Case ReportOn Neuropsychiatric Manifestations of Basal Ganglia Injury:A Report of Three Cases and Literature Review

Heela Azizi ,1 Alexander Kilpatrick,1 Olaniyi Olayinka ,2

Olusegun Poopola ,2 Maleeha Ahmad,1 Alexa Kahn,1 Tasmia Khan,3

Dina Rimawi,1 ShantaleWilliams ,1 Sinthuja Jayaraj,1 Ivan Leung,4

Sherina Langdon,1 Mirna Iskander,1 Ali Chohan,1 Deepa Nuthalapati,1

Ulunma Umesi,3 Geetha Vyas,1 Ayotomide Oyelakin ,2 Kodjovi Kodjo,2

Chiedozie Ojimba,2 Oluwole Jegede ,2 Carolina Nisenoff,2 and Ayodeji Jolayemi2

1American University of Antigua College of Medicine, Department of Psychiatry, Interfaith Medical Center, Brooklyn, New York, USA2Department of Psychiatry, Interfaith Medical Center, Brooklyn, New York, USA3Medical University of the Americas, Department of Psychiatry, Interfaith Medical Center, Brooklyn, New York, USA4Saba University School of Medicine, Department of Psychiatry, Interfaith Medical Center, Brooklyn, New York, USA

Correspondence should be addressed to Heela Azizi; heelaa@auamed.net

Received 19 January 2019; Revised 21 March 2019; Accepted 24 March 2019; Published 4 April 2019

Academic Editor: Peter Berlit

Copyright © 2019 Heela Azizi et al. This is an open access article distributed under the Creative Commons Attribution License,which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

The basal ganglia have been considered to primarily play a role in motor processing. A growing body of theoretical and clinicalevidence shows that in addition to the motor functions the basal ganglia play a key role in perceptual and visual disturbances.Thisrole may be evident in patients with basal ganglia pathology and subsequent manifestation of symptoms that include cognitive,perceptual, and affective disturbances.We present three cases with basal ganglia pathology that demonstrate affective and psychoticsymptoms. Two of the cases presentedwith late onset psychotic disturbances suggesting likely neurological etiologies. The third casepresented with treatment refractory psychosis and symptoms that are rare for a diagnosis of schizophrenia. The role of incidentalbilateral basal ganglia calcifications in all the cases is discussed. A review of current literature highlighting various neuropsychiatricmanifestations of basal ganglia pathologies in various patients with psychiatric symptoms is presented.

1. Introduction

The basal ganglia neural circuits are utilized in movementselection, planning, and learning. A much less researchedfeature of the basal ganglia is its function in cognition andperceptual function [1–3]. A few cases in literature havesuggested the possible link between basal ganglia damageand visual hallucinations and psychosis [4, 5]. The symptomsexhibited by patients are consistent with varying degreesof hallucinations, delusions and flashbacks [6, 7]. The uti-lization of advanced imaging techniques such as MagneticResonance Imaging (MRI) might provide the means toidentify organic causes of new onset psychoses and furtherknowledge of the functions of the basal ganglia.These lesionsand associated symptoms give us insight into the complex

pathways within the basal ganglia that have not been fullyexplored. A comprehensive investigation exploring damagedbasal ganglia will allow us to elucidate potential causes ofpsychosis and hallucinations and open up new avenues ofpharmacotherapy targeting the neural networks involved inhallucinations and psychosis. We present three patients withaffective and psychotic symptoms who share brain imagingfindings of incidental basal ganglia pathology. We also reviewrelevant literature of symptoms reported for patients withbilateral basal ganglia calcifications (BGC).

2. Case Presentations

2.1. Case One. The first case was a 59-year-old AfricanAmerican male with a past medical history notable for

HindawiCase Reports in Neurological MedicineVolume 2019, Article ID 3298791, 11 pageshttps://doi.org/10.1155/2019/3298791

2 Case Reports in Neurological Medicine

Figure 1: CT scan without contrast showing bilateral basal ganglia calcification.

schizoaffective disorder, depression, and substance abusewho was brought in to the emergency room for disorganizedbehavior and agitation in the community. At the time ofadmission the patient demonstrated disorientation, repetitivemotor behavior, and an alternation between agitation andpsychomotor retardation. He had poor response to commu-nication and tactile stimuli. A suspicion of altered mentalstatus due to organic causes was suspectedwith the possibilityof catatonic excitement and retardation. He was admittedto the medical floor, with a work-up revealing a positivetoxicology screen for cocaine and opioids. The patients CBCand BMP were within normal limits except for his ammonialevel which was 80mg/dl. The patient was initially treatedwith Chlorpromazine Hcl 50mg orally daily for his agitatedbehavior aswell asNaltrexone 50 mgorally daily for his opiateintoxication.

