Hopkins syndrome: T2-weighted high intensity of anterior horn on spinal MR imaging

doi:10.1016/0887-8994(95)00181-E

Pediatric Neurology

Volume 13, Issue 3, October 1995, Pages 263-265

https://doi.org/10.1016/0887-8994(95)00181-E Get rights and content

Abstract

Hopkins syndrome is a poliomyelitis-like illness manifesting flaccid paralysis of an extremity in the recovery stage after an asthmatic attack. A 7-year-old boy who developed acute flaccid paralysis of the left upper extremity 4 days after an asthmatic attack is reported. T₂-weighted magnetic resonance imaging of the cervical spine revealed a local high-intensity area in the left anterior horn at the C₄ to C₆ level. There have been few pathologic or radiologic studies of this syndrome. We suspect that the cause is an anterior horn lesion.

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Cited by (20)

Management of the 2014 Enterovirus 68 Outbreak at a Pediatric Tertiary Care Center
2015, Clinical Therapeutics
Citation Excerpt :
A similar entity, known as Hopkins syndrome or acute flaccid paralysis associated with an asthma attack, has been reported previously.30 Anterior horn lesions on either magnetic resonance imaging or electromyography are reported commonly.31,32 To further complicate the etiology of the paralysis, many of these children have EVs or adenoviruses isolated from specimens.30,33
Enterovirus 68 (EV-D68) is an uncommonly recognized cause of acute respiratory tract infections. During the late summer of 2014, an international EV-D68 outbreak occurred. We review the steps of outbreak recognition and management in the context of 1 hospital’s experience with the EV-D68 outbreak.
We reviewed the role of Children’s Mercy Hospital as one of the first hospitals to recognize the 2014 EV-D68 outbreak in the United States. The steps of outbreak management were applied to real-life examples as the outbreak unfolded at our hospital.
Management of the 2014 EV-D68 outbreak was a multifaceted effort requiring close coordination with hospitals, local and state health departments, and the Centers for Disease Control and Prevention. The importance of clear and frequent communication is highlighted both intra- and interinstitutionally. Increased respiratory disease−related pediatric admissions at hospitals nationally were attributed to EV-D68. Outcomes for these children, including the association of EV-D68 with acute flaccid myelitis, remain under investigation.
Following the steps of outbreak management is critical to providing optimal patient care and ensuring the health of the public. During the 2014 EV-D68 outbreak, close adherence to outbreak principles led to swift recognition of illness, rapid diagnostic measures, institution of appropriate therapies, and dissemination of information to health care providers and the public. Equally important was the subsequent identification of an increase in acute flaccid myelitis cases against the backdrop of an increase in EV-D68 detections nationally. Future prospective studies are needed to determine the true burden of EV-D68 disease, potential vaccines and therapeutics, and outcomes of children with EV-D68 infection.
Myopathies of Systemic Disease
2015, Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach
When evaluating a child with myopathy, the clinician must not only determine the pattern and extent of muscle weakness but also the cause. In addition to the many primary disorders of muscle, there are many systemic diseases that produce myopathies. These generalized disorders include endocrine abnormalities, renal disorders, infections, medication-related effects, connective tissue diseases, and eosinophilic syndromes. In this chapter, we detail the disorders that may have a significant impact on skeletal muscle function in childhood.
A cluster of acute flaccid paralysis and cranial nerve dysfunction temporally associated with an outbreak of enterovirus D68 in children in Colorado, USA
2015, The Lancet
Citation Excerpt :
First, neurological disease might be an extremely rare complication of a previously uncommon infection and might not have been detected in previous outbreaks of enterovirus-D68, which were much smaller in size. Second, detection of rhinoviruses or enteroviruses in respiratory specimens has only become widely available in the past few years with the advent of new PCR assays, and previous clinically consistent cases, such as those described as Hopkins' syndrome, might not have been fully investigated.25–27 Last, acute flaccid paralysis and cranial nerve dysfunction associated with enterovirus D68 might truly be a new occurrence indicating a newly emerged strain, viral mutation, or viral recombination.28
Clusters of acute flaccid paralysis or cranial nerve dysfunction in children are uncommon. We aimed to assess a cluster of children with acute flaccid paralysis and cranial nerve dysfunction geographically and temporally associated with an outbreak of enterovirus-D68 respiratory disease.
We defined a case of neurological disease as any child admitted to Children's Hospital Colorado (Aurora, CO, USA) with acute flaccid paralysis with spinal-cord lesions involving mainly grey matter on imaging, or acute cranial nerve dysfunction with brainstem lesions on imaging, who had onset of neurological symptoms between Aug 1, 2014, and Oct 31, 2014. We used Poisson regression to assess whether the numbers of cases during the outbreak period were significantly greater than baseline case numbers from a historical control period (July 31, 2010, to July 31, 2014).
