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NCC – DIAGNOSIS &
MANAGEMENT
ARUN
Laboratory findings
• Peripheral eosinophilia - up to 37% of cases
• Ventricular NCC – CSF
• Moderate mononuclear pleocytosis (cell counts rarely exceeding 300
per mm3),
• Moderate increase in CSF protein (50–300 mg/dL)
• CSF glucose levels are usually normal
• Hypoglycorrhachia - poor prognosis
Immunologic Diagnosis
• Assess prevalence of cysticercosis in populations
• Exclude or confirm diagnosis – neuroimaging findings inconclusive
• Enzyme-linked immunoelectrotransfer blot assay (EITB)
• detects antibodies to T. solium glycoprotein antigen in serum - lentil
lectin purified glycoprotein antigens (LLGP)
• Only reliable serologic test – specificity up to 100% and sensitivity of
up to 98% for patients with two or more lesions
• Major weakness - high rate of false-negative results (up to 50%) – SCG
• Poor sensitivity - calcified cysticerci
• In hospital-based series of SCG, however, the yield of the EITB was
considerably lower, 20% – 80%
Neuroimaging
• Objective evidence on the number and topography of lesions
• Burden of infection
• Stage of involution
• Degree of the host’s inflammatory reaction
• MRI - imaging modality of choice - cystic lesions located in the
ventricular system, the brainstem, subarachnoid space
• CT - best screening neuroimaging – suspected NCC - parenchymal
brain calcifications may be sole evidence of the disease
PARENCHYMAL
NEUROCYSTICERCOSIS
• Depend on the stage of involution
• Vesicular cysticerci
• small and rounded cysts, well demarcated
• eccentric hyperdense nodule in the interior - scolex
• pathognomonic “hole-with-dot” appearance
• no edema/contrast enhancement
• heavy nonencephalitic form of neurocysticercosis - brain looks like a
‘‘Swiss cheese /Starry sky ,’’ - another imaging finding that is
pathognomonic
• Colloidal cysticerci
• ill-defined lesions surrounded by edema
• ring pattern of enhancement
• Scolex - not usually visualized
• diagnostic challenge - tuberculomas, toxoplasma, brain abscesses,
primary or metastatic brain tumors – similar neuroimaging findings
• DWI and ADC maps facilitates the diagnosis - recognition of the scolex
• Cysticercotic encephalitis - severe form of the disease
• diffuse brain edema and collapse of the ventricular system without
midline shift
• multiple small ring-like or nodular lesions appear disseminated within
the brain parenchyma
• Granular cysticerci / cysticercus granuloma
• CT - discretely hyperdense nodular-enhancing lesion + surrounding
edema
• MRI – hypo onT1- and T2, surrounded by hyperintense rim -
representing gliosis
• Thicker ring or disk enhancement
• Calcified (dead) cysticerci
• CT - small hyperdense nodules without perilesional edema or
abnormal enhancement
• Sensitivity of conventional MRI - poor, SWI may enhance the
identification of calcifications
Solitary cysticercus granuloma
• Contrast CT - enhancing ring- or disk-shaped lesion
• usually less than 20 mm in diameter
• surrounded by a variable amount of perifocal edema
• eccentric scolex is frequently seen within the ring lesion
• Seen throughout the cerebral hemispheres, parietal and frontal lobes
- most frequent
• Plain CT - irregular white matter hypodensity due to vasogenic edema
• tiny speck of calcification may be seen within the area of hypodensity
Radiologic features consistent with a diagnosis of SCG
• A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar,
J.M.K. Murthy, et al. Neurology 2010;75;2236
DD of SCG
• small intracranial tuberculoma
• Tuberculomas - greater than 20 mm in size, irregular in outline, may
be associated with a midline shift
• Cysticercus granulomas - less than 20 mm in size, regular in outline,
not associated with a midline shift
• Visualization of the scolex as eccentric dot – characteristic, appears as
a hyperintense nodule within the live cyst, this gives a characteristic
hole-with dot appearance
• Earlier, ‘target sign’ (central nidus of calcification surrounded by a
ring of enhancement) was pathognomonic for tuberculoma
• In HIV - imaging picture resembling ‘the eccentric target sign’ -
suggestive of toxoplasmosis, eccentric core is produced by the leash
of inflamed leaky vessels entering through a sulcus into the lesion
• Mets - target’ lesion with a central core of calcification
• ‘target sign’ - nonspecific neuroimaging finding and is not specific for
NCC
• MRS in cysticercosis - elevated levels of lactate, alanine, succinate
and choline and reduced levels of N-acetyl aspartate and creatine.
