[Review] Pathogenesis of progressive multifocal leukoencephalopathy and risks associated with treatments for multiple sclerosis: a decade of lessons learned
Progressive multifocal leukoencephalopathy (PML) is a rare, devastating demyelinating disease of the CNS caused by the JC virus (JCV) that occurs in patients with compromised immune systems. Detection of PML in systemically immunocompetent patients with multiple sclerosis treated with natalizumab points to a role for this drug in the pathophysiology of PML. Emerging knowledge of the cellular and molecular biology of JCV infection and the pathogenesis of PML —including interplay of this common virus with the human immune system and features of natalizumab that might contribute to PML pathogenesis—provides new op...
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: Eugene O Major, Tarek A Yousry, David B Clifford Tags: Review Source Type: research

[In Context] Impairment of creativity by anaesthesia
Miguel Delibes (1920 –2010) was one of the most important Spanish writers of the 20th century. In his novels, he used a distinctly clear and elegant language to convey the beauty of rural Castilla. He was a professor, journalist, and director of the newspaper El Norte de Castilla. He wrote 68 fiction books and was awa rded the most prominent Spanish literary prizes, including the Premio Nadal in 1947, the Prince of Asturias Award for Literature in 1982, and the Miguel de Cervantes Prize in 1993. His literary career was cut short in 1998, after he had surgery for colon cancer. (Source: Lancet Neurology)
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: Justo Garc ía de Yébenes Tags: In Context Source Type: research

[In Context] Sven van der Lee
Sven van der Lee is a medical doctor and postdoctoral researcher at the Alzheimer Center, VU University Medical Center (Amsterdam, Netherlands). He earned his MD at Leiden University Medical Center (Leiden, Netherlands), is trained as a genetic epidemiologist, and obtained his PhD at Erasmus Medical Center (Rotterdam, Netherlands). In his research he identified, in large scale collaborative analyses, multiple genetic and metabolic factors that influence the risk of Alzheimer's disease. He is particularly interested in the translation of findings from basic science to clinically useful markers of dementia. (Source: Lancet Neurology)
Source: Lancet Neurology - April 25, 2018 Category: Neurology Tags: In Context Source Type: research

[Correspondence] Classification of epilepsies in the 18th century
Classifications provide “a framework on which to conceptualize knowledge and research”1 through organisation of items according to their fundamental relationships; they also allow standardised communication among the scientific community, and they tailor patient-oriented treatment decisions. Throughout history, several attempts have been made to organise epileptic seizures and epilepsies into clinical categories1,2 for diagnostic purposes, epilepsy research, development of antiepileptic therapies, and communication around the world. (Source: Lancet Neurology)
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: Francesco Brigo, Nicola Luigi Bragazzi, Simona Lattanzi, Raffaele Nardone, Mariano Martini, Eugen Trinka Tags: Correspondence Source Type: research

[Correspondence] Intravenous versus subcutaneous immunoglobulin – Authors' reply
We thank Ravi Uniyal and colleagues for their comments on our results from the PATH trial1 on subcutaneous immunoglobulin (SCIg) for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (CIDP). They express concern about the relapse rates in the treatment groups and hypothesise the cause being the pharmacokinetics of SCIg. (Source: Lancet Neurology)
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: Ivo N van Schaik, Orell Mielke, PATH study group Tags: Correspondence Source Type: research

[Correspondence] Intravenous versus subcutaneous immunoglobulin
We read with interest the Article by Ivo van Schaik and colleagues1 about the use of weekly subcutaneous immunoglobulin (SCIg) in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). After an IgG dependency test, the investigators randomly assigned 172 patients to low-dose SCIg (0 ·2 g/kg), high-dose SCIg (0·4 g/kg), or placebo. Relapse or withdrawal rate was significantly higher in the placebo group (63%) than in the high-dose SCIg group (33%) or low-dose SCIg group (39%). (Source: Lancet Neurology)
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: Ravi Uniyal, Ravindra Kumar Garg, Hardeep Singh Malhotra, Neeraj Kumar, Shweta Pandey Tags: Correspondence Source Type: research

