British Journal of Neurosurgery (1992) 6, 365-370

Br J Neurosurg Downloaded from informahealthcare.com by Flinders University of South Australia on 01/03/15
For personal use only.

SHORT REPORT

Cysticercosis producing various neurological presentations
in a patient: case report
DOUGLAS C. BILLS & LINDSAY SYMON

The Gough-Cooper Department, The National Hospital for Neurology and Neurosurgery, Queen
Square, London, UK

Abstract
We present an unusual case of neurocysticercosis supported by characteristic lesions on computed tomography and
positive serum and CSF titres. The patient came to medical attention on various occasions over a decade with three
clearly separate neurological presentations (acute psychosis, cerebral infarction, and hydrocephalus) before diagnosis was
made.
Key words: Cysticercosis, hydrocephalus, neurocysticercosis, Praziquantel.

Introduction
Infestation of the brain with the larval form of
the pork tapeworm, Taenia solium (neurocysticercosis), is one of the most common and
clinically significant parasitic infections of
nervous tissue in the world at present. It is,
however, largely associated with developing
countries, and the occasional case presenting in
Britain may be missed through lack of recognition of the possibility. We report here a patient
with a variety of neurological symptoms probably caused by the disease, initially presenting
10 years before diagnosis was reached and
specific treatment given.

Case report

The patient had been born in present day
Malawi, and had spent her first 18 years there
and in neighbouring Zimbabwe. She first came
to medical attention with neurological symp-

toms in 1981 at the age of 34 years, having
been previously well; she presented with several weeks’ history of confusion, auditory
hallucinations and paranoid delusions. At this
stage she refused investigations, and no computed tomography (CT) was performed. She
was discharged from hospital after 2 weeks
with a presumed diagnosis of schizophrenia,
and continued to suffer from auditory hallucinations periodically for at least a further 5
years. Treatment with antipsychotic medication resulted in the emergence of oro-facial
dyskinesia.
In 1985 she re-presented with a few days of
severe headaches, vomiting, disorientation and
blurred vision. Her in-patient course was
characterized by an intermittent fever and
deterioration in vision. Antibiotics and steroids
were administered for a presumed diagnosis of
encephalitis. The initial CT was performed
then, and revealed a group of cystic lesions
situated between the frontal lobes, and also
bilateral posterior cerebral territory low densi-

365

366

Douglas C. Bills 6.Lindsay Symon

Br J Neurosurg Downloaded from informahealthcare.com by Flinders University of South Australia on 01/03/15
For personal use only.

ties (Fig. 1). As a result of this episode she
became cortically blind, with vision reduced to
discerning hand movements only.

FIG. 1. CT performed in 1985, demonstrating frontal
cystic lesions and bilateral posterior cerebral artery
territory infarction.

She presented again in January 1990, with a
few weeks’ history of confusion and deterioration in her self-care. On examination she was
alert but disorientated, with very poor shortterm memory. Her confusion settled, but
short-term memory loss persisted. There was
no papilloedema at that time, but C T showed a
moderate hydrocephalus associated with a
collection of basal cisternal cysts (Fig. 2). It
was elected not to shunt her ventricles at this
stage and she was discharged, to re-present 12
months later with nausea, vomiting, dizziness,
and headache of an indeterminate length of
time (due to her memory deficit). On examination she was partially orientated, had chronic
papilloedema, no change in visual acuity, slight

limitation af upward gaze, and a markedly
ataxic gait. She was also occasionally incontinent of urine. Repeat C T revealed worsening
of her hydrocephalus but almost complete
resolution of the basal cysts (Fig. 3). Her
cysticercosis antibody titre (measured by immunofluoresence) in serum was positive at
1/40, and in CSF at 1/20.

FIG. 2. CT performed in January 1990, demonstrating
cysts clustered in the basal cisterns and dilated temporal
horns.

A ventriculo-peritonea1 shunt was installed
with resolution of her nausea, vomiting and
headaches. Her gait also improved considerably, allowing her to ambulate independently
again, and her incontinence decreased. Followup C T showed reduction in the hydrocephalus
(Fig. 4). Revision of the shunt was necessary 2
months later due to a distal block. In view of
the few residual cysts on C T a course of
Praziquantel
(pyrazinoisoquinolone) (50
mg/kg/day for 2 weeks) was given with
steroid cover (dexamethasone). Whilst taking
Praziquantel she suffered a single generalized
seizure, but otherwise her neurological symptoms and signs were unaltered.

Cysticercosis

367

Discussion

Br J Neurosurg Downloaded from informahealthcare.com by Flinders University of South Australia on 01/03/15
For personal use only.

Epidemiology and pathogenesis

FIG. 3. CT performed in January 1991, showing gross
hydrocephalus and partial resolution of the basal cysts.