• MRS in tuberculoma shows a high peak of lipids, more choline and
less N-acetylaspartate and creatine.
• The choline/creatine ratio was greater than 1 in all tuberculomas
Radiologic resolution of SCG
• Majority - resolve by 1 year
• Natural historymay take one of the following paths:
• lesion may completely resolve
• lesion may resolve by leaving behind a punctuate calcific residue
Neuroimaging - Recommendations
• The initial imaging evaluation of SCG may be either contrast CT or
MRI
• Once CT demonstrates a single enhancing lesion consistent with a
diagnosis of SCG, further evaluation with MRI may not be required
•A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar,
J.M.K. Murthy, et al. Neurology 2010;75;2236
Appropriate timing and modality of
follow-up imaging
• Follow-up imaging (either contrast-enhanced CT or MRI) should be
undertaken at 6 months following initial symptomatic presentation in
all individuals with SCG
• Earlier CT might be useful in order to identify those lesions that
enlarge or change morphology - suggesting an alternative etiology
(e.g., neoplasm, tuberculoma, or fungal granuloma)
SUBARACHNOID
NEUROCYSTICERCOSIS
• Most common neuroimaging finding - hydrocephalus
• inflammatory occlusion of Luschka and Magendie foramina
• fibrous arachnoiditis - responsible for hydrocephalus, seen as
abnormal basal leptomeningeal enhancement
• Cystic subarachnoid lesions
• Small - located within cortical sulci,
• Large - Sylvian fissure or within the basal cisterns
• Multilobulated appearance - racemose form, displace neighboring
structures, and behave as SOL
VENTRICULAR
NEUROCYSTICERCOSIS
• CT – Ventricular cysts are isodense with CSF; cannot be directly
visualized
• Appear as hypodense lesions that distort the ventricular system,
causing asymmetric obstructive hydrocephalus
• MRI - ventricular cysts are readily visualized because the signal
properties of the cystic fluid or the scolex differ from those of the CSF
• Cyst mobility within the ventricular cavities in response to
movements of the head - ventricular migration sign
SPINAL CORD
NEUROCYSTICERCOSIS
• MRI - imaging modality of choice - suspected cysticercosis of the
spinal cord or the spinal subarachnoid space
• Intramedullary cysticerci - rounded or septated lesions that may have
an eccentric hyperintense nodule representing the scolex
• If scolex is not identified - difficult to differentiate from spinal tumors
• Leptomeningeal cysts - freely mobile within the spinal subarachnoid
space and change their position according to movements of the
patient on the exploration table
Diagnostic criteria
• Diagnosis - challenge in many patients
• Clinical manifestations are nonspecific
• Neuroimaging findings are most often not pathognomonic
• Serologic tests - relatively poor specificity and sensitivity
• Histologic demonstration of the parasite is not possible in most cases
• Diagnostic criteria based on the objective evaluation of clinical,
radiologic, immunologic and epidemiologic data has been proposed
Diagnostic Criteria for Neurocysticercosis
• Del Brutto OH, Rajshekhar V, White AC Jr et al. (2001). Proposed diagnostic criteria for neurocysticercosis. Neurology 57: 177–183
Revised diagnostic criteria of neurocysticercosis applicable to poor countries
• Garg RK. Diagnostic criteria for neurocysticercosis: some modifications are needed for Indian patients. Neurol. India 52(2), 171–177 (2004).
Diagnostic criteria for solitary cysticercus granuloma
• Rajshekhar V, Chandy MJ. Validation of diagnostic criteria for solitary cerebral cysticercus granuloma in patients
presenting with seizures. Acta Neurol. Scand. 96(2), 76–81 (1997).