[Comment] Understanding risk of PML through multiple sclerosis
Progressive multifocal leukoencephalopathy (PML), first described in 1959, is an often-fatal disease caused by an opportunistic infection by JC virus (JCV), which most human beings carry throughout life without consequences.1,2 PML was a major cause of death in patients with AIDS, but its prevalence decreased sharply with the introduction of retroviral therapy. PML is now mostly seen in patients during chemotherapy or immunosuppression. The first PML cases in patients with multiple sclerosis who were treated with natalizumab came as a surprise in 20053,4 because PML had not been observed with other drugs for multiple scler...
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: Roland Martin Tags: Comment Source Type: research

[Comment] Evidence-based care in Duchenne muscular dystrophy
Duchenne muscular dystrophy (DMD) is a severely progressive X-linked recessive neuromuscular disorder. It is caused by mutations in the dystrophin gene that result in absent or insufficient functional dystrophin protein, and manifests as progressive muscle degeneration and weakness with symptom onset between ages 3 and 5 years. The disease primarily affects boys and men, but in rare cases it can affect girls and women. Prevalence of DMD has been reported as one case per 5000 –6000 live male births. (Source: Lancet Neurology)
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: Craig M McDonald, Eugenio Mercuri Tags: Comment Source Type: research

[Comment] Prediction to prevention in Alzheimer's disease and dementia
Risks of dementia and Alzheimer's disease reflect the combined effects of age, genetic variants, and social and behavioural determinants.1 In The Lancet Neurology, Sven van der Lee and colleagues2 use data from the Rotterdam Study to address the important task of predicting whether an individual is likely to develop Alzheimer's disease or dementia in his or her lifetime. In this cohort, a genetic risk score (GRS) of 23 genetic variants modified the risk of late-onset Alzheimer's disease or dementia above and beyond the effect of the gene for apolipoprotein E (APOE). (Source: Lancet Neurology)
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: Carole Dufouil, M Maria Glymour Tags: Comment Source Type: research

[Editorial] Hope and foresight for your patients with ALS
The astrophysicist Stephen Hawking, arguably the most famous patient with amyotrophic lateral sclerosis (ALS) to date, died on March 14, 2018. He was exceptional in many respects, and so was his disease. Hawking's long survival after an ALS diagnosis (over 50 years) gave hope to other patients, and his case helped ALS specialists illustrate disease variability and prognostic uncertainty. When caring for people with a neurodegenerative disease that usually progresses rapidly, like ALS, prognosis is key to plan medical care and future needs. (Source: Lancet Neurology)
Source: Lancet Neurology - April 25, 2018 Category: Neurology Authors: The Lancet Neurology Tags: Editorial Source Type: research

[Review] Pathogenesis of progressive multifocal leukoencephalopathy and risks associated with treatments for multiple sclerosis: a decade of lessons learned
Progressive multifocal leukoencephalopathy (PML) is a rare, devastating demyelinating disease of the CNS caused by the JC virus (JCV) that occurs in patients with compromised immune systems. Detection of PML in systemically immunocompetent patients with multiple sclerosis treated with natalizumab points to a role for this drug in the pathophysiology of PML. Emerging knowledge of the cellular and molecular biology of JCV infection and the pathogenesis of PML —including interplay of this common virus with the human immune system and features of natalizumab that might contribute to PML pathogenesis—provides new op...
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: Eugene O Major, Tarek A Yousry, David B Clifford Tags: Review Source Type: research

