Neurocysticercosis

1. NEUROCYSTICERCOSIS
By Dr. Sheelendra Shakya
Dept. of Paediatrics, KMCTH

2. INTRODUCTION
•A major cause of adult-
onset epilepsy in the
developing world.
•Cysticercosis is a
disease caused by the
presence of cysticercus
cellulosae and
cysticercus racemose,
the larval forms of T.
solium

3. TYPES OF CYSTS
Cysticercus cellulosae
•Less virulent form
•Small (<2cm), round,
thin walled
•Lodges in the
parenchyma or the
subarachnoid space
•Provokes only a minor
inflammation
•Often remain silent

4. TYPES OF CYSTS
Cysticercus racemose
•Large lobulated cysts with
predilection for basal cisterns
•Causes cysticercotic arachnoiditis
and presents as meningitis
•Causes obstruction of 4
th
ventricle
and resultant raised ICP and
hydrocephalus
•Can cause occlusion of vessels and
vasculits resulting in stroke
•Causes intense inflammatory
reaction and seizures

5. PREVALENCE

6. MODE OF INFECTION
•Humans are both
intermediate and definitive
hosts.
•Cysticercosis develops
when humans become
intermediate hosts by
ingesting the embryonated
eggs of the tapeworm, which
release oncospheres that
penetrate the intestinal wall,
enter the bloodstream, and
disseminate into the tissue.

7. MODE OF INFECTION
•HETEROINOCULATION
–eggs may come from the environment
•INTERNAL AUTOINOCULATION
–regurgitated from proglottids into the stomach
•EXTERNAL AUTOINOCULATION
–from the fingers of an infected person

8. TARGET TISSUES
•Predilection for migration to eyes, CNS
and striated muscles, probably due to high
glycogen and glucose content of these
tissues.
•CNS and Eye involvement is termed as
Neurocysticercosis.

9. PRESENTATION
The manifold and
diverse clinical
presentation of NC is
determined by
•Location of cysts
•Size of cysts
•Cyst load (number of
cysts)
•Host’s immune
response

10. CLASSIFICATIONS
•Anatomical classification
•Sotelo et al classification
•Carpio et al classification
•Chorobski Classification

11. Anatomical Classification
•Parenchymal NC
•Intraventricular NC
•Meningeal NC
•Spinal NC
•Ocular NC
Nelson

12. CLINICAL PRESENTATION
•Parenchymal NC
–Seizures (87%)
•Simple partial with secondary generalization, generalized,
complex partial or complex partial with secondary
generalization
–Headache, nausea and vomiting
–Stroke
•Hemiparesis
•Focal neurologic deficits
–Frontal lobe involvement
•Psychosis, dementia, parkinsonism, intellectual impairment
–Cerebellar Ataxia
–Fulminant encephalitis in massive initial infection

13. CLINICAL PRESENTATION
•Intraventricular NC
–5- 10% of all cases
–4
th
ventricle most common site for obstruction
–Cysts in lateral ventricles less likely to cause
obstruction
–Hydrocephalus and acute, subacute or
intermittent signs of raised ICP without
localizing signs

14. CLINICAL PRESENTATION
•Meningeal NC
–Meningeal irritation resembling TBM
–Raised ICP from oedema, inflammation and
presence of cyst obstructing flow of CSF

15. CLINICAL PRESENTATION
•Spinal NC
–Spinal cord compression
–Nerve root pain
–Transverse myelitis
–Arachnoiditis
•Ocular NC
–Visual impairment (decreased visual acquity)
–Scotoma, retinal detachment, iridocyclitis

16. Sotelo et al classification
•Active forms of NC
(represents both viable, live and degenerating
parenchymal cysts)
–Arachnoiditis
–Hydrocephalus secondary to meningeal inflammation
–Parenchymal cysts
–Brain infraction secondary to vasculitis
–Mass effect due to large cyst or cyst clumps
–Intraventricular cysts
–Spinal cysts
•Inactive forms of NC
–Parenchymal calcifications
–Hydrocephalus secondary to meningeal fibrosis

