Letter re: Ischemic Lesions, blood pressure dysregulation, and poor outcomes in intracerebral hemorrhage
We appreciated the article by Kidwell et al.1 that showed higher first-recorded blood pressure (BP) and greater delta mean arterial pressure are associated with the development of diffusion-weighted imaging (DWI) lesions in acute intracerebral hemorrhage (ICH). The most desirable BP target and optimal antihypertensive strategy in ICH are controversial.2 The relationship between BP and outcome is more complex than simply linear, and mechanisms other than BP reduction play a role. There is accruing evidence that not only absolute BP levels but their variation over time affect ICH prognosis.3 Besides increasing the risk of he...
Source: Neurology - October 16, 2017 Category: Neurology Authors: Lattanzi, S., Silvestrini, M. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In "Ischemic lesions, blood pressure dysregulation, and poor outcomes in intracerebral hemorrhage," the authors concluded that acute blood pressure (BP) dysregulation in primary intracerebral hemorrhage (ICH) patients was associated with the development of diffusion-weighted imaging (DWI) lesions and, subsequently, worse outcomes. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Alcauskas, M., Galetta, S. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Fatigable ptosis as an initial presentation of adult-onset Leigh syndrome
A 20-year-old man presented with bilateral fatigable ptosis for 1 month. On examination, there was bilateral incomplete ptosis, which deteriorated during upward gaze and improved at rest (figure, A and B). Tests for myasthenia gravis were all negative. Brain MRI showed symmetric hyperintensities at periaqueductal gray matter on T2- and diffusion-weighted images (figure, C). CSF lactic acid was elevated. Mitochondrial genome test demonstrated a homoplasmic T9176C mutation in the MT-APT6A gene, known as pathogenic mutation of Leigh syndrome.1 In our patient, fatigable ptosis may be ascribed to the dysfunction at centrally lo...
Source: Neurology - October 16, 2017 Category: Neurology Authors: Oh, E. H., Chae, S.-H., Cho, J.-W., Baik, S. K., Choi, S.-Y., Choi, K.-D., Choi, J.-H. Tags: MRI, Eyelids, Mitochondrial disorders NEUROIMAGES Source Type: research

Did Jules Dejerine describe AMAN at the end of the 19th century?
We describe his observations, which provide us with valuable information on the course of pathologic lesions in this disease. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Mathis, S., Magy, L., Le Masson, G., Vallat, J.-M. Tags: Autoimmune diseases, Clinical neurology history, Peripheral neuropathy, Guillain-Barre syndrome HISTORICAL NEUROLOGY Source Type: research

Genome editing technologies and their potential to treat neurologic disease
Genome editing refers to a process of making precise and permanent changes in the genetic code of cells, tissues, and whole organisms. The first step in this process is to program an enzyme called a nuclease to bind to a precise DNA sequence, whereby the enzyme will cut the DNA. This double-stranded break will in turn induce the cell to make a repair at that site, which will change the DNA sequence. The repair mechanism can be utilized to either knock out or introduce selected genes. Given how rapidly gene editing systems are influencing diverse applications in biomedical research, biotechnology, and agriculture,1 clinical...
Source: Neurology - October 16, 2017 Category: Neurology Authors: Madigan, N. N., Staff, N. P., Windebank, A. J., Benarroch, E. E. Tags: CLINICAL IMPLICATIONS OF NEUROSCIENCE RESEARCH Source Type: research

Qualitative study of burnout, career satisfaction, and well-being among US neurologists in 2016
Conclusions: Our results further characterize burnout among US neurologists through respondents' own voices. They clarify the meaning respondents attributed to ambiguous survey questions and highlight the barriers neurologists must overcome to practice their chosen specialty, including multiple regulatory hassles and increased work hours. Erosion of professionalism by external factors was a common issue. Our findings can provide strategic direction for advocacy and programs to prevent and mitigate neurologist burnout and promote well-being and engagement. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Miyasaki, J. M., Rheaume, C., Gulya, L., Ellenstein, A., Schwarz, H. B., Vidic, T. R., Shanafelt, T. D., Cascino, T. L., Keran, C. M., Busis, N. A. Tags: Electronic medical records, Insurance, Administration ARTICLE Source Type: research

Smoking cessation and outcome after ischemic stroke or TIA
Conclusion: Cessation of cigarette smoking after an ischemic stroke or TIA was associated with significant health benefits over 4.8 years in the IRIS trial cohort. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Epstein, K. A., Viscoli, C. M., Spence, J. D., Young, L. H., Inzucchi, S. E., Gorman, M., Gerstenhaber, B., Guarino, P. D., Dixit, A., Furie, K. L., Kernan, W. N., For the IRIS Trial Investigators Tags: Stroke prevention, Prognosis, All Cerebrovascular disease/Stroke, Infarction ARTICLE Source Type: research

Serum magnesium is associated with the risk of dementia
Conclusions: Both low and high serum magnesium levels are associated with an increased risk of all-cause dementia. Our results warrant replication in other population-based studies. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Kieboom, B. C. T., Licher, S., Wolters, F. J., Ikram, M. K., Hoorn, E. J., Zietse, R., Stricker, B. H., Ikram, M. A. Tags: Electrolyte, All Cognitive Disorders/Dementia, Alzheimer's disease, Cohort studies, Incidence studies ARTICLE Source Type: research

