Headache Medicine 2021, 12(1) p-ISSN 2178-7468, e-ISSN 2763-6178

ASAA

DOI: 10.48208/HeadacheMed.2021.11

Headache Medicine

Case Report

Hydrocephaly and secondary intracranial hypertension to racemosa

neurocysticercosis

Patrick Giordanni Gomes Sampaio Hiago Diniz Maracajá Túlio Carneiro Monteiro Temoteo Sara

Raquel Nóbrega Figueiredo Francielle Lopes de Araújo Batista

Unifacisa, Medicina, Campina Grande, Paraíba, Brazil

Abstract

Case report

G.F.M, 71 years old, male, farmer, presented a history of holocranial headache of a pulsatile type,

more intense on waking, and associated with photophobia. The complaint lasted six months. He also

reported episodes of tinnitus and unilateral hearing loss on the left. After conducting screening tests in

the emergency department, communicating hydrocephalus and cystic lesions were found in the subara-

chnoid and intraparenchymal space. In the neurological examination, the positive data were bilateral

papilledema and hearing loss in the left boné conduction. Given these ndings, the racemous variant

of neurocysticercosis and secondary hydrocephalus was suspected, causing intracranial hypertension.

Thus, it was decided to start specic treatment with albendazole 15 mg/kg/day (two daily doses), as-

sociated with praziquantel 50 mg/kg/day (three daily doses), for 14 days. To reduce the inammatory

process of the lesions, it was proposed to keep the patient on dexamethasone 8 mg/day for 90 days. In

addition to this scheme, topiramate 100 mg/day associated with acetazolamide 1 g/day was started,

along with serial lumbar puncture to relieve intracranial pressure.

Conclusion

The combination of modern diagnostic tests, use of antiparasitic drugs, optimization of anti-inamma-

tory treatments and minimally invasive neurosurgical procedures have improved results in patients

with NCC. Even so, this is the helminth infection that most affects the CNS and represents a major

public health problem in most parts of the world, since it remains a neglected pathology, like so many

other parasites, given that it is susceptible to containment through simple preventive actions as well as

eradication.

Hiago Diniz Maracajá

hiago_maracaja@yahoo.com.br

Edited by:

Marcelo Moraes Valença

Keywords:

Hydrocephalus

Intracranial Hypertension

Neurocysticercosis

Received: June 6, 2021

Accepted: June 30, 2021

ASAA

Sampaio PGG, Maracajá HD, Temoteo TC, Figueiredo SRN, Batista FLA

Hydrocephaly and secondary intracranial hypertension to racemosa neurocysticercosis

Introduction

ysticercosis is an infection caused by the larvae of Tae-

nia solium, which is an intestinal parasite of animals

- like a pig - and has a secondary life cycle in humans. This

is the parasitic infection that mostly affects the brain, at

the time being called neurocysticercosis (NCC), and may

inltrate intra or extraparenchymal areas.

NCC is highly endemic in most low-income countries, in-

cluding areas such as Latin America, African Sahara, and

parts of Asia

2,3

, being considered an emerging condition in

developed countries in Europe, as well as in the USA and

Canada, due to the migratory movements. It is estimated

that between 1320 and 5050 new cases of NCC occur

per year in the USA.

In Latin America and the Caribbean,

an estimated 75 million people are at risk of infection. In

Brazil, the Southeast, South, and Midwest regions are con-

sidered endemic. A study presented in 2003 showed that

the frequency of NCC cases ranged from 0.12% to 9% in

autopsies and from 0.03% to 13.4% in clinical studies. The

lethality rate ranged from 4.8-25.9%, and asymptomatic

forms were detected in 48-55% of cases with a diagnosis

conrmed by autopsy.

Racemic neurocysticercosis is a variant of the subarachnoid

form of NCC. It is extremely rare and clinically more ag-

gressive than the intraparenchymal form, characterized by

the appearance of an image in the form of a grape cluster,

the result of an aberrant proliferation of the cysticercar

membrane with simultaneous degeneration of the scolex.

