Presentation on theme: "Title Here 1.Messacar K, Schreiner TL, Maloney JA, Wallace A, Ludke J, Oberste MS, Nix WA, Robinson CC, Glodé MP, Abzug MJ, Dominguez SR.; A cluster of."— Presentation transcript:
Title Here 1.Messacar K, Schreiner TL, Maloney JA, Wallace A, Ludke J, Oberste MS, Nix WA, Robinson CC, Glodé MP, Abzug MJ, Dominguez SR.; A cluster of acute flaccid paralysis and cranial nerve dysfunction temporally associated with an outbreak of enterovirus D68 in children in Colorado, USA. Lancet Jan 28. pii: S (14) Pastula DM, Aliabadi N, Haynes AK, Messacar K, Schreiner T, Maloney J, Dominguez SR, Davizon ES, Leshem E, Fischer M, Nix WA, Oberste MS, Seward J, Feikin D, Miller L; Centers for Disease Control and Prevention (CDC). Acute neurologic illness of unknown etiology in children - Colorado, August-September MMWR Morb Mortal Wkly Rep Oct 10;63(40): Maloney JA, Mirsky DM, Messacar K, Dominguez SR, Schreiner T, Stence NV. MRI findings in children with acute flaccid paralysis and cranial nerve dysfunction occurring during the 2014 enterovirus D68 outbreak. AJNR Am J Neuroradiol Feb;36(2): Results References Conclusions Background Methods Acute Flaccid Myelitis and Cranial Nerve Dysfunction in Children Temporally Associated with an Enterovirus D68 Outbreak in Colorado, 2014 Teri L Schreiner, MD MPH, Kevin Messacar, MD, John Maloney, MD, Adam Wallace, MD, Jan Ludke, MD and Samuel Dominguez, MD Children’s Hospital Colorado, Anschutz Medical Campus, CO Implications This first geographically and temporally defined cluster of AFM and cranial nerve dysfunction in children associated with an outbreak of EV D68 respiratory illness suggests the possibility of a link between EV D68 and severe neurologic damage in children Case definition: any child admitted to Children’s Hospital Colorado between August 1 and October 31, 2014 with AFM, and/or cranial nerve dysfunction with spinal cord gray matter, and/or brainstem lesions on MRI Cases were enrolled into an existing, IRB approved study of encephalitis in children Clinical, laboratory and neuroimaging results were collected Evaluation for viral and bacteria pathogens was undertaken in nasopharyngeal, blood, CSF and stool samples An epidemiologic review of historical cases and respiratory admissions was conducted Thirteen children met the case definition (median age 10 years, range 1-18 years, nine male); 12 seen at Children’s Hospital Colorado, 1 seen at Denver Health Medical Center Twelve were fully vaccinated; one was immunosuppressed Clinical Features: All had prodromal febrile illness preceding neurologic symptoms by a median of 7 days (range 3-16) Neurologic deficits included: Flaccid limb weakness (n=9, 6 asymmetric), Bulbar weakness (n=7), Cranial nerve VI dysfunction (n=3), and Cranial nerve VII dysfunction (n=2) No patient had encephalopathy Laboratory results: Nasopharyngeal specimens from nine of twelve children tested on admission were positive for rhinovirus/enterovirus by polymerase chain reaction (PCR) Five subsequently typed as EV D68 EV PCR of cerebrospinal fluid, blood, and rectal swabs were negative MRI results: Eleven had confluent, longitudinally extensive spinal cord lesions of the central gray matter Nine had brainstem lesions Six had nerve root enhancement None had supratentorial lesions Electromyography and nerve conduction studies: Electrophysiological studies were done on six children and consistent with motor neuronopathy/neuropathy All 6 children had normal sensory nerve conduction studies Cluster of AFM cases occurred concurrent to respiratory outbreak of EV D68 Recovery to date has been slow and incomplete for most in this cohort of patients despite treatment with a variety of therapies Clusters of acute flaccid paralysis (AFP) with cranial nerve dysfunction are uncommon August - October 2014, we identified a cluster of children with AFP and cranial nerve dysfunction temporally associated with an enterovirus D68 respiratory outbreak Acute Flaccid Myelitis (AFM): sudden onset of weakness in one or more arms or legs with MRI scans with inflammation of the gray matter in the spinal cord Contact: Axial T1-weighted post- contrast image at the level of the conus medullaris demonstrating ventral nerve root enhancement. Sagittal T2-weighted image of the thoracic spinal cord showing longitudinally extensive T2 hyperintense lesion involving the gray matter. The authors have no relevant disclosures. Respiratory Outbreak Between August 1, 2014 and September 30, 2014, there was a 77% increase in admissions to Children’s Hospital Colorado for respiratory illnesses Cluster of AFM Cluster of cases with AFM was three times higher than any 3 month period over the prior 4 years Axial T2-weighted image in the thoracic spine showing T2 hyperintense lesions involving the anterior horns. Axial FLAIR image through the posterior fossa with hyperintense lesion in the right dorsolateral pons Outcomes Twelve children have residual deficits to date Next Steps National studies are underway to investigate viral pathogenicity, genetic factors that may predispose patients to AFM, epidemiologic studies to link EV D68 exposure to AFM, and surveillance for reemergence of EV D68 and AFM In Colorado, we have established a multidisciplinary clinic with representation from neurology, rehabilitation, psychology, occupational therapy and physical therapy to address the many physical and emotional needs of these children Patient-reported outcomes are collected to better characterize the implications of AFM This 18 yo M presented with bulbar weakness, back pain and left arm weakness following upper respiratory infection. Respiratory PCR was positive for enterovirus/rhinovirus. Additional testing at the CDC specified EV D68. MRI showed T2 nonenhancing lesions in the dorsal medulla, cervicomedullary junction and a longitudinally extensive ventral-predominant spinal cord lesion Seven months after presentation, he has muscle atrophy of left scapular muscles, left arm and shoulder. The left shoulder is anteriorly subluxed. Acute Flaccid Myelitis and Cranial Nerve Dysfunction in Children Temporally Associated with an Enterovirus D68 Outbreak in Colorado, 2014 Teri L Schreiner, MD MPH, Kevin Messacar, MD, John Maloney, MD, Adam Wallace, MD, Jan Ludke, MD and Samuel Dominguez, MD Children’s Hospital Colorado, Anschutz Medical Campus, CO Cases in purple represent the AFM cluster. Cases in light gray are historical cases that met the case definition, but had clinical or radiographic findings that distinguished them from the current cluster. Cases in black met case definition without any incongruent features.