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Bilateral rhinocerebral phycomycosis.

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Right
Lett
F i g 2. Superimposed subtrar-tim rorrztgezogramsramparing the
right and left carotid arteries. The left carotid siphon is displar-ed laterally (armw).
oculomotor palsy in T H S to spare the parasympathetic fibers. Of 10 patients in the world literature
with third nerve deficits, only 2 had pupillary involvement. They hypothesized that, as with the more
familiar diabetic oculomotor palsy, this was due to
nerve infarction with sparing of the more peripherally located pupillomotor fibers. In our patient the
granulomatous mass could have directly compressed
the oculomotor nerve as well as the carotid artery and
sella turcica, thus producing the complete third nerve
palsy more commonly seen with tumor in this area.
T h e opinions o r assertions contained herein are the private views
of the authors and are not to be construed as reflecting the views of
the Department o f the Army o r the Department of Defense.
References
1. Schatz NJ: Pain associated with ophthalmoplegia, in New
Orleans Academy of Ophthalmology Symposium on Neuroophthalmology. St. Louis, Mosby, 1076, p 80
2 . Takeoka T, Gotoh F, Fukuuchi Y, e t al: Tolosa-Hunt syndrome. Arch Neurol 35:219-223, 1078
3. Tolosa E: Periarteritic lesions of carotid siphon with clinical
features of a carotid infraclinoid aneurysm. J Neurol
Neurosurg Psychiatry 17:300-302, 1954
Bilateral Rhinocerebral
Phycomycosis
Lloyd H. Kasper, MD, James L. Bernat, MD,
Richard E. Nordgren, MD, and Alexander G. Reeves, MD
A 57-year-old diabetic woman developed bilateral
rhinoorhitocerebral phycomycosis. Successful treatment was accomplished by correction of the ketoacidosis, surgical debridement, and amphotericin
B therapy. Rhinoorhitocerebral phycomycosis is a
fulminant and frequently fatal disease most often seen
in debilitated hosts. This survival suggests that aggressive therapy is indicated in patients with extensive
fungal involvement.
Kasper LH, Bernat JL, Nordgren RE, et al:
Bilateral rhinocerebral phycomycosis.
Ann Neurol 6:131-133, 1979
Infection with Phycomycetes, a group of nonseptate
saprophytes, is an uncommon yet rapidly fatal disease
that usually occurs in debilitated hosts. Patients with
diabetes mellitus, with or without ketoacidosis, account for up t o S O T j of all reported cases and most
frequently suffer from rhinoorbitocerebral involvement.
From the Section of Neurology, Department of Medicinc,
Dartmouth Medical School, Hanover, N H .
Accepted for publication Feb 26, 1070.
Address reprint requests to D r Kaspcr, Section of Neurology,
Department of Medicine, Dartmouth Medical School, Hanover,
N H 03755.
0364-5134/79/080131-03$01.25 @ 1978 by Lloyd H . Kasper
131
Rhinoorbitocerebral phycomycosis is almost uniformly unilateral, and recovery is unusual. T h e r e
have b e e n 2 reported cases of bilateral involvement,
w i t h o u t survival [ 4 , 91. We describe a patient w h o
recovered f r o m bilateral orbital apex a n d cavernous
sinus infection.
A 57-year-old woman with a six-month history of untreated
polyuria and polydipsia was admitted t o an outside hospital
because of lethargy and headache. She was noted to have a
blood glucose level of 680 mgldl, p H of 7.34, and positive
serum acetone. A diagnosis of diabetic ketoacidosis was
made, and she was treated with low-dose intravenous insulin, fluids, and potassium, which resulted in prompt resolution of the ketoacidosis. O n the second day the patient
noticed left facial numbness and periorbital edema followed by visual loss, decreased extraocular movements,
and proptosis o f tht- left eye. Lumbar puncture revealed 13
red cells and 4 Lvhite cells ( 3 neutrophils, 1 lymphocyte)
per mm:', glucose of 108 mgidl, and protein of 59 mgidl.
The patient was transferred to Dartmouth-Hitchcock
Medical Center.
H e r past medical history was unremarkable except for
hypothyroidism, for which she was receiving sodium
levothyroxine, 0. I S mg daily. Examination revealed a disoriented, lethargic woman complaining of diffuse body discomfort. Temperature was 38.4"C. There was bilateral tender edema of the periorbital tissues and globe. The left
tympanic membrane was injected, and a black, necrotic
palatal lesion was seen. Nuchal rigidity was present. Cranial
nerve testing revealed bilateral anosmia. Blurring of the
disc margin with vitreous clouding was present on the left.
There was no vision in the left eye, and she had only a
minimal response t o threat i n the right eye. The left pupil
was 6 mm, irregular. and unreactive. The right pupil was 5
mm and reacted minimally t o light. The eyes were disconjugate. There was complete bilateral external ophthalmoplegia with no voluntary or reflex response. The left
side of the face w 3 s anesthetic. The left corneal reflex was
absent and the right, depressed. N o other abnormalities
were found on examination.