Clinical manifestations include hydrocephalus, mass effect,

chronic meningitis, seizure, stroke, reversible dementia, and

cranial neuropathy. This variant accounts for 10% of cases.

The diagnosis is established based on the clinical presenta-

tion and neuroimaging exams - computed tomography and

magnetic resonance.

Case report

G.F.M, 71 years old, male, farmer, presented a history of

holocranial headache of a pulsatile type, more intense on

waking, and associated with photophobia. The complaint

lasted six months. He also reported episodes of tinnitus

and unilateral hearing loss on the left. The case progres-

sively evolved to the point that the patient complained

of changes in visual acuity. After this new symptom, the

patient developed a generalized tonic-clonic seizure and

was then referred for hospital evaluation at a neurological

referral service.

After conducting screening tests in the emergency depart-

ment, communicating hydrocephalus and cystic lesions

were found in the subarachnoid and intraparenchymal

space. Laboratory tests showed no signicant changes.

In the neurological examination, the positive data were

bilateral papilledema and hearing loss in the left bone

conduction. Given these ndings, the racemous variant

of neurocysticercosis and secondary hydrocephalus was

suspected, causing intracranial hypertension. As there

were no signs of contraindication to lumbar puncture, this

test was carried out, which presented an opening pressure

of 38 cmH20, conrming the presence of ICH, cellularity

102 mm3, proteinorchia of 158 mg/dL, hypoglycorrhea

of 42 mg/dL, and test from chemiluminescence to positive

neurocysticercosis.

Thus, it was decided to start specic treatment with alben-

dazole 15 mg/kg/day (two daily doses), associated with

praziquantel 50 mg/kg/day (three daily doses), for 14

days. To reduce the inammatory process of the lesions,

it was proposed to keep the patient on dexamethasone 8

mg/day for 90 days. In addition to this scheme, topiramate

100 mg/day associated with acetazolamide 1 g/day

was started, along with serial lumbar puncture to relieve

intracranial pressure.

The patient evolved with a satisfactory improvement of

headache and low visual acuity and maintained the con-

dition of hearing loss even after the treatment performed.

Coments

Some factors predispose to contagion, among them the

underdevelopment and prevalence of the rural region

with the absence of basic sanitary measures.

8,9

Neuro-

cysticercosis is classied into extra and intraparenchymal

forms. In the intraparenchymal form, seizures are almost

pathognomonic for this condition.

8,10

And in general, in up

to 70% of patients, they represent the rst manifestation of

the disease and occur, mostly, due to the degeneration of

the parasite or the release of antigens from the calcied

cysticercus.

Thus, it is justiable that the main stages of

cysticerci found by neuroimaging are the nal stages, the

granular stage, and the calcied stage.

8,10

In many cases,

there are recurrences of seizures, directly related to residual

calcications and, mainly, with hippocampal sclerosis.

The extraparenchymal form includes the presence of cysts

in the subarachnoid, intraventricular space, spinal cord, or

ASAA

Sampaio PGG, Maracajá HD, Temoteo TC, Figueiredo SRN, Batista FLA

Hydrocephaly and secondary intracranial hypertension to racemosa neurocysticercosis

intraocular space. In subarachnoid involvement, cysticerci

may be present in the cortical grooves. However, if these

cysts are located in the basal cisterns, they have a great

chance of having the appearance of a “grape bunch”,

characterizing the racemic variant, whose main clinical

manifestation - ICH - is caused by the “mass effect” of the

cysts, obstructing CSF ow

9,11

and causing non-obstructive

hydrocephalus, with nocturnal worsening and morning

improvement, justied by the orthostatic position, which

favors the drainage of cerebrospinal uid (CSF).

According to the criteria of Del Brutto, it is possible to close

a diagnosis through a biopsy of the lesion with histopatho-

logical study, the visualization of subretinal parasites by fun-

doscopy or the visualization of the cystic of the cysticercus in

the neuroimaging. In the absence of these absolute criteria,

it is possible to associate the image with epidemiological,

clinical, and/or serological criteria. It is important to note

that the ELISA is not the most suitable immunological test,

due to the inconsistency in sensitivity and specicity, since

intraparenchymal lesions are usually not enough to cause

changes in the serology of the CSF sample.