The patient exhibited incoherent thought process inaddition to mumbled speech that made a significant portionof his assessment evaluation difficult. During evaluation,he displayed abnormal movements of his arms and face,with tremors and restlessness. His affect was flat. He didnot display any perceptual disturbances or delusions. Anassessment for cognitive impairment was noncontributoryduring his most recent admission. The patient receivedMirtazapine 45mg orally at bedtime and Olanzapine 10mgorally daily in his treatment and by day three of admis-sion had shown improvement in his disorganized behaviorwith supportive care. The patient demonstrated more effortto directly communicate with house staff after treatmentbegan.

The patient reported a past history of psychiatric illnessthat was late in onset. His first presentation at the age of 51yearswas significant for depressedmood, paranoid delusions,and auditory hallucinations for which he was diagnosedwith a major mood disorder. His symptoms respondedpoorly to medications including antidepressants. His diseasecourse involved increasing periods of impulsive behaviorand agitation. He became noncompliant with his prescribed

medications. He was later admitted to the medical floors atthe age of 54 years for “repetitive behavior” during which hewas found moving from his bed to the bathroom repeatedlyas if he wanted to use the bathroom all the time. He alsoshowed some abnormal rocking movements during this timeperiod. A medical work-up for seizure was negative. Hewas discharged with a presumptive diagnosis of a psychoticdisorder. Thereafter, at the age of 56 years he had an episodeof property destruction in the community and it was notedthat he had “abnormal body movements” in addition tolability of mood. His diagnosis was revised to schizoaffectivedisorder and he was treated for mood lability at the time withrisperidone.

Given the late onset of his neuropsychiatric symptoms,a computed tomography scan (CT) of his brain was doneduring his presentation, as seen in Figure 1. Reviewing hischart, it was noted that the calcifications were apparent in hisfirst head CT taken in January of 2012 with no changes to thecurrent CT in January of 2019.

2.2. Case Two. The second case is of a 71-year-old AfricanAmerican man, who presented following an episode ofagitation and combativeness in the community. The patienthad a late onset psychiatric history starting at the age of57 with a diagnosis of schizophrenia. He was found todemonstrate symptoms of pressured speech, loose associa-tion, paranoid delusions, grandiose delusions, and labilityof mood. He was very restless during the interview andpaced around multiple times as he could not sit still. Healso demonstrated constant oral movements initially thoughtto be responding to internal stimuli. However, he deniedauditory hallucinations and stated he could not control hismouth movements. A mental status examination (MontrealCognitive Assessment) revealed a score of 22/30 with sig-nificant deficits in memory and executive control. This wasconsistent with his psychiatric evaluation which revealedsignificant confabulation in his history. The patient wasstarted on haloperidol 2mg orally twice daily and risperidone

Case Reports in Neurological Medicine 3

Figure 2: CT scan without contrast showing bilateral basal ganglia calcification.

0.5mg orally twice daily for his psychotic symptoms. Thepatient reports he has been noncompliant with medicationspreviously. On systemic review, the patients CBC was withinnormal limits and he had a negative toxicology screen. Thepatients BMP had abnormal glucose values of 172mg/dl andvitamin D level of 6.4ng/dl. An initial CT scan at the timeof first diagnosis noted basal ganglia calcifications. A repeatCT scan of the brain was done (shown in Figure 2) andshowed small bilateral basal ganglia calcifications, consistentwith the image findings during his first episode of psychoticdisturbance.

The patient showed some improvement in lability withmedication compliance but his cognitive impairment, para-noid delusions, and grandiose delusions did not significantlyimprove. His extrapyramidal symptoms were not amenableto pharmacological interventions of anticholinergic ben-ztropine.