Although uncommon in Britain, cysticercosis is
endemic in areas of the Far East, Africa
(including Zimbabwe), South and Central
America.' Migration from Mexico is leading to
a rising incidence in the south-western United
state^.^-^ The disease represents infection with
the larval stage of Taenia solium, the pork
tapeworm. In its adult form this parasite
inhabits the human small intestine, shedding
eggs into the faeces. Ingestion of these by the
intermediate host (the pig or human) results in
the embryos being released into the bowel and
penetrating its wall, to be spread haematogenously to other tissues, such as the eye, muscle,
subcutaneous tissue, or brain (neurocysticercosis), where they enter the encysted larval
stage known as cysticerci. Ingestion of these
cysticerci by the human (by eating undercooked pork) completes the cycle. The racemose strain of cysticercosis classically gives
cysts in groups, resembling a 'bunch of grapes',
often lodged in the basal cisterns, whilst the
cellulosae variety has a predilection for isolated
parenchymal lesion^.^

Presentation and diagnosis

FIG. 4. CT performed in February 1991, following
shunting, showing lessening of the hydrocephalus.

Based on C T appearances, lesions are described as being intraventricular, basal cisternal, meningeal or parenchymal. Patients may
exhibit only one or a mixture of types, which in
turn will affect the symptoms, natural course,
and response to treatment of the infection. The
neurological manifestations of neurocysticercosis are protean, the most frequent symptom
being seizures,4ys which commonly occur in
patients with discrete enhancing parenchymal
l e s i ~ n s .Symptoms
~~~
of raised intracranial
pressure (headache, vomiting, papilloedema)
are common, either from multiple cerebral
cysts,' cerebral oedema (an initial response to
parenchymal invasion, or to the by-products of
dying larvae at a later stage)6 or hydrocephalus
from blockage by intraventricular or basal
cisternal cysts, or secondary to basal arachnoi d i t i ~ . ' ,Other
~
well-recognized complaints are

Br J Neurosurg Downloaded from informahealthcare.com by Flinders University of South Australia on 01/03/15
For personal use only.

368

Douglas C. Bills & Lindsay Symon

signs of meningeal irritation: cranial nerve
palsies: cerebral infarction$8 dementia,'*4
acute p s y c h ~ s i s ~or, ~gait ataxia (the latter
usually associated with hydrocephalus).'
The diagnosis of neurocysticercosis can
often be made with reasonable confidence from
characteristic C T appearan~e?~with support
from a history of living in an endemic area and
tests for cysticercosis antibody in the serum or
CSF. Examination should include a search for
subcutaneous nodules and retinal cysts on
fundoscopy, and plain radiographs of the
thighs may show intramuscular calcified cystic e r ~ i . ' ?A~positive antibody titre supports the
diagnosis, but may be negative in up to 15%for
CSF, and 27% for serum ~ a m p l e s . ~

Features of this case

The case presented here demonstrates the
variety of presentations of neurocysticercosis,
and the long time course possible between
infestation and Occurrence of symptoms. Although C T was not performed at the time of
this patient's initial presentation with an acute
psychotic episode, such episodes have been
well described in neurocysticercosis. Signore
and Lahmeyer9 reported the case of a young
man presenting with recurrent acute episodes
involving paranoid feelings, auditory hallucinations and tangential thinking, who had
calcified cerebral hemisphere cysts on CT, and
positive cysticercosis serology. In his review of
238 cases, S c h d noted 'psychosis' as being
part of the presenting symptomatology in eight
Natural history
(3.4%).
Cerebral infarcts associated with neurocystiThe natural history of the disease is variable.
cercosis
are also well documented, with a
The C T changes in the parenchymal form of
'cerebrovascular
accident' occurring in 4.2% of
the disease have been well documented, where
the
presentations
in Scharf s ~ e r i e s .Infarcts
~
in the chronic phase lesions may calcify or
are
often
of
the
type
expected
with
small
vessel
resolve spontane~usly.~.~
The patient may
involvement,
but
major
intracranial
vessel
remain asymptomatic until the death of the
cysticerci many years after infection, resulting occlusion has been documented. Rodriguezin a marked inflammatory reaction and conse- Carbajal et aL8 reported three cases of middle
quent cerebral oedema. Spontaneous resolu- cerebral artery occlusion, with large basal
tions of meningeal, basal and ventricular cysts subarachnoid cysticerci surrounding the occluded vessels in two of the cases, and diffuse
has also been described.2.6
thickening of the surrounding leptomeninges
in the other. McCormick et al." reported a
Management
case of complete occlusion of the supraclinoid