Clinical and radiologic features consistent with a diagnosis of SCG
• A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar,
J.M.K. Murthy, et al. Neurology 2010;75;2236
Clinical and radiologic features consistent with a diagnosis of SCG
• A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar,
J.M.K. Murthy, et al. Neurology 2010;75;2236
TREATMENT
• Accurate characterization of disease - important for a rational therapy
• viability of cysts
• degree of the host’s immune response to the parasites
• location of the lesions
• Therapeutic approaches - include a combination of
• Symptomatic therapy
• Cysticidal drugs
• Surgical resection of lesions
• Placement of ventricular shunts
Treatment approach - Intraparenchymal
Treatment approach - Extraparenchymal
Parenchymal
Neurocysticercosis
Parenchymal brain calcifications
• Represent sequelae of previous infections
• should not be treated with cysticidal drugs
• may be an incidental finding on neuroimaging – endemic areas
• prophylactic AED therapy - not justified
• AEDs - advised when associated with seizures
• Neuroimaging studies performed immediately after seizure relapse -
focal edema and abnormal contrast enhancement around previously
inert calcifications
Symptomatic therapy
• AEDs - adequate control of seizures - respond well to first-line AEDs
• Risk of seizure recurrence remains high as long as the granuloma is
visible on imaging as an enhancing lesion
• Outcome improves following resolution of SCG
• Seizure relapses are associated with the presence of residual
calcification
• Optimal length of antiepileptic drug therapy - has not been settled
• Should receive AED therapy for at least 2 years after the last seizure,
followed by gradual withdrawal
• Withdrawal is not recommended in patients with multicystic disease
– will end up with calcified lesions, and a substantial proportion will
have further seizure relapses
SCG - Recommendations
• Risk of seizure recurrence - related to the persistence of the
enhancement
• Currently used AEDs effectively prevent seizure recurrence
• Appropriate to continue AEDs until such time that the lesion is
actively degenerating - appears as an enhancing lesion on imaging
studies
• AED may be withdrawn once complete resolution of the granuloma is
demonstrated on follow-up imaging studies
• Risk of seizure recurrence - remains high if the granuloma resolves
leaving behind a calcific residue, longer duration of AED should be
considered
• Any AED may be used, newer non-enzyme-inducing AED might be
considered for the period of time that antihelminthic treatment is
administered - Both phenytoin and carbamazepine were shown to
increase metabolism of praziquantel and albendazole
 A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G.
Singh, V. Rajshekhar, J.M.K. Murthy, et al. Neurology 2010;75;2236
Cysticidal drugs - Albendazole
• Initially administered at doses of 15 mg/kg/day for 1 month
• Further studies - length of therapy could be shortened to 1 week
without lessening the efficacy of the drug
• usual dose 15 mg/kg per day for 2 weeks
• even to 3 days if the patient has SCG
• Albendazole destroys 70–80% of parenchymal brain cysts
• superior to praziquantel in trials comparing the efficacy
• Another advantage - also destroys subarachnoid and ventricular cysts
due to its different mechanism of action
Cysticidal drugs - Praziquantel
• destroys 60–70% of parenchymal brain cysticerci
• usual dose 50 mg/kg per day for 2 weeks
• single-day course – SCG - three individual doses of 25–30 mg/kg at 2
hour intervals
Vesicular cysts
• Reached a state of immune tolerance with the host
• may remain for years in the brain parenchyma
• only way to destroy these cysts is by the use of a cysticidal drug
• evidence favors the use of cysticidal drugs - provides clinical
improvement and resolution of lesions
• Single cyst: Albendazole 15 mg/kg/d for 3 days or praziquantel 30
mg/kg in three divided doses every 2 hours
Antihelminthics in SCG
• Two recent meta-analyses of randomized trials have evaluated the
effect of cysticidal drugs on neuroimaging and clinical outcomes of
patients with neurocysticercosis
• Better resolution of both colloidal and vesicular cysticerci
• Lower risk of seizure recurrence in patients with colloidal cysticerci,
and a reduction in the rate of generalized seizures in patients with
vesicular cysticerci
• Del Brutto OH, Roos KL, Coffey CS, Garcia HH. Metaanalysis: cysticidal drugs for
neurocysticercosis: albendazole and praziquantel. Ann Intern Med 2006
Seizure freedom with anthelminthic treatment
• Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta-
analysis. Neurology. 2013 Jan 8;80(2):152-62
