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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: Apr 2017. | This topic last updated: May 11, 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

Anesthesia for patients with acute ischemic stroke undergoing mechanical thrombectomy (January 2017)

The type of anesthesia used for patients with acute ischemic stroke undergoing mechanical thrombectomy may affect outcomes, but data are limited and inconsistent. In a randomized trial testing general anesthesia versus conscious sedation in this setting, there was no significant difference between the two groups for the outcome of early neurologic improvement [1]. Subjects assigned to general anesthesia were more likely to achieve functional independence at three months, but small patient numbers lower the confidence in this result. The findings of this trial conflict with earlier reports that general anesthesia was associated with worse outcomes. (See "Reperfusion therapy for acute ischemic stroke", section on 'Mechanical thrombectomy'.)

DEMENTIA

Antipsychotic drugs and risk of falls and fracture (March 2017)

In a large, population-based sample of Finnish people with Alzheimer disease, new users of antipsychotic medication had an increased risk of hip fractures from the first days of use [2]. Subsequent to multiple similar reports in patients with varied disorders, the US Food and Drug Administration (FDA) issued a warning that antipsychotic drugs may cause falls and fractures as a result of somnolence, postural hypotension, and/or motor and sensory instability, and recommended that a fall risk assessment be completed when initiating antipsychotic treatment and recurrently for patients continuing on long-term antipsychotics. (See "Second-generation antipsychotic medications: Pharmacology, administration, and side effects", section on 'Falls'.)

Air pollution and the risk of dementia (February 2017)

Two recent studies highlight the potential role of air pollution as a contributor to cognitive decline and dementia. In a population-based study of older adults in Ontario, Canada, residing in close proximity to a major traffic road was associated with a 7 percent increase in the relative risk of incident dementia after adjusting for multiple potential confounders [3]. In another cohort study, the adjusted relative risk of all-cause dementia was nearly twofold higher among older women living in areas with high concentrations of fine particulate matter compared with those living in areas with low concentrations [4]. In a subgroup analysis, the effect size was largest in women with genetic susceptibility to Alzheimer disease, and in mouse models, fine particulate exposure promoted amyloid deposition and caused selective hippocampal atrophy. (See "Risk factors for cognitive decline and dementia", section on 'Toxins and air pollution'.)

High-risk drug prescribing in adults with dementia (February 2017)

Older adults with dementia are at heightened risk for adverse drug effects from anticholinergic drugs, benzodiazepines, and opioids, among many others. Despite these risks, polypharmacy remains common in this population. In a study that included over 75,000 adults with dementia, 44 percent of patients were prescribed at least one potentially unsafe medication (mostly drugs with high anticholinergic activity), and rates were consistently higher in patients receiving care from multiple providers [5]. These results highlight the need for careful monitoring of drug therapy in patients with dementia and the importance of communication among providers before starting new therapies. (See "Safety and societal issues related to dementia", section on 'Polypharmacy'.)

Decision aids for advance care planning in dementia (February 2017)

Structured interventions to improve advance care planning among families of patients with advanced dementia have not been well studied. In a cluster randomized trial, use of a video decision aid and structured family meeting resulted in better communication about end of life care, greater use of medical orders defining scope of treatment, and fewer hospital transfers compared with a control intervention (informational video and usual care plan meeting) [6]. Audiovisual decision aids and structured meetings may thus be effective components of multidimensional decision-making support for families of patients with advanced dementia. (See "Palliative care of patients with advanced dementia", section on 'Advance care planning'.)

DEMYELINATING DISEASE

Ocrelizumab for primary progressive multiple sclerosis (April 2017)

Ocrelizumab is the first drug to reduce the risk of disability progression among patients with primary progressive multiple sclerosis (PPMS), as shown by the multicenter ORATORIO randomized trial [7]. Compared with placebo, ocrelizumab modestly reduced the proportion of patients with disability progression at 24 weeks (30 versus 36 percent). In addition, ocrelizumab slowed deterioration from baseline to week 120 on the timed 25-foot walk and led to improvements on other endpoints. While the long-term risks of infection and neoplasm with ocrelizumab are uncertain, there are no other disease-modifying therapies for PPMS. Therefore, we suggest treatment with ocrelizumab for most patients with PPMS. Ocrelizumab has also been approved by the US Food and Drug Administration for use in relapsing-remitting MS (RRMS), although its role in the early treatment of this form of the disease remains to be determined. (See "Treatment of progressive multiple sclerosis in adults", section on 'Ocrelizumab'.)

