Neurocysticercosis Association with Cognitive and Aberrant Behavioural Symptoms: A Case Report and Review

doi:10.4236/jbbs.2011.14029

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Journal of Behavioral and Brain Science, 2011, 1, 229-233

doi:10.4236/jbbs.2011.14029 Published Online November 2011 (http://www.SciRP.org/journal/jbbs)

Neur ocysticercosis Association with Cognitive and Aberra n t

Behavioural Symptoms: A Case Report and Review

Rohit Verma1, Kuljeet Singh Anand2, Mina Chandra1, Neha Prakash2, Ankur Sachdeva1

1Department of Psych iatry, Post Graduate Institute of Medical Education and Research,

Dr Ram Manohar Lohia Hospital, New Delhi, India

2Department of Neurology, Post Graduate Institute of Medical Education and Research,

Dr Ram Manohar Lohia Hospital, New Delhi, Indi a

E-mail: [email protected]

Received June 10, 2011; revised July 7, 2011; accepted August 26, 2011

Abstract

Neurocysticercosis (NCC) is a leading cause of seizures and epilepsy worldwide. Its clinical manifestations

are varied, non specific and pleomorphic, depending on multiple factors. Since NCC mimic large number of

neurological disorders, it is important that clinicians should be familiar with these rare presentations to avoid

delay in diagnosis and management. We herein describe an unusual case of multiple NCC’s involving entire

cortex with only cognitive and behavioural manifestations and review pertinent literature.

Keywords: Neurocysticercosis, Dementia, Neuropsychiatric

1. Introduction

Neurocysticercosis (NCC) is the common parasitic infes-

tation of the central nervous system worldwide. The cli-

nical manifestations of NCC are varied and depend on

the topography, number, the size and stage of lesions, as

well as the status of the host’s immune response to the

parasite [1]. While some cases of NCC may remain as-

ymptomatic, most have been reported to present seizures,

headache, raised intra cranial tension (ICT) due to

blockage of CSF flow, stroke, neuropsychiatric symp-

toms, ophthalmologic and endocrinological manifesta-

tions [2]. Dementia, being the first and only presenting

symptom, has been an unusual association with NCC.

We herein describe an unusual case of multiple NCC’s

involving entire cortex with only cognitive and behav-

ioural manifestations and review pertinent literature.

2. Case Report

A 44 year old non-diabetic, normotensive non-vegetarian

male of middle socio economic status, educated up to higher

secondary, presented acute onset behavioral disturbances

and forgetfulness for two and half month. The symptoms

started after a mild continuous fever of 4 days followed

by B/L fronto-temporal headache which was followed

few days later by abrupt onset inappropriate behaviour

like walking in circles on bed, patting his body parts, and

shouting inappropriately. Patient would not stop despite

the efforts of family members. He received some medi-

cations from a nearby hospital (records not available) but

they showed no improvement but only put pati ent t o sleep.

In a few days, patient started g iving inappropriate response

to asked questions, irrelevan t and abusive talking . Patient

had become irritable and started exhibiting symptoms of

forgetfulness like difficulty finding rooms in his own house,

unable to find way back home from known places. Patient

seemed confused most of the time and disoriented to time

and place. His activities o f daily living were affected and

he needed constant nagging to carry out work. He was

also not able to maintain hygiene. Patient would wander

inside and outside the hou se for no reason. His sleep du-

ration was decreased with difficulty in initiating sleep.

There was no history suggestive of coronary artery dis-

ease, cerebro-vascular disease, trauma, blood transfusion,

joint pain, rashes, photosensitivity, seizure-like activity,

and substance use. The pati e n t did not gi ve a ny history of

loss of consciousness, vomiting, dysphagia, dysarthria,

gait ataxia or any weakness. There was no history of any

perceptual disturbances, delusion, suspiciousness, or de-

pressed mood. There was no past or family history of any

similar, psychiatric or neurological illness. His premor-

bid personality was well adjusted.

The general physical examination was normal. Visual

acuity, including pupils, intraocular pressure and ocular

fundi were normal. Both upper and lower limb bulk, power,

R. VERMA ET AL.

230

reflex and plantar response were normal. Sensory and ce-

rebellar examinations were also normal. Cranial nerves-

were intact. Extrapyramidal system showed no abnorma-

lity. No involvement of other system was found on ex-

amination. On mental state examination, rapport was for-

med easily. There was no abnormality in psychomotor

activity and speech was relevant, coherent, with normal

tone and productivity. Affect was euthymic with no per-

ceptual or abnormalities in though t—form, flow, con tent,

or possession. Attention was arousable but ill-sustained.

He was oriented to person but not to time or place. Im-

mediate, recent and remote memory was impaired with

confabulations. Judgement was impaired with no insight

into the illness. His mini-mental state examination (MMSE)

score was 10 [3]. On lobar function testing using Strub

and Black scheme, impairment was observed in working

and episodic verbal memory, executive functions, verbal

fluency, constructive praxis, and visual-spatial orientation.