Granuloma resolution with anthelminthic treatment
• Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta-
analysis. Neurology. 2013 Jan 8;80(2):152-62
Conclusion on Antihelminthics
• Conclusively established the benefit of anthelminthic treatment
• Significantly improved resolution rate of the granuloma
• Better seizure-freedom rates
• Anthelminthics might hasten the involution of the granuloma as well
as offer the clinical benefit of improved possibility of being seizure-
free
• No evidence, however, for either an increased or decreased risk of
residual calcification associated with anthelminthic treatment
Role of corticosteroids - SCG
• Administration of a short course of corticosteroids in conjunction
with anthelminthic treatment - to control edema and symptoms due
to the host inflammatory response - common practice
• Many variations of steroidal drugs, doses, and lengths of treatment
have been used
• most common regimen is 0·1 mg/kg per day of dexamethasone given
1 day before antiparasitic therapy commences and maintained for 1
or 2 weeks, followed by a slow taper
• Anecdotal observations of the benefits of corticosteroid treatment
regarding amelioration of certain manifestations (including seizures
and headache) - attributable to the inflammatory degeneration of
SCG
Seizure freedom with corticosteroid treatment
• Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta-
analysis. Neurology. 2013 Jan 8;80(2):152-62
Granuloma resolution with corticosteroid treatment
• Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta-
analysis. Neurology. 2013 Jan 8;80(2):152-62
Conclusion on corticosteriods
• No overall significant difference between the corticosteroid-treated
and control subjects -
• rates of seizure freedom
• granuloma resolution
• residual calcification
• Lack of benefit of corticosteroid treatment on various outcomes -
important finding in guiding treatment policies for SCG
• Corticosteroids are useful in the management of inflammatory
symptoms in multiple neurocysticercosis
Cysticercotic encephalitis
• Cysticidal drugs must not be used, may exacerbate the inflammatory
response within the brain parenchyma
• High doses of corticosteroids and osmotic diuretics are advised as the
first therapeutic measures - to reduce the severity of brain edema
• Should be prolonged for 2 to 3 weeks until the edema subsides.
• Refractory cases – decompressive craniotomies - to avoid the life-
threatening risk of intracranial hypertension
Extraparenchymal
Neurocysticercosis
Subarachnoid cysts
• Medical treatment of small subarachnoid cysts localised to the
convexity of the cerebral hemispheres - similar parenchymal brain
cysts
• only difference - albendazole is the preferred drug because it
penetrates the subarachnoid space better and reaches higher
concentrations in the CSF
• Treatment of giant cysts in the Sylvian fissure is controversial - some
authors recommend surgical resection, others suggest medical
therapy with albendazole and corticosteroids might be an equally
effective but less aggressive approach
Hydrocephalus
• Intracranial hypertension – cysticercotic arachnoiditis, mass effect of
cysts located in basal subarachnoideal cisterns, or the obstruction of
CSF pathway by ventricular cysts.
• due to cysticercotic arachnoiditis - Immediate CSF drainage or shunt
placement
• high dose corticosteroids (dexamethasone, 16 mg/kg per day or
more) - frequently lead to temporary control of hydrocephalus
Ventricular cysts and ependymitis
• Could be treated by surgical resection or by antiparasitic treatment
• Consensus guidelines favour surgical
• Possible exception - small cysts located in lateral ventricle
• Favoured surgical approach –
• endoscopic removal of cysts in the lateral and third ventricles with a
flexible ventriculoscope
• Posterior approach for removal of fourth ventricular cysts
• Absence of ependymitis, permanent shunt placing is not necessary
• Shunt placement should follow or even precede the excision of
ventricular cysts associated with ependymitis
References
• Garcia HH, Nash TE, Del Brutto OH. Clinical symptoms, diagnosis, and
treatment of neurocysticercosis. Lancet Neurol. 2014 Dec
• Del Brutto OH. Neurocysticercosis. Handb Clin Neurol. 2014
• Singh G, Rajshekhar V, Murthy JM, Prabhakar S, Modi M, Khandelwal
N, Garcia HH. A diagnostic and therapeutic scheme for a solitary
cysticercus granuloma. Neurology. 2010 Dec 14
• Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary
cysticercus granuloma: a systematic review and meta-analysis.
Neurology. 2013 Jan
• Del Brutto OH, Roos KL, Coffey CS, García HH. Meta-analysis:
Cysticidal drugs for neurocysticercosis: albendazole and praziquantel.

