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Brainstem abscess and the syndrome of acute tegmental encephalitis.

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BRIEF COMMUNICATIONS AND CASE REPORTS
Brainstem Abscess and
the Syndrome of Acute
Tegmental Encephalitis
Frederick H . Harvey, MD, and Thomas J. Carlow, M D
A 63-year-old m a n developed the acute onset and rapid
progression of signs of lower brainstem dysfunction
accompanied by a sterile cerebrospinal fluid containi n g moderate pleocytosis. Autopsy examination disclosed diffuse acute bacterial inflammation of the
brainstem tegmentum. The findings i n 55 additional
cases of brainstem bacterial infections are reviewed
and discussed w i t h respect to differential diagnosis
and potential treatment.
Harvey FH, Carlow TJ: Brainstem abscess and the
syndrome of acute tegmental encephalitis.
Ann Neurol 7:371-376, 1980
Bacterial infections of the brainstem a r e u n c o m m o n
b u t should be a diagnostic consideration w h e n
rapidly progressive tegmental signs evolve. D o c u m e n t e d survivors have all u n d e r g o n e neurosurgical
drainage p r o c e d u r e s [7, 8 , 2 4 , 36,381. A n illustrative
autopsied case of tegmental encephalitis is discussed.
A 63-year-old right-handed man experienced nausea,
headache, vertigo, and horizontal double vision. Over the
next two days he noted numbness of the right half of the
tongue and face and paralysis of right facial muscles. By the
fourth day of his illness he had developed bilateral sixth
nerve palsies, a right peripheral seventh nerve paralysis,
sensory loss in the distribution of the right fifth nerve,
truncal ataxia, and a temperature of 38.3"C. Jerk nystagmus
of the right eye with no nystagmus in the left was observed
o n gaze to the right. Visual acuity, visual fields, convergence, vertical gaze, optic fundi, and pupillary reactions
were normal. Mental status and motor and reflex examinations were normal. Dexamethasone, 10 mg intravenously, was administered initially, followed by 4 mg every
six hours.
Cerebrospinal fluid (CSF) examination revealed a normal
pressure, protein of 85 mgldl, 80 white blood cells/mm3
with 7 5 F neutrophils and 25% lymphocytes, glucose of 64
rng/dl, and sterile cultures. Cytological examination of the
CSF showed no malignant cells or microorganisms. Blood
cultures were sterile initially. Complete blood count, urinFrom the Department of Pathology (Neuro-Pathology) and Department of Neurology, University of New Mexico School of
Medicine, Albuquerque, NM.
Accepted for publication Aug 19, 1979.
Address reprint requests to Dr Harvey, Office of Medical Investigator, University of New Mexico School of Medicine, Albuquerque, N M 87131.
alysis, antinuclear antibody titer, and routine chemistry
determinations were normal. An erythrocyte sedimentation rate was 57 mm/hr (Westergren).
O n the fifth day of his illness the patient complained of
difficulty swallowing and developed slurred speech. Skull
and chest roentgenograms, electroencephalogram, vertebral angiogram, technetium brain scan, and computerized
axial tomography (CT scan) with contrast were normal. On
the seventh day of the illness he stopped breathing but was
resuscitated. His temperature rose to 40"C, but the mental
status examination remained normal. His pupils became
pinpoint and nonreactive. Cephalothin (Keflin), 1 gm every
4 hours, and gentamicin, 80 mg every 8 hours, were begun.
Several hours later he developed bilateral seventh nerve
motor paralyses, absent corneal reflexes, inability to open
o r close the mouth o r protrude the tongue, and complete
bilateral ophthalmoplegia. H e was, however, receptive to
commands to grasp and was mentally alert. Two of the five
blood cultures drawn the previous day revealed growth of
microaerophilic streptococcus. O n the ninth day of illness
he developed a flaccid quadriparesis with hypoactive deep
tendon reflexes and died.
The immediate cause of death was acute pulmonary embolism. The general autopsy was otherwise negative except
for the presence of focal aggregates of neutrophils in the
liver compatible with sepsis. The source of the sepsis was
not found.
Neuropathological Findings
T h e brain was unremarkable externally. Cross sections of
the brainstem revealed brown-gray discoloration throughout the tegmentum without brainstem enlargement, gross
abscess formation (Fig 1), or softening. The discolorations
extended through both middle cerebellar peduncles to surround the dentate nuclei and rostrally into the posterior
subthalamic region. Sections through the cerebral hemispheres were normal.
Histological examination revealed extensive diffuse
acute inflammation throughout the brainstem tegmentum.