Laboratory values included a hemoglobin of 11.6 gmidl
and a white cell count o f 14,600 per mm:' (619f neutrophils, 2 6 q ' stab forms, 1057 lymphocytes, and 3 9 monocytes). The serum glucose was 124 mgidl, and no ketones
were present in either serum o r urine. Arterial blood p H
was 7.45. Repeat cerebrospinal fluid examination showed
26 white cells (70"; lymphocytes, 3 0 T neutrophils) and 4
red cells per mm3, protein of 32 mgldl, and glucose of 119
mgidl. A chest roentgenogram showed bilateral atelectasis;
sinus tomography was normal. Control of blood glucose
was achieved with 12 units of N P H Insulin and 6 units of
regular insulin every morning.
A biopsy of t h e inferior turbinate showed acute inflammation; no fungi \\ere identified. She was begun on an
intravenous regimen of methicillin, 12 gm per day, penicillin, 12 million units per day, and amphotericin B, 0.3 mg
per kilogram of hoJy weight per day initially, then 0.5
mgikgiday, to providc coverage for the wide variety of organisms anticipnted in septic cavernous sinus thrombosis.
132 Annals of Neurology
Vol 6
No 2
August 1979
Right retinal artery occlusion was noted on the third
hospital day. Computerized tomography showed thickening of the right maxillary sinus. O n the sixth hospital day a
repeat biopsy from the posterior ethmoid area identified
RhizopuJ organisms histologically and in culture.
O n the twelfth hospital day she underwent a left middle
and inferior turbinectomy, right middle turbinectomy with
removal of two-thirds of the posterior septum, and bilateral
antrostomies. Following surgery the cavity that remained
was irrigated twice daily with amphotericin B, 1 mdkg. The
penicillin and methicillin were stopped one day postoperatively, but intravenous amphotericin B, 0.5 mgikgiday, was
continued. The patient's creatinine initially rose but
stabilized at 2.7 mgldl when the amphotericin dosage was
changed to 0.5 mglkg on alternate days.
The amphotericin B was discontinued after a total dose
of 3,100 mg, and repeat nasal exploration disclosed no evidence of recurrent fungus. Two years later the patient remained blind. H e r diabetes was controlled on a restricted
diet and daily NPH Insulin therapy. N o further fungal infection has been evident.
Discussion
Infection of t h e cavernous sinus c a n be p r o d u c e d
by a variety of organisms including staphylococcus,
streptococcus, p r o t e u s , p s e u d o m o n a s , meningococcus, aspergillus, a n d Phycomycetes. T h e r e are t h r e e
p r e s u m e d r o u t e s of invasion: anteriorly, via t h e o p h thalmic artery a n d frontal sinus; a m i d d l e r o u t e
t h r o u g h t h e s p h e n o i d sinus; a n d posteriorly from t h e
petrosal sinus [8]. Generally, t h e anterior r o u t e of
infection gives rise t o t h e m o r e fulminant f o r m o f
septic thrombosis including high fever, headache,
nausea, a n d prostration. Usually p r e s e n t are cyanosis
of t h e u p p e r face, pupillary abnormalities, chemosis,
corneal haziness, a n d ophthalmoplegia f r o m e i t h e r
n e r v e paralysis or mechanical obstruction d u e to o r bital swelling. T h e prognosis is grave w h e n pyogenic
cavernous thrombosis spreads t o t h e contralateral
side. Prior t o this r e p o r t , d e a t h has always occurred
in reported patients with bilateral orbital phycomycotic infection.
H a r r i s [ 5 ] r e p o r t e d t h e first h u m a n survival of cerebral phycomycosis. Recently, Schwartz e t a1 [71
reviewed t h e literature, compiling 100 d o c u m e n t e d
cases of phycomycosis with 28 r e p o r t e d survivals.
M o s t of these patients w e r e treated by correction of
t h e underlying metabolic imbalance, surgical deb r i d e m e n t , a n d amphotericin B therapy.
Our case again illustrates t h e close link b e t w e e n
uncontrolled ketoacidosis a n d rhinocerebral phycomycosis. T h e k e t o n e reductase system of t h e Rhizofius organism utilizes a high glucose concentration,
a n d peak metabolic activity occurs a t low p H .
Accordingly, rapid correction of the acidosis remains
a mainstay o f p r e s e n t therapy.
Pathologically, Phycomycetes organisms invade
small arteries, leading to extensive thrombosis and
infarction of infected tissue. Systemically administered drugs may not be able to reach inhibitory concentration at ischemic sites, and surgical debridement
appears necessary. The review by Pillsbury and
Fischer [6] describes 11 of 13 patients with rhinocerebral phycomycosis who survived, all after extensive surgical debridement.