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Neurocysticercosis

  • 1. NCC – DIAGNOSIS & MANAGEMENT ARUN
  • 2. Laboratory findings • Peripheral eosinophilia - up to 37% of cases • Ventricular NCC – CSF • Moderate mononuclear pleocytosis (cell counts rarely exceeding 300 per mm3), • Moderate increase in CSF protein (50–300 mg/dL) • CSF glucose levels are usually normal • Hypoglycorrhachia - poor prognosis
  • 3. Immunologic Diagnosis • Assess prevalence of cysticercosis in populations • Exclude or confirm diagnosis – neuroimaging findings inconclusive • Enzyme-linked immunoelectrotransfer blot assay (EITB) • detects antibodies to T. solium glycoprotein antigen in serum - lentil lectin purified glycoprotein antigens (LLGP) • Only reliable serologic test – specificity up to 100% and sensitivity of up to 98% for patients with two or more lesions
  • 4. • Major weakness - high rate of false-negative results (up to 50%) – SCG • Poor sensitivity - calcified cysticerci • In hospital-based series of SCG, however, the yield of the EITB was considerably lower, 20% – 80%
  • 5. Neuroimaging • Objective evidence on the number and topography of lesions • Burden of infection • Stage of involution • Degree of the host’s inflammatory reaction • MRI - imaging modality of choice - cystic lesions located in the ventricular system, the brainstem, subarachnoid space • CT - best screening neuroimaging – suspected NCC - parenchymal brain calcifications may be sole evidence of the disease
  • 6. PARENCHYMAL NEUROCYSTICERCOSIS • Depend on the stage of involution • Vesicular cysticerci • small and rounded cysts, well demarcated • eccentric hyperdense nodule in the interior - scolex • pathognomonic “hole-with-dot” appearance • no edema/contrast enhancement • heavy nonencephalitic form of neurocysticercosis - brain looks like a ‘‘Swiss cheese /Starry sky ,’’ - another imaging finding that is pathognomonic
  • 7.
  • 8.
  • 9. • Colloidal cysticerci • ill-defined lesions surrounded by edema • ring pattern of enhancement • Scolex - not usually visualized • diagnostic challenge - tuberculomas, toxoplasma, brain abscesses, primary or metastatic brain tumors – similar neuroimaging findings • DWI and ADC maps facilitates the diagnosis - recognition of the scolex
  • 10.
  • 11. • Cysticercotic encephalitis - severe form of the disease • diffuse brain edema and collapse of the ventricular system without midline shift • multiple small ring-like or nodular lesions appear disseminated within the brain parenchyma
  • 12.
  • 13. • Granular cysticerci / cysticercus granuloma • CT - discretely hyperdense nodular-enhancing lesion + surrounding edema • MRI – hypo onT1- and T2, surrounded by hyperintense rim - representing gliosis • Thicker ring or disk enhancement
  • 14.
  • 15. • Calcified (dead) cysticerci • CT - small hyperdense nodules without perilesional edema or abnormal enhancement • Sensitivity of conventional MRI - poor, SWI may enhance the identification of calcifications
  • 16.
  • 17. Solitary cysticercus granuloma • Contrast CT - enhancing ring- or disk-shaped lesion • usually less than 20 mm in diameter • surrounded by a variable amount of perifocal edema • eccentric scolex is frequently seen within the ring lesion • Seen throughout the cerebral hemispheres, parietal and frontal lobes - most frequent • Plain CT - irregular white matter hypodensity due to vasogenic edema • tiny speck of calcification may be seen within the area of hypodensity
  • 18. Radiologic features consistent with a diagnosis of SCG • A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar, J.M.K. Murthy, et al. Neurology 2010;75;2236
  • 19.
  • 20. DD of SCG • small intracranial tuberculoma • Tuberculomas - greater than 20 mm in size, irregular in outline, may be associated with a midline shift • Cysticercus granulomas - less than 20 mm in size, regular in outline, not associated with a midline shift • Visualization of the scolex as eccentric dot – characteristic, appears as a hyperintense nodule within the live cyst, this gives a characteristic hole-with dot appearance
  • 21. • Earlier, ‘target sign’ (central nidus of calcification surrounded by a ring of enhancement) was pathognomonic for tuberculoma • In HIV - imaging picture resembling ‘the eccentric target sign’ - suggestive of toxoplasmosis, eccentric core is produced by the leash of inflamed leaky vessels entering through a sulcus into the lesion • Mets - target’ lesion with a central core of calcification • ‘target sign’ - nonspecific neuroimaging finding and is not specific for NCC
  • 22. • MRS in cysticercosis - elevated levels of lactate, alanine, succinate and choline and reduced levels of N-acetyl aspartate and creatine. • MRS in tuberculoma shows a high peak of lipids, more choline and less N-acetylaspartate and creatine. • The choline/creatine ratio was greater than 1 in all tuberculomas
  • 23.
