Brain Pathology Case of the Month - May 1998

FINAL DIAGNOSIS:

CMV ventriculo-encephalitis, polyradiculitis, and multiple cranial neuritis

DISCUSSION

CMV has been associated with a variety of central (CNS) and peripheral (PNS) nervous system syndromes in AIDS patients. Among the PNS syndromes, a rapidly progressive lumbosacral polyradiculopathy is the most frequent, occurring in about 1% of AIDS patients (1-6). Cranial nerve involvement in this PNS CMV syndrome has been mentioned only rarely and only in occasional nerves (7,8). Our case is unique in the presence of multiple cranial neuropathies resulting from direct infection of each with CMV. The manifestations of CMV ventricular encephalitis are consistent with this previously described distinct clinical-pathological entity known to occur in HIV-infected patients (9). Another unique aspect in this case is the discrepancy between the minimal MRI findings in the periventricular regions in the presence of diffuse necrotizing ventriculo-encephalitis. A possible explanation for this is the fact that inflammation, which may be the primary reason for the abnormal MRI signal, in these areas was scarce.

In conclusion, the affinity of CMV for peripheral nerves also includes the cranial nerves. Thus, in an AIDS patient with cranial neuropathy and CSF polymorphonuclear pleocytosis, the diagnosis of CMV infection should be strongly considered.

ACKNOWLEDGMENT

Supported in part by a PHS grant PO1-NS27405.

REFERENCES

  1. Roullet E, Assuerus V, Gozlan J, et al. Cytomegalovirus multifocal neuropathy in AIDS: analysis of 15 consecutive cases. Neurology 1994; 44: 2174-2182.
  2. Simpson DM, Olney RK. Peripheral neuropathies associated with human immunodeficiency virus infection. Neurologic Clinics 1992; 10: 685-711.
  3. Fuller GN. Cytomegalovirus and the peripheral nervous system in AIDS. J. Acq. Immun. Defic. Syn. 1992; 5: S33-6.
  4. Robert ME, Geraghty JJ3, Miles SA, Cornford ME, Vinters HV. Severe neuropathy in a patient with acquired immune deficiency syndrome (AIDS). Evidence for widespread cytomegalovirus infection of peripheral nerve and human immunodeficiency virus-like immunoreactivity of anterior horn cells. Act. Neuropathol. 1989; 79: 255-61.
  5. Grafe MR, Wiley CA. Spinal cord and peripheral nerve pathology in AIDS: the roles of cytomegalovirus and human immunodeficiency virus. Ann Neurol. 1989; 25:561-566.
  6. Mahieux F, Gray F, Fenelon G, et al. Acute myeloradiculitis due to cytomegalovirus as the initial manifestation of AIDS. J. Neurol. Neurosurg. Psychiat. 1989; 52: 270-274.
  7. Chimelli L, de Freitas MR, Bazin AR, et al. Cytomegalovirus encephalo-myelo-radiculitis in acquired immunodeficiency. Revue Neurologique 1990; 246: 354-360.
  8. Behar R, Wiley C, McCutchan JA. Cytomegalovirus polyradiculoneuropathy in acquired immune deficiency. Neurology 1987; 37: 557-561.
  9. Kalayjian RC, Cohen ML, Bonomo RA, Flanigan TP. Cytomegalovirus ventriculoencphalitis in AIDS - a syndrome with distinct clinical and pathological features. Medicine 72: 67-77, 1993.

Contributed by Lawrence C. Kenyon, MD, PhD, Herbert I. Goldberg, MD, Dennis L. Kolson, MD, PhD, Ronald G. Collman, MD, and Ehud Lavi, MD

International Society of Neuropathology