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Cerebral vasculitis in coccidioidal meningitis.

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BRIEF COMMUNICATIONS A N D CASE REPORTS
Cerebral Vasculitis in
Coccidioidal Meningitis
R o n a l d M. Kobayashi, MD, M a r c Coel, M D , G e n Niwayama, MD,
a n d D o r i s T r a u n e r , MD
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Cerebral vasculitis p r o d u c i n g focal central nervous system manifestations was observed i n 2 patients w i t h meningitis
due to Coccidioides immitis. Cerebral angiography demonstrated t h e characteristic changes of arteritis, including
irregular narrowing of arteries a t the base of t h e brain i n 1 patient a n d occlusion of a middle cerebral artery branch i n
the other. Pathological examination of t h e brain i n the latter p a t i e n t revealed extensive basilar meningitis along w i t h
prominent vasculitis a n d organisms. O n e p a t i e n t responded to intrathecal a n d intravenous amphotericin B treatment
combined with a ventriculopleural shunt, t h e o t h e r died despite amphotericin B therapy.
Kobayashi RM, Coel hi,Niwayama G, e t al: Cerebral vasculitis in coccidioidal meningitis.
Ann Neurol 1:281-284, 1977
Meningitis is t h e m o s t c o m m o n f o r m of central nervous system involvement with Coccidioides immitis b u t
occurs i n less than 12)of t h e estimated 100,000
infections annually [ 11. T h i s r e p o r t describes 2 cases
of coccidioidal meningitis diagnosed a n t e m o r t e m in
w h i c h cerebrovascular involvement was p r o m i n e n t .
In 1 patient, occlusion of a m i d d l e cerebral artery was
associated with pathologically d e m o n s t r a t e d arteritis.
A second patient exhibited bilateral arteriris o n cerebral angiography.
Patietit 1
A Wyear-old male farm worker presented with 3 six-month
history of progressive weakness, anorexia, weight loss, and
slurred speech. O n examination he was alert, but his recent
memory was moderately impaired and speech was
moderately dysarthric. There was generalized muscle atrophy, and strength was mildly decreased in all four limbs.
Deep tendon reflexes were normal except for absent ankle
jerks; plantar responses were flexor. Gait was broad based
and slow. Vibratory sensation was diminished in both legs.
Skin testing was positive for Corridi0ide.i. The cerebrospinal
fluid contained 380 mg/dl of protein, 200 mononuclear
cells, and 17 mddl of glucose; the pressure was 200 mm of
CSF. Complement fixation on the spinal fluid was positive for
C. inrrrcitis at a titer of 1 : 32; culture subsequently grew C .
immitis. Over the next two weeks response to verbal stimuli
was lost, nuchal rigidity appeared, and right hemiparesis and
hemianopia developed. A brain scan revealed increased
uptake in the left frontotemporal region. Bilateral carotid
angiography demonstrated nonvisualization of the left ascending frontoparietal artery with a moderate-sized avascular area in t h e frontotemporal region (Fig 1). Delayed
collateral flow to this region via branches of the left perical-
From the Veterans Administration Hospital, San Diego, and the
Departments of Neurosciences, Radiology, and Pathology, University of California, San Diego, School of Medicine, La Jolla, CA.
Accepted for publication Sept 10, 1976.
losal artery and early filling of the left basal vein were seen.
The right anterior and middle cerebral arteries were normal.
Moderate enlargement of the lateral ventricles was indicated by the course of the thalamostriate vein. Despite
treatment with intravenous and intrathecal amphotericin B,
the patient died six weeks after admission.
Postmortem examination revealed endospores of C. in?niitii in the lung, spleen, lymph nodes, and bone marrow,
with granulomas, some with giant cells. The brain weighed
1,350 gm. The meninges were opaque and thickened, and a
large amount of purulent exudate was present over the base
of the hrain. A 2 to 3 cm area of softening was present in the
left frontotemporal and temporoparicml regions. Postmortem brain cultures grew C. in2r)zitis. Microscopical examination revealed marked meningeal thickening wich prominent
infiltration by plasma and mononuclear cells, numerous
cndosporcs, and multiple granulomas. Widespread focal
cortical necrosis and hemorrhages were observed. Small- to
medium-sized arteries (Fig 2) and veins were involved with
subiritimal edema, lymphocytic arid plasmacytic infiltration,
and periadventitial inflammarion. Fungi were demonstrated
in the subintima, media, and adventia ofvessels (see Fig 2 ) .
The leptomeninges of the spinal cord were thickened, with
granulomatous inflammation and numerous endospores of
C . in?rilitk.