[In Context] Impairment of creativity by anaesthesia
Miguel Delibes (1920 –2010) was one of the most important Spanish writers of the 20th century. In his novels, he used a distinctly clear and elegant language to convey the beauty of rural Castilla. He was a professor, journalist, and director of the newspaper El Norte de Castilla. He wrote 68 fiction books and was awa rded the most prominent Spanish literary prizes, including the Premio Nadal in 1947, the Prince of Asturias Award for Literature in 1982, and the Miguel de Cervantes Prize in 1993. His literary career was cut short in 1998, after he had surgery for colon cancer. (Source: Lancet Neurology)
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: Justo Garc ía de Yébenes Tags: In Context Source Type: research

[In Context] Sven van der Lee
Sven van der Lee is a medical doctor and postdoctoral researcher at the Alzheimer Center, VU University Medical Center (Amsterdam, Netherlands). He earned his MD at Leiden University Medical Center (Leiden, Netherlands), is trained as a genetic epidemiologist, and obtained his PhD at Erasmus Medical Center (Rotterdam, Netherlands). In his research he identified, in large scale collaborative analyses, multiple genetic and metabolic factors that influence the risk of Alzheimer's disease. He is particularly interested in the translation of findings from basic science to clinically useful markers of dementia. (Source: Lancet Neurology)
Source: Lancet Neurology - April 13, 2018 Category: Neurology Tags: In Context Source Type: research

[Correspondence] Classification of epilepsies in the 18th century
Classifications provide “a framework on which to conceptualize knowledge and research”1 through organisation of items according to their fundamental relationships; they also allow standardised communication among the scientific community, and they tailor patient-oriented treatment decisions. Throughout history, several attempts have been made to organise epileptic seizures and epilepsies into clinical categories1,2 for diagnostic purposes, epilepsy research, development of antiepileptic therapies, and communication around the world. (Source: Lancet Neurology)
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: Francesco Brigo, Nicola Luigi Bragazzi, Simona Lattanzi, Raffaele Nardone, Mariano Martini, Eugen Trinka Tags: Correspondence Source Type: research

[Correspondence] Intravenous versus subcutaneous immunoglobulin – Authors' reply
We thank Ravi Uniyal and colleagues for their comments on our results from the PATH trial1 on subcutaneous immunoglobulin (SCIg) for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (CIDP). They express concern about the relapse rates in the treatment groups and hypothesise the cause being the pharmacokinetics of SCIg. (Source: Lancet Neurology)
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: Ivo N van Schaik, Orell Mielke, PATH study group Tags: Correspondence Source Type: research

[Correspondence] Intravenous versus subcutaneous immunoglobulin
We read with interest the Article by Ivo van Schaik and colleagues1 about the use of weekly subcutaneous immunoglobulin (SCIg) in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). After an IgG dependency test, the investigators randomly assigned 172 patients to low-dose SCIg (0 ·2 g/kg), high-dose SCIg (0·4 g/kg), or placebo. Relapse or withdrawal rate was significantly higher in the placebo group (63%) than in the high-dose SCIg group (33%) or low-dose SCIg group (39%). (Source: Lancet Neurology)
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: Ravi Uniyal, Ravindra Kumar Garg, Hardeep Singh Malhotra, Neeraj Kumar, Shweta Pandey Tags: Correspondence Source Type: research

[Comment] Understanding risk of PML through multiple sclerosis
Progressive multifocal leukoencephalopathy (PML), first described in 1959, is an often-fatal disease caused by an opportunistic infection by JC virus (JCV), which most human beings carry throughout life without consequences.1,2 PML was a major cause of death in patients with AIDS, but its prevalence decreased sharply with the introduction of retroviral therapy. PML is now mostly seen in patients during chemotherapy or immunosuppression. The first PML cases in patients with multiple sclerosis who were treated with natalizumab came as a surprise in 20053,4 because PML had not been observed with other drugs for multiple scler...
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: Roland Martin Tags: Comment Source Type: research