Most of the inflammatory cells were intact neutrophils; in
many areas degenerated neutrophils and numerous monocytes were present, some with phagocytic activity. White
matter tracts showed vacuolation, axon swelling, and interruption of the myelin sheaths. Focal necrosis of inflammatory elements formed purulent foci but were not
surrounded by granulation tissue. Some capillaries had necrotic, inflamed walls. Vein walls showed acute inflammation; the arteries were normal. This process spread into the
subarachnoid space, where acute phlebitis and mild diffuse acute inflammation (meningitis) were noted (Fig 2).
Gram stains demonstrated numerous intraparenchymal
gram-positive pleomorphic cocci compatible with microaerophilic streptococcus.
The extent of the lesion was localized histologically
through serial evaluation of brainstem blocks. InAammation was found in the following regions; oculomotor nuclei,
brachium conjunctivum, right fourth nucleus, both medial
longitudinal fasciculi, left pontine tegmentum, right fifth
nucleus (severe), left fifth nucleus (minimal), left fifth
nerve, left sixth nucleus, bilateral sixth nerves, bilateral
pontine paramedian reticular formation, both seventh nu-
0364-5 134/80/040371-06$01.25@ 1078 by Frederick H . Harvey
371
F i g 1 . Brainstem in serial blocks demonstrating brown-gray
areas of acute injlatmmation throughout the tegmentum (cut on
the right side).
Fig 2. Histological section through subarachnoid space of the
brainstem showing an injltrate of neutrophih ( N ) and
mononuclear cells. Mild phlebitis (P) cap1 be seen. ( H 6 E ;
x 100 before 20% reduction.)
372
Annals of Neurology
Vol 7
No 4
April 1080
Fig 3. Pons: histological section just rostral to the abducens
nuclei, showing several areas of severe inflammatory necrosis
(N) indicated by arrowheads. There is extensive neuron loss in
both facial nuclei (7). The facial (f)and abducens (a) nerves
are indicated. The latter pass through an extensiveh inflamed
and necroticpontine paramedian reticular formation (P).
(LFBfPASfhematoxylin; x l .)
clei (Fig 3), both pyramids, bilateral medial lemnisci, bilateral inferior olives, bilateral rostral medullary tegmentum,
bilateral hypoglossal nuclei and nerves, right vestibular nucleus, left vagal nucleus, right spinal trigeminal tract and
nucleus, and the extramedullary portions of both fifth and
seventh nerves.
The clinical neurological signs can be readily correlated
with the observed histopathological findings. The temporal
evolution of the clinical course suggests initial localization
in the caudal pontine tegmentum with subsequent rostral
and caudal extension.
Discussion
Only 55 cases of brainstem abscess appear in the literature 17, 8, 11, 17, 21, 24, 34, 36, 37,40,451. This
entity is not as rare as these reports would indicate
since 1 brainstem abscess should be encountered for
every 5,000 autopsies [12]. Only 4 were found in
330,000 autopsies filed at the Armed Forces Institute
of Pathology [41]; however, a brainstem abscess
would not commonly be referred to that institution
for evaluation. Approximately 1% of all autopsies
show brain abscesses [12], and 2% of these localize
to the brainstem [ 18,401; correspondingly, the brainstem represents 2% of total brain weight [25]. Although the incidence of brainstem abscess has not
appreciably changed since the preantibiotic era [ 1,
13, 271, the mortality rate has decreased [l].
Streptococcus and staphylococcus are the most
common organisms identified with brainstem abscess
[8, 15, 18, 24, 29, 32, 33, 34, 35, 411. Proteus
mirubilis has been encountered only once [401. Bacteria identified with brainstem inflammation without
abscess formation include Mycoplusmu pneumoniue
[ 161 and Listeriu monocytogenes [9, 141. Toxoplasmosis
may cause brainstem encephalitis [39].
Four recognized routes of bacterial spread into the
central nervous system exist: (1) metastatic extension, e.g., septic penumonia; (2) contiguous extension, e.g., otitis; (3) undetermined; and (4) direct implantation, e.g., trauma [32]. Thirty-four to 57% of
brainstem abscesses are metastatic [33, 411. Bacteremia, rather than septic embolism, appears to be
the underlying mechanism [12]. Thirteen cases with
clinical and pathological characteristics of embolic
spread have been reported [15, 331, with most
Case Report: Harvey and Carlow: Acute Tegmental Encephalitis
373
initially having a unilateral brainstem focus. A
hypothetical spread through Batson’s plexus with venous embolism is unlikely [32]. Contiguous extension has occurred in 25, to 4394 of reported cases [33,
411, most commonly from the ear. The routes of extension from the mastoid bone to the brainstem have
been well outlined [40].Approximately one-third of
cases have an undetermined origin [6]. Direct implantation has not been reported to have caused a
brainstem abscess.
