disease (ADPKD). Clinicians and patients want to know the risk of aneurysm growth, rupture, or de novo development, predictors of clinical outcomes, and the optimal initial and follow-up screening for individuals with UIA and ADPKD. Such data are unavailable. The recent American Heart Association–American Stroke Association statement on UIAs recommends that patients with familial risk and patients with conditions in which an...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Torner, J. C., Brown, R. D. Tags: EDITORIALS Source Type: research

Spotlight on the October 31 issue
(Source: Neurology)
Source: Neurology - October 30, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Virtual reality may relieve pain in patients with spinal cord injury
Body ownership is the ongoing feeling that our body and body parts belong to us, and are related to us in a different way than things in the external world. This sense of body ownership may seem trivial to us, yet our brain needs to connect sensory inputs (e.g., touch, pain, and visual information), internal inputs (from our body's internal milieu), and higher cognitive inputs like memories of our body in different events to generate the sense of ownership. For instance, hand ownership is generated by the visual input we get from our eyes that tracks its location, sensory input on its posture (known as proprioception), and...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Saadon-Grosman, N., Arzy, S. Tags: All Spinal Cord, Spinal cord trauma; see Trauma/spinal cord trauma PATIENT PAGES Source Type: research

Teaching Video NeuroImages: Hemifacial spasm due to cerebellopontine angle mass
A 21-year-old woman presented with tremor of the right eyelid that progressed in 1 year to involve the ipsilateral cheek and lip and ipsilateral hearing loss since childhood. Her lack of medical comorbidities prompted imaging that revealed a mass in the right cerebellopontine angle cistern. She underwent debulking of the mass, which resolved the tremors. Her pathology revealed pilocytic astrocytoma (figure; video at Neurology.org). Cranial nerve 7 is predisposed to injury due to its anatomic location. There are many case reports of hemifacial spasms due to mass effect.1 Imaging should be performed in otherwise healthy pati...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Guha, R., Cathcart, K., Arteaga, D. Tags: Tremor, Primary brain tumor, Adolescence RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Bilateral hypertrophic olivary degeneration following posterior circulation stroke
A 40-year-old comatose man was brought to the hospital with a history of posterior circulation stroke 4 months earlier due to hypertension. On examination, he had spastic quadriplegia, bilateral extensor plantar reflex, and palatal myoclonus. MRI revealed enlarged olives (figure 1) and chronic infarcts involving midbrain and pons (figure 2) suggestive of hypertrophic olivary degeneration seen after 4 months of insult. Hypertrophic olivary degeneration is a transsynaptic degeneration involving interconnecting fibers of inferior olivary nucleus, red nucleus, and contralateral dentate nucleus forming the 3 corners of the Guil...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Venkatesh, M., Prasad, V. R. S., Basha, S. U., Priya, G. H. J. Tags: MRI, Coma, All Cerebrovascular disease/Stroke, All Education, Infarction RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A case of ataxia, seizure, and choreoathetosis in a 34-year-old woman
A 34-year-old woman of Chinese Han descent was admitted to the neurology department after complaining of gait instability for 20 years and seizure attacks for 6 years. At age 14, she began experiencing slurred speech and unsteady gait. Meanwhile, she had learning difficulties and her academic performance was poor. Since then, her intelligence regressed gradually. At age 28, she developed recurrent generalized tonic-clonic seizures and myoclonus refractory to valproic acid and oxcarbazepine. She developed choreoathetosis at age 29. In the following years, the seizures and choreoathetosis progressively worsened. Two years la...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Xiao, F., Wang, X.-f. Tags: Gait disorders/ataxia, Chorea, Myoclonus RESIDENT AND FELLOW SECTION Source Type: research