12 children met the case definition (median age 11·5 years [IQR 6·75–15]). All had a prodromal febrile illness preceding neurological symptoms by a median of 7 days (IQR 5·75–8). Neurological deficits included flaccid limb weakness (n=10; asymmetric n=7), bulbar weakness (n=6), and cranial nerve VI (n=3) and VII (n=2) dysfunction. Ten (83%) children had confluent, longitudinally extensive spinal-cord lesions of the central grey matter, with predominant anterior horn-cell involvement, and nine (75%) children had brainstem lesions. Ten (91%) of 11 children had cerebrospinal fluid pleocytosis. Nasopharyngeal specimens from eight (73%) of 11 children were positive for rhinovirus or enterovirus. Viruses from five (45%) of 11 children were typed as enterovirus D68. Enterovirus PCR of cerebrospinal fluid, blood, and rectal swabs, and tests for other causes, were negative. Improvement of cranial nerve dysfunction has been noted in three (30%) of ten children. All ten children with limb weakness have residual deficits.
We report the first geographically and temporally defined cluster of acute flaccid paralysis and cranial nerve dysfunction in children associated with an outbreak of enterovirus-D68 respiratory illness. Our findings suggest the possibility of an association between enterovirus D68 and neurological disease in children. If enterovirus-D68 infections continue to happen in an endemic or epidemic pattern, development of effective antiviral or immunomodulatory therapies and vaccines should become scientific priorities.
National Center for Advancing Translational Sciences, National Institutes of Health.
Imaging of Topographic Viral CNS Infections
2008, Neuroimaging Clinics of North America
Citation Excerpt :
Also, these lesions have been described with Hopkins syndrome, a poliomyelitis-like illness manifesting flaccid paralysis of an extremity in the recovery stage after an asthmatic attack. A patient with Hopkins syndrome had T2 hyperintensity of the left anterior horn in the cervical spinal cord [21]. On the other hand, enteroviruses may cause CNS infections with nonspecific imaging appearance or simulate other topographic involvement characteristics for other infectious agents.
Infections caused by enteroviruses, rabies, adenoviruses, and Nipah and Hanta viruses are discussed. Several studies defined the pattern of MR imaging findings in these disease processes that reflect parenchymal infiltration with inflammatory cells, typically visualized as areas of low attenuation on CT, as well as of low T1 and high T2 signal intensity on MR imaging. Diffusion-weighted MR imaging has been shown to be superior to conventional magnetic resonance imaging for the detection of early signal abnormalities in encephalitis. Focal unilateral hyperperfusion as visualized by SPECT appears to be an indicator of severe inflammation of the brain tissue and was found to be an independent predictor of poor prognosis, whereas clinical outcome variables, CSF, or EEG findings are not.
Erratum: Cervical myelitis from herpes simplex virus type I (Pediatric Neurology (2004) 30:1 (54-57))
2004, Pediatric Neurology
Although subacute ascending paralysis without sensory involvement is typically evocative of Guillain-Barré syndrome, it can alternatively be due to infection or inflammation of the spinal cord. We describe a 16-month-old female who presented with ascending flaccid paresis after an upper respiratory tract infection. She then developed signs of upper motor neuron involvement of the lower limbs associated with upper motor neuron involvement of the upper limbs. Motor nerve conduction and electromyographic studies of upper limbs demonstrated anterior horn cell involvement. Neuroimaging was consistent with cervical myelitis, and cerebrospinal fluid polymerase chain reaction was positive for herpesvirus-1. Although association with the primary infection of the respiratory tract may be fortuitous, possible neurotropic or hematogenous spread of herpesvirus-1 to the cervical spinal cord cannot be excluded.
Cervical myelitis from herpes simplex virus type 1
2004, Pediatric Neurology
Although subacute ascending paralysis without sensory involvement is typically evocative of Guillain-Barré syndrome, it can alternatively be due to infection or inflammation of the spinal cord. We describe a 16-month-old female who presented with ascending flaccid paresis after an upper respiratory tract infection. She then developed signs of upper motor neuron involvement of the lower limbs associated with upper motor neuron involvement of the upper limbs. Motor nerve conduction and electromyographic studies of upper limbs demonstrated anterior horn cell involvement. Neuroimaging was consistent with cervical myelitis, and cerebrospinal fluid polymerase chain reaction was positive for herpesvirus-1. Although association with the primary infection of the respiratory tract may be fortuitous, possible neurotropic or hematogenous spread of herpesvirus-1 to the cervical spinal cord cannot be excluded.

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Case reportHopkins syndrome: T2-weighted high intensity of anterior horn on spinal MR imaging

Abstract

J Pediatr

Pediatr Neurol

Pediatr Neurol

A new syndrome: Poliomyelitis-like illness associated with acute asthma in childhood

Aust Pediatr J

Electrophysiological study of amytrophy associated with acute asthma (asthmatic amytrophy)

J Neurol Neurosurg Psychiatry

Poliomyelitis-like illness after acute asthma

Arch Dis Child

Immunological abnormalities in the syndrome of poliomyelitis-like illness associated with acute bronchial asthma (Hopkins syndrome)

Arch Dis Child

Case report
Hopkins syndrome: T₂-weighted high intensity of anterior horn on spinal MR imaging