Patient 2
A 33-year-old construction worker complained of intense
midline headaches with occasional vomiting and a 9 kg
weight loss of several months' duration. Two days before
admission, he lost the ability to speak and was incontinent of
urine but remained conscious. H e subsequently exhibited
left hemiparesis and hemianesthesia. Spontaneous improvement occurred over the next two days.
Address reprint requests to Dr Kobayashi, Neurology Service,
Veterans Administration Hospital, 33SU La Jolla Viliaggc Dr, San
Diego, CA 92161.
281
Pig 2. (Patienl 1.I Grairzrlomutous 2 asrulitis.
Coccidioides orgurzisms (arrows)are .reen u ithin the i d 1
o/an artery. (a, PAS jtain; b, elustiru stain: both x I O before
10cG red1drtion.j
282
Annals of Neurology
Vol 1 No 3 March 1977
O n admission the patient was mildly lethargic, with slow
responses, poor calculating ability, concrete proverb interpretation, and poor memory for recent events. Bilateral
papilledema and mild left-sided hpperreflexia were present.
The findings of the remainder of the examination were
normal. A chest roentgenogram showed apical thickening.
Bilateral carotid angiography revealed prominent narrowing of the distal portions of both internal carotid arteries and
the horizontal portions of both middle cerebral arteries;
these changes were much more prominent on the left (Fig
3). The genu of the middle cerebral arteries was displaced
upward, indicating bilateral temporal lobe swelling. O n the
left there were occlusions of multiple smaller vessels and
irregular narrowing of the middle cerebral branches at the
level of the frontal and parietal opercula, compatible with
arteritis. The lateral ventricles were moderately dilated. Air
ventriculography through a parietal burr hole revealed
communicating hydrocephalus consisting of moderate dilacation of the lateral ventricles, enlarged posterior fossa
cisterns, and normal aqueduct and third and fourth ventricles. Air failed to pass over the convexities. The ventricular
fluid contained 143 mononuclear and 10 polpmorphonuclear cells, 23 mgidl of glucose, and 87 mgidl of protein. A
coccidioidal complement-fixation rest was positive in the
CSF at 1:32 and in the serum at 1:bi dilutions. A culture of
the CSF was sterile.
Fig 3. (Patioit 2.1Right and left rurotrd arteriogranij.
anteriorpro;rntzvm There i J narrou ing ufthe mprailiviotd
portion! of both intermi/ rarotzd arteries arid horizontal
portiom of both vaddle cerehrul arteries (iz-repromtilent o u the
left, The peiru of both nuddk cerebral urterieJ i r dzrplaced
upward arid niedtall? The left frontoparzetal area 11a v a s d u r
The patient was treated with aventriculopleural shunt and
steroids, which led to remission of che headache and papilledema and restoration of alertness. When the diagnosis of
coccidioidal meningitis was established, he was treated with
intrathecal and intravenous amphotericin B. H e was discharged six weeks after admission to continue on intermittent amphotericin B treatment.
Discussion
Coccidioides immitis is a natural inhabitant of t h e soil in
t h e semiarid southwestern U n i t e d States, Mexico, a n d
South America [ 2 ] . Infections with this f u n g u s occur
with higher incidence in t h o s e with occupational exp o s u r e to dust, such as farm laborers a n d other o u t d o o r workers, than i n w o r k e r s in o t h e r occupations
[2]. The 2 patients in this r e p o r t were both long-term
residents of k n o w n e n d e m i c areas and, f u r t h e r m o r e ,
worked o u t d o o r s .
Since the organism usually e n t e r s via the respiratory tract, p u l m o n a r y manifestations are t h e m o s t
c o m m o n f o r m of clinical disease, a n d m a n y cases are
subclinical [2]. Dissemination occurs i n a b o u t 1% of
clinically a p p a r e n t infections, and meningitis is present i n one-third to three-quarters of t h e s e disseminated cases [1,3]. Of 39 cases of meningitis in which
t h e patient was studied at autopsy, 29 w e r e associated
with dissemination and 10 with p u l m o n a r y disease [4].
Cerebrovascular involvement by fungi i n t h e 2
cases described is s u p p o r t e d b y t h e focal clinical manifestations, radiographic findings [ 5 ] ,a n d (in Patient 1)
pathological examination.
Case Report: Kobayashi et al: Cerebral Vasculitis in Coccidioidal Meningitis 283
A review of the literature for clinical, radiological,
and pathological features disclosed only limited reference to cerebral vasculitis iri coccidioidal meningitis
12, 3, 6, 71. Vascular involvement has been reported
more frequently in pulmonary coccidioidomycosis
[8]. There is a single report of a case involving the
thyroid gland [91.
Clinically, coccidioidal meningitis usually produces
a subacute illness lacking many of the usual signs of
meningeal irritation such as nuchal rigidity [ 1, 2 , 101.