Mystery Case: Widespread plexiform neurofibromas in neurofibromatosis type 1: An uncommon cause of back pain
A 23-year-old man with known neurofibromatosis type 1 (NF1) presented with low back pain described as a "wringing" sensation, with radiation to both legs and painful spasms upon awakening. Examination was significant for reduced reflexes bilaterally. The figure is a coronal T2-weighted MRI revealing extensive plexiform neurofibromas arising from all lumbosacral nerve roots. Neurofibromas appear as high signal intensity on T2-weighted images due to increased fluid in myelin associated with dysplastic glial proliferation.1 In patients with NF1 presenting with back pain or polyradiculopathy, imaging should be obtain...
Source: Neurology - October 30, 2017 Category: Neurology Authors: Grimsrud, K. W., Porter, A. B. Tags: MRI, Peripheral neuropathy, Nerve tumor, Neuropathic pain, Neurofibromatosis RESIDENT AND FELLOW SECTION Source Type: research

Genetics, white matter, and cognition: The effects of methylation on FMR1
(Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Author response: Teaching NeuroImages: Acute Parinaud syndrome
We thank Vanikieti et al. for their comments on our NeuroImage.1 (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Swinkin, E., Bui, E. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Teaching NeuroImages: Acute Parinaud syndrome
Drs. Swinkin and Bui1 presented a patient with bilateral ptosis, light-near dissociation, impaired vertical eye movements, paresis of superior greater than inferior rectus, and upgaze-induced convergence nystagmus (saccades) from infarction of the bilateral oculomotor nuclei and mesial thalami. They concluded that these findings were consistent with Parinaud syndrome. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Vanikieti, K., Bouffard, M., Gilbert, A., Rizzo, J. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Alterations of functional connectivity of the motor cortex in Fabry disease: An RS-fMRI study
We thank Sechi et al. for the comments on our article.1 We agree that investigating the relationship between functional changes and disease course is a core issue for defining the role of functional imaging in both pathophysiology and clinical studies in Fabry disease (FD). The findings of Ortu et al.2 help define the role of the functional connectivity (FC) alterations in patients. Indeed, in their study,2 motor cortex hyperexcitability partly subsided when enzyme replacement therapy was started (although the small number of patients and the incomplete reversal of transcranial magnetic stimulation findings, possibly relat...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Cocozza, S., Pisani, A., Brunetti, A., Quarantelli, M., Tedeschi, E. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Alterations of functional connectivity of the motor cortex in Fabry disease: An RS-fMRI study
Cocozza et al.1 confirmed, by use of resting-state (RS)–fMRI, previous clinical and electrophysiologic studies showing involvement of cerebral motor circuits in Fabry disease (FD), independently of cerebrovascular signs/symptoms and brain MRI lesions.1–3 In particular, they showed alterations of functional connectivity in a polysynaptic motor circuit involving motor cortices, basal ganglia, and right cerebellar hemisphere.1 In metabolic/lysosomal storage diseases, a subclinical CNS impairment is common and may be related, regardless of brain MRI findings, either to a structural irreversible or to a biochemical ...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Sechi, G., Demurtas, R., Boadu, W., Ortu, E. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: Motor involvement is not considered a typical feature of Fabry disease (FD). However, recent studies reported slower gait, reduced hand speed, and poorer fine manual dexterity, independently of cerebrovascular symptoms, in patients with FD. To further study alterations in motor circuits of patients with FD, Cocozza et al. used resting-state (RS)–fMRI. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Karam, C., Galetta, S. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Head flutter
A previously healthy 30-year-old man presented with oscillopsia, involuntary head movements, and imbalance. His examination showed intermittent ocular flutter (OF), bursts of back-to-back horizontal head movements time-locked with OF (head flutter) (figure; video at Neurology.org), and generalized ataxia. Head MRI, paraneoplastic antibody testing, CSF analysis, toxicology and serology panel, and CT of chest/abdomen/pelvis were unrevealing. The patient fully recovered after 6 months with monthly IV immunoglobulin. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Lemos, J., Duque, C., Araujo, R., Castelhano, J., Eggenberger, E., Nunes, C. Tags: Ocular motility VIDEO NEUROIMAGES Source Type: research