[Comment] Evidence-based care in Duchenne muscular dystrophy
Duchenne muscular dystrophy (DMD) is a severely progressive X-linked recessive neuromuscular disorder. It is caused by mutations in the dystrophin gene that result in absent or insufficient functional dystrophin protein, and manifests as progressive muscle degeneration and weakness with symptom onset between ages 3 and 5 years. The disease primarily affects boys and men, but in rare cases it can affect girls and women. Prevalence of DMD has been reported as one case per 5000 –6000 live male births. (Source: Lancet Neurology)
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: Craig M McDonald, Eugenio Mercuri Tags: Comment Source Type: research

[Comment] Prediction to prevention in Alzheimer's disease and dementia
Risks of dementia and Alzheimer's disease reflect the combined effects of age, genetic variants, and social and behavioural determinants.1 In The Lancet Neurology, Sven van der Lee and colleagues2 use data from the Rotterdam Study to address the important task of predicting whether an individual is likely to develop Alzheimer's disease or dementia in his or her lifetime. In this cohort, a genetic risk score (GRS) of 23 genetic variants modified the risk of late-onset Alzheimer's disease or dementia above and beyond the effect of the gene for apolipoprotein E (APOE). (Source: Lancet Neurology)
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: Carole Dufouil, M Maria Glymour Tags: Comment Source Type: research

[Editorial] Hope and foresight for your patients with ALS
The astrophysicist Stephen Hawking, arguably the most famous patient with amyotrophic lateral sclerosis (ALS) to date, died on March 14, 2018. He was exceptional in many respects, and so was his disease. Hawking's long survival after an ALS diagnosis (over 50 years) gave hope to other patients, and his case helped ALS specialists illustrate disease variability and prognostic uncertainty. When caring for people with a neurodegenerative disease that usually progresses rapidly, like ALS, prognosis is key to plan medical care and future needs. (Source: Lancet Neurology)
Source: Lancet Neurology - April 13, 2018 Category: Neurology Authors: The Lancet Neurology Tags: Editorial Source Type: research

[Corrections] Corrections
Nonnekes J, Post B, Tetrud JW, Langston JW, Bloem BR. MPTP-induced parkinsonism: an historical case series. Lancet Neurol 2018; 17: 300 –01—The appendix of this Correspondence has been corrected as of March 27, 2018. (Source: Lancet Neurology)
Source: Lancet Neurology - March 27, 2018 Category: Neurology Tags: Corrections Source Type: research

[Review] Diagnosis and assessment of neuropathic pain through questionnaires
Neuropathic pain —pain caused by a lesion or disease of the somatosensory nervous system—is underdiagnosed and difficult to treat. However, development of easy-to-use questionnaires based mainly on self-reports of symptoms has improved diagnosis and management. Two types of questionnaires (screening and assessme nt) have been validated, and rapidly translated and revalidated in several languages, probably because of their simplicity. Screening questionnaires help clinicians to identify neuropathic pain easily, particularly in patients with complex medical conditions (eg, spinal cord injury). (Source: Lancet Neurology)
Source: Lancet Neurology - March 26, 2018 Category: Neurology Authors: Nadine Attal, Didier Bouhassira, Ralf Baron Tags: Review Source Type: research

[Articles] Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model
We have developed an externally validated model to predict survival without tracheostomy and non-invasive ventilation for more than 23 h per day in European patients with ALS. This model could be applied to individualised patient management, counselling, and future trial design, but to maximise the benefit and prevent harm it is intended to be used by medical doctors only. (Source: Lancet Neurology)
Source: Lancet Neurology - March 26, 2018 Category: Neurology Authors: Henk-Jan Westeneng, Thomas P A Debray, Anne E Visser, Ruben P A van Eijk, James P K Rooney, Andrea Calvo, Sarah Martin, Christopher J McDermott, Alexander G Thompson, Susana Pinto, Xenia Kobeleva, Angela Rosenbohm, Beatrice Stubendorff, Helma Sommer, Bas Tags: Articles Source Type: research