2.3. Case �ree. The third case reported is a 69-year-oldEnglish/Creole speaking Haitian female. Her initial admis-sion was for an acute episode of mixed mood symptoms andpsychotic symptoms at the age of 61. The patient reportedconstant restlessness with inner anxiety and preoccupationwith delusions of control. She had a past history of treat-ment for chronic progressive paranoid delusions, cognitivedysfunction, and disorganized thought believed to be dueto schizophrenia that responded poorly to treatment. At thetime of her initial admission to our clinic, the diagnosiswas revised to schizoaffective disorder considering the mooddisturbances. A change in medications from risperidone3mg orally twice daily to fluphenazine 5mg orally twicedaily was also done. She showed no improvement in herpsychosis and affective symptoms. At the time of her secondadmission, the patient was brought in by husband on accountof bizarre behavior and disorganized thought in the contextof medication noncompliance. Her symptoms had evolved toinclude visual hallucinations of Buddha, visual hallucinationsof demons, and perceptual distortions of the floor. Sheendorses bizarre delusions stating there is a demon inside ofher and that an “agent” took the place of her husband. She also

exhibited depressive symptoms with worsening restlessnessand cognitive functioning. Urine toxicology was negative onadmission, with full blood count and metabolic panel withinnormal limits. Risperdal 2mg orally twice daily was con-tinued for psychosis. Paliperidone 156mg intramuscularlyone-time depot shot was added, with a second dose fivedays later of 117mg intramuscularly one time. She continuedto endorse visual hallucinations of “the head of the devil”that “moves like a shadow”. Other notable findings were aMontreal Cognitive Assessment score of 22/30 with deficitsin memory and executive functioning. Given the refractorynature of her disease and onset of new symptoms specificallyof a visual nature, a head CT without contrast was orderedto rule out organic pathology. The images showed smallbilateral basal ganglia calcifications, in addition to mild tomoderate bilateral periventricular and deep white matterlow attenuation suggestive for chronic small vessel ischemicdisease (Figure 3).

On day 9 of admission, patient remained internally pre-occupied with a disorganized thought process. Throughoutthe following week, she continued to hear voices of “thedevil” with beliefs that the “evil spirits attack me and myhusband”. Haloperidol 5mg orally twice daily was added fortreatment of psychosis, and no side effects of the medicationswere reported by the patient. Her antipsychotic regimenof fluphenazine 5mg orally twice daily that was eventuallyincreased to 7.5mg orally twice daily, as delusions of perse-cution with auditory and visual hallucinations, continued tobe present.

A differential diagnosis of psychosis due to a neuro-logical condition was added, with possible role of basalganglia injury considered in light of the visual hallucinations,akathisia symptoms, and cognitive dysfunction. The patientwas treated symptomatically with fluphenazineHcl (Prolixin)5mg orally twice daily, hydroxyzine Hcl 10mg orally twicedaily, and benztropine 1mg twice daily. Although her visualhallucinations and delusions did not resolve significantlywith medications, an augmentation with psychoeducation,supportive therapy, and cognitive behavior therapy helpedthe patient cope with her symptoms.

4 Case Reports in Neurological Medicine

Figure 3: CT scan without contrast showing mild to moderate bilateral periventricular and deep white matter low attenuation along withtrace bilateral basal ganglia calcification.

3. Discussion

Thebasal ganglia have been postulated to play a role inmotorcontrol, emotional processing, perception, and cognitivefunction [8]. Consequently, an insult to any part of this circuitshould reveal abnormal symptoms of perception, emotionalprocessing, and cognition. Our cases demonstrated pro-gressive disturbances in mood and perception (particularlyauditory and visual) in the context of basal ganglia pathology.The possibility of attributing these symptoms to basal gangliapathology is informed by the evolving understanding of therole of the basal ganglia and brain imaging [8].

In the first and second cases, the late onset of thesymptoms raises the index of suspicion for an underlyingneurological cause.Mufaddel et al. reported that patients withBGC tend to develop psychiatric symptoms later in life thanother psychiatric patients and have higher rates of medicalcomorbidities [9]. In the third case, the patient had a chronicdiagnosis of schizophrenia. The presence of refractory symp-toms and visual hallucinations usually motivate a medicalwork-up for underlying organic etiology which in her caserevealed bilateral basal ganglia calcification like the othertwo cases. Cummings et al. [10] reported a case of basalganglia calcification that presented with schizophrenia likesymptoms as early as age of 17 years; similar observationshave been noted in the literature [11]. The image findingsreflected a pathology of the basal ganglia bilaterally alongwith peripheral calcifications of the posterior occipital lobeand central tentorium like those reported in prior literature[8, 10, 12, 13].