Pilocytic astrocytoma with anaplasia arising from the optic chiasm in a very elderly patient
An 81-year-old man presented with a 3-week history of impaired visual acuity (100/200 right eye, 2/200 left eye). He had a central scotoma (left eye) and bitemporal hemianopsia, with no abnormality in the optic discs. Cranial MRI suggested a tumor predominantly located at the optic chiasm and extending to the optic nerves, identified pathologically as pilocytic astrocytoma with anaplasia (PAA) (figure). He received temozolomide (for 14 months) and irradiation, but the tumor eventually extended to the hypothalamus. This unusual case of histologically proven PAA demonstrates an origin from the optic chiasm,1 which is extreme...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Hayashi, S., Akao, N., Nakazato, Y., Okamoto, K. Tags: MRI, All Neuro-ophthalmology, Optic nerve, Visual loss, Primary brain tumor NEUROIMAGES Source Type: research

COL4A2 is associated with lacunar ischemic stroke and deep ICH: Meta-analyses among 21,500 cases and 40,600 controls
Objective: To determine whether common variants in familial cerebral small vessel disease (SVD) genes confer risk of sporadic cerebral SVD. Methods: We meta-analyzed genotype data from individuals of European ancestry to determine associations of common single nucleotide polymorphisms (SNPs) in 6 familial cerebral SVD genes (COL4A1, COL4A2, NOTCH3, HTRA1, TREX1, and CECR1) with intracerebral hemorrhage (ICH) (deep, lobar, all; 1,878 cases, 2,830 controls) and ischemic stroke (IS) (lacunar, cardioembolic, large vessel disease, all; 19,569 cases, 37,853 controls). We applied data quality filters and set statistical signific...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Rannikmäe, K., Sivakumaran, V., Millar, H., Malik, R., Anderson, C. D., Chong, M., Dave, T., Falcone, G. J., Fernandez-Cadenas, I., Jimenez-Conde, J., Lindgren, A., Montaner, J., O'Donnell, M., Pare, G., Radmanesh, F., Rost, N. S., Slowik, A., So& Tags: All Cerebrovascular disease/Stroke, Infarction, Intracerebral hemorrhage, All Genetics, Association studies in genetics ARTICLE Source Type: research

UFM1 founder mutation in the Roma population causes recessive variant of H-ABC
Conclusions: UFM1 encodes ubiquitin-fold modifier 1 (UFM1), a member of the ubiquitin-like family involved in posttranslational modification of proteins. Its exact biological role is unclear. This study associates a UFM1 gene defect with a disease and sheds new light on possible UFM1 functional networks. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Hamilton, E. M. C., Bertini, E., Kalaydjieva, L., Morar, B., Dojcakova, D., Liu, J., Vanderver, A., Curiel, J., Persoon, C. M., Diodato, D., Pinelli, L., van der Meij, N. L., Plecko, B., Blaser, S., Wolf, N. I., Waisfisz, Q., Abbink, T. E. M., van der Kna Tags: MRI, Leukodystrophies, All Pediatric, All Genetics ARTICLE Source Type: research

A phase 3 randomized placebo-controlled trial of tadalafil for Duchenne muscular dystrophy
Conclusions: Tadalafil did not lessen the decline in ambulatory ability in boys with DMD. Further studies should be considered to confirm the hypothesis-generating upper limb data and to determine whether ambulatory decline can be slowed by initiation of tadalafil before 7 years of age. Clinicaltrials.gov identifier: NCT01865084. Classification of evidence: This study provides Class I evidence that tadalafil does not slow ambulatory decline in 7- to 14-year-old boys with Duchenne muscular dystrophy. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Victor, R. G., Sweeney, H. L., Finkel, R., McDonald, C. M., Byrne, B., Eagle, M., Goemans, N., Vandenborne, K., Dubrovsky, A. L., Topaloglu, H., Miceli, M. C., Furlong, P., Landry, J., Elashoff, R., Cox, D., For the Tadalafil DMD Study Group Tags: Muscle disease, Clinical trials Randomized controlled (CONSORT agreement) ARTICLE Source Type: research

