Fulminating encephalopathy with perivenular demyelination and vacuolar myelopathy as the initial presentation of human immunodeficiency virus infection
B. Silver, K. McAvoy, S. Mikesell and T. W. Smith
Department of Neurology, University of Massachusetts Medical Center, Worcester, USA.
OBJECTIVE: To study the neuropathologic features in a case involving a
22-year-old woman in whom a fulminating encephalopathy developed as the
initial manifestation of human immunodeficiency virus (HIV) infection.
DESIGN: Case report. SETTING: Tertiary care hospital. PATIENT: The patient
presented with rapidly progressive mental status, changes, cranial nerve
abnormalities, and quadriplegia, which led to her death 5 months later.
Serologic tests for HIV were initially indeterminate on Western blot
analysis but were positive 1 week later. METHODS: A complete autopsy,
including examination of the brain and spinal cord, was performed.
Paraffin-embedded sections of the brain and spinal cord were examined using
standard histologic staining procedures and immunohistochemical techniques.
RESULTS: Neuropathologic examination revealed discrete foci of perivenular
demyelination disseminated throughout the brain and spinal cord, as well as
severe vacuolar myelopathy. Lesions typical of HIV encephalitis were not
present. Human immunodeficiency virus-infected monocytes and microglia were
observed in the vicinity of, but not restricted to, the perivenular
demyelinating lesions. No other infectious agents were identified.
CONCLUSIONS: The patient's acute encephalopathy was most likely the direct
result of a widespread demyelinating process resembling acute disseminated
encephalomyelitis. We suggest that the perivenular demyelination may
represent an autoimmune reaction, possibly due to a nonspecific viral
infection, occurring in the setting of chronic immunosuppression secondary
to HIV. Although less likely, we cannot exclude the possibility that HIV
could have directly triggered an autoimmune response that caused the acute
disseminated encephalomyelitis-like lesions.