  • 24. Radiologic resolution of SCG • Majority - resolve by 1 year • Natural historymay take one of the following paths: • lesion may completely resolve • lesion may resolve by leaving behind a punctuate calcific residue
  • 25. Neuroimaging - Recommendations • The initial imaging evaluation of SCG may be either contrast CT or MRI • Once CT demonstrates a single enhancing lesion consistent with a diagnosis of SCG, further evaluation with MRI may not be required •A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar, J.M.K. Murthy, et al. Neurology 2010;75;2236
  • 26. Appropriate timing and modality of follow-up imaging • Follow-up imaging (either contrast-enhanced CT or MRI) should be undertaken at 6 months following initial symptomatic presentation in all individuals with SCG • Earlier CT might be useful in order to identify those lesions that enlarge or change morphology - suggesting an alternative etiology (e.g., neoplasm, tuberculoma, or fungal granuloma)
  • 27. SUBARACHNOID NEUROCYSTICERCOSIS • Most common neuroimaging finding - hydrocephalus • inflammatory occlusion of Luschka and Magendie foramina • fibrous arachnoiditis - responsible for hydrocephalus, seen as abnormal basal leptomeningeal enhancement • Cystic subarachnoid lesions • Small - located within cortical sulci, • Large - Sylvian fissure or within the basal cisterns • Multilobulated appearance - racemose form, displace neighboring structures, and behave as SOL
  • 28.
  • 29. VENTRICULAR NEUROCYSTICERCOSIS • CT – Ventricular cysts are isodense with CSF; cannot be directly visualized • Appear as hypodense lesions that distort the ventricular system, causing asymmetric obstructive hydrocephalus • MRI - ventricular cysts are readily visualized because the signal properties of the cystic fluid or the scolex differ from those of the CSF • Cyst mobility within the ventricular cavities in response to movements of the head - ventricular migration sign
  • 30.
  • 31. SPINAL CORD NEUROCYSTICERCOSIS • MRI - imaging modality of choice - suspected cysticercosis of the spinal cord or the spinal subarachnoid space • Intramedullary cysticerci - rounded or septated lesions that may have an eccentric hyperintense nodule representing the scolex • If scolex is not identified - difficult to differentiate from spinal tumors • Leptomeningeal cysts - freely mobile within the spinal subarachnoid space and change their position according to movements of the patient on the exploration table
  • 32.
  • 33. Diagnostic criteria • Diagnosis - challenge in many patients • Clinical manifestations are nonspecific • Neuroimaging findings are most often not pathognomonic • Serologic tests - relatively poor specificity and sensitivity • Histologic demonstration of the parasite is not possible in most cases • Diagnostic criteria based on the objective evaluation of clinical, radiologic, immunologic and epidemiologic data has been proposed
  • 34. Diagnostic Criteria for Neurocysticercosis • Del Brutto OH, Rajshekhar V, White AC Jr et al. (2001). Proposed diagnostic criteria for neurocysticercosis. Neurology 57: 177–183
  • 35. Revised diagnostic criteria of neurocysticercosis applicable to poor countries • Garg RK. Diagnostic criteria for neurocysticercosis: some modifications are needed for Indian patients. Neurol. India 52(2), 171–177 (2004).
  • 36. Diagnostic criteria for solitary cysticercus granuloma • Rajshekhar V, Chandy MJ. Validation of diagnostic criteria for solitary cerebral cysticercus granuloma in patients presenting with seizures. Acta Neurol. Scand. 96(2), 76–81 (1997).