8,11,12

NCC presents a wide variety of possible differential diag-

noses concerning its neuroimaging, the main ones being:

benign and malignant tumors. Simulating the intraparen-

chymal form, it is necessary to remember, especially in the

presence of single lesions, of brain tuberculomas, which,

in addition to sharing endemic zones with the NCC, also

present the “hole-in-dot” sign, previously considered to be

pathognomonic of the NCC. Regarding the extraparen-

chymal form, cystic hemangioblastomas and colloid cysts

should be excluded, which can also obstruct the CSF ow,

mimicking ICH and headache present in some cases of

NCC.

About communicating hydrocephalus is initially indicated

to perform ventricular shunt due to ICH, and then to carry

out conservative drug treatment.

However, given the risk of

bleeding during the procedure, socioeconomic conditions,

and the not so exacerbated clinic, conservative treatment

may be the rst option. In the case in question, an assess-

ment was requested from the neurosurgery team, which

chose not to proceed with a peritoneal shunt, against what

the literature recommends.

It is worth mentioning that the proposed treatment for the

racemous form is not the typical treatment performed for

NCC with single and mild lesions, in which only one an-

tiparasitic can be prescribed, lasting an average of 10 to

14 days depending on the radiological resolution of the

cyst. Generally, the treatment of racemosa is carried out by

combining albendazole, 15 mg/kg/day, maximum dose

of 1200 mg, divided into two doses, with praziquantel,

50 mg/kg/day divided into 3 doses, until radiological

resolution of the condition, associated with the use of cor-

ticosteroids or methotrexate, as an alternative to prolonged

treatment.7 This, in turn, requires monitoring of the patient's

biochemical parameters, due to the potential hepatotoxic

and leukopenic effects of this treatment.

Corticosteroids (Prednisone 1 mg / day or dexamethasone

0.1 mg/day) are used before antiparasitic therapy has

been prescribed to reduce inammation/edema of brain

tissue caused mainly by cyst degeneration, resulting from

antiparasitic treatment.

13,14

In patients with subarachnoid

cysts, mainly in the racemous form, as well as in those with

multiple intraparenchymal, ventricular and medullary cysts,

the administration of corticosteroids before and during an-

tiparasitic therapy is mandatory, since, in addition to being

necessarily longer, the inammatory reaction caused by

the destruction of the cyst can be exacerbated to the point

of occluding vessels, causing cerebral infarctions, as well

as massive cerebral edema.

When there is calcication of cysticerci, antiparasitic ther-

apy is no longer necessary, as the cysts are no longer

viable. Also, the prescription of corticosteroids is not indi-

cated even in the presence of perilesional edema, to avoid

rebound effect at the end of this treatment.

Conclusion

From the report presented and the association between

the radiological images and the presented clinical picture,

the diagnosis of the racemic variant of neurocysticercosis

was reached.

This is the helminth infection that most affects the CNS and

represents a major public health problem in most parts

of the world, since it remains a neglected pathology, like

so many other parasites, given that it is susceptible to

containment through simple preventive actions, as well

as eradication. This, in turn, depends on individualized

clinical treatment, with increased time for subarachnoid

cysts (mainly in the racemous variant), use of corticosteroids

before and concomitant with the use of antiparasitic drugs

in most cases, and use of anticonvulsants when necessary.

For this, a diagnosis based on neuroimaging is essential

but supplemented by the individual's epidemiology, clinic,

and even serology.