Comparison of symptomatic and asymptomatic persons with primary age-related tauopathy
Conclusions: These findings support the hypothesis that participants with PART have an amyloid-independent dementing Alzheimer disease–like temporal lobe tauopathy. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Besser, L. M., Crary, J. F., Mock, C., Kukull, W. A. Tags: All Clinical Neurology, Prognosis, Depression, All Cognitive Disorders/Dementia, Cognitive aging ARTICLE Source Type: research

Pretreatment behavior and subsequent medication effects in childhood absence epilepsy
Conclusions: Pretreatment and ongoing behavioral problems exist in CAE. Valproic acid is associated with worse behavioral outcomes than ethosuximide or lamotrigine, further reinforcing ethosuximide as the preferred initial therapy for CAE. Clinicaltrials.gov identifier: NCT00088452. Classification of evidence: This study provides Class II evidence that for children with CAE, valproic acid is associated with worse behavioral outcomes than ethosuximide or lamotrigine. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Shinnar, R. C., Shinnar, S., Cnaan, A., Clark, P., Dlugos, D., Hirtz, D. G., Hu, F., Liu, C., Masur, D., Weiss, E. F., Glauser, T. A., For the Childhood Absence Epilepsy Study Group Tags: Clinical trials Randomized controlled (CONSORT agreement), Attention, All Pediatric, Antiepileptic drugs, Absence seizures ARTICLE Source Type: research

Variable course of Unverricht-Lundborg disease: Early prognostic factors
Conclusions: A younger age at onset, early severe myoclonus, and seizure persistence are predictors of a more severe outcome. All of these factors may be genetically determined, but the greater hyperexcitability underlying more severe seizures and myoclonus at onset may also play a role by increasing cell damage due to reduced cystatin B activity. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Canafoglia, L., Ferlazzo, E., Michelucci, R., Striano, P., Magaudda, A., Gambardella, A., Pasini, E., Belcastro, V., Riguzzi, P., Fanella, M., Granata, T., Beccaria, F., Trentini, C., Bianchi, A., Aguglia, U., Panzica, F., Franceschetti, S. Tags: Myoclonus; see Movement Disorders/myoclonus, Natural history studies (prognosis), EEG, Generalized seizures ARTICLE Source Type: research

Randomized open-label trial of dextromethorphan in Rett syndrome
Conclusions: Dextromethorphan is a potent noncompetitive antagonist of the NMDA receptor channel that is safe for use in young girls with Rett syndrome. Preliminary evidence suggests that dextromethorphan may improve some core features of Rett syndrome. Classification of evidence: This study provides Class IV evidence that dextromethorphan at various doses does not change EEG spike counts over 6 months, though precision was limited to exclude an important effect. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Smith-Hicks, C. L., Gupta, S., Ewen, J. B., Hong, M., Kratz, L., Kelley, R., Tierney, E., Vaurio, R., Bibat, G., Sanyal, A., Yenokyan, G., Brereton, N., Johnston, M. V., Naidu, S. Tags: Clinical trials Observational study (Cohort, Case control), Rett Syndrome, All Genetics ARTICLE Source Type: research

Analysis of blood-based gene expression in idiopathic Parkinson disease
Conclusions: We present a large-scale study of PD gene expression profiling. This work identifies a reliable blood-based PD signature and highlights the importance of large-scale patient cohorts in developing potential PD biomarkers. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Shamir, R., Klein, C., Amar, D., Vollstedt, E.-J., Bonin, M., Usenovic, M., Wong, Y. C., Maver, A., Poths, S., Safer, H., Corvol, J.-C., Lesage, S., Lavi, O., Deuschl, G., Kuhlenbaeumer, G., Pawlack, H., Ulitsky, I., Kasten, M., Riess, O., Brice, A., Pete Tags: All Movement Disorders, Parkinson's disease/Parkinsonism, Gene expression studies, All Genetics ARTICLE Source Type: research

Comorbidity in multiple sclerosis is associated with diagnostic delays and increased mortality
Conclusions: An increased awareness of both the necessity of neurologic evaluation of new neurologic symptoms in persons with preexisting chronic disease and of optimum treatment of comorbidity in MS is critical. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Thormann, A., Sorensen, P. S., Koch-Henriksen, N., Laursen, B., Magyari, M. Tags: Multiple sclerosis, Cohort studies ARTICLE Source Type: research

Spinal cord perfusion pressure predicts neurologic recovery in acute spinal cord injury
Conclusions: We demonstrate that maintaining SCPP above 50 mm Hg is a strong predictor of improved neurologic recovery following spinal cord injury. This suggests that SCPP (the difference between MAP and CSFP) can provide useful information to guide the hemodynamic management of patients with acute spinal cord injury. (Source: Neurology)
Source: Neurology - October 16, 2017 Category: Neurology Authors: Squair, J. W., Belanger, L. M., Tsang, A., Ritchie, L., Mac-Thiong, J.-M., Parent, S., Christie, S., Bailey, C., Dhall, S., Street, J., Ailon, T., Paquette, S., Dea, N., Fisher, C. G., Dvorak, M. F., West, C. R., Kwon, B. K. Tags: Clinical trials Observational study (Cohort, Case control), Spinal cord trauma; see Trauma/spinal cord trauma, Cerebrospinal Fluid ARTICLE Source Type: research