  • 37. Clinical and radiologic features consistent with a diagnosis of SCG • A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar, J.M.K. Murthy, et al. Neurology 2010;75;2236
  • 38. Clinical and radiologic features consistent with a diagnosis of SCG • A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar, J.M.K. Murthy, et al. Neurology 2010;75;2236
  • 39. TREATMENT • Accurate characterization of disease - important for a rational therapy • viability of cysts • degree of the host’s immune response to the parasites • location of the lesions • Therapeutic approaches - include a combination of • Symptomatic therapy • Cysticidal drugs • Surgical resection of lesions • Placement of ventricular shunts
  • 40. Treatment approach - Intraparenchymal
  • 41. Treatment approach - Extraparenchymal
  • 43. Parenchymal brain calcifications • Represent sequelae of previous infections • should not be treated with cysticidal drugs • may be an incidental finding on neuroimaging – endemic areas • prophylactic AED therapy - not justified • AEDs - advised when associated with seizures • Neuroimaging studies performed immediately after seizure relapse - focal edema and abnormal contrast enhancement around previously inert calcifications
  • 44. Symptomatic therapy • AEDs - adequate control of seizures - respond well to first-line AEDs • Risk of seizure recurrence remains high as long as the granuloma is visible on imaging as an enhancing lesion • Outcome improves following resolution of SCG • Seizure relapses are associated with the presence of residual calcification
  • 45. • Optimal length of antiepileptic drug therapy - has not been settled • Should receive AED therapy for at least 2 years after the last seizure, followed by gradual withdrawal • Withdrawal is not recommended in patients with multicystic disease – will end up with calcified lesions, and a substantial proportion will have further seizure relapses
  • 46. SCG - Recommendations • Risk of seizure recurrence - related to the persistence of the enhancement • Currently used AEDs effectively prevent seizure recurrence • Appropriate to continue AEDs until such time that the lesion is actively degenerating - appears as an enhancing lesion on imaging studies • AED may be withdrawn once complete resolution of the granuloma is demonstrated on follow-up imaging studies
  • 47. • Risk of seizure recurrence - remains high if the granuloma resolves leaving behind a calcific residue, longer duration of AED should be considered • Any AED may be used, newer non-enzyme-inducing AED might be considered for the period of time that antihelminthic treatment is administered - Both phenytoin and carbamazepine were shown to increase metabolism of praziquantel and albendazole  A diagnostic and therapeutic scheme for a solitary cysticercus granuloma G. Singh, V. Rajshekhar, J.M.K. Murthy, et al. Neurology 2010;75;2236
  • 48. Cysticidal drugs - Albendazole • Initially administered at doses of 15 mg/kg/day for 1 month • Further studies - length of therapy could be shortened to 1 week without lessening the efficacy of the drug • usual dose 15 mg/kg per day for 2 weeks • even to 3 days if the patient has SCG • Albendazole destroys 70–80% of parenchymal brain cysts • superior to praziquantel in trials comparing the efficacy • Another advantage - also destroys subarachnoid and ventricular cysts due to its different mechanism of action
  • 49. Cysticidal drugs - Praziquantel • destroys 60–70% of parenchymal brain cysticerci • usual dose 50 mg/kg per day for 2 weeks • single-day course – SCG - three individual doses of 25–30 mg/kg at 2 hour intervals
  • 50. Vesicular cysts • Reached a state of immune tolerance with the host • may remain for years in the brain parenchyma • only way to destroy these cysts is by the use of a cysticidal drug • evidence favors the use of cysticidal drugs - provides clinical improvement and resolution of lesions • Single cyst: Albendazole 15 mg/kg/d for 3 days or praziquantel 30 mg/kg in three divided doses every 2 hours
  • 51. Antihelminthics in SCG • Two recent meta-analyses of randomized trials have evaluated the effect of cysticidal drugs on neuroimaging and clinical outcomes of patients with neurocysticercosis • Better resolution of both colloidal and vesicular cysticerci • Lower risk of seizure recurrence in patients with colloidal cysticerci, and a reduction in the rate of generalized seizures in patients with vesicular cysticerci • Del Brutto OH, Roos KL, Coffey CS, Garcia HH. Metaanalysis: cysticidal drugs for neurocysticercosis: albendazole and praziquantel. Ann Intern Med 2006
  • 52. Seizure freedom with anthelminthic treatment • Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta- analysis. Neurology. 2013 Jan 8;80(2):152-62
