[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 opportunit...
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 opportunit...
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