[Comment] What if you knew the prognosis of your patients with ALS?
More than 60 years ago, Karl Jaspers1 stated that when patients plead for the truth, most of them just want assurance. Forbes Norris, one of the most respected American experts on amyotrophic lateral sclerosis (ALS), used to tell his patients: “I think you have ALS, but I might be wrong”. His intention was to try to convey some uncertainty regarding the diagnosis, which could provide hope for some patients. After so many years, have we made any progress in relaying diagnoses and prognoses to patients with ALS? In The Lancet Neurology, a research team led by Leonard van den Berg and colleagues now reports on a c...
Source: Lancet Neurology - March 26, 2018 Category: Neurology Authors: Hiroshi Mitsumoto Tags: Comment Source Type: research

[Review] Diagnosis and assessment of neuropathic pain through questionnaires
Neuropathic pain —pain caused by a lesion or disease of the somatosensory nervous system—is underdiagnosed and difficult to treat. However, development of easy-to-use questionnaires based mainly on self-reports of symptoms has improved diagnosis and management. Two types of questionnaires (screening and assessme nt) have been validated, and rapidly translated and revalidated in several languages, probably because of their simplicity. Screening questionnaires help clinicians to identify neuropathic pain easily, particularly in patients with complex medical conditions (eg, spinal cord injury). (Source: Lancet Neurology)
Source: Lancet Neurology - March 26, 2018 Category: Neurology Authors: Nadine Attal, Didier Bouhassira, Ralf Baron Tags: Review Source Type: research

[Articles] Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model
We have developed an externally validated model to predict survival without tracheostomy and non-invasive ventilation for more than 23 h per day in European patients with ALS. This model could be applied to individualised patient management, counselling, and future trial design, but to maximise the benefit and prevent harm it is intended to be used by medical doctors only. (Source: Lancet Neurology)
Source: Lancet Neurology - March 26, 2018 Category: Neurology Authors: Henk-Jan Westeneng, Thomas P A Debray, Anne E Visser, Ruben P A van Eijk, James P K Rooney, Andrea Calvo, Sarah Martin, Christopher J McDermott, Alexander G Thompson, Susana Pinto, Xenia Kobeleva, Angela Rosenbohm, Beatrice Stubendorff, Helma Sommer, Bas Tags: Articles Source Type: research

[Comment] What if you knew the prognosis of your patients with ALS?
More than 60 years ago, Karl Jaspers1 stated that when patients plead for the truth, most of them just want assurance. Forbes Norris, one of the most respected American experts on amyotrophic lateral sclerosis (ALS), used to tell his patients: “I think you have ALS, but I might be wrong”. His intention was to try to convey some uncertainty regarding the diagnosis, which could provide hope for some patients. After so many years, have we made any progress in relaying diagnoses and prognoses to patients with ALS? In The Lancet Neurology, a research team led by Leonard van den Berg and colleagues now reports on a c...
Source: Lancet Neurology - March 26, 2018 Category: Neurology Authors: Hiroshi Mitsumoto Tags: Comment Source Type: research

[In Context] Frederik Barkhof: building bridges between disciplines
The early academic journey of Frederik Barkhof was a winding one. As a child he was fascinated by the engineering of buildings and bridges, but then crossed over to study dentistry —“which was a mistake” (a bridge too far, presumably). He next stopped to study at the intersection between philosophy and medicine, until he landed at VU University (Amsterdam, the Netherlands) where he received his MD in 1988. Combining his love of engineering and medicine, Barkhof is now a neuroradiology professor who shares his expertise with both VU University Medical Centre and University College London (UCL, London, UK) ...
Source: Lancet Neurology - March 22, 2018 Category: Neurology Authors: Jules Morgan Tags: In Context Source Type: research