17. Carpio et al
•Active form
–Refers to live, viable parenchymal or extraparenchymal
cysts, rarely produce symptoms apart from the rare
instance of mass effect
•Transitional form
–Symptomatic seizures occur with this form.
Degenerating subarachnoid cysts produce meningitis,
arachnoiditis and hydrocephalus; ventricular cysts lead
to acute hydrocephalus.
•Inactive form
–Single or multiple parenchymal calcification/s and /or
hydrocephalus secondary to meningeal fibrosis

18. Chorobski Classification
CSF diversion
procedure
Basal meningeal or ventricular
cysticercus giving rise to
hydrocephalus and intracranial
HTN, rarely focal signs and mental
disturbances
Group III
Exeresis rarely useful
but may be undertaken
in life-threatening
conditions
Diffuse cerebral syndrome due to
numerous cysticerci leading to
cerebral oedema, intracranial
HTN, organic brain syndrome
Group II
Exeresis often indicatedSpace occupying intracranial
tumor like behaviour producing
focal neurological manifestations
and ultimately raised ICP
Group I
SURGICAL
IMPLICATION
CLINICAL FEATURESGROUP

19. INVESTIGATIONS
•Stool Routine and
Microscopy
•Fundoscopy
•Biopsy and histopathology
•CT with contrast
•MRI
•Serology
–EITB
•sensitivity of 98%
specificity of 100%
–ELISA in CSF
•sensitivity of 87%
specificity of 95%

20. RADIOLOGICAL STAGING
(A) Viable cyst with scolex

21. (B) degenerating cyst

22. (C) calcified cyst (non-contrast CT)

23. STAGES OF NC
•Cystic or vesicular stage
•Cyst wall & scolex do not enhance
•Cyst is viable & has a well defined, fluid-filled membrane contains
only one scolex.
•Colloid stage
•Enhancing walls with perilesional oedema
•Earliest stage in the involution of the cyst.
•the fluid contents of the cyst become more turbid and the scolex
begins to degenerate.
•Necrotic, granular stage
•Characterized by parasite necrosis and surrounding inflammation
•Gives an appearance of an eosinophilic structure in which the
bladder and scolex are in various stages of disintegration
•Oedema and/or necrosis of the surrounding neural tissue may be
present in some cases
•Fibro-calcified nodule
•With time, fibrosis develops, progressively occupying the entire
lesion
D.Sharada et al

24. DIAGNOSTIC CRITERIA
•Absolute criteria
–Demostration of cysticerci by histologic or microscopic examination of biopsy
material
–Visualization of the parasite in the eye by fundoscopy
–Neuroradiologic demostration of cystic lesions containing a characteristic scolex
•Major criteria
–Neuroradiologic lesions suggestive of NC
–Demostration of antibodies to cysticerci in serum by enzyme linked
immunoelectrotransfer blot
–Resolution of intracranial cystic lesions spontaneously or after therapy with
albendazole or praziquantel alone
•Minor criteria
–Lesions compatible with NC detected by neuroimaging studies
–Clinical manifestations suggestive of NC
–Demonstration of antibodies to cysticerci or cysticercal antigen in CSF by ELISA
–Evidence of cysticercosis outside the CNS (eg. Cigar shaped soft tissue
calcification)
•Epidemiologic criteria
–Residence in a cysticercosis-endemic area
–Frequent travel to a cysticercosis- endemic area
–Household contact with an individual infected with T. solium
Del Brutto et al

25. DIAGNOSTIC CRITERIA
•Definitive
–1 absolute
–2 major
–1 major + 2 minor + 1 epidemiological
•Probable
–1 major + 2 minor
–1 major + 1 minor + 1 epidemiological
–3 minor + 1 epidemiological
•Possible
–1 major
–2 minor
–1 minor + 1 epidemiological
Del Brutto et al

