Enterovirus rhombencephalitis

Acute onset of decreaseddisturbed conscious level of consciousness, fever and squint. History of flu-like symptomsrespiratory infection 2 weeks before.

PCR on CSF from lumbar puncture Enterovirus puncture Enterovirus 71: +ve

Human enteroviruses can cause a variety of disease states including respiratory infections, hand-foot-mouth disease, and aseptic meningitis. Enteroviruses have also been associated with neurological syndromes, particularly acute flaccid paralysis and brainstem encephalitis.  

Infection with enterovirus 71 predominantly results in a vesicular rash of the hands and feet that follows a prodrome of symptoms including fever, vomiting and oral mucosal ulceration.

In most cases, the initial infection wanes and symptoms and signs resolve. However in rare cases, it may be followed a by severe neurological infection resulting in aseptic meningitis, brainstem encephalitis and even acute flaccid paralysis akin to paralytic poliomyelitis.

Infections with enteroviruses are common during summer and fall, especially in children, whose immunity is still being developed. Immunocompromised children may be at greater risk of complications. The virus can be found in an infected person’s respiratory secretions, such as saliva, nasal mucus, or sputum. EV-D68 likely spreads from person to person when an infected person coughs, sneezes, or touches a surface that is then touched by others. 

Amongst the worst complications is rhombencephalitis, which causes acute and severe neurologic disorders: ataxia, tremor, myoclonic jerks, oculomotor problems, or bulbar palsy. In some cases, neurologic affection can extend to the spinal cord. Diagnosis is achieved by MRI findings and confirmed by faeces analysis. 

Usually it is a sporadic, self­limited, and reversible disease. However, rhombencephalitis may be potentially epidemic and fatal.

MR imaging of affected patients typically demonstrates a rhombencephalitis , the most common site of involvement is the brain stem, being the pontine tegmentum the most common site of brain stem involvement. It shows increased T2 signal. Ventral pontine lesions, substantia nigra and dentate nuclei affection can be found. Usually, no supratentorial lesions are identified. Spinal cord lesions are seen as T2 hyperintensity throughout the entire central spinal cord gray matter.

Rdiculomyelitis with a predilection for the anterior horn cells of the spinal cord and ventral nerve roots. Cord lesions most typically effect the cervical spine, and the findings can evolve over time (originally effecting the central gray matter, and then the region of the anterior horn cells). Enhancement of the cauda equine and other nerve roots can also be seen.

Clinical and laboratory correlation with radiological findings is mandatory to achieve a proper diagnosis.

Treatment is based on support therapy: steroids, intravenous immunoglobulin and/or plasma exchange. The antiviral drug pleconaril may be useful. Most patients recover completely within a year, although some deaths have been reported (mainly immunocompromissed children or affected by other severe concurrent illnesses).