Eleven cases of rhinocerebral mucormycosis (RM) encountered over a 13-
year period were reviewed. Predisposing factors included leukemia (36%
), diabetes mellitius (27%), aplastic anemia (9%), myelodysplastic syn
drome (9%), and treatment with immunosuppressive medications necessary
to maintain solid organ or bone marrow graft viability (64%). Two pat
ients had no predisposing factors. Clinical findings included headache
(73%), fever (55%), black nasal eschar (45%), orbitofacial cellulitis
(36%), cranial nerve palsy (36%), altered sensorium (36%), and hemipa
resis (27%). Seven patients presented with destruction of the paranasa
l sinuses and local invasion; three with direct extension to the front
al or temporal lobes. Four patients displayed hematogenous disseminati
on to the cerebrum, brain stem, and cerebellum from a primary pulmonar
y focus. The seven patients with sinus involvement were treated with a
ggressive surgical debridement. Two patients with focal intracerebral
lesions underwent either open craniotomy or stereotactic biopsy. Ampho
tericin B was administered intravenously to all patients. Local irriga
tion via a percutaneous catheter was performed in the seven patients w
ith sinus disease and in one case of intracranial abscess. All seven p
atients with intracranial infection died, in contrast to four patients
that survived with infection localized to the sinuses and orbits. All
survivors had been treated with a combination of surgery and amphoter
icin B therapy. This review demonstrates that RM is increasingly affec
ting patients with sources of immunosuppression other than diabetes me
llitus. Early aggressive therapy to prevent cerebral involvement by th
is severe infection provides the best chance for a good outcome.