Amphotericin B remains the drug of choice for
rhinocerebral phycomycosis. Its mechanism of action
is probably through alteration of cellular permeability with subsequent loss of intracellular cations. It is
most effective when administered intravenously. The
present recommended dosage is 0.3 mgikg over 1 to
3 hours daily, which is advanced during the first two
weeks to 0.5 to 0.6 mg/kg/day, depending on renal
toxicity [ 3 ] .Abramson e t a1 [ 11 and later Battock and
associates [2] advocated achieving a cumulative dose
of 3 gm, to be given on an alternate-day basis if nephrotoxicity intervened. It may be necessary to continue treatment for weeks, since amphotericin B is
fungistatic and premature discontinuation of the drug
may result in recrudescence of infection.
A combination of correcting the underlying metabolic disorder, surgical debridement, and amphotericin B therapy is the treatment of choice for
rhinocerebral phycomycosis. Survival may be possible even in patients who have extensive bilateral
phycomycotic infections.
References
1. Abramson E, Wilson D , Arky RA: Rhino-cerebral phycomycosis in association with diabetic ketoacidosis. Report
of two cases and a review of clinical and expcrimental cxperience with amphotericin B therapy. Ann Intern Med 66:735742, 1967
2. Battock DJ, Granoz H , Bobrowsky M, e t al: Altcrnatc day
amphotericin B therapy in the treatment of rhino-cerebral
phycornycosis. Ann Intern Mcd 68: 122-137, 1968
3. Bennett JE: Drug therapy. Chemotherapy of systemic mycosis
(Part 1). N Engl J Med 290:30-32, 1974
4. Dwyer GK, Changus GW: Rhino mucorrnycosis resulting in
fatal cerebral mucormycosis. AMA Arch Otolaryngol 67:
619-623, 1958
5. Harris J: Mucormycosis: report of a case. Pediatrics 16:857867, 1955
6. Pillsbury H C , Fischer N D : Rhino-cerebral mucormycosis.
Arch Otolaryngol 103:600-604, 1977
7. Schwartz JN, Donnelly EH, Klintworth GK: Ocular and orbital
phycomycosis. SUITOphthalmol 22:3-28, 1977
8. Smith BH: Infections of the cranial dura and the dural sinuses,
in Vinken PJ, Bruhn GW (eds): Handbook of Clinical Neurology. Vol 33, Infections of the Nervous System. Amsterdam,
North-Holland, 1978
9. Wasserman AJ, Shiels WS: Cerebral mucormycosis. South Med
J 54:403-410, 1961
Presence of Lymphocyte
Membrane Surface
Markers on “Small Cells”
in a Pineal Germinoma
Edward A. Neuwelt, MD, and R. Graham Smith, MD
A 16-year-old boy was operated on via an occipital
transtentorial craniotomy for a pineal tumor. Routine
histological examination of the tissue revealed it to
be a germinoma, as characterized by the presence of
two cell populations: large cells and small, lymphoidappearing cells. T h e cells were evaluated in a singlecell suspension for the presence of lymphocyte membrane surface markers; small cells exclusively were
found to have such markers. A T-lymphocyte membrane marker was present in 81% of the small cells,
whereas 15 to 189; of the small cells had a Blymphocyte membrane marker.
Neuwelt EA, Smith RG: Presence of lymphocyte
membrane surface markers o n “small cell?”
in a pineal germinoma.
Ann Neurol 6:133-136, 1979
In most series, germinomas account for as many as
50% of tumors of the pineal region [1]. Histologically [4]and ultrastructurally [ 111, the tumor is identical to seminoma of the testes [ 7 ] .It is characterized
by the presence of two types of cells: large cells with
abundant clear cytoplasm and sharp cytoplasmic borders, and small mononuclear cells with only scanty
cytoplasm. Both histologically [4] and ultrastructurally [ 111 the “small cells” appear to be lymphoid,
although at least at the level of the light microscope
they are somewhat similar to pinealoblasts and
medulloblasts. To characterize this small cell population further, we assayed for surface markers of lymphocytes in a single-cell suspension prepared from a
pineal germinoma removed from a 16-year-old boy.
A large subpopulation of these cells formed sheep
erythrocyte (E) rosettes, and a smaller subpopulation
bore surface immunoglobulins.
Case History
A 16-year-old boy was first seen with an 18-month history o f progressive blurring of vision, bifronral headaches,
From the Departments of Neurosurgery, Biochemistry, and Internal Medicine, The university of Texas Health Science Center at
Dallas, Dallas, T X .
Accepted for publication Feb 13, 1979.
Address reprint requests to Dr Neuwelt, Division of Neurosurgery, The University of Texas Health Science Center at Dallas,
5323 Harry Hines Blvd, Dallas, TX 75235.
0364-5134/79/080133-04$01.25 @ 1978 by Edward A. Neuwelt
133
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