26. SUGGESTED DIAGNOSTIC
CRITERIA
•Absolute criteria
•Histopathological demostration of the parasite in the tissues obtained from
the biopsy of a brain or spinal cord lesion
•Multiple cystic lesions with or without scolex on CT or MRI
•Major Criteria
•Lesion highly suggestive of NC in neuroimaging studies
•Spontaneous resolution or eventual calcification
•Positive serum EITB assay for the detection of antibodies against T. solium
•Minor criteria
•Presence of a characteristic clinical picture
•Positive CSF ELISA
•Cysticercosis outside the CNS
•Aggravation of existing symptoms or appearance of a new symptom
following anticysticercal therapy
•Diagnosis with caution
•Old age
•Patients with pre existing systemic tuberculosis or malignancy
•HIV infection
•Grossly abnormal neurological examination
Garg

27. Tuberculoma Versus Cysticercus
Granuloma
Cysticercus Granuloma
•Round in shape
•Cystic
•20mm or less with ring
enhancement or visible
scolex
•Cerebral edema not
enough to produce
midline shift or focal
neurological deficit
Tuberculoma
•Irregular in shape
•Solid
•Greater than 20mm
•Associated with severe
perifocal edema and
focal neurological deficit
Rajshekhar et al
Target lesions:
Lesions with central nidus of calcification or a dot enhancement

28. Magnetic Resonance Spectroscopy
•Tuberculomas had a high peak of lipids,
more choline, and less N-acetylaspartate
and creatine.
•The choline/creatine ratio was greater
than 1 in all tuberculomas but in none of
the cysticerci.
Cysticercosis working group in Peru

30. Understanding a controversy

31. NATURAL COURSE
Rate of spontaneous resolution of a
solitary cysticercus granuloma in
patients with seizures
210 patients presenting with seizures with
a solitary cerebral cysticercus on CT
•3 months - 18.8%
•6 months - 36.4%
•1 year - 62.5%
Vedantam Rajshekhar

32. TREATMENT
Efficacy of albendazole and short-course
dexamethasone treatment in children with 1
or 2 ring-enhancing lesions of NC:
•Dexamethasone 0.15 mg/kg per day for 5 days
plus
•Albendazole 15 mg/kg per day for 28 days,
starting on the third day of dexamethasone
•Anti-epileptic therapy was given to both the
study groups
AIIMS

33. RESULTS
AIIMS
33%13%SEIZURES at 6 mths
32%10%SEIZURES at 3mths
57%79%RESOLUTION OF
LESION (Complete or partial)
CONTROLTREATEDPARAMETERS

34. STEROIDS
JULIO SOTELO, M.D., AND OSCAR H. DEL BRUTTO, M.D
•Corticosteroids represent the primary form of
therapy for cysticercal encephalitis and
arachnoiditis causing hydrocephalus and
progressive entrapment of cranial nerves.
•High doses of iv Dexamethasone along with
Mannitol at 2mg/kg/day can be followed by
chronic oral therapy with Prednisolone
1mg/kg/day or Dexamethasone 0.1mg/kg/day
administered 3 times a week.

35. NEW PROTOCOLS
•Praziquantel three doses of 25-30 mg/kg
at 2-hour intervals on a single day equally
effective to the 50mg/kg 8 hourly dose for
15 days. Corona T, Lugo R, Medina R, et al
•Albendazole 15mg/kg/day in 2 divided
doses for 1 week equally effective to the
15 mg/kg/day 12 hrly for a 1-month period.
Cysticercosis working group in Peru

36. Surgery restricted to:
•Placement of ventriculo-peritoneal shunts for
hydrocephalus
•Excision of single big cysts causing mass effect
•Endoscopical excision of intraventricular
parasites.
•Unfortunately shunts are frequently occluded by the high
protein content and debris in the CSF of patients with
extraparenchymal neurocysticercosis, requiring multiple
revisions.
•Deaths due to shunt dysfunction may occur in up to 50% of
cases, mainly in the initial 1–2 years after placement.
Garcia et al