Disparate effects of training on brain activation in Parkinson disease
Objective: To compare the effects of 2 forms of exercise, i.e., a 6-week trial of treadmill training with virtual reality (TT + VR) that targets motor and cognitive aspects of safe ambulation and a 6-week trial of treadmill training alone (TT), on brain activation in patients with Parkinson disease (PD). Methods: As part of a randomized controlled trial, patients were randomly assigned to 6 weeks of TT (n = 17, mean age 71.5 ± 1.5 years, disease duration 11.6 ± 1.6 years; 70% men) or TT + VR (n = 17, mean age 71.2 ± 1.7 years, disease duration 7.9 ± 1.4 years; 65% men). A previously validated f...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Maidan, I., Rosenberg-Katz, K., Jacob, Y., Giladi, N., Hausdorff, J. M., Mirelman, A. Tags: fMRI, Parkinson's disease/Parkinsonism, Executive function, Plasticity ARTICLE Source Type: research

Comment: Caffeine and PD--Time to consider other interventions
Caffeine had shown promise in a small (61 patients), short (6 weeks), randomized study1 for improving motor function in patients with Parkinson disease. Caffeine holds appeal as an inexpensive intervention that is well-tolerated in most individuals. The investigators for that small study now report the results of a multicenter randomized study2 using the same outcome. They designed the study to have approximately 4 times as many participants, an extended follow-up to evaluate the persistence of any effects, and adequate power to detect a similar effect size as observed in the smaller study. The study focused on symptomatic...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Hall, C. B. Tags: ARTICLE Source Type: research

Caffeine as symptomatic treatment for Parkinson disease (Cafe-PD): A randomized trial
Conclusion: Caffeine did not provide clinically important improvement of motor manifestations of PD (Class I evidence). Epidemiologic links between caffeine and lower PD risk do not appear to be explained by symptomatic effects. Clinicaltrials.gov identifier: NCT01738178. Classification of evidence: This study provides Class I evidence that for patients with PD, caffeine does not significantly improve motor manifestations. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Postuma, R. B., Anang, J., Pelletier, A., Joseph, L., Moscovich, M., Grimes, D., Furtado, S., Munhoz, R. P., Appel-Cresswell, S., Moro, A., Borys, A., Hobson, D., Lang, A. E. Tags: Parkinson's disease/Parkinsonism ARTICLE Source Type: research

Association of metabolic syndrome and change in Unified Parkinson's Disease Rating Scale scores
Objective: To explore the association between metabolic syndrome and the Unified Parkinson’s Disease Rating Scale (UPDRS) scores and, secondarily, the Symbol Digit Modalities Test (SDMT). Methods: This is a secondary analysis of data from 1,022 of 1,741 participants of the National Institute of Neurological Disorders and Stroke Exploratory Clinical Trials in Parkinson Disease Long-Term Study 1, a randomized, placebo-controlled trial of creatine. Participants were categorized as having or not having metabolic syndrome on the basis of modified criteria from the National Cholesterol Education Program Adult Treatment Pa...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Leehey, M., Luo, S., Sharma, S., Wills, A.-M. A., Bainbridge, J. L., Wong, P. S., Simon, D. K., Schneider, J., Zhang, Y., Perez, A., Dhall, R., Christine, C. W., Singer, C., Cambi, F., Boyd, J. T. Tags: All Medical/Systemic disease, Endocrine, Parkinson's disease/Parkinsonism ARTICLE Source Type: research

Neurogranin, a synaptic protein, is associated with memory independent of Alzheimer biomarkers
Conclusions: Synaptic dysfunction (assessed via neurogranin) may be an early pathologic process in age-related neurodegeneration, and a sensitive marker of age-related cognitive abilities, potentially preceding or even acting independently from AD pathogenesis. Synaptic functioning may be a useful early marker of cognitive aging and possibly a target for future brain aging interventions. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Casaletto, K. B., Elahi, F. M., Bettcher, B. M., Neuhaus, J., Bendlin, B. B., Asthana, S., Johnson, S. C., Yaffe, K., Carlsson, C., Blennow, K., Zetterberg, H., Kramer, J. H. Tags: Alzheimer's disease, Cognitive aging ARTICLE Source Type: research