The similar natures of the three cases led the teamto conduct a literature review on the varying symptomsreported in patients with basal ganglia pathology. Conse-quently, PubMedwas searched for peer-reviewed articles thatreported psychiatric symptoms and also had evidence ofbasal ganglia calcification(s). The search was not restrictedby timeframe or language. The search was conducted usingthe keywords andMeSH terms: “basal ganglia” “calcification”and “function.” We also searched the reference list of eligiblearticles to identify additional papers relevant to this study.

Screening for eligible articles was conducted independentlyby five authors. Eligible studies were those that focusedon symptomatology of basal ganglia injury and respectivebehavior in human subjects.

Given the paucity of articles on this topic, all types ofstudies were considered for analysis including experimental,cohort, meta-analysis, case-control, case series, and casereports. Any disagreement regarding the eligibility of anarticle was resolved by discussion among the authors. Rel-evant data from eligible articles were extracted and enteredonto a data abstraction form designed by the authors usingMicrosoft Excel. Data extracted from eligible articles includetitle of the article, gender and age of the patient, signs andsymptoms elicited, and lab findings/imaging recorded. Theliterature review table is shown in Table 1.

Bilateral basal ganglia calcification was confirmed by CTscan in a total of 316 patients, ranging from 7 to 96 years old.125 patients were male and 191 were female. Sixteen percentwere ages 7-30 years old, 31% were ages between 20 and 50years old, about 32% were ages 50 to 70 years old, and 21%were 70 years and older. In terms of location, 33% had nospecification of the basal ganglia calcification, while 51%werein the globus pallidum and 18% in the putamen. Twenty-twopercent of the cases had other areas of calcification outside thebasal ganglia including the thalamus, cerebral white matter,the cerebellar dentate nuclei, and the putamen.

About 53% of cases reported no psychiatric symptoms.Of the 47% that reported symptoms, the symptoms includedboth affective symptoms (19% of cases) and psychotic symp-toms (26% of cases).Themost commonly reported symptomsincluded delusions (12%), extrapyramidal symptoms (9%),mood lability (9%), visual hallucinations (3%), and agitation(1%). Cognitive dysfunctions were reported in less than 1%of cases. Five percent of cases presented with symptoms thatmet the criteria for schizophrenia, and 2% presented withsymptoms that met criteria for mania. No cross correlationwas reported between the different symptoms.There was alsono report of correlation between the size of the calcification,location of the calcifications in the basal ganglia, the dura-tion of the calcifications, and the symptoms presented. In

Case Reports in Neurological Medicine 5

Table1:Areview

ofselected

literatureo

nbasalgangliainjury

resulting

inneurop

sychiatricmanifesta

tions.

Researchers

ArticleTitle

Num

ber&

Gender

ofParticipants

Age

Sign

s&Symptom

sLabFind

ings/Im

agingStud

ies

Shira

hama,M.,

Akiyoshi,J.,

Ishito

bi,Y.,etal.

Ayoun

gwo

man

with

visual

hallu

cinatio

ns,delu

sions

ofpersecution

andah

istoryo

fperform

ingarsonwith

possiblethree-generatio

nFahr

disease

[14]

1F23

Visualhallu

cinatio

ns,delu

sionof

injury,

irritability,moo

dlability

CT:bilateralcalcifications

oftheb

asal

gang

lia(globu

spallid

us)

Ber,I.L.,M

arie,

R.,C

habo

t,B.,et

al.

Neuropsycho

logicaland

18FD

G-PET

studies

inafam

ilywith

idiopathicbasal

gang

liacalcificatio

ns[2]

5M

Pts.

3FPts.

7-73

Schizoph

renialikep

sychosiswith

late

EPS

CT&MRI:basalgang

liacalcificatio

n

Pan,

B.,Liu,W

.,Ch

en,Q

.,etal.

Idiopathicbasalgangliacalcificatio

npresentin

gas

schizoph

renia-lik

epsycho

sisandob

sessive-compu

lsive

symptom

s:Acase

repo

rt[15]

1M41

Schizoph

renia-lik

epsychosisand

obsessive-compu

lsive

symptom

sCT

:sym

metric

alcalcificatio

nsin

the

basalganglia

Kasuga,K

.,Ko

nno,T.,Saito,

K.,etal.