Headache is usually prominent. In about half the cases
fever, weight loss, progressive personality changes,
and confusion are presenting complaints. Most patients are exposed in endemic areas, but the illness
may become manifest in a nonendemic area [lo].
T h e most useful diagnostic procedure is the CSFspecific complement-fixation test [ 101, which is positive in 769;’of cases of active coccidioidal meningitis
[ 1 I]. False-positive results do not occur, but since the
CSF in 24Cf of patients fails to fix complement, a
negative result does not exclude the diagnosis [ill.
T h e coccidiodin skin test is usually negative when
dissemination and meningitis have occurred [ 101. Culture of the organism is confirmatory b u t is not as
helpful an early diagnostic tool as CSF complement
fixation. Initial CSF findings are usually abnormal,
with mononuclear pleocytosis, elevated protein, and
reduced glucose [l, 2 , 101. T h e pressure may not be
elevated in the presence of normal-pressure communicating hydrocephalus.
Treatment with amphotericin B has reduced the
niortality from uniform fatality within o n e year to
approximately 40(?; [ 11. Combined intravenous and
intrathecal administration, including use of the Ommaya reservoir for intraventricular injection, has been
advocated [ I , 121. Relief of hydrocephalus by ventricular drainage (as performed in Patient 2 ) has been
reported to be a useful adjunct [13- 151.
Particularly in patients from endemic areas such as
the southwestern LJnitcd States, coccidioidal meningitis with vasculitis should be considered in the presence of focal neurological deficits, especially when
accompanied by headache and fever.
Dr Kohavashi
284
IS
the recipicnt ot a VA Clinical Investigatorship
Annals of Neurnlugy
Vol 1 No 3
March 19”
References
1. Einstein HE: Coccidioidomycosis of rhe central nervous system. Adv Neurol 6:101-105, 1974
2 . Fetter BF, Klintworth GK, Hendry WS: Coccidioidomycosis,
in Mycoses of the Central Nervous System. Baltimore,
Williams & Wilkins Company, 1967) pp 74-78
3 . Muntingtoti RW. Waldmaiin WJ, Sargent JA, et al: Parhologic
and clinical observations o n 1 4 2 c s c s of fatal coccidioidomyco~is with necropsy. i n Ajello L (4):Coccidioidomycosis. Tucson. IJniversiry of Arizonia Press, 1965,
pp 143-167
4 . HLntingron RW: Morphology and racial distribution of fatal
coccidioiduniycusis: report of a ten year autopsy Feries in an
endemic area.JAMA 169:115-118, 1959
5 . Ferris EJ, Leviiie H1.: Cerebral arreritis: classification. Radiology 109:32:-341, 1973
6 . Case Records of the Massachusetts General Hospital: Case
36-1971. N Engl J Mecl 285:621-630. 1971
CA, T e n g Y : Pneumoencephalo~raphic
, . I’apatheodorou
findings in coccidioiilal meningoencephalitis. Calif M e d
L O l:4Y 9 M 8 I , 1964
8 . Deppisch LM, Donwvho EM: Pulmonary coccidioidomycosis.
A m J Clin Pathol 5H:4X1I-500, I972
9. Winn WR, Finegold SM, Huntingcon RW: Coccidioidomycosis with fungemia, in Ajello L (ed): Coccidioidomycosis. Tucson, University of Arizonia Press, 1965, p p
93-109
10. Caudill R G , Smith CE, Reinarz JA: Coccidioidal meningitis: a
&agnostic challenge. Am J &led 40:360-?65. 1970
1 1 . Smith CE, Saito MT, Sirnons SA. Pattern of 39,500 serologic
tests in coccidioidomycosis. JAMA 160:546-552, I956
12. Winn WA: Coccidioidal meningitis: a f o l l o ~ + u p report, i n
Ajello L (ed): Cociiclioidoniytosis. Tucsun, Llniversity of
Arizonia Press, 1965, pp 55-61
13. Ramseyer J L , Baker R N , Torniyasu LT: Venrriculovrnous
shunt in treatment of obstructive hydrocephalus due to cocLidioidomycotic meningiris. Neurology (Minneap! l6.7Ol708, 1960
14. Locks h10,Hawkins J A : Ventriculo-arriosromy in coccidioidal
nieniiigiris. Am Rev Resp Dis P8:331-136, 1953
1 5 . Zealear DS, Winn W’A: T h e neurosurgical approach in the
trearment of coccidioidal mengitis: report of ten cascs, i n
Ajello I, Icd): (~orridioi~i~,m!cosis.
Tucson, University of
Arimnia Press, 1965, pp 45-53
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