Once bacteria invade the parenchyma, inflammation promptly ensues and clinical signs result from
either necrosis or inflammation of cranial nerves, nuclei, long tracts, or all three. Compression of adjacent structures follows abscess encapsulation and
expansion. Under favorable circumstances, inflammation is controlled by fibrous encapsulation; this is dependent on the number of organisms and their virulence [2]. Bacteria enter the brainstem tegmentum
via arterioles. When localization is prompt, intrinsic
spread is minimal 1.351. Pathologically, a tubular
abscess configuration suggests spread along white
matter tracts [ 4 1 , 4 2 ] ,as with spinal cord abscess [27,
4 11. Once the parenchyma is involved, toxins are released which can reach the reticular perivascular
spaces [44] to incite a cellular (neutrophilic and
mononuclear) response in the CSF [ 5 , 181. An initial
CSF pleocytosis recedes as the abscess capsule matures [43]. Because bacteria remain trapped in the
parenchyma, the CSF remains sterile; CSF cultures
were sterile in the 16 cases of brainstem abscess with
CSF culture reported in the literature [8, 11, 15,
18-21, 32-34, 40-421.
A clinical profile suggests [8, 11, 1 5 , 18-21, 27,
32-35,40-421 the average age to be 33 years, with a
range extending from 8 months to 87 years. Males
outnumber females 2 to 1, which is the same ratio
found in the cerebral abscess population. The average duration of the illness is 33 days, with 54% surviving less than two weeks. Most have no underlying
chronic disease; diabetes was documented in only 1
case [33] and congenital heart disease in 1 (large intraventricular septa1 defect) [34]. Facial weakness,
fever, headache, hemiparesis, dysphagia, and vomiting are commonly described [6]. Vomiting may
reflect irritation of the floor of the fourth ventricle
(area postrema) [32].Death within the first weeks of
the illness may be due to compression of medullary
centers [ 8 , 41, 421.
Most patients show tegmental signs, with
hemiparesis as the initial neurological manifestation.
Multiple cranial nerve or nuclear palsies depend on
the site of inflammation or abscess. If the initial
localization is in the rostral pontine tegmentum,
overt signs may be subtle until rostral or caudal
spread involves cranial nerves, nuclei, or both. An
374 Annals of Neurology
Vol 7
No 4 April 1980
initial unilateral localization with ipsilateral cranial
nerve palsies and contralateral hemiparesis is typical.
All of Weickhardt and Watts’ [42]collected cases of
medullary abscess showed cranial nerve palsies initially on the right side, but this tendency has not been
borne out in subsequent cases, which show a random
right-left distribution. Eventually, all patients demonstrate long tract signs; however, this is less common with a medullary o r mesencephalic abscess. The
most common initial site of involvement is the pons.
The process then extends diffusely throughout most
of the brainstem. Primary spinocerebellar tract involvement may mimic a cerebellar lesion [42].
CSF changes have been documented in 22 cases [8,
11, 15, 17, 18, 20, 21, 24, 28, 29, 32, 33, 34, 36,
40-42, and this report]. The CSF pressure was measured in 14 patients and was reported as normal.
Pleocytosis, either mixed or lymphocytic, was noted
in 20 patients: 0 to 5 WBC/mm3 in 7 , 5 to 20 in 4 , 2 0
to 50 in 2 , and greater than 50 in 5 . The earlier the
CSF was examined, the fewer cells-predominantly
mononuclear-were
observed. When lumbar puncture was delayed days to months, the cellular content of the CSF increased and the dominant cell
type became the neutrophil. CSF protein was recorded in 21 patients and was 0 to 50 mgldl in 5 , 50
to 85 in 7, 85 to 150 in 4 , and greater than 150 in 5 .
CSF glucose was normal in all 11 recorded cases. CSF
cultures were sterile in the 16 documented cases. It is
thus apparent that CSF studies can be entirely normal
early in the clinical course.
