Acute Flaccid Paralysis With Anterior Myelitis — California, June 2012–June 2014

Patrick Ayscue, DVM; Keith Van Haren, MD; Heather Sheriff; Emmanuelle Waubant, MD, PhD; Paul Waldron, MD; Shigeo Yagi, PhD; Cynthia Yen, MPH; Anna Clayton, MPH; Tasha Padilla; Chao Pan, MPH; John Reichel; Kathleen Harriman, PhD; James Watt, MD; James Sejvar, MD; William Allan Nix; Daniel Feikin, MD; Carol Glaser, DVM, MD

Disclosures

Morbidity and Mortality Weekly Report. 2014;63(40):903-906. 

In This Article

Abstract and Introduction

Introduction

In August 2012, the California Department of Public Health (CDPH) was contacted by a San Francisco Bay area clinician who requested poliovirus testing for an unvaccinated man aged 29 years with acute flaccid paralysis (AFP) associated with anterior myelitis (i.e., evidence of inflammation of the spinal cord involving the grey matter including anterior horn cell bodies) and no history of international travel during the month before symptom onset. Within 2 weeks, CDPH had received reports of two additional cases of AFP with anterior myelitis of unknown etiology. Testing at CDPH's Viral and Rickettsial Disease Laboratory for stool, nasopharyngeal swab, and cerebrospinal fluid (CSF) did not detect the presence of an enterovirus (EV), the genus of the family Picornaviridae that includes poliovirus. Additional laboratory testing for infectious diseases conducted at the CDPH Viral and Rickettsial Disease Laboratory did not identify a causative agent to explain the observed clinical syndrome reported among the patients. To identify other cases of AFP with anterior myelitis and elucidate possible common etiologies, CDPH posted alerts in official communications for California local health departments during December 2012, July 2013, and February 2014. Reports of cases of neurologic illness received by CDPH were investigated throughout this period, and clinicians were encouraged to submit clinical samples for testing. A total of 23 cases of AFP with anterior myelitis of unknown etiology were identified. Epidemiologic and laboratory investigation did not identify poliovirus infection as a possible cause for the observed cases. No common etiology was identified to explain the reported cases, although EV-D68 was identified in upper respiratory tract specimens of two patients. EV infection, including poliovirus infection, should be considered in the differential diagnosis in cases of AFP with anterior myelitis and testing performed per CDC guidelines.[1]

A case was defined as AFP in at least one limb consistent with anterior myelitis, as indicated by neuroimaging of the spine or electrodiagnostic studies (e.g., nerve conduction studies and electromyography), and with no known alternative etiology, in a person with symptom onset during January 2012–June 2014. Among the 23 cases identified, younger persons and males were more frequently affected, with a median age of 10 years (range = 1–73 years); 15 were aged <15 years, and 56% were male. Similar to the race/ethnicity distribution in California, seven (30%) patients were white, six (26%) were Asian, six (26%) were Hispanic, one (4%) was black, one (4%) was of multiple race, and two (9%) were of unknown race. Affected patients resided in diverse geographic areas throughout California with no indication of clustering. During the 30-month inquiry, no indication of seasonality or temporal trends in disease onset was established (Figure).

Figure.

Number of cases of acute flaccid paralysis with anterior myelitis (N = 23), by month of neurologic symptom onset — California, 2012–2014

Common features among the clinical presentations of patients included an upper respiratory or gastrointestinal prodrome <10 days before AFP onset (83%), CSF pleocytosis (83%), and absence of sensory deficits (78%). Ten patients (43%) also had concomitant mental status changes; eight patients (34%) had cranial nerve abnormalities. Patients typically had extended hospital stays (median = 17 days), and of 13 patients with available information, all had prolonged paralysis persisting at 60 days follow-up. Five patients were ventilator-dependent when discharged from the hospital to rehabilitation facilities, and one death was reported in an adult. Of 10 patients with mental status changes, eight (80%) had returned to baseline cognitive function at the time of discharge.

Specimens were available for testing from 19 (83%) of the patients. The CDPH Viral and Rickettsial Disease Laboratory tested nasopharyngeal or throat swabs (18), stool or rectal swabs (14), serum (17), and CSF (17) for evidence of recent infection with numerous infectious agents, including EVs (including poliovirus), arboviruses, herpes viruses (HSV-1, HSV-2, VZV, and EBV), parechoviruses, adenoviruses, rabies, influenza A and B, human metapneumovirus, respiratory syncytial virus, parainfluenza 1–4, Mycoplasma pneumoniae, rickettsial pathogens, and free-living amoebas. Results were unremarkable except for the following: 1) mycoplasma immunoglobulin M–positive serologies in two patients (these same patients had negative mycoplasma throat polymerase chain reaction [PCR] tests), 2) rhinovirus-positive PCR from a respiratory specimen in one patient, and 3) EV-positive PCR from throat or upper respiratory tract specimens in two patients; these EVs were sequenced as EV-D68. Testing was limited by incomplete and late collection of specimens; respiratory samples collected <7 days of paralysis onset were submitted for nine (39%) patients, and stool or rectal specimens were submitted for 15 (65%) patients. Specimens meeting World Health Organization (WHO) or CDC guidelines for poliovirus detection (e.g., two stool specimens collected ≥24 hours apart and <14 days after symptom onset) were submitted for only two of the patients. Serologic testing was of limited value because specimens often were collected after patients had received intravenous immunoglobulin (IVIG) therapy.

Poliovirus was determined to be an unlikely etiology for any of the cases based on epidemiologic and limited laboratory investigation findings. Nonetheless, because AFP with anterior myelitis is the classic presentation of paralytic poliomyelitis, CDPH attempted to rule out poliovirus infection. Of 14 patients with available information, 12 had previously received polio vaccine; one child and one adult were unvaccinated because of personal belief exemptions. Pre-IVIG serum was available from the unvaccinated child and tested negative for neutralizing antibodies against poliovirus at CDC laboratories. None of the patients reported travel out of the United States during the month before symptom onset.

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