Clinical Dementia Rating scale score was 2 suggesting

moderate dementia [4]. The Hamilton Depression Rating

Scale score was 7, thus limiting the influence of negative

affect over cognitive integrity [5].

Complete blood count, blood sugar, electrolyte, LFT,

KFT, and lipid profile were normal. X-ray chest, EEG

and echocardiogram were normal. On magnetic reso-

nance imaging (MRI) of brain, bilateral cerebral hemi-

spheres, cerebellum and brain stem region a multitude of

tiny cystic lesions of a similar nature were seen (Figure

1). There was adjoin ing hyperinten sity on T2 and FLAIR

images in few of the lesions. There was involvement of

bilateral gangliocapsular region, thalami, and caudate

nucleus. Bilateral parotid showed multiple tiny similar

cystic lesions. Bilateral mastoids showed hyperintensity

within. Ventricles were normal with basal cisterns, syl-

vian fissures and intracranial vascular flow voids pre-

served. Ophthalmic evaluation was normal including

B-scan. Enzyme linked immunoabsorbent assay (ELISA)

of the cerebrospinal fluid (CSF) confirmed NC C infection.

3. Discussion

Common infective causes of dementia include prion

Figure 1. (MRI brain scan showing bilateral cerebral hemi-

spheres, cerebellum and brain stem region were seen stud-

ded with multip le 1 - 2 mm size ring lesion with cy stic centre

and eccentric dark signal foci within).

disease, herpes virus, toxoplasmosis, cryptococcus, cytome-

galovirus, neurocysticercosis, syphillis, and borelia [6].

NCC is an infestation of the brain by Taenia solium, the

tissue-invading larval form of the pork tapeworm. The

larvae are introduced into the body by the ingestion of

Taenia solium eggs in fecally contaminated food or wa-

ter. Once ingested, the eggs hatch in the duodenum and

release larvae. The larvae then penetrate intestinal muco-

sa and enter the circulatory system, where they eventually

localize as cysts within the skeletal muscles, the eyes, or

the brain [2,7]. NCC is mainly lo cated in gray matter or at

the junction of gray and white matter with a rich blood sup-

ply. In this intra-parenchymal NCC, seizure is the most

common clinical manifestation. Extraparenchymal NCC

refers primarily to infection of the ventricles and subara-

chnoid spaces [2]. Clinical presentation is of more aggres-

sive behaviour as compared to the parenchymal lesions,

and generally, the prognosis of these patients is worse [8].

Leptomeningeal involvement of NCC may cause intrasell a r

arachnoiditis, which is one of the most severe complica-

tions of NCC, associated with hydrocephalus, multiple cra-

nial nerves dysfunction, and death [9]. Subarachnoid in-

volvement of NCC is in 3.5% of the patients with NCC

presen t with parasites in vesicles of the subarachnoid space,

presented with visual and hormonal impairment by direct

compression of the hypophyseal ste m and the optic nerves

[8]. Tuberculous granuloma, microab sc es s, focal meningo-

encephalitis, neoplasms, and vascular lesions should be

considered in the differential diagnosis [10].

Prevalence of NCC approaches 4% in rural areas of

the developing world [11]. Although neurocysticercosis

can cause almost any neurological symptom, late-onset

epilepsy and intracranial hypertension are its most com-

mon clinical manifestations [2]. Cognitive and behav-

ioural deteriorations in NCC are more commonly associ-

ated with hydrocephalus or multiple lesions [2,8].

Psychiatric symptoms have been frequently associated

with NCC; severity of psychiatric symptoms may corre-

late with treatment of NCC with anti-parasitic drugs,

associated with an increase in CNS inflammation [11].

The mental changes most frequently reported are: confu-

sion, disorientation, memory loss, hallucinations, psy-

chomotor incoordination, progressive deterioration of

language ability, and mental deterioration [12].

Forlenza et al. [11] reviewed 38 cases of neurocysti-

cercosis and reported psychiatric illness and cognitive de-

cline in 65.8% and 87.5% of cases, respectively. Amongst

psychiatric illness, depressive disorders and psychosis was

seen in 52.6% and 14.2% of cases, respectively. Progres-

sion of disease and intracranial hypertension correlated

with h igher levels of psychi atric como rbidity. Pr evious ep i-

sodes of depression correlated with recurrence of depres-

s ion in act ive dise ase. Sote lo et al. [13] reviewed 753 case s

231

R. VERMA ET AL.

and reported intellectual deterioration in 15.8%, psycho-

sis in 4.7%, and behavioral disturbance in 2.7%. These

s ympto ms w ere mor e commo n wi th hyd roceph alus o r mul -

tiple lesions. In a review of 23 cases, Loo and Braude

found that in 56%, the presenting symptom was psychi-

atric, while abnormal mental status examination results

were a presenting sign in 43% [14]. McCormick et al.