Many brainstem disorders can mimic acute tegmental encephalitis. T h e advent of polytomography
and contrast C T scanning has made it possible to
identify most intrinsic and extrinsic tumors of the
posterior fossa, vascular malformations, hemorrhages, and infarcts. Hydrocephalus with indirect
brainstem compression and congenital abnormalities,
e.g., Arnold-Chiari malformation, can also be documented with this technique. Tuberculous, fungal,
carcinomatous, sarcoid, and acute purulent meningitis can usually be identified after thorough analysis
of the CSF [ l o ] .Major CSF hemorrhage with brainstem signs would suggest a hemorrhage or aneurysm
in the vertebrobasilar distribution. A differentiation
of acute posterior fossa disorders without neuroradiological signs and absent to moderate CSF pleocytosis can be found in the Table.
Since acute brainstem tegmentitis is rapidly fatal
and other entities with similar presentations would
not be adversely affected by antibiotic therapy, initial
broad-spectrum coverage is suggested, e.g., with
penicillin and chloramphenicol. As soon as the infecting organism has been identified, usually from an
associated infection, broad-spectrum coverage can be
changed to the optimal antibiotic. Steroids and os-
Differential Diagnosis of Acute Tegmental Encephalitis a
Disease
CSF Pleocvtosis
Differential Aspects
Treatment
N ( 5 0 9 ; see text)
Associated infection
Broad-spectrum antibiotics
10-500 P. then L
Epidemic, unvaccinated, +
culture
Tick exposure
Pharyngeal pseudomembrane, peripheral neuropathy, paralysis of accommodation
Epidemic, ingestion of contaminated food, no sensory signs
virus identification, benign
course
Associated cerebral and deep
nuclear signs, abnormal
EEG
Supportive
Infectious and toxic
Brainstem bacterial tegmentitis
Acute bulbar poliomyelitis
[231
Tick paralysis [22]
Diphtheria [191
N
N , MPE
Botulism [24]
N
Viral brainstem encephalitis
[ 3 , 261
Viral meningitis and encephalomyelitis [ l o , 301
10-153 L
Demyelinating
Acute multiple sclerosis [ 101
+
6-1,000 P, then L, MPE
5-50 L
Postinfectious encep halom y eli tis [ 301
Central pontine myelinolysis
[221
Multifocal leukoencephalopathy [311
N (20%)
Infectious polyneuritis
(Landry -Guillain-Bark
syndrome) [ 101
Other
Cavernoma [41
Wernicke's encephalopathy
[221
Syringobulbia [231
Amyotrophic lateral sclerosis
L231
Porphyria [22]
N (85%)
Elevated gamma globulin,
multiple lesions in time
and space
Recent vaccination or viral
infection
Chronic alcoholism, malnutrition, hyponatremia
Immunosuppressed, leukemia, lymphoma, ascending radiculopathy
N
N, MPE
Family history of seizures
Chronic alcoholism, malnutrition, nystagmus
Other congenitial defects
Tongue fasciculations, no
sensory findings
Abdominal pain, elevated
urinary porphyrin precursors
Hypertensive, nonprogressive
Responds to anticholinesterase
N, MPE
N
N
N
Myasthenia gravis [22]
N
Anti toxin
Supportive
Supportive ( ? adenine
arabinoside)
Supportive ( ? steroids)
Supportive
Supportive
Treat primary disease
Supportive ( ? steroids)
N
N (80%)
Acute lacunar state
Remove tick
Antibiotics, antitoxin
Supportive
Thiamine
? neurosurgery
Supportive
Avoid barbiturates
Antihypertensives
Thymectomy, steroids, anticholinesterase
"Normal neuroradiological and CSF studies except for pleocytosis.
N
=
normal; P
=
polymorphonuclear cell; L
=
lymphocyte; MPE = minimal protein elevation.
motic agents should be considered in the presence of
either a mass effect or eminent brainstem herniation.
If survival to abscess formation is achieved and
identified roentgenographically, a neurosurgical
drainage procedure can be attempted. Five patients
with confirmed fully formed abscesses have survived
following neurosurgical drainage [6, 7 , 2 4 , 31, 341. It
is possible that vigorous and prompt antibiotic therapy during the first 24 hours of hospitalization-the
acute inflammatory phase-could
be curative. To
date, no such treated cases have been reported. This
phase would consist of the advent in a febrile, usually
Case Report: Harvey and Carlow: Acute Tegmental Encephalitis
375
toxic patient of rapid evolution of multiple cranial
nerve deficits, long tract involvement, no cerebral
signs, sterile CSF with mild o r no pleocytosis, and
negative neuroradiological studies.
Presented at the Ninth Annual Neuro-Ophthalmologic Pathology
Symposium, St. Louis, MO, Feb 18-19, 1977.
The authors gratefully acknowledge the efforts and assistance of
Stephen Markowitz, MD, in the preparation of this paper.
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