Encephalomyeloradiculitis: Same Clinical Features But Two Different Viruses
July 1, 2002
Abstracts & Commentary
Sources: Majid A, et al. Brain. 2002;125:159-165; Kusuhara T, et al. Clin Infect Dis. 2002;34:1199-1205.
The recent admission to New York Presbyterian Hospital of a young woman with acute transverse myelitis and encephalopathy with CSF pleocytosis prompted the review of these 2 reports.
Majid and colleagues described the clinical CSF, imaging, and virological analysis of 4 patients with combined central and peripheral nervous system disease produced by Epstein-Barr virus (EBV) infection. Three patients acutely developed myeloradiculitis, encephalomyeloradiculitis, or meningoencephalomyeloradiculitis. One patient had a subacute meningomyeloradiculitis. In all 4, CSF contained a predominantly mononuclear pleocytosis (420-33 cells/mm3) with elevated levels of protein (298-49 mg/dL) but a normal glucose level. EBV, DNA, and EBV IgM and IgG antibodies existed in the CSF of all 4 patients. The antibody profile in serum was consistent with recent infection.
In the 2 patients with myeloradiculitis and meningomyeloradiculitis, MRIs had an increased signal in the spinal cord with lumbosacral roots accompanied by lower extremity weakness; the 2 other patients’ MRIs of the brain and spinal cord were normal but they also had residual neurological defects. Each of the 4 patients was treated empirically with antiviral agents and in 3 cases with corticosteroids without obvious benefit. No definitive treatment exists for EBV infection of the nervous system.
The mechanism by which EBV produces neurological disease is unknown. The development of neurological deficits over weeks is consistent with either viral infection or a postinfectious process.
Kusuhara and colleagues reported a previously healthy 59-year-old man with acute encephalomyeloradiculitis due to herpes simplex virus 1 (HSV-1). The patient presented with fever, stiff neck, lethargy, and urinary retention. CSF contained a mononuclear pleocytosis (127 cells/mm3) with a protein level of 360/mg/dL and a normal glucose level. HSV-1 DNA sequences were present in the PCR products of CSF samples obtained on day 1 of hospitalization but were not present in CSF obtained on day 25. HSV-1 IgG, but not HSV-1 IgM antibody, was detected in the acute phase serum and CSF specimens. Titers decreased in convalescent-phase specimens.
Treatment with acyclovir was followed by clinical improvement during the first week of hospitalization. Nevertheless, on day 8 he again became febrile and developed myoclonus of the upper limbs and flaccid paraplegia. Brain MRIs during week 1 of hospitalization showed meningeal enhancement and multiple contrast-enhancing lesions in the basal ganglia, pons, and cerebral white matter. MRIs of the spine performed during week 3 showed an intramedullary lesion of the thoracic spinal cord as well as meningeal enhancement in the conus medullaris and cauda equina regions. The patient improved with corticosteroids and immunoabsorption plasmapheresis but 6 months later he had residual leg weakness and a neurogenic bladder although cognitive function was normal.
Commentary
Both papers reported patients with postherpes viral syndromes of encephalomyeloradiculitis. Acute EBV infection was established in the 4 cases of Majid et al. Primary EBV infection is prevalent worldwide and frequently results in infectious mononucleosis which has a low incidence of neurological complications (Epstein SH, Dameshek W. N Engl J Med. 1931;205:1238-1241). Nevertheless, EBV infection of the CNS and peripheral nerves has been reported to cause aseptic meningitis, encephalitis, myelitis, and neuritis in isolation or in overlapping combinations (Cohen JI. N Engl J Med. 2000;343:481-492).
The presentation of Kusuhara et al’s patient is noteworthy because HSV-1 infection usually produces focal medial temporal lobe encephalitis rather than a more disseminated syndrome (Shyu W-C, et al. Ann Neurol. 1993;34:625-627). The patients in both reports resemble cases of acute disseminated encephalomyelitis. This is most true in Kusuhara et al’s patient who developed postinfectious encephalomyelitis after the apparently successful treatment of HSV-1 encephalitis with acyclovir (Koenig H, et al. N Engl J Med. 1979;300:1089-1093). Both reports are models of the correlative clinical, radiological, virological, and immunological studies needed to fully understand virus-associated neurological disease. —John J. Caronna
Dr. Caronna, Vice-Chairman, Department of Neurology, Cornell University Medical Center; Professor of Clinical Neurology, New York Hospital, is Associate Editor of Neurology Alert.
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