[Correspondence] Accelerated long-term forgetting in asymptomatic APOE ε4 carriers
In The Lancet Neurology, Philip Weston and colleagues report that presymptomatic individuals carrying familial Alzheimer's disease gene mutations demonstrate accelerated long-term forgetting over an extended 1 week retention interval compared with gene-negative controls.1 These findings raise the question of whether accelerated long-term forgetting is also detectable in presymptomatic individuals at genetic risk of the much more common sporadic form of Alzheimer's disease. (Source: Lancet Neurology)
Source: Lancet Neurology - March 22, 2018 Category: Neurology Authors: Jacqueline F Zimmermann, Christopher R Butler Tags: Correspondence Source Type: research

[Corrections] Corrections
Grisold W, Wasay M. The advocacy role of the World Federation of Neurology. Lancet Neurol 2018; 17: 204 —The name of the second author, Mohammad Wasay, was written incorrectly. This name has been corrected in the online version as of March 22, 2018. (Source: Lancet Neurology)
Source: Lancet Neurology - March 22, 2018 Category: Neurology Tags: Corrections Source Type: research

[In Context] Frederik Barkhof: building bridges between disciplines
The early academic journey of Frederik Barkhof was a winding one. As a child he was fascinated by the engineering of buildings and bridges, but then crossed over to study dentistry —“which was a mistake” (a bridge too far, presumably). He next stopped to study at the intersection between philosophy and medicine, until he landed at VU University (Amsterdam, the Netherlands) where he received his MD in 1988. Combining his love of engineering and medicine, Barkhof is now a neuroradiology professor who shares his expertise with both VU University Medical Centre and University College London (UCL, London, UK) ...
Source: Lancet Neurology - March 22, 2018 Category: Neurology Authors: Jules Morgan Tags: In Context Source Type: research

[Correspondence] Accelerated long-term forgetting in asymptomatic APOE ε4 carriers
In The Lancet Neurology, Philip Weston and colleagues report that presymptomatic individuals carrying familial Alzheimer's disease gene mutations demonstrate accelerated long-term forgetting over an extended 1 week retention interval compared with gene-negative controls.1 These findings raise the question of whether accelerated long-term forgetting is also detectable in presymptomatic individuals at genetic risk of the much more common sporadic form of Alzheimer's disease. (Source: Lancet Neurology)
Source: Lancet Neurology - March 22, 2018 Category: Neurology Authors: Jacqueline F Zimmermann, Christopher R Butler Tags: Correspondence Source Type: research

[Corrections] Corrections
Grisold W, Wasay M. The advocacy role of the World Federation of Neurology. Lancet Neurol 2018; 17: 204 —The name of the second author, Mohammad Wasay, was written incorrectly. This name has been corrected in the online version as of March 22, 2018. (Source: Lancet Neurology)
Source: Lancet Neurology - March 22, 2018 Category: Neurology Tags: Corrections Source Type: research

[Articles] The effect of APOE and other common genetic variants on the onset of Alzheimer's disease and dementia: a community-based cohort study
Common variants with small individual effects jointly modify the risk and age at onset of Alzheimer's disease and dementia, particularly in APOE ε4 carriers. These findings highlight the potential of common variants in determining Alzheimer's disease risk. (Source: Lancet Neurology)
Source: Lancet Neurology - March 16, 2018 Category: Neurology Authors: Sven J van der Lee, Frank J Wolters, M Kamran Ikram, Albert Hofman, M Arfan Ikram, Najaf Amin, Cornelia M van Duijn Tags: Articles Source Type: research

[In Context] The storm and stress in the adolescent brain
Sarah-Jayne Blakemore, Professor of Cognitive Neuroscience at University College London (London, UK), is sick of hearing the same joke over and over again. When she informs people that she studies the adolescent brain, she inevitably hears a now familiar refrain: “What? Teenagers have brains?” She's right to take offence, not only on behalf of the young people she works with, but because—as her book, Inventing Ourselves: the Secret Life of the Teenage Brain demonstrates—adolescence is a fascinating and important period of neurological change. (Source: Lancet Neurology)
Source: Lancet Neurology - March 15, 2018 Category: Neurology Authors: Robert Stirrups Tags: In Context Source Type: research