AJapanese

family

with

idiopathicbasal

gang

liacalcificatio

nwith

novelSLC

20A2

mutationpresentin

gwith

late-onset

hallu

cinatio

nanddelusio

n[16]

1F73

Auditory

Hallucinatio

ns,heard

swear

wordso

fneigh

borin

gchild

ren,and

delusio

nssheh

asbeen

eavesdropp

edon

CT:sym

metric

calcificatio

nin

theb

asal

gang

lia,thalamus,w

hitematter,occipital

cortex,and

cerebellard

entatenu

clei

Chabot,B

.,Ro

ulland

,C.,&

Dollfu

s,S.

Schizoph

reniaa

ndfamilialidiopathic

basalgangliacalcificatio

n:ac

aser

eport

[12]

3M

2F

36-6

8(36,38,

39,

46,68)

Schizoph

reniar

elapse

CT:bilateraland

symmetric

albasal

gang

liacalcificatio

ns

6 Case Reports in Neurological Medicine

Table1:Con

tinued.

Researchers

ArticleTitle

Num

ber&

Gender

ofParticipants

Age

Sign

s&Symptom

sLabFind

ings/Im

agingStud

ies

Nicolas,G

.,Guillin,

O.,

Borden,A

.,etal.

Psycho

sisrevealingfamilialidiopathic

basalgangliacalcificatio

n[17]

F39

Auditory

hallu

cinatio

nsanddelusio

nsCT

:basalgang

liacalcificatio

nsin

the

prob

andandin

hertwo

asym

ptom

atic

parents.

Lauterbach,E.C

.,Spears,T.,

Prew

ett,M.J.,e

tal.

Neuropsychiatric

disorders,myoclon

us,

anddysto

niain

calcificatio

nof

basal

gang

liapathways[18]

2M

Patie

nt1:44

Patie

nt2:40

Both

patie

ntsh

adcogn

itive

dysfu

nctio

n,tempo

rallob

e-lik

esym

ptom

s(inclu

ding

amnestics

tate,perceptualdistortio

ns,or

complex

visualhallu

cinatio

ns),and

myoclon

us

Patie

nt1:CT

with

contrast:

Bilateral

medialand

lateralpallid

alcalcificatio

nanteromedially.Th

yroidT3

,I''4,T,and

thyroid-stimulatingho

rmon

ewerea

llno

rmal.

Patie

nt2:MRI:bilateralh

olo-anterio

rglob

uspallidu

scalcificationmeasurin

g1

cmon

ther

ight

and1/2

cmon

theleft

atthetim

eofexamination.

Flint,J.,&

Goldstein,L.H

.

Familialcalcificatio

nof

theb

asalgang

lia:

acaser

eportand

review

oftheliterature

[19]

Pt1:M

Pt2:F

Patie

nt1:31

Patie

nt2:67

Delu

sions

thatcelebrities

were

jealou

sof

hisson

g-writingtalent

andwe

rethreateninghim,third

person

auditory

hallu

cinatio

ns,tho

ught

broadcastin

gand

theb

eliefthatp

eoplek

newwhath

ewas

thinking

.Slig

htchoreiform

movem

ents

ofthea

rms;repetitivem

ovem

entswe

reslo

wandpo

orcoordinatio

n.

Serum

calcium,pho

sphate,alkaline

phosph

atasea

ndPT

Hwe

realln

ormal.

Radiologicevaluatio

n:bilateral

calcificatio

nof

theg

lobu

spallid

us.

Forstl,H.,

Krum

m,B

.,Eden,

S.,etal.

Neurologicald

isordersin166patie

nts

with

basalgangliacalcificatio

n:a

statistic

alevaluatio

n[20]

59M

107F

16-86

Noevidence

ofas

ignificantly

increased

riskof

dementia

,cerebralinfarction,

epilepsy,vertigo,headache,ora

lcoh

olism

CTs:97%of

BGCgrou

phadcalcificatio

nin

theg

lobu

spallid

usand46

%had

calcificatio

nin

thep

utam

en.25B

GC

patie

ntssho

wedac

ircum

scrib

edun

ilateralcalcificationof

theg

lobu

spallidu

s,and143p

atientssho

wedbilateral

mineralization

Case Reports in Neurological Medicine 7

Table1:Con

tinued.

Researchers

ArticleTitle

Num

ber&

Gender

ofParticipants

Age

Sign

s&Symptom

sLabFind

ings/Im

agingStud

ies

Francis,A.