Treatment naturally depends upon the size, internal carotid artery with cysticerci adherent
location, and effects of the cysts. Hydrocepha- to the artery at the site of occlusion. We
lus, if present, may require shunting. Surgical believe that this is the first reported case of
posterior cerebral artery territory
removal of large cysts can also be p e r f ~ r m e d . ~ ?bilateral
~
infarction
in neurocysticercosis. Although the
Symptoms of meningeal irritation may respond
patient
exhibited
basal cysts of the racemose
to steroids, and seizures to normal anticonvulsant therapy. Praziquantel has been shown to variety on C T at the time of evolution of the
be effective in improving the C T appearance infarcts, these were at that time situated more
in all forms of neurocysticercosis.'O Side ef- frontally, and presumably the vessel occlusion
fects attributable to observed raised intracran- was associated with a basal leptomeningitis
ial pressure, believed due to inflammatory that was more diffuse than the location of the
changes associated with death of cysticerci, cysts.
Our patient also exhibited a profound shorthave been observed during the first few days of
Praziquantel administration, but concomitant term memory loss. ScharP noted 'dementia' in
steroid cover drastically reduces such ef- 2.1% of his series on presentation, and Yang et
fect~.~.'~
al.' noted that 33 patients in their series of 143

Br J Neurosurg Downloaded from informahealthcare.com by Flinders University of South Australia on 01/03/15
For personal use only.

Cysticercosis
(29.8%) suffered mental deterioration, with
memory loss a specific feature of this. All of
Yang et al.’s patients had parenchymal disease,
and the degree of memory loss in our case,
when first apparent, seemed out of proportion
to her hycrocephalus at that time (Fig. 2). It
may be that there was also an element of
parenchymal disease not obvious on CT, to
explain this amnesia.
Hydrocephalus is a common presentation
for neurocysticercosis (21.4% of Scharf‘s
series4), and such cases almost invariably have
or have had racemose cysts obstructing the
basal cisterns. The distribution of cysts on CT
of our case was seen to have changed by the
time her hydrocephalus first became evident,
with an increase in the number of perimesencephalic cysts. However, her hydrocephalus worsened despite spontaneous C T resolution of
most of the cysts (Fig. 3), supporting Lebl a d s statement that arachnoiditis and aqueductal ependymitis associated with basal cysts
often contributes significantly to the obstruction of CSF o ~ t f l o wFor
. ~ this reason, surgical
removal of the cysts is often not helpful in
treatment of the hydrocephalus, and shunting
is required. Unfortunately shunt obstruction
from inflammatory debris, which may have
been responsible for the shunt blockage in our
case, is c ~ m m o n . ~

Conc1usions
Neurocysticercosis is a potentially debilitating
disease that can now be safely treated with the
drug Praziquantel. A high index of suspicion,
coupled with a knowledge of the disease’s
many possible presentations and the character-

369

istic CT appearances, is necessary for clinicians to diagnose and treat the disease before
permanent sequelae, such as the above patient’s memory loss and blindness, occur.

Address for correspondence: Prof Lindsay
Symon, The Gough-Cooper Department, The
National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.

References
1 Yang S, Wang M, Xue Q. Cerebral cysticercosis. Surg
Neurol 1990; 34:286-93.
2 Miller B, Grinnell V, Goldberg MA, Heiner D.
Spontaneous radiographic disappearance of cerebral
cysticercosis: three cases. Neurology 1983; 33:1377-9.
3 Leblanc R, Knowles KF, Melanson D, et al. Neurocysticercosis: surgical and medical management with
praziquantel. Neurosurgery 1986; 18:419-27.
4 Scharf D. Neurocysticercosis: two hundred and thirtyeight cases from a California hospital. Arch Neurol
1988; 45777-80.
5 Bhoopat W, Poungvarin N, Issaragrisil R, Suthipongchai S, Khanjanasthiti P. C T diagnosis of cerebral
cysticercosis. J Med Assoc Thai 1989; 72:673-81.
6 Kramer LD, Locke GE, Byrd SE, Daryabagi J.
Cerebral cysticercosis: documentation of natural history with CT. Radiology 1989; 171:459-62.
7 Kennedy A, Schon F. Epilepsy: disappearing lesions
appearing in the United Kingdom. BMJ 1991;
302~933-5.
8 Rodriguez-Carbajal J, Del Brutto OH, Penagos P,
Huebe J, Escobar A. Occlusion of the middle cerebral
artery due to cysticercotic angiitis. Stroke 1989;
201095-9.
9 Signore RJ, Lahmeyer HW. Acute psychosis in a
patient with cerebral cysticercosis. Psychosomatics
1988; 1:106-8.
10 Van Dellen JR, McKeown CP. Praziquantel (pyrazinoisoquinolone) in active cerebral cysticercosis. Neurosurgery 1988; 22(1):92-6.
11 McCormick GF, Giannotta S, Zee C, Fisher M.
Carotid occlusion in cysticercosis. Neurology 1983;
33~1078-80.