[Personal View] The roles of surgery and technology in understanding focal epilepsy and its comorbidities
Intracranial electrophysiological recording in patients with refractory focal epilepsy is the gold standard for defining epileptogenic tissue. Although the concordance of intracranial electrophysiology, structural MRI, and pathology can identify brain regions for resection, complete seizure control after surgery is not achieved in all patients with focal epilepsy. Repetitive identical behavioural seizures suggest one onset area, but epileptogenesis might be distributed and connected by functional and structural brain networks outside the seizure onset area, which could explain poor postsurgical outcomes in some patients. (...
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Dennis D Spencer, Jason L Gerrard, Hitten P Zaveri Tags: Personal View Source Type: research

[Articles] Survival in patients with spinocerebellar ataxia types 1, 2, 3, and 6 (EUROSCA): a longitudinal cohort study
Our study provides quantitative data on the survival of patients with the most common spinocerebellar ataxias, based on a long follow-up period. These results have implications for the design of future interventional studies of spinocerebellar ataxias; for example, the prognostic survival nomogram could be useful for selection and stratification of patients. Our findings need validation in an external population before they can be used to counsel patients and their families. (Source: Lancet Neurology)
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Alhassane Diallo, Heike Jacobi, Arron Cook, Robyn Labrum, Alexandra Durr, Alexis Brice, Perrine Charles, Cecilia Marelli, Caterina Mariotti, Lorenzo Nanetti, Marta Panzeri, Maria Rakowicz, Anna Sobanska, Anna Sulek, Tanja Schmitz-H übsch, Ludger Schöls, Tags: Articles Source Type: research

[In Context] Yogeshwar Kalkonde
Yogeshwar Kalkonde is a neurologist and a public health researcher working at the Society for Education, Action and Research in Community Health in Gadchiroli, India. His research focuses on stroke as a public health problem in rural India, and he is currently investigating community-based interventions to reduce stroke mortality. (Source: Lancet Neurology)
Source: Lancet Neurology - March 14, 2018 Category: Neurology Tags: In Context Source Type: research

[In Context] Hemiplegic migraine and stroke in Mary Shelley
Mary Wollstonecraft Godwin Shelley (1797 –1851) was the author of Frankenstein; or, the Modern Prometheus. In 1839, she had recurrent, severe headaches whose severity, location, and duration were variable.1 These headaches were associated with dizziness; pain in the legs, right hand, and right eye; and episodes of transient partial paral ysis of the right leg and hand, with tremors, convulsive seizures, and severe pain on the top of the head.2 She was diagnosed with psychosomatic illness in 1842, “functional derangement in the nerves or brain” in 1845, and back pain and “neuralgia of the heart&rdquo...
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Philippe Charlier, Otto Appenzeller, Saudamini Deo, Antonio Perciaccante, Raffaella Bianucci Tags: In Context Source Type: research

[Correspondence] MPTP-induced parkinsonism: an historical case series
In 1982, seven young adults developed severe and irreversible parkinsonism shortly after they injected themselves with a new synthetic heroin.1 Sample analyses revealed that this synthetic heroin consisted of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP),2 a potent neurotoxin targeting neurons within the substantia nigra.3 (Source: Lancet Neurology)
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Jorik Nonnekes, Bart Post, James W Tetrud, J William Langston, Bastiaan R Bloem Tags: Correspondence Source Type: research

[Correspondence] Outcome assessment after traumatic brain injury – Authors' reply
We thank Thomas McMillan and colleagues for their thoughtful comments on assessment of outcomes, with many of which we fully agree. As with many outcome assessments in traumatic brain injury (TBI), the Glasgow Outcome Scale (GOS) is open to a wide variety of influences other than brain injury: factors related to acute TBI appear to explain at best 35% of the variance.1 The predictors, moderators, and mediators of outcome after TBI are incompletely understood. There is thus much progress to be made in identifying confounding covariates for the effects of interventions. (Source: Lancet Neurology)
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Lindsay Wilson, Nicole von Steinbuechel, David K Menon, Andrew I R Maas Tags: Correspondence Source Type: research