ASAA

Sampaio PGG, Maracajá HD, Temoteo TC, Figueiredo SRN, Batista FLA

Hydrocephaly and secondary intracranial hypertension to racemosa neurocysticercosis

Financing source: No

Conflict of interests: No

Authors' contribution: PGGS - Data Collection, Preparation and

Writing of the Original; HDM and FLAB – Methodology, Prepa-

ration and Writing of the Original; TCMT and SRNF- Writing of

the Original

Patrick Giordanni Gomes Sampaio

https://orcid.org/0000-0003-0952-9798

Hiago Diniz Maracajá

https://orcid.org/0000-0002-5032-3436

Túlio Carneiro Monteiro Temoteo

https://orcid.org/0000-0001-6160-3321

Sara Raquel Nóbrega Figueiredo

https://orcid.org/0000-0002-3832-4065

Francielle Lopes de Araújo Batista

https://orcid.org/0000-0002-7256-4070

References

1. Frackowiak M, Sharma M, Singh T, Mathew A

and Michael BD. Antiepileptic drugs for seizure

control in people with neurocysticercosis.

Cochrane

Database Syst Rev

2019;10(10):Cd009027 Doi:

10.1002/14651858.CD009027.pub3

2. Coyle CM. Neurocysticerosis: An Individualized

Approach.

Infect Dis Clin North Am

2019;33(1):153-

168 Doi: 10.1016/j.idc.2018.10.007

3. White Jr AC, Coyle CM, Rajshekhar V, Singh G,

Hauser WA, Mohanty A, . . . Nash TE. Diagnosis and

treatment of neurocysticercosis: 2017 clinical practice

guidelines by the Infectious Diseases Society of

America (IDSA) and the American Society of Tropical

Medicine and Hygiene (ASTMH).

J Clinical Infectious

Diseases

2018;66(8):e49-e75 Doi: 10.1093/cid/

cix1084

4. Hong BY, Bean A, Vickers A, Cykowski M and Lee

AG. Fourth Nerve Palsy Due to Neurocysticercosis.

Neuroophthalmol

2020;40(3):414-416 Doi:

10.1097/wno.0000000000000785

5. Krupa K, Krupa K, Pisculli ML, Athas DM and Farrell

CJ. Racemose neurocysticercosis.

Surg Neurol Int

2016;7:12 Doi: 10.4103/2152-7806.175881

6. Costa PS, Santiago IG, Lima WR, Santos CS, Rocha

AV and Fortes PM. Racemose neurocysticercosis: a

cluster of bad grapes.

Int J Infect Dis

2016;48:5-6

Doi: 10.1016/j.ijid.2016.04.012

7. McClugage SG, Lee RA, Camins BC, Mercado-Acosta

JJ, Rodriguez M and Riley KO. Treatment of racemose

neurocysticercosis.

Surg Neurol Int

2017;8:168 Doi:

10.4103/sni.sni_157_17

8. White AC, Jr. and Garcia HH. Updates on the

management of neurocysticercosis.

Curr Opin

Infect Dis

2018;31(5):377-382 Doi: 10.1097/

qco.0000000000000480

9. Gripper LB and Welburn SC. Neurocysticercosis

infection and disease-A review.

Acta Trop

2017;166:218-224 Doi: 10.1016/j.

actatropica.2016.11.015

10. Delgado-García G, Méndez-Zurita VA, Bayliss L,

Flores-Rivera J and Fleury A. Neurocysticercosis:

mimics and chameleons.

Pract Neurol

2019;19(2):88-

95 Doi: 10.1136/practneurol-2017-001788

11. P N and G B. Handbook of Clinical Neurology:

Neurologic Aspects of Systemic Disease Part III.

Elsevier

2014;121(97):1445 -1459

12. Reddy DS and Volkmer R, 2nd. Neurocysticercosis

as an infectious acquired epilepsy worldwide.

Seizure

2017;52(1)176-181 Doi: 10.1016/j.

seizure.2017.10.004

13. Garcia HH. Neurocysticercosis.

Neurol Clin

2018;36(4):851-864 Doi: 10.1016/j.ncl.2018.07.003

14. White AC. Cysticercosis: Treatment [Internet]. UpToDate. 2019

[cited 2020 Dec 9]. Available from: https://www.uptodate.com/

contents/cysticercosistreatment?search=neurocisticercose%20

racemosa&source=search_result&selectedTitle=3~20&usage_

type=default&display_rank=3#H1312982321.