Letter re: Physician-assisted death in chronic neurologic diseases
I read with interest the editorial by Drs. Bernat and McQuillen1 on physician-assisted death (PAD) in chronic neurologic disease. I fully support their view that "physicians have a concurrent duty to respect our patients' liberty-based right to determine themselves what constitutes their best outcome." However, it is important to remember that, among legislative criteria granting the right to practice PAD, there are 4 requirements: requests must be voluntary, persistent, well-thought, and well-informed.2 Also, and perhaps more importantly, I do not agree that in the Netherlands and Belgium "voluntary active euthanasia now ...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Bier, J.-C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: High hypothetical interest in physician-assisted death in multiple sclerosis
Reading the article by Marrie et al.1 reminded me of Leo Alexander's horrifying report, which was published as the lead article in a 1949 issue of the New England Journal of Medicine (NEJM).2 As an expert at the Nuremberg trial, Alexander carefully reviewed the extensive medical records left behind by the Nazi regime. In the NEJM article,2 he mentioned one of the Nazi propaganda movies, I Accuse, which depicted the life history of a woman with multiple sclerosis. In the movie, the woman's husband, a doctor, finally kills her to the accompaniment of soft piano music rendered by a sympathetic colleague in an adjoining room. ...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Masdeu, J. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In the article "High hypothetical interest in physician-assisted death in multiple sclerosis," the opinions of persons with multiple sclerosis (MS) regarding physician-assisted death (PAD) were assessed. Investigators surveyed participants in the North American Research Committee on Multiple Sclerosis Registry regarding PAD. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Karam, C., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Benefit of dual-energy CT iodine overlay technique for T1-hyperintense brain lesion
A 47-year-old woman was admitted for radiation therapy of a left frontal glioblastoma, resected 2 months prior. On enhanced MRI, it was difficult to identify the tumor enhancement due to intrinsic T1 hyperintensity, possibly related to postoperative hemorrhage1 (figure, A–C). However, the iodine overlay image derived from the dual-energy CT technique (figure, D) was revealing: subsequent surgical resection confirmed residual tumor cells along the region that showed the elevated iodine concentration (figure, E). The dual-energy CT iodine overlay technique may be particularly useful in the evaluation of a T1-hyperinten...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Lee, Y., Seo, H. S., Je, B.-K., Kim, S.-D., Oh, H. E. Tags: CT, MRI, Clinical neurology history, Primary brain tumor, Surgical therapy-tumor NEUROIMAGES Source Type: research

Abdominal tremor in thyrotoxicosis
Hyperthyroidism is a common medical condition in the general population and neurologic symptoms can be the first presentation. Neurologic manifestations include cognitive abnormalities and seizures, movement disorders such as chorea and tremors, and peripheral nervous system conditions including myopathy and neuropathy. Tremor, usually limb tremor, has been observed in 78% of patients with thyrotoxicosis.1 Here we report isolated abdominal tremor, reminiscent but different from belly dancer’s dyskinesia, in a patient with thyrotoxicosis. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Kelly, D. M., Lynch, T., Casserly, L. F. Tags: Endocrine, Tremor CLINICAL/SCIENTIFIC NOTES Source Type: research

Thalamic deep brain stimulation for tremor in Parkinson disease, essential tremor, and dystonia
Conclusions: VIM DBS is an effective long-term (beyond 10 years) treatment for tremor in PD and ET. Effects on dystonic tremor were modest and transient. Classification of evidence: This provides Class IV evidence. It is an observational study. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Cury, R. G., Fraix, V., Castrioto, A., Perez Fernandez, M. A., Krack, P., Chabardes, S., Seigneuret, E., Alho, E. J. L., Benabid, A.-L., Moro, E. Tags: Dystonia, Parkinson's disease/Parkinsonism, Tremor, Surgery/Stimulation ARTICLE Source Type: research

Association of intracranial aneurysm rupture with smoking duration, intensity, and cessation
Conclusions: Current cigarette smoking, smoking intensity, and smoking duration are significantly associated with ruptured IAs at presentation. However, the significantly increased risk persists after smoking cessation, and smoking cessation does not confer a reduced risk of aneurysmal subarachnoid hemorrhage beyond that of reducing the cumulative dose. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Can, A., Castro, V. M., Ozdemir, Y. H., Dagen, S., Yu, S., Dligach, D., Finan, S., Gainer, V., Shadick, N. A., Murphy, S., Cai, T., Savova, G., Dammers, R., Weiss, S. T., Du, R. Tags: All Cerebrovascular disease/Stroke, Subarachnoid hemorrhage ARTICLE Source Type: research

Comment: Capacity, consent, and country in acute stroke research
As a general principle, any research involving humans requires voluntary participation based on informed consent.1 This also applies to enrollment in clinical trials und usually requires participants to give written informed consent after having received detailed information about potential benefits and risks as well as alternative treatment options, and after having had adequate time for consideration. Trials in acute stroke, however, present several challenges to this approach. Reperfusion therapies in acute stroke show a clear time-dependent effect, being more effective the earlier treatment is started, or reperfusion a...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Chen, D. T., Worrall, B. B. Tags: ARTICLE Source Type: research