FamilialBa

salG

angliaCalcificationand

Schizoph

reniform

Psycho

sis[21]

5M 3F

9-53

(9,13,

27,32

,35,42,44

,53)

Persecutoryd

elusio

ns,aggressive

behavior

andauditory

andvisual

hallu

cino

sis,coarseinvolun

tary

movem

entsof

theh

eadandtrun

k,Parkinsonian

facies

andgait,

flatte

ned

affect,elevationof

moo

d,pressured

speech,paranoia,EP

S.

SkullX

-ray:bilateralcalcifications

ofthe

basalganglia

Vakaet,A

.,Ru

bens,R

.,Re

uck,J.D.,etal.

Intracranialbilateralsym

metric

alcalcificatio

non

CT-scann

ing[22]

1F57

Tonicfi

t,LO

C,tong

uebitin

g,urinary

incontinence

(hxof

epilepsy),global

dementia

,dysarthria

,EPS

(rigidity

)inall

four

limbs,p

ositive

Chvoste

k’ssig

n.

CT:extensiv

ebilateralsym

metric

alcalcificatio

nin

ther

egionof

theb

asal

gang

lia,nucleiofthe

cerebellu

mandthe

cerebralandcerebellarw

hitematter.

Geschwind,D.

H.,Lo

gino

v,M.,

&Stern,J.M.

Identifi

catio

nof

aLocus

onCh

romosom

e14qforIdiop

athicB

asalGanglia

Calcification(FahrD

isease)[23]

3M9F

9-73

(9,10

,38,40

,41,46,43,58,

52,50,70,76

)

Psycho

sisandschizoph

reniform

psycho

sis

Who

le-genom

escanusingpo

lymorph

icmicrosatellitemarkers:G

enom

icstu

dyestablish

esthefi

rstchrom

osom

allocus

(IGBC

1)andlin

kage

analysisregarding

ageo

fonsetof

familialIBGCprod

uced

results

consistentw

ithgenetic

antic

ipation.

CTscan:IBG

C

Roiter,B.,Pigato,

G.,&Perugi,G

.

Late-onsetManiain

aPatient

with

Movem

entD

isorder

andBa

salG

anglia

Calcifications:A

Challengefor

Diagn

osis

andTreatm

ent[24]

1M58

Severe

delirious-m

anicepiso

deconsisting

ofirr

itability,dyspho

ria,talkativ

eness,

racing

thou

ghts,

hyperactivity,

distr

actib

ility,grand

iosity,persecutory

delusio

ns,psychom

otor

agitatio

n,aggressiv

eness,insomnia,andmental

confusion

CT:m

ilddiffu

secorticalatroph

yand

smallbasalgang

liacalcificatio

nsbu

tthese

finding

swerec

onsid

ered

notclin

ically

relevant.

CTandbrainMRI

(forthe

second

episo

de):mod

erated

iffusec

ortic

alatroph

y,especiallyin

frontalandoccipital

region

s,po

sterio

rwhitematterlesions

duetochronicv

ascularischemia,and

smallbilateralpallid

alcalcificatio

ns

8 Case Reports in Neurological Medicine

Table1:Con

tinued.

Researchers

ArticleTitle

Num

ber&

Gender

ofParticipants

Age

Sign

s&Symptom

sLabFind

ings/Im

agingStud

ies

John

son,

J.M.,

Legesse,B.,

Cam

prod

on,J.A

.,etal.

Thec

linicalsig

nificance

ofbilateralbasal

gang

liacalcificatio

npresentin

gwith

maniaanddelusio

ns[25]

1M37

New

-onsetpsycho

ticmaniapresented

with

increase

ingu

ilt,elatedand

expansivem

ood,pressuredspeech,

grandiosed

elusio

ns(i.e.claiminghe

was

Chris

t),increasedenergy,erratic

thou

ghtsandactiv

ity,impu

lsive

and

risk-taking

.Presenceo

fdelu

sions

ofreference.

Com

pleteb

lood

coun

t,basic

metabolic

panel,liver

functio

ntests

,TSH

,CRP

,ES

R,we

reun

remarkable,with

negativ

eRP

R.Serum

calcium

andintactPT

Hwe

rewith

inno

rmallim

its.U

rined

rug

screen

was

negativ

e.Hed

idhave

apo

sitiveA

NA,at1:30,in

anon

specific

speckled

patte

rn.A

heavy-metalscreen

was

negativ

efor

arsenic,lead,m

ercury,

andcadm

ium.

CT:smallbilateralbasalgang

liacalcificatio

ns.

Philp

ot,M

.P.,&

Lewis,

S.W.