[15] reviewed 127 cases and found that 38% had intrac-

ranial hypertension; 10% had increased intracranial pres-

sure and dementia. In a review of 17 cases, Grisolia and

Wiederholt reported that 18% exhibited altered mental

status because of increased intracranial pressure [16].

Scharf reviewed 238 cases; at some point during the course

of illness, 33% experienced altered mental status [17].

Two percent presented with dementia, 3% with psycho-

sis. Wadia et al. reported three cases of disseminated

cysticercosis (involving brain and muscle tissue); all ex-

hibited dementia, and two cases experienced psychosis

[18]. Ther e are a lso c ase re ports of v aried presentatio n of

NCC including pure motor hemiparesis, alexia and

manic episodes [19-22].

Dixon and Lipscomb reported schizophrenic and manic-

like episodes as possible initial signs of neurocysticerco-

sis and found only 5 cases of true dementia in 450 patients

(1.1%) [23]. Andrade et al. [24] in a cross-sectional con-

trolled study reported dementia in 5 (12.5%) of 40 pa-

tients with NCC and attributed this to a synergistic inter-

action of cysticercus lesions and local inflammation. In

their view, antiepileptic drug use and seizure frequency

could not account for the cognitive features. They found

no correlation between cognitive scores and number, lo-

calization, or type of NCC lesions on MRI. Rosselli et al.

[25] reported a severe case of dementia associated with a

parenchymous miliary neurocysticercosis without motor

seizures whose EEG showed epileptic-like focal dis-

charges. The clinical presentation was of headaches, as-

sociated with apathy, poor school work and finally hallu-

cinations, confusion and excitement in apparently par-

oxysmal episodes. Diagana et al. [26] studied cognitive

impairment in an Ecuadorian urban Andean community

known to be an endemic zone for Taenia solium taeniasis

and cysticercosis. In a sample of 227 adults over the age

of 40 years assessed using Mini mental state tests (MMS),

the overall prevalence of cognitiv e impairment was 8.4%

(19/227). In the elderly above the age of 75 years the

prevalence of cognitive impairment was 23.5%. The au-

thors linked this high prevalence of dementia to the en-

demic nature of Taenia solium taeniasis and cysticercosis

in this community. The authors postulated that parasitic

encephalopathy which was present in 20% persons in

this Andean community could play a part in early dam-

age of cognitive function and hence result in such high

prevalence of dementia. The single case report of NCC

presenting only dementia as per our knowledge was by

Jha and Ansari [27].

Various pathophysiological mechanisms have been sug-

gested to explain the symptom production by NCC infec-

tion. Fuentes suggested that psychiatric symptoms asso-

ciated with NCC were not due to the direct effect of the

parasite on a definite region of the brain but were rather

related to mechanical alterations in CSF pressure. More-

over, inflammatory injury of the brain parenchyma may

also play some role [28]. Parasites may induce expres-

sion of several classes of genes in the CNS of the host

and exceeds the gene expression seen in inflammatory

processes. These genes encode proteins or neuropeptides

involved in the regulation of vital physiologic and be-

havioral processes, which have led to the behavioral ma-

nipulation hypothesis [29,30]. It has been observed that

NCC affects brain regions that are distant from the loca-

tion of the cysticerci [31]. Thus location of lesion itself

appears insufficient to caus e cognitive changes.

Dementia syndrome observed in patients with NCC

could be a combined effect, resulting from multiple pa-

rasitic and vascular lesions, disrupting frontal-parietal-tem-

poral networks related to intellectual functioning in pa-

tients with vulnerable brains (because of repeated epilep-

tic seizures, low educational levels, advanced age) [32].

One study suggests that dementia occurs frequently in

patients with untreated NCC, and it is reversible in most

cases [32]. Most studies for evaluating cognition in NCC

have taken patients having associated seizures and their

influence over assessment is indeed a bias.

Dementia as an initial presentation of NCC is a rare

occurrence, and even rarer is its occurrence solely. The

case described here is of a miliar cysticercosis, affecting

both cortical and subcortical structures within the cere-

bral parenchyma. Our case did not present with any of

the common symptoms of NCC and the diagnosis was

made on the basis of MRI and ELISA reports.

4. Conclusions

We describe a 44-year-old man with a multiple NCC stud-

ded over whole cortex. Patients with unusual clinical ma-

nifestations should be investigated more thoroughly. NCC

should be considered in the differential diagnosis of pa-

tients presenting with cognitive and disorganised behav-

ioural symptoms in absence of any other psychopatho-

logy even in absence of gross neurological signs. Prompt

intervention is also recommended for accurate diagnosis,

to prevent progression of the disease, and to avoid ire-

versible loss of the cognitive functions.

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