Mixed neuropathologies and associations with domain-specific cognitive decline
Conclusions: ADNC + LBD (but not ADNC + VBI) was associated with poorer executive function and attention compared to other pathology groupings. However, the effect of co-occurring pathologies on cognitive trajectories may depend on the severity of ADNC. Future studies using antemortem biomarkers should seek to replicate these neuropathologic observations. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Brenowitz, W. D., Hubbard, R. A., Keene, C. D., Hawes, S. E., Longstreth, W. T., Woltjer, R. L., Kukull, W. A. Tags: Prognosis, Alzheimer's disease, Dementia with Lewy bodies, Vascular dementia, Cognitive neuropsychology in dementia ARTICLE Source Type: research

Brain network connectivity differs in early-onset neurodegenerative dementia
Conclusions: Disease-specific patterns of functional network topology and connectivity alterations were observed in patients with EOAD and bvFTD. Graph analysis and connectomics may aid clinical diagnosis and help elucidate pathophysiologic differences between neurodegenerative dementias. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Filippi, M., Basaia, S., Canu, E., Imperiale, F., Meani, A., Caso, F., Magnani, G., Falautano, M., Comi, G., Falini, A., Agosta, F. Tags: MRI, Alzheimer's disease, Frontotemporal dementia ARTICLE Source Type: research

Virtually reducing fall risk in Parkinson disease
Falls are common and often represent devastating events for patients with advanced Parkinson disease (PD). Prospective studies report that 70% of people with PD have at least one fall in a year, and that 39% fall recurrently. Falls have serious consequences (fractures and other injury, hospital admission, fear of falls, and an increase in caregiver burden).1,2 The few available treatment options are not highly effective. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Moreau, C., Barton, B. R., Devos, D. Tags: Parkinson's disease/Parkinsonism, All Rehabilitation, Assessment of cognitive disorders/dementia EDITORIALS Source Type: research

Can lifestyle modification slow progression of Parkinson disease?
Parkinson disease (PD), similar to other neurodegenerative conditions, is characterized by relentless clinical progression with gradual worsening of both motor and nonmotor features. Potential neuroprotective therapies focusing on aspects of neurodegeneration in PD such as impaired mitochondrial function with abnormalities of oxidative phosphorylation, increased oxidative stress, and suppressed neuroinflammation, have failed to alter the clinical course of PD.1,2 New insights into PD pathophysiology have identified potential molecular targets, including accumulation and potential prion-like spreading of aggregates containi...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Hedera, P., Davis, T. L. Tags: Parkinson's disease/Parkinsonism EDITORIALS Source Type: research

Deciphering neurodegeneration: A paradigm shift from focality to connectivity
"Wet or dry" is the fundamental dichotomy of biomarker research, referring to biofluid vs imaging-based approaches. Biomarker development in neurodegeneration has gained unprecedented momentum in recent years. Despite the substantial diagnostic advantages of molecular PET and biofluid markers, accurate prognostic indicators and sensitive monitoring markers are urgently required both for clinical trial designs and individualized patient care. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Bede, P. Tags: EDITORIALS Source Type: research