Effect of informed consent on patient characteristics in a stroke thrombolysis trial
Conclusions: Patients recruited by proxy consent were older, had more severe strokes, and had higher prevalence of aphasia than those with capacity to give personal consent. Variations in the manner of consent across countries may influence trial results. Clinicaltrials.gov and Clinicaltrialsregister.eu identifiers: NCT01525290 (clinicaltrials.gov); 2011-005906-32 (clinicaltrialsregister.eu). (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Thomalla, G., Boutitie, F., Fiebach, J. B., Simonsen, C. Z., Nighoghossian, N., Pedraza, S., Lemmens, R., Roy, P., Muir, K. W., Heesen, C., Ebinger, M., Ford, I., Cheng, B., Cho, T.-H., Puig, J., Thijs, V., Endres, M., Fiehler, J., Gerloff, C. Tags: MRI, All Clinical trials, All Cerebrovascular disease/Stroke, Clinical trials Methodology/study design, All Ethics in Neurology/Legal issues ARTICLE Source Type: research

1H-MRS metabolites and rate of {beta}-amyloid accumulation on serial PET in clinically normal adults
Conclusion: Among CN older adults, early metabolic alterations on 1H-MRS and APOE 4 status are independently associated with an increased rate of Aβ accumulation. Our findings could have important implications for early diagnosis and identification of individuals for secondary prevention trials, because an increased rate of Aβ accumulation in CN older adults may confer a higher risk for cognitive decline and mild cognitive impairment. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Nedelska, Z., Przybelski, S. A., Lesnick, T. G., Schwarz, C. G., Lowe, V. J., Machulda, M. M., Kremers, W. K., Mielke, M. M., Roberts, R. O., Boeve, B. F., Knopman, D. S., Petersen, R. C., Jack, C. R., Kantarci, K. Tags: MRS, Cognitive aging ARTICLE Source Type: research

Sex differences in Alzheimer risk: Brain imaging of endocrine vs chronologic aging
Conclusions: Multimodality brain imaging indicates sex differences in development of the AD endophenotype, suggesting that the preclinical AD phase is early in the female aging process and coincides with the endocrine transition of perimenopause. These data indicate that the optimal window of opportunity for therapeutic intervention in women is early in the endocrine aging process. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Mosconi, L., Berti, V., Quinn, C., McHugh, P., Petrongolo, G., Varsavsky, I., Osorio, R. S., Pupi, A., Vallabhajosula, S., Isaacson, R. S., de Leon, M. J., Brinton, R. D. Tags: PET, Alzheimer's disease, Cognitive aging ARTICLE Source Type: research

Brain network efficiency is influenced by the pathologic source of corticobasal syndrome
Conclusions: Our results indicate that the underlying pathologic sources of corticobasal syndrome can be classified more accurately using graph theoretical statistics derived from patterns of white matter network organization in association cortex than by regional gray matter density alone. These results highlight the importance of a multimodal neuroimaging approach to diagnostic analyses of corticobasal syndrome. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Medaglia, J. D., Huang, W., Segarra, S., Olm, C., Gee, J., Grossman, M., Ribeiro, A., McMillan, C. T., Bassett, D. S. Tags: Alzheimer's disease, Frontotemporal dementia, Corticobasal degeneration, MCI (mild cognitive impairment) ARTICLE Source Type: research

Developmental outcomes of cord blood transplantation for Krabbe disease: A 15-year study
Conclusions: The surviving patients function at a much higher level than untreated children or symptomatic children who underwent HSCT. These results show that early HSCT changes the natural history of this disease by improving both lifespan and functional abilities. Classification of evidence: This study provides Class IV evidence that for children with early-infantile Krabbe disease, early HSCT improves lifespan and functional abilities. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Wright, M. D., Poe, M. D., DeRenzo, A., Haldal, S., Escolar, M. L. Tags: Leukodystrophies, Developmental disorders, All Demyelinating disease (CNS) ARTICLE Source Type: research

A natural history study of X-linked myotubular myopathy
Conclusions: MTM is one of the most severe neuromuscular disorders, with affected individuals requiring extensive mechanical interventions for survival. However, among study participants, the disease course was more stable than predicted, with more individuals surviving infancy and early childhood. These data reflect the disease burden of MTM but offer hope in terms of future therapeutic intervention. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Amburgey, K., Tsuchiya, E., de Chastonay, S., Glueck, M., Alverez, R., Nguyen, C.-T., Rutkowski, A., Hornyak, J., Beggs, A. H., Dowling, J. J. Tags: Muscle disease, Natural history studies (prognosis) ARTICLE Source Type: research

High frequency of gastrointestinal manifestations in myotonic dystrophy type 1 and type 2
Conclusions: GI manifestations were common in both DM1 and DM2, with a relatively high frequency of gallbladder removal in DM1 and DM2 occurring at a younger age compared to normative data in the literature. Studies are needed to determine the pathomechanism of how sex, weight gain, and duration of disease contribute to GI manifestations and how these manifestations affect quality of life and clinical care for patients with DM1 and DM2. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Hilbert, J. E., Barohn, R. J., Clemens, P. R., Luebbe, E. A., Martens, W. B., McDermott, M. P., Parkhill, A. L., Tawil, R., Thornton, C. A., Moxley, R. T., On behalf of the National Registry Scientific Advisory Committee/Investigators Tags: All Clinical Neurology, Gastrointestinal, Muscle disease ARTICLE Source Type: research