Acute Neurologic Illness with Focal Limb Weakness of Unknown Etiology in Children

Submitted by Connie Cutler, MLH Director, Infection Prevention and Control


The following was distributed by the Hospital and Healthsystem Association of Pennsylvania:

Issue and Recommended Action:  The United States currently is experiencing a nationwide outbreak of EV-D68 associated with severe respiratory disease. The Centers for Disease Control and Prevention (CDC) is closely working with the Colorado Department of Public Health and Environment (CDPHE) and Children’s Hospital Colorado to investigate a cluster of nine pediatric patients hospitalized with acute neurologic illness of undetermined etiology for a possible linkage of this cluster to the EV-D68 outbreak. Please disseminate this information to heath care staff as appropriate.


All suspected cases of Acute Neurologic Illness with Focal Limb Weakness of Unknown Etiology in Children should be reported to the Pennsylvania Department of Health at 1-877-PA-HEALTH or to the local health department where the patient resides.


Patients who meet the following case definition should be reported to state and local health departments:

  • Patient’s under the age of 21 with:
    o Acute onset of focal limb weakness occurring on or after August 1, 2014
    o A magnetic resonance imaging (MRI) showing a spinal cord lesion largely restricted to gray matter


Providers treating patients meeting the above case definition should consult with their local and state health department for laboratory testing of stool, respiratory, and cerebrospinal fluid specimens for enteroviruses, West Nile virus, and other known infectious etiologies.


In addition, the CDC is seeking information about other similar neurologic illnesses in all states, especially cases clustered in time and place, with particular interest in characterizing the epidemiology and etiology of such cases. HAP wants to make you aware of this neurologic syndrome under investigation with the aim of determining if children with similar clinical and radiographic findings are being cared for in other geographic areas.


Background:  The CDPHE, Children’s Hospital Colorado, and CDC are investigating nine cases of acute neurologic illness among pediatric patients. The cases were identified during August 9 through September 17, 2014, among children aged 1–18 years (median age 10 years). Most of the children are from the Denver metropolitan area. All were hospitalized.


The illness is characterized by focal limb weakness and abnormalities of the spinal cord gray matter on MRI. These illnesses have occurred since August 1, 2014, coincident with an increase of respiratory illnesses among children in Colorado.


Common features included acute focal limb weakness and specific findings on MRI of the spinal cord consisting of non-enhancing lesions largely restricted to the gray matter. In most cases, these lesions spanned more than one level of the spinal cord. Some also had acute cranial nerve dysfunction with correlating, non-enhancing brainstem lesions on MRI. None of the children experienced altered mental status or seizures. None had any cortical, subcortical, basal ganglia, or thalamic lesions on MRI. Most children reported a febrile respiratory illness in the two weeks preceding development of neurologic symptoms. In most cases, cerebrospinal fluid (CSF) analyses demonstrated mild-moderate pleocytosis (increased cell count in the CSF) consistent with an inflammatory or infectious process. CSF testing to date has been negative for enteroviruses, including poliovirus and West Nile virus. Nasopharyngeal specimens were positive for rhinovirus/enterovirus in six out of eight patients that were tested. Of the six positive specimens, four were typed as EV-D68, and the other two are pending typing results. Testing of other specimens is still in process. Eight out of nine children have been confirmed to be up to date on polio vaccinations. Epidemiologic and laboratory investigations of these cases are ongoing.


For More Information:  Please visit the CDC enterovirus website for general information about enterovirus infections, including EVD-68, and for up-to-date guidance about infection control measures. For information about poliovirus, please visit the CDC poliovirus website.


Additionally, HAP would like to make you aware of the availability to register for the Pennsylvania Department of Health’s (DOH) Health Alert Network. The registration is simple and can be done by visiting the DOH website.


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