[Correspondence] Outcome assessment after traumatic brain injury
In their wide ranging and impressive Commission of traumatic brain injury (TBI), Andrew Maas and colleagues1 recommend the development and validation of multidimensional outcome constructs that quantify overall burden of disability from TBI. Their observation that there are already nearly 1000 assessment instruments to consider is one of many challenges that need to be overcome to achieve this aim. (Source: Lancet Neurology)
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Thomas McMillan, Erin D Bigler, Graham Teasdale, Jennie Ponsford, Gordon D Murray Tags: Correspondence Source Type: research

[Corrections] Corrections
Weston P S J, Nicholas J M, Henley S M D, et al. Accelerated long-term forgetting in presymptomatic autosomal dominant Alzheimer's disease: a cross-sectional study. Lancet Neurol 2018; 17: 123 –32—In table 3 of this Article, data for figure 30-min recall were incorrectly recorded. Data in this row have been corrected in the online version as of March 13, 2018. (Source: Lancet Neurology)
Source: Lancet Neurology - March 14, 2018 Category: Neurology Tags: Corrections Source Type: research

[Comment] Mapping brain networks in patients with focal epilepsy
Epilepsy is one of the most common neurological disorders, affecting more than 50 million people worldwide. Around 70% of patients with epilepsy respond to antiepileptic drugs.1 For patients who do not respond, surgery is an accepted treatment option.2 Seizure recurrence after resection is seen in 30 –70% of patients and follows predictable slopes based on multiple factors, including the duration of epilepsy, history of generalised tonic seizures, frequency of preoperative seizures, and the presumptive pathological cause of epilepsy on MRI. (Source: Lancet Neurology)
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Imad M Najm Tags: Comment Source Type: research

[Comment] A crystal ball for survival for spinocerebellar ataxias?
Death is an unequivocal clinical endpoint. Although each of us must accept the harsh reality of 100% mortality, prolonging survival has been, and will be, one of the major goals of medicine. Spinocerebellar ataxias are a group of autosomal dominant neurodegenerative disorders, most of which show relentless progression, often resulting in total disability and premature death.1 SCA1, SCA2, SCA3, and SCA6 are among the most common spinocerebellar ataxias and caused by expanded CAG trinucleotide repeats encoding polyglutamine (polyQ), which belong to the same class of mutations as those of Huntington's disease, spinal and bulb...
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Tetsuo Ashizawa Tags: Comment Source Type: research

[Editorial] Accelerating research for Parkinson's disease
Despite considerable ongoing translational and clinical research efforts, no disease-modifying drugs have been approved for Parkinson's disease. The Accelerating Medicines Partnership Parkinson's disease (AMP PD) programme, launched on Jan 30, 2018, is the most ambitious initiative so far to find a cure for Parkinson's disease. Formed by the US National Institutes of Health (NIH), the US Food and Drug Administration (FDA), five biopharmaceutical and life science companies, and one non-profit organisation, this partnership is focused on discovering novel therapeutic targets and on developing biomarkers to help validate exis...
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: The Lancet Neurology Tags: Editorial Source Type: research

[Correspondence] International Classification of Headache Disorders
The third edition of the International Classification of Headache Disorders (ICHD-3) has now been published.1 This classification and the corresponding diagnostic criteria were initially released as a beta document in 2014; since then, they have been widely accepted and should now form the basis of all diagnosis and management of headache disorders in clinical practice as well as in research. The ICHD-3, like its predecessors, is hierarchical and allows diagnosis in different clinical settings, diagnosing at the first or second level in general practice and at the third, fourth, or fifth level in neurological practice or i...
Source: Lancet Neurology - March 14, 2018 Category: Neurology Authors: Jes Olesen Tags: Correspondence Source Type: research