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Holly E. Hinson, MD, MCR, FAAN Associate Professor of Neurology and Emergency Medicine Oregon Health & Science University. What’s New in Neurology? Recent Discoveries. Courtney Takahashi, MD MCR Assistant Professor of Neurology and Neurosurgery Boston University Medical Center.
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Holly E. Hinson, MD, MCR, FAAN Associate Professor of Neurology and Emergency Medicine Oregon Health & Science University What’s New in Neurology? Recent Discoveries Courtney Takahashi, MD MCR Assistant Professor of Neurology and Neurosurgery Boston University Medical Center
Overview: Updates in Neurology Neurologists are traditionally characterized as “diagnosticians” but this stereotype is rapidly changing! In the past 5 years, new treatments and diagnostic advances have changed every subspecialty of neurology.
Vascular Neurology: Background • Before 1995: Stroke patients are managed for secondary prevention only • 1996: tPA approved for the treatment of acute stroke, shown to improve functional outcomes (modified rankin scores) at 3 months • 2004: Thrombectomy devices approved • 2012: Stent-trievers introduced
Expanding the Window for Acute Stroke Treatment with Imaging CT-perfusion and MR perfusion are more readily accessible to evaluate patients outside the classic 6-hour time window for intervention Donahue. Journal of NeuroradiologyVolume 42, Issue 1, 2015
Hyperacute MRI May Change tPA Time Windows NEJM published data that suggest MRI mismatch may be able to supplement and possibly replace time windows based on last known well times.
Results: MRI Guided Thrombolysis for Stroke / WAKE up Gerloff et al, MRI-Guided Thrombolysis for Stroke with Unknown Time of Onset, New England Journal of Medicine, 16 May 2018
IN 2015: New Era of Acute Stroke Therapy Five recent randomized controlled trials Clear efficacy and overwhelming benefit: Intra-arterial thrombectomy Standard of care: Class I, level of evidence A
Summary of Thrombectomy Trials Gory et al, Stent retriever thrombectomy for acute ischemic stroke: Indications, results, and management in 2015, Diagnostics and Interventional Imaging (2016) 97, 141-149
Background: Chronic Traumatic Encephalopathy (CTE) • CTE is a progressive tauopathy that occurs after repeated traumatic brain injury • Pathological changes associated with the disease and the clinical manifestations associated with each pathological stage Stage I: Headache, loss of attention, concentration Stage 2: Depression, memory loss Stage 3: Executive dysfunction, cognitive impairment Stage 4: Dementia, word finding difficulty, aggression McKee et al, The spectrum of disease in chronic traumatic encephalopathy, Brain, 2013: Jan 136 (1):43-63
Traumatic Brain Injury: New Innovations • 50 million people per year worldwide suffer from TBI • Given long term effects with CTE, TBI research deserves promotion • Many TBI sufferers, however, do not have access to CT scanner
Detecting Traumatic Brain Injury • Adults with GCS 9-15 within 12 hours of injury • Alert TBI: Banyan UCH-L1/GFAP Detection Assay • Correlation of assay result with absence of acute intracranial lesions Bazarian. Lancet Neurol. 2018 Jul 24
ALERT-TBI: Results • Can blood-based biomarkers (GFAP, UCH-L1) predict head injury after TBI? • Primary study outcomes: test sensitivity and negative predictive value (NPV) • 1977 patients enrolled; sensitivity 0.976 and NPV 0.996. Less than 1% of patients had a positive CT scan when the test was negative. Bazarian et al, Serum GFAP and UCH-L1 for prediction of absence of intracranial injuries on head CT (ALERT-TBI): a multicentre observational study, The Lancet Neurology, Vol 17, issue 9, September 2018, 782-789
Background: Spinal Muscular Atrophy • Genetic disorders that cause degeneration of anterior horn cells, leading to weakness and muscle atrophy • Four phenotypes defined by severity and age of onset. 60% of cases occur before age 1 and classify most severe form Image courtesy of Prime Health Channel: Spinal Muscular Atrophy
Treatments for Spinal Muscular Atrophy • Degenerative neuromuscular disorder due to insufficient SMN1 protein • Manifests at truncal weakness before age 1 in the most severe cases. Prognosis historically poor—usually death in childhood. Crawford et al, Two breakthrough gene-targeted treatments for spinal muscular atrophy: challenges remain. Journal of Clinical Investigation. 2018; 128 (8): 3219-3227
Treatment Options: Spinal Muscular Atrophy • Nusinersen/Spinraza (ASO) alters gene splicing mechanisms in pre-mRNA quantities of SMN2 proteins • Avexis-101 (scAAV9-SMN) delivers exogenous SMN1cDNA through viral vectors
Background: Myasthenia Gravis • Myasthenia gravis is an autoimmune disease that prevents transmission between motor neuron and muscle • Traditional treatments include symptom control with pyridostigmine and immunosuppression with steroids, azathioprine, and mycophenolatemofetil
Myasthenia Gravis: Eculizumab • Eculizumab is the first FDA-approved drug since the 1950s for the treatment of refractory, generalized, antibody positive myasthenia gravis • Originally distributed for the treatment of paroxysmal nocturnal hemoglobinuria • Blocks complement (C5) • Advantages include FASTER onset and evaluation for efficacy compared to traditional immunosuppressants
Background: Dystonia • Dystonia consists of abnormal, sustained muscle contractions, usually in agonist and antagonist groups, which lead to odd posturing • First line medications: anticholingergics, benzodiazepines, baclofen, PT/OT • Deep brain stimulation may be considered for treatment
Background: Dystonia Images Globus Pallidus
Deep Brain Stimulation for Generalized Dystonia http://www.youtube.com/watch?v=wk3wz4gQZ3Q
Acknowledgement to Dr. Mohamad Abdalkader from Boston University Medical Center for assistance with these slides