37. •Group A (150 patients) were treated with
15 mg/kg/day albendazole for 14 days,
plus 2 mg dexamethasone orally at 8-h
intervals for 14 days, plus antiepileptic
drugs at appropriate doses. The
dexamethasone was tapered off over time.
•Group B (150 patients) were treated with
antiepileptic drugs and placebo.
Current Consensus Guidelines for
Treatment of Neurocysticercosis
Garcia et al

38. RESULTS
•During the first year of treatment the
incidences of seizure, encephalopathy,
and readmission were greater for group A
than group B.
•Two patients in group A died from
intractable seizures and encephalopathy
in the first 3 months of treatment.
Garcia et al

39. •The proportion of patients with complete
resolution of lesions was greater in group
B than in group A
•The proportion of patients with calcification
of lesions was greater in group A than in
group B. Calcified lesions may be the focal
pathology for seizure recurrence.
Garcia et al
randomised controlled study and follow-up of at least 5 years (the longest follow-
up period yet for this type of study) for 300 neurocysticercosis patients with more
than one lesion

40. Calcification Vs Natural Resolution
•There is increasing evidence that calcified
neurocysticercosis is not clinically inactive, and
that perilesional oedema may at times be
present around the apparently calcified foci.
•Therefore, it is better to wait for spontaneous
resolution of lesions, while treating the patient
with appropriate antiepileptic drugs for control of
seizures.
Garcia et al

41. RECOMMENDATIONS
1.Individualize therapeutic decisions,
including whether to use antiparasitic
drugs, based on the number, location,
and viability of the parasites within the
nervous system;
3.Actively manage growing cysticerci either
with antiparasitic drugs or surgical
excision;
Garcia et al

42. 1.Prioritize the management of intracranial
hypertension secondary to
neurocysticercosis before considering
any other form of therapy; and
3.Manage seizures as done for seizures
due to other causes of secondary
seizures (remote symptomatic seizures)
because they are due to an organic focus
that has been present for a long time.
Garcia et al

43. CONCLUSION
•Treatment with albendazole plus antiepileptic
drugs has no benefit over treatment with
antiepileptic drugs alone.
•Albendazole treatment may cause problems or
have adverse effects with regard to increased
seizure frequency, encephalopathy and hospital
readmissions in the early part of the treatment.
•Albendazole treatment may be disadvantageous
from an economical perspective because of the
direct and indirect treatment costs and the loss
of working days.
Garcia et al

44. REFERENCES
•Review of neurocysticercosis Julio Sotelo M.D., and Oscar H. Del Brutto, M.D
•New Concepts in the diagnosis and management of neurocysticercosis (Taenia
Solium) Hector H. Garcia, Oscar H. Del Brutto, Theodore E. Nash, A. Clinton White,
Jr., Victor C. W. Tsang, and Robert H. Gilman
•Neurocysticercosis: some of the essentials Hector H Garcia, Armando E Gonzalez,
Victor C W Tsang, Robert H Gilman, for the Cysticerocosis Working Group in Peru
•Diagnostic criteria for neurocysticercosis: Some modifications are needed for Indian
patients Garg Ravindra Kumar
•Medical Management of Neurocysticercosis Garg RK
•Current Consensus Guidelines for Treatment of Neurocysticercosis. Garcia et al
•Rate of spontaneous resolution of a solitary cysticercus granuloma in patients with
seizures Vedantam Rajshekhar, MCh
•Differential diagnosis between cerebral tuberculosis and neurocysticercosis by
magnetic resonance spectroscopy.Cysticercosis working group in Peru
•Harrison Textbook of Medicine 19
th
Edition
•Bailey and Love’s Short Practice of Surgery 21
st
edition
•Rudolph’s Pediatrics 21
st
edition
•Nelson’s Textbook of Pediatrics
•Others: D. Sharada et al, Carpio et al, Sotelo et al, Chorobski et al