Spotlight on the October 24 issue
(Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching NeuroImages: New-onset focal epilepsy: The curious case of the camel and the jawbone
A 54-year-old right-handed woman was referred for evaluation of drug-resistant epilepsy. At age 16, while riding in a park in Perth, Western Australia, she had been thrown from her horse, which had been startled by a rogue camel. There was no significant head injury but the patient subsequently noted a "clicky" jaw. At age 37, she developed recurrent episodes involving a sense of disconnection followed by dysphasia. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Triplett, J. D., Lawn, N., Edis, R. Tags: All Imaging, All Trauma, EEG; see Epilepsy/Seizures, EEG, Partial seizures RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Hypoglossal nerve palsy due to basilar invagination
A woman in her 50s presented to the emergency room with complaints of gait instability. Her examination was notable for tongue deviation to the left. Imaging demonstrated basilar invagination and compression of the preolivary sulci near the cranial nerve 12 nerve root exit zone (figures 1 and 2). Basilar invagination is a rare craniocervical malformation, which can cause neurologic deficits and instability of the craniocervical junction.1,2 Imaging is also notable for platybasia, which is often associated with basilar invagination. The patient underwent a posterior occiput-C3 decompression and fusion without any complicati...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Bakhsheshian, J., Goshtasbi, K., Acharya, J., Buchanan, I. A., Fredrickson, V., Reid, P. Tags: All Imaging, All Spinal Cord RESIDENT AND FELLOW SECTION Source Type: research

Pearls & Oy-sters: CNS lymphoma in a patient with relapsing-remitting multiple sclerosis treated with interferon
Early clinical suspicion for CNS lymphoma is important for appropriate diagnosis and treatment in patients with preexisting multiple sclerosis (MS). (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Chiang, S., Kesari, N. K., Bradshaw, A., Chen, W., Samudralwar, R., Alobaidy, A. M., Kass, J. S. Tags: Primary brain tumor, Multiple sclerosis RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A common cause for Phelan-McDermid syndrome and neurofibromatosis type 2: One ring to bind them
An 8-year-old girl with mild dysmorphic features presented for evaluation of developmental delay and staring spells. She had been born late preterm and spent 1 month in the neonatal intensive care unit. She was generally healthy other than her developmental delay, which improved somewhat with physical, occupational, and speech therapy. At the first clinic visit, her mother reported loss of previously mastered vocabulary and struggles with fine motor skills such as buttoning. She also noted repetitive movements, obsessive behaviors, and hand flapping. (Source: Neurology)
Source: Neurology - October 23, 2017 Category: Neurology Authors: Lyons-Warren, A. M., Cheung, S. W., Holder, J. L. Tags: All Pediatric, Developmental disorders, Neurofibromatosis, Chromosomes RESIDENT AND FELLOW SECTION Source Type: research

Senses
Scent of newborn wrapped in a knitted blanket Watch her grow, first day of school Track her on the playground, never a moment of rest Chew on oatmeal raisin cookies baked together Shut our eyes for hide-and-seek Hear her cry, she skinned her knees Cheer as she scores a goal Carry her when she falls down Run to catch up, now she's too fast Clap and celebrate, it's graduation Point to her suitcase, she is ready to pack Hug her tight and hope she comes back She walks to the gate, college here she comes Reflex to wake her up for school, no longer there Home feels off-balance, too quiet Sharp pain from the tough goodbye Cold sh...
Source: Neurology - October 23, 2017 Category: Neurology Authors: Aghajan, Y. Tags: Clinical neurology examination REFLECTIONS: NEUROLOGY AND THE HUMANITIES Source Type: research

Letter re: Efficacy and safety of brivaracetam for partial-onset seizures in 3 pooled clinical studies
We thank Dr. French1 for her Epilepsy Currents commentary on our article.2 We agree that time will tell regarding the usefulness of brivaracetam's (BRV's) addition to the antiepileptic drug (AED) armamentarium, as there is no substitute for real-world clinical experience. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Klein, P., Benbadis, S. R., Diaz, A., Moseley, B. D., Ben-Menachem, E. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Ischemic Lesions, blood pressure dysregulation, and poor outcomes in intracerebral hemorrhage
We appreciate the comments by Drs. Lattanzi and Silvestrini on our article,1 and agree that further research is needed to better understand the complex relationship among blood pressure (BP) dysregulation, small vessel disease, and development of remote ischemic lesions in the setting of acute intracerebral hemorrhage.2 (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Kidwell, C. S., Rosand, J., Norato, G., Dixon, S., Worrall, B. B., James, M. L., Elkind, M. S. V., Flaherty, M. L., Osborne, J., Vashkevich, A., Langefeld, C. D., Moomaw, C. J., Woo, D. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research