TheP

sychop

atho

logy

ofbasalganglia

calcificatio

n[26]

12M

24F

Meanaffected

M-6

0Mean

affectedF-

71

Nopsychiatric

diagno

siswas

specifically

associated

with

BGCalthou

ghcalcificatio

nof

thep

utam

enandthe

caud

atew

ason

lyfoun

din

patie

ntsw

ithfunctio

nald

isorders.

Noabno

rmalities

ofcalcium

orph

osph

atem

etabolism

were

foun

d.

Trautner,R

.J.,

Cummings,J.L.,

Read,S.L

.,etal.

Idiopathicbasalgangliacalcificatio

nand

organicm

ooddisorder

[27]

5M

Pt1:55

Pt2:47

Pt3:56

Pt4:

56Pt

5:49

Organicmoo

dchanges,inclu

ding

one

patie

ntwith

second

arymania.Sym

ptom

sresembletho

seof

otherd

isorders

affectin

gsubcortic

alstr

ucturesand

supp

ortanassociationbetweenmoo

d,affect,cogn

ition

,and

thee

xtra-pyram

idal

nuclearsystem.

Labstu

dies

were

norm

alam

ongstall

cases.

CTof

Case1:dense

basalganglia

calcificatio

nandperiv

entricular

gray

matterstructures.

CTof

Case2

:calcificationof

putamen

inbasalganglia.

CTof

Case3

:calcificationin

the

putamen,caudate,pulvinar,deep

sulciof

frontalcortex

anddentaten

uclei.

SkullX

-ray

ofCase3

:dense

basalganglia

calcificatio

n.CT

ofCase4

:extensiv

ebasalgang

liacalcificatio

nin

caud

aten

uclei,thalam

i,anddentaten

ucleiofcerebellum.

CTof

Case5

:massiv

eintracranial

calcificatio

nsof

basalganglia,cerebellum,

andareaso

fcerebralw

hitematter.

Case Reports in Neurological Medicine 9

Table1:Con

tinued.

Researchers

ArticleTitle

Num

ber&

Gender

ofParticipants

Age

Sign

s&Symptom

sLabFind

ings/Im

agingStud

ies

Cummings,J.L.,

Gosenfeld,L.F.,

Hou

lihan,J.P.,e

tal.

Neuropsychiatric

distu

rbancesa

ssociated

with

idiopathiccalcificatio

nof

theb

asal

gang

lia[10]

1M56

Schizoph

renialikep

sychosis:

auditory

hallu

cinatio

ns,ideas

ofreferencea

ndinflu

ence,psychom

otor

retardation,

and

circum

stantialspeech.Depressed

moo

d.

CT:calcifications

symmetric

ally

depo

sited

inthep

utam

en,the

pulvinar,a

fewsulciofthe

frontalcortex

andthe

dentaten

uclei.

SkullX

-rays:densec

alcifications

ofthe

basalgangliaandthed

entatenu

cleiof

the

cerebellu

m.C

SFproteinwas

elevated

Shakibai,S.V

.,John

son,

J.P.,

&Bo

urgeois,J.A.

Fahr’sDise

ase:AnIncidentalFind

ingin

aCaseP

resentingwith

Psycho

sis[28]

1M24

Aggressiveb

ehavior,abusivelangu

age,

smiling

toself,

talkingto

selfandgh

osts,

fear

ofothers,irregular

sleep

patte

rns,

delusio

nsof

persecution,auditory

hallu

cinatio

ns.

MRI:sym

metric

allargea

reas

andfociof

calcificatio

nin

bilateralbasalgang

lia,

thalam

i,cerebellarp

arenchym

aand

subcortic

alregion

sofb

ilateralcerebral

hemisp

heres

Konig,P.

Psycho

pathologicalalteratio

nsin

caseso

fsymmetric

albasalgangliasclerosis

[29]

25M

37F

Varie

s

31casesinitia

llypresentedneurological

symptom

s,25

psychiatric

,and

6cases

hadno

symptom

s.InitialSymptom

sin

BilateralSym

metric

alBa

salG

anglia

Sclerosis

(n=62;6

Cases

Incidental

Disc

overs)Prim

aryNeurological50%

,Seizures

6%,T

IA6%

syncop

es5%

Paresis/apo

plexia10%Cephalea/vertigo

13%,E

PS-m

ovem

entd

isorders10%

,Prim

aryPsychiatric

40%,O

rganic

affectiv

e21%

Organicparano

id/

hallu

cinatory

1.6%Com

pulsion

s1.6%

Alcoh

olism

8%.O

ligop

hrenia1.6

%Dem

entia

8%.