  • 53. Granuloma resolution with anthelminthic treatment • Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta- analysis. Neurology. 2013 Jan 8;80(2):152-62
  • 54. Conclusion on Antihelminthics • Conclusively established the benefit of anthelminthic treatment • Significantly improved resolution rate of the granuloma • Better seizure-freedom rates • Anthelminthics might hasten the involution of the granuloma as well as offer the clinical benefit of improved possibility of being seizure- free • No evidence, however, for either an increased or decreased risk of residual calcification associated with anthelminthic treatment
  • 55. Role of corticosteroids - SCG • Administration of a short course of corticosteroids in conjunction with anthelminthic treatment - to control edema and symptoms due to the host inflammatory response - common practice • Many variations of steroidal drugs, doses, and lengths of treatment have been used • most common regimen is 0·1 mg/kg per day of dexamethasone given 1 day before antiparasitic therapy commences and maintained for 1 or 2 weeks, followed by a slow taper • Anecdotal observations of the benefits of corticosteroid treatment regarding amelioration of certain manifestations (including seizures and headache) - attributable to the inflammatory degeneration of SCG
  • 56. Seizure freedom with corticosteroid treatment • Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta- analysis. Neurology. 2013 Jan 8;80(2):152-62
  • 57. Granuloma resolution with corticosteroid treatment • Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta- analysis. Neurology. 2013 Jan 8;80(2):152-62
  • 58. Conclusion on corticosteriods • No overall significant difference between the corticosteroid-treated and control subjects - • rates of seizure freedom • granuloma resolution • residual calcification • Lack of benefit of corticosteroid treatment on various outcomes - important finding in guiding treatment policies for SCG
  • 59. • Corticosteroids are useful in the management of inflammatory symptoms in multiple neurocysticercosis
  • 60. Cysticercotic encephalitis • Cysticidal drugs must not be used, may exacerbate the inflammatory response within the brain parenchyma • High doses of corticosteroids and osmotic diuretics are advised as the first therapeutic measures - to reduce the severity of brain edema • Should be prolonged for 2 to 3 weeks until the edema subsides. • Refractory cases – decompressive craniotomies - to avoid the life- threatening risk of intracranial hypertension
  • 62. Subarachnoid cysts • Medical treatment of small subarachnoid cysts localised to the convexity of the cerebral hemispheres - similar parenchymal brain cysts • only difference - albendazole is the preferred drug because it penetrates the subarachnoid space better and reaches higher concentrations in the CSF • Treatment of giant cysts in the Sylvian fissure is controversial - some authors recommend surgical resection, others suggest medical therapy with albendazole and corticosteroids might be an equally effective but less aggressive approach
  • 63. Hydrocephalus • Intracranial hypertension – cysticercotic arachnoiditis, mass effect of cysts located in basal subarachnoideal cisterns, or the obstruction of CSF pathway by ventricular cysts. • due to cysticercotic arachnoiditis - Immediate CSF drainage or shunt placement • high dose corticosteroids (dexamethasone, 16 mg/kg per day or more) - frequently lead to temporary control of hydrocephalus
  • 64. Ventricular cysts and ependymitis • Could be treated by surgical resection or by antiparasitic treatment • Consensus guidelines favour surgical • Possible exception - small cysts located in lateral ventricle • Favoured surgical approach – • endoscopic removal of cysts in the lateral and third ventricles with a flexible ventriculoscope • Posterior approach for removal of fourth ventricular cysts • Absence of ependymitis, permanent shunt placing is not necessary • Shunt placement should follow or even precede the excision of ventricular cysts associated with ependymitis
  • 65. References • Garcia HH, Nash TE, Del Brutto OH. Clinical symptoms, diagnosis, and treatment of neurocysticercosis. Lancet Neurol. 2014 Dec • Del Brutto OH. Neurocysticercosis. Handb Clin Neurol. 2014 • Singh G, Rajshekhar V, Murthy JM, Prabhakar S, Modi M, Khandelwal N, Garcia HH. A diagnostic and therapeutic scheme for a solitary cysticercus granuloma. Neurology. 2010 Dec 14 • Otte WM, Singla M, Sander JW, Singh G. Drug therapy for solitary cysticercus granuloma: a systematic review and meta-analysis. Neurology. 2013 Jan • Del Brutto OH, Roos KL, Coffey CS, García HH. Meta-analysis: Cysticidal drugs for neurocysticercosis: albendazole and praziquantel.