Ocrelizumab for relapsing multiple sclerosis (January 2017)

Two randomized, controlled trials in patients with relapsing-remitting multiple sclerosis (RRMS) have found that ocrelizumab, a recombinant anti-CD20 monoclonal antibody, is more effective than interferon beta-1a for reducing relapses and may also slow disability progression [8]. In both trials, ocrelizumab significantly reduced the relapse rate and the mean number of gadolinium-enhancing brain lesions on magnetic resonance imaging (MRI) scan. In a pooled analysis, ocrelizumab also reduced the proportion of subjects with confirmed disability progression at 24 weeks. Infusion reactions were the most common adverse effect of ocrelizumab, and there were more neoplasms with ocrelizumab than with interferon beta-1a (0.5 versus 0.2 percent). In March 2017, The US Food and Drug Administration (FDA) approved ocrelizumab for relapsing MS. The role of this agent in the treatment of RRMS remains to be clarified pending further safety data on rates of infection and neoplasm. (See "Disease-modifying treatment of relapsing-remitting multiple sclerosis in adults", section on 'Ocrelizumab'.)

EPILEPSY

Revised ILAE classification of seizures and epilepsy (March 2017)

The International League Against Epilepsy (ILAE) has revised its working classification of seizures and epilepsy [9-11]. This is the first major update since 2010. The current framework allows for diagnosis at four levels, according to seizure type (level 1) (table 1), epilepsy type (level 2), epilepsy syndrome (level 3) (table 2), and epilepsy with etiology (level 4). The proposal recognizes six etiologic categories of epilepsy: genetic, structural, metabolic, immune, infectious, and unknown (figure 1). (See "ILAE classification of seizures and epilepsy", section on 'Introduction'.)

Premature mortality in people with epilepsy (February 2017)

People with epilepsy are at increased risk for premature mortality compared with the general population. In systematic reviews commissioned by the International League Against Epilepsy (ILAE), standardized mortality ratios (SMRs) across all age groups indicate that the observed number of deaths in people with epilepsy is two- to threefold higher than in reference populations without epilepsy [12,13]. SMRs are substantially higher in children compared with adults and among those with structural/metabolic etiologies, medically-refractory epilepsy, and convulsive seizures. Causes of death attributable to epilepsy include convulsive status epilepticus, unintentional injuries, suicide, and sudden unexpected death in epilepsy (SUDEP). (See "Seizures and epilepsy in children: Refractory seizures and prognosis", section on 'Mortality' and "Overview of the management of epilepsy in adults", section on 'Mortality'.)

No benefit of hypothermia in convulsive status epilepticus (January 2017)

Convulsive status epilepticus is prolonged and refractory to standard first-line medications in approximately one-third of patients, and outcomes associated with refractory status epilepticus are often poor. Induced hypothermia has been proposed as a potential neuroprotective strategy. However, in a randomized trial of 250 critically ill adults with convulsive status epilepticus, induced hypothermia did not improve 90-day mortality, functional outcomes, or rate of progression to refractory status epilepticus when added to standard first-line therapies [14]. (See "Convulsive status epilepticus in adults: Treatment and prognosis", section on 'Others'.)

MOVEMENT DISORDERS

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 [15]. 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

Nusinersen for spinal muscular atrophy (January 2017)

Nusinersen, an antisense oligonucleotide, is the first drug approved to treat spinal muscular atrophy (SMA) by the US Food and Drug Administration (FDA). In an interim analysis of the double-blind ENDEAR trial, which enrolled 82 infants with SMA, improvement in motor milestones was observed in 40 percent of patients treated with intrathecal nusinersen, versus none for those who received the sham procedure [16]. The FDA based its approval upon data from this trial and open-label studies in older patients with SMA [17,18]. We recommend nusinersen for most infants with SMA and select children ages 2 to 12 years with SMA. (See "Spinal muscular atrophy", section on 'Nusinersen'.)