Forb

othpsychiatric

andneurological

symptom

s,no

correlations

betweeneither

localizationor

volumeo

fintracerebral

calcificatio

nwe

reno

ted,exceptingcases

ofdementia

,which

show

edlarger

hyperdensities.Asb

asalgang

liacalcificatio

ninflictsm

orph

ological

damagetotheC

NS,ad

eterioratio

nof

brainfunctio

nshou

ldoccura

nd,infact,

was

identifi

edin

term

sofeith

erneurologicaland/or

psychiatric

symptom

s.

Shakibai,S.V

.,John

son,

J.P.,

&Bo

urgeois,J.A.

Parano

idDelu

sions

andCognitiv

eIm

pairm

entSug

gesting

Fahr’sDise

ase[7]

1F62

Multip

leparano

iddelusio

ns;beliefsthat

peop

lewe

recontrolling

herb

ymanipulatingelectricaldials,un

know

notherp

eoplew

ere“

listening

throug

hthe

walls”

ofherh

ome,fearsthath

erbedwas

“magnetized”.

CT:bilateralsym

metric

basalganglia

calcificatio

ns

“M”,males;“F”,fem

ales;“Pt”,patient;“MRI”,Magnetic

ResonanceIm

aging;“C

T”,com

putedtomograph

y.

10 Case Reports in Neurological Medicine

addition, there were no reports on the course and evolutionof the symptoms reported in the literature. Further studiesmay be needed to explore these factors in understanding theneuropsychiatry of basal ganglia pathology.

Limitations. (1) To explore functional image studies that mayalso reveal dysfunction in other areas of the brain causingthe pathology (2). Being a report of three individual cases,the need for a larger number of cases may provide a clearerpicture of correlation.

4. Conclusion

A growing body of theoretical and clinical evidence showsthat the basal ganglia play a key role in perceptual dis-turbances in addition to motor function. Our three casesdemonstrate affective and psychotic symptoms concurrentwith basal ganglia pathology. It is consistent with a literaturereview of the symptoms reported in patients with basalganglia pathology. More appreciation of neuropsychiatricpresentation of basal ganglia injury is needed with studiescharacterizing the correlation between basal ganglia pathol-ogy including onset and subnuclei location with symptoms.In doing so, we may improve the diagnosis, management andtreatment following injury.

Consent

The patient’s consent was obtained orally.

Conflicts of Interest

The authors have no conflicts of interest to declare.

Authors’ Contributions

All authors have participated in the procurement of thisdocument and agree with the submitted case report.

References

[1] F. A. Middleton and P. L. Strick, “Basal ganglia and cerebellarloops: motor and cognitive circuits,” Brain Research Reviews,vol. 31, no. 2-3, pp. 236–250, 2000.

[2] I. Le Ber, R.-M. Marie, B. Chabot, C. Lalevee, and G.-L.Defer, “Neuropsychological and 18FDG-PET studies in a familywith idiopathic basal ganglia calcifications,” Journal of theNeurological Sciences, vol. 258, no. 1-2, pp. 115–122, 2007.

[3] S. A. Kotz, M. Schwartze, andM. Schmidt-Kassow, “Non-motorbasal ganglia functions: A review and proposal for a model ofsensory predictability in auditory language perception,”Cortex,vol. 45, no. 8, pp. 982–990, 2009.

[4] N. Adachi, T. Watanabe, H. Matsuda, and T. Onuma, “Hyper-perfusion in the lateral temporal cortex, the striatum andthe thalamus during complex visual hallucinations: singlephoton emission computed tomography findings in patientswith charles bonnet syndrome,” Psychiatry and Clinical Neuro-sciences, vol. 54, no. 2, pp. 157–162, 2000.

[5] F. A. Middleton and P. L. Strick, “Basal ganglia output andcognition: Evidence from anatomical, behavioral, and clinicalstudies,” Brain and Cognition, vol. 42, no. 2, pp. 183–200, 2000.

[6] A. Rosenblatt and I. Leroi, “Neuropsychiatry of Huntington’sdisease and other basal ganglia disorders,” Psychosomatics, vol.41, no. 1, pp. 24–30, 2000.

[7] S. V. Shakibai, J. P. Johnson, and J. A