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What's new in neurology
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What's new in neurology
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Sep 2017. | This topic last updated: Oct 19, 2017.

The following represent additions to UpToDate from the past six months that were considered by the editors and authors to be of particular interest. The most recent What's New entries are at the top of each subsection.

CEREBROVASCULAR DISEASE

Head position in acute stroke (July 2017)

A supine position with the head flat has been preferred for patients with acute ischemic stroke because it maximizes cerebral perfusion. However, the benefit of maintaining the head flat in this setting remains unproven. In the HeadPoST controlled trial of over 11,000 subjects with acute stroke (85 percent ischemic) who were randomly assigned to either a lying-flat position or a sitting-up position with the head elevated to at least 30 degrees, there was no difference between treatment groups in disability outcomes, mortality, or serious adverse events [1]. For most patients with acute ischemic stroke, we suggest keeping the head of the bed in the position that is most comfortable for the patient. Exceptions include those at risk for elevated intracranial pressure, aspiration, cardiopulmonary decompensation, or oxygen desaturation, where we recommend elevating the head of the bed to 30 degrees. (See "Initial assessment and management of acute stroke", section on 'Head and body position'.)

DEMENTIA

Dementia risk factors and prevention (September 2017)

Two major reports released by a Lancet Commission in the United Kingdom and the Agency for Healthcare Research and Quality in the United States review the literature on risk factors for dementia and the impact of risk factor modification on dementia incidence [2,3]. The Lancet Commission estimates that approximately one-third of dementia cases are attributable to a combination of nine potentially modifiable risk factors: low educational attainment, midlife hypertension, midlife obesity, hearing loss, late-life depression, diabetes, physical inactivity, smoking, and social isolation [2]. While the overall evidence is generally of low quality and does not support any single intervention, there is optimism that intensive risk factor modification, especially during midlife, has the potential to delay or prevent dementia. (See "Risk factors for cognitive decline and dementia" and "Prevention of dementia".)

Revised diagnostic criteria for dementia with Lewy bodies (July 2017)

Revised consensus criteria for the clinical diagnosis of dementia with Lewy bodies (DLB) have been published [4]. The updated criteria now recognize rapid eye movement (REM) sleep behavior disorder as a core clinical feature of DLB, along with three previously recognized features: cognitive fluctuations, visual hallucinations, and parkinsonism (table 1). In patients with dementia, probable DLB can be diagnosed in the presence of two or more core clinical features or at least one core feature plus an indicative biomarker (reduced dopamine transporter [DAT] uptake in basal ganglia, abnormal 123-iodine-metaiodobenzylguanidine [MIBG] myocardial scintigraphy, or REM sleep without atonia on polysomnography). (See "Clinical features and diagnosis of dementia with Lewy bodies", section on 'Clinical features'.)

DEMYELINATING DISEASE

Minocycline for clinically isolated syndrome suggestive of multiple sclerosis (June 2017)

In a recent trial, 142 subjects with a first central nervous system demyelinating event (ie, a clinically isolated syndrome or CIS) were randomly assigned to minocycline or placebo [5]. At six months, the difference in the risk of conversion to multiple sclerosis was lower for minocycline compared with placebo, and MRI outcomes also favored minocycline. However, at 24 months, there was no difference between groups for any of these outcomes. Thus, more data are needed from larger trials to determine if minocycline has any benefit for patients with CIS or early multiple sclerosis. (See "Clinically isolated syndromes suggestive of multiple sclerosis", section on 'Minocycline'.)

EPILEPSY

Cannabidiol in patients with Dravet syndrome and refractory epilepsy (May 2017)

Although cannabidiol (CBD), a component of cannabis, has received interest in the epilepsy community, particularly in children with Dravet syndrome (DS), controlled trials have not been available. In the first multicenter trial comparing oral CBD solution with placebo (in addition to standard antiseizure treatment) in 120 children and young adults with DS, seizure frequency was decreased at 14 weeks in the CBD group [6]. Common side effects of CBD were diarrhea, sedation, and fatigue. Further study of CBD in patients with refractory epilepsy is indicated. In the absence of an available regulated preparation of CBD, we do not advocate use of cannabis or its derivatives outside of the context of a clinical trial. (See "Dravet syndrome: Management and prognosis", section on 'Cannabinoids'.)

MOVEMENT DISORDERS

Exenatide for Parkinson disease (August 2017)

Exenatide, a synthetic glucagon-like peptide-1 (GLP-1) receptor agonist, has shown neuroprotective effects in animal models of Parkinson disease (PD). In a 60-week randomized trial of 60 patients with PD, the group assigned to subcutaneous exenatide 2 mg weekly showed a small improvement in motor scores, while the placebo group exhibited a slight worsening [7]. However, there were no differences between the groups on any of the secondary clinical outcome measures. Given the small size of the trial and other limitations, more data are needed to confirm whether exenatide is beneficial in patients with PD. (See "Neuroprotective therapy for Parkinson disease", section on 'Exenatide'.)

Association between Parkinson disease and melanoma (August 2017)

Several studies have suggested an association between Parkinson disease (PD) and melanoma. A retrospective study using medical records from 1976 to 2013 found that, compared with controls, PD patients had nearly a four-fold increased likelihood of having a history of melanoma; likewise, patients with melanoma had approximately a four-fold risk of developing PD after the diagnosis of melanoma [8]. Although the underlying cause of this reciprocal association remains unclear, these findings suggest that PD and melanoma may share genetic risk factors. (See "Etiology and pathogenesis of Parkinson disease", section on 'Risk factors' and "Risk factors for the development of melanoma", section on 'Other proposed risk factors'.)

Valbenazine for tardive dyskinesia (May 2017)

Tetrabenazine and valbenazine are vesicular monoamine transporter 2 inhibitors that deplete presynaptic dopamine and may be useful therapeutic agents for tardive dyskinesia (TD). Data from old, small studies supported the utility of tetrabenazine for this indication. Now there is evidence from the placebo-controlled KINECT 3 trial that valbenazine 40 mg once daily reduces dyskinesia in patients with TD [9]. For patients who have disturbing and intrusive tardive dyskinesia or tardive dystonia not amenable to treatment with botulinum toxin, we suggest treatment with tetrabenazine or valbenazine. (See "Tardive dyskinesia: Prevention and treatment", section on 'Valbenazine'.)

NEUROMUSCULAR DISEASE

Edaravone for amyotrophic lateral sclerosis (May 2017)

Edaravone is a free radical scavenger that is thought to reduce oxidative stress, which has been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). Edaravone may slow functional deterioration in some patients with ALS. An earlier trial found no benefit for edaravone compared with placebo, but a post-hoc analysis showed a possible treatment effect in a subgroup of individuals with early-stage ALS. A subsequent trial enrolled 137 Japanese patients within two years of ALS diagnosis who were living independently and had a forced vital capacity (FVC) of ≥80 percent [10]. Compared with placebo, functional decline at 24 weeks was smaller in the edaravone group, and the difference was considered clinically meaningful. Edaravone was approved in 2015 for the treatment of ALS in Japan and Korea and has now received regulatory approval to treat patients with ALS in the United States [11]. We now suggest edaravone for patients with early-stage disease as well as for those with more advanced disease, although the data are less compelling for the latter group. (See "Disease modifying treatment of amyotrophic lateral sclerosis", section on 'Edaravone'.)

NEUROONCOLOGY

Duration of postradiation temozolomide in glioblastoma (July 2017)

Postradiation monthly temozolomide is a standard component of initial therapy for glioblastoma, but the number of cycles has been subject to variation, with some centers treating beyond six cycles for patients with stable disease. In a retrospective study of over 600 patients with glioblastoma enrolled in four randomized trials who were free of progression after six cycles of adjuvant temozolomide, receipt of more than six cycles was associated with a slight improvement in progression-free survival but no difference in overall survival [12]. Since extended adjuvant therapy exposes patients to ongoing treatment-related toxicities, these results support our practice of stopping adjuvant therapy after six cycles of monthly temozolomide. (See "Initial postoperative therapy for glioblastoma and anaplastic astrocytoma", section on 'Treatment duration'.)

Postoperative stereotactic radiosurgery for resected brain metastases (July 2017)

Two randomized trials lend further support to the use of postoperative stereotactic radiosurgery (SRS) rather than whole brain radiation therapy (WBRT) or observation after resection of a single brain metastasis. In one trial, patients treated with single-fraction SRS to the surgical cavity had improved neurocognitive function and similar overall survival compared with those treated with WBRT [13]. In the second trial, single-fraction SRS decreased rates of local recurrence compared with observation, especially for tumors smaller than 2.5 cm preoperatively [14]. Larger tumors may be better suited for multiple-fraction SRS, which allows for a higher dose of radiation to be delivered to the surgical cavity. (See "Overview of the treatment of brain metastases", section on 'Postoperative radiation'.)

PEDIATRIC NEUROLOGY

Hematopoietic stem cell gene therapy for adrenoleukodystrophy (October 2017)

Childhood cerebral adrenoleukodystrophy (ALD) is a severe neurologic disease that rapidly progresses to total disability and death unless treated with allogeneic hematopoietic cell transplantation (HCT), which has considerable morbidity and mortality. Gene therapy with autologous hematopoietic stem cells is emerging as a possible alternative treatment. A study of 17 boys with early-stage cerebral ALD enrolled to undergo transplantation with autologous CD34+ cells transfected with Lenti-D (a lentiviral vector containing manufactured ABCD1 complementary DNA) reported 88 percent were alive with no major functional disabilities at 24 months posttransplantation [15]. One boy died from disease progression that began during pretransplantation conditioning, and one was withdrawn from the study and died from complications of subsequent allogeneic HCT. None of the survivors had evidence of graft failure or graft-versus-host disease. These results suggest that autologous hematopoietic stem cell gene therapy may be as effective as, and safer than, HCT for treatment of early cerebral ALD. The treatment has not received regulatory approval. The clinical trial is ongoing and important uncertainties remain. (See "Adrenoleukodystrophy", section on 'Gene therapy'.)

Complex motor behaviors during REM sleep in children with narcolepsy type 1 (September 2017)

Complex motor behaviors during rapid eye movement (REM) sleep are well described in adults with narcolepsy, but their prevalence in children has not been well documented. In a series of 40 children with narcolepsy type 1 who underwent video polysomnography, nearly one-third of patients exhibited motor behaviors during REM sleep ranging from classic dream enactment (eg, vigorous reaching or throwing movements) to more calm, slow pantomime-like events [16]. Events were more common among children with impaired nighttime sleep, worse daytime sleepiness, and severe cataplexy. (See "Narcolepsy in children", section on 'Other sleep disturbances'.)

Genetic testing in neonates with epileptic encephalopathy (August 2017)

The role of genetic testing in the clinical care of neonates with epilepsy is evolving as the number of monogenetic causes of early epileptic encephalopathy increases and specific treatments become available for some syndromes. In a prospective cohort study of over 600 consecutive newborns with seizures, 13 percent had an epilepsy syndrome, including 35 infants (6 percent) with epileptic encephalopathy [17]. Among these, the large majority had a genetic etiology identified by genetic testing, most commonly KCNQ2 encephalopathy, for which sodium channel blocking antiseizure drugs are a preferred therapy. We pursue genetic testing in neonates with epilepsy who do not have an acute symptomatic cause identified on initial history, examination, and neuroimaging. (See "Clinical features, evaluation, and diagnosis of neonatal seizures", section on 'Genetic testing'.)

Low yield of lumbar puncture after complex febrile seizure (July 2017)

After a febrile seizure, lumbar puncture to assess for infection can be avoided in most well-appearing children who have returned to their baseline, even when the febrile seizure has complex features (ie, focal onset, >15 minutes in duration, or recurrent within 24 hours). In a multicenter cohort study of more than 800 children age six months to five years presenting to a pediatric emergency department with a complex febrile seizure, rates of bacterial meningitis and herpes simplex encephalitis were 0.7 and 0 percent, respectively [18]. All five cases of infection occurred in children with a clinical examination suggestive of meningitis. (See "Clinical features and evaluation of febrile seizures", section on 'Lumbar puncture'.)

Consensus panel guidelines on Dravet syndrome in children and adults (April 2017)

A North American consensus panel has published guidelines on the diagnosis and management of Dravet syndrome (DS), an early-onset epileptic encephalopathy most often due to de novo mutations in the voltage-gated sodium channel, alpha-1 subunit (SCN1A) gene [19]. The document includes a description of the typical clinical presentation of DS; guidance on genetic testing and family counseling; and recommendations for first-line treatment with clobazam and/or valproic acid, avoidance of sodium channel blocking drugs, and provision of home rescue medications and an emergency seizure protocol. DS should be suspected in previously healthy infants presenting with recurrent tonic-clonic seizures, often in the setting of fever, beginning before one year of age and associated with neurodevelopmental regression after the onset of seizures. (See "Dravet syndrome: Genetics, clinical features, and diagnosis" and "Dravet syndrome: Management and prognosis".)

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REFERENCES

  1. Anderson CS, Arima H, Lavados P, et al. Cluster-Randomized, Crossover Trial of Head Positioning in Acute Stroke. N Engl J Med 2017; 376:2437.
  2. Livingston G, Sommerlad A, Orgeta V, et al. Dementia prevention, intervention, and care. Lancet 2017.
  3. Kane RL, Bulter M, Fink HA, et al. Interventions to prevent age-related cognitive decline, mild cognitive impairment, and clinical Alzheimer's-type dementia: Comparative effectiveness review No. 188. AHQR Pub. No. 17-EHC008-EF, Agency for Healthcare Research and Quality, Rockville, MD 2017.
  4. McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology 2017; 89:88.
  5. Metz LM, Li DKB, Traboulsee AL, et al. Trial of Minocycline in a Clinically Isolated Syndrome of Multiple Sclerosis. N Engl J Med 2017; 376:2122.
  6. Devinsky O, Cross JH, Laux L, et al. Trial of Cannabidiol for Drug-Resistant Seizures in the Dravet Syndrome. N Engl J Med 2017; 376:2011.
  7. Athauda D, Maclagan K, Skene SS, et al. Exenatide once weekly versus placebo in Parkinson's disease: a randomised, double-blind, placebo-controlled trial. Lancet 2017.
  8. Dalvin LA, Damento GM, Yawn BP, et al. Parkinson Disease and Melanoma: Confirming and Reexamining an Association. Mayo Clin Proc 2017; 92:1070.
  9. Hauser RA, Factor SA, Marder SR, et al. KINECT 3: A Phase 3 Randomized, Double-Blind, Placebo-Controlled Trial of Valbenazine for Tardive Dyskinesia. Am J Psychiatry 2017; 174:476.
  10. Writing Group, Edaravone (MCI-186) ALS 19 Study Group. Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial. Lancet Neurol 2017; 16:505.
  11. FDA approves drug to treat ALS. https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm557102.htm (Accessed on May 09, 2017).
  12. Blumenthal DT, Gorlia T, Gilbert MR, et al. Is more better? The impact of extended adjuvant temozolomide in newly diagnosed glioblastoma: a secondary analysis of EORTC and NRG Oncology/RTOG. Neuro Oncol 2017; 19:1119.
  13. Brown PD, Ballman KV, Cerhan JH, et al. Postoperative stereotactic radiosurgery compared with whole brain radiotherapy for resected metastatic brain disease (NCCTG N107C/CEC·3): a multicentre, randomised, controlled, phase 3 trial. Lancet Oncol 2017; 18:1049.
  14. Mahajan A, Ahmed S, McAleer MF, et al. Post-operative stereotactic radiosurgery versus observation for completely resected brain metastases: a single-centre, randomised, controlled, phase 3 trial. Lancet Oncol 2017; 18:1040.
  15. Eichler F, Duncan C, Musolino PL, et al. Hematopoietic Stem-Cell Gene Therapy for Cerebral Adrenoleukodystrophy. N Engl J Med 2017.
  16. Antelmi E, Pizza F, Vandi S, et al. The spectrum of REM sleep-related episodes in children with type 1 narcolepsy. Brain 2017; 140:1669.
  17. Shellhaas RA, Wusthoff CJ, Tsuchida TN, et al. Profile of neonatal epilepsies: Characteristics of a prospective US cohort. Neurology 2017; 89:893.
  18. Guedj R, Chappuy H, Titomanlio L, et al. Do All Children Who Present With a Complex Febrile Seizure Need a Lumbar Puncture? Ann Emerg Med 2017; 70:52.
  19. Wirrell EC, Laux L, Donner E, et al. Optimizing the Diagnosis and Management of Dravet Syndrome: Recommendations From a North American Consensus Panel. Pediatr Neurol 2017; 68:18.
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