Diaphragmatic pacing harmful in patients with amyotrophic lateral sclerosis (January 2017)

Pacing the diaphragm in patients with amyotrophic lateral sclerosis (ALS) is controversial. A recent randomized trial of patients with early respiratory impairment from ALS reported that, compared with sham pacing, diaphragmatic pacing resulted in increased mortality and did not delay time to noninvasive ventilation [19]. We continue to prefer to avoid diaphragmatic pacing in this population until future trials demonstrate a clear benefit. (See "Pacing the diaphragm: Patient selection, evaluation, implantation, and complications", section on 'Amyotrophic lateral sclerosis'.)

NEUROONCOLOGY

Revised clinical criteria for neurofibromatosis type 2 (March 2017)

Clinical criteria for neurofibromatosis type 2 (NF2), caused by mutations in the NF2 gene, have been modified to include requirement for negative LZTR1 genetic testing in patients with a unilateral vestibular schwannoma and two or more non-intradermal schwannomas [20]. Previously, these patients would have met clinical criteria for NF2. The change was prompted by recognition that the phenotypic spectrum of both NF2 and LZTR1-related schwannomatosis includes unilateral vestibular schwannoma in selected patients. (See "Neurofibromatosis type 2", section on 'Clinical criteria'.)

PEDIATRIC NEUROLOGY

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 [21]. 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

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  2. Koponen M, Taipale H, Lavikainen P, et al. Antipsychotic Use and the Risk of Hip Fracture Among Community-Dwelling Persons With Alzheimer's Disease. J Clin Psychiatry 2017; 78:e257.
  3. Chen H, Kwong JC, Copes R, et al. Living near major roads and the incidence of dementia, Parkinson's disease, and multiple sclerosis: a population-based cohort study. Lancet 2017; 389:718.
  4. Cacciottolo M, Wang X, Driscoll I, et al. Particulate air pollutants, APOE alleles and their contributions to cognitive impairment in older women and to amyloidogenesis in experimental models. Transl Psychiatry 2017; 7:e1022.
  5. Thorpe JM, Thorpe CT, Gellad WF, et al. Dual Health Care System Use and High-Risk Prescribing in Patients With Dementia: A National Cohort Study. Ann Intern Med 2017; 166:157.
  6. Hanson LC, Zimmerman S, Song MK, et al. Effect of the Goals of Care Intervention for Advanced Dementia: A Randomized Clinical Trial. JAMA Intern Med 2017; 177:24.
  7. Montalban X, Hauser SL, Kappos L, et al. Ocrelizumab versus Placebo in Primary Progressive Multiple Sclerosis. N Engl J Med 2017; 376:209.
  8. Hauser SL, Bar-Or A, Comi G, et al. Ocrelizumab versus Interferon Beta-1a in Relapsing Multiple Sclerosis. N Engl J Med 2017; 376:221.
  9. Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia 2017; 58:522.
  10. Fisher RS, Cross JH, D'Souza C, et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia 2017; 58:531.
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  14. Legriel S, Lemiale V, Schenck M, et al. Hypothermia for Neuroprotection in Convulsive Status Epilepticus. N Engl J Med 2016; 375:2457.
  15. 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.
  16. FDA approves first drug for spinal muscular atrophy. U.S. Food & Drug Administration. www.fda.gov/newsevents/newsroom/pressannouncements/ucm534611.htm (Accessed on January 03, 2017).
  17. Finkel RS, Chiriboga CA, Vajsar J, et al. Treatment of infantile-onset spinal muscular atrophy with nusinersen: a phase 2, open-label, dose-escalation study. Lancet 2016; 388:3017.
  18. Chiriboga CA, Swoboda KJ, Darras BT, et al. Results from a phase 1 study of nusinersen (ISIS-SMN(Rx)) in children with spinal muscular atrophy. Neurology 2016; 86:890.
  19. Gonzalez-Bermejo J, Morélot-Panzini C, Tanguy ML, et al. Early diaphragm pacing in patients with amyotrophic lateral sclerosis (RespiStimALS): a randomised controlled triple-blind trial. Lancet Neurol 2016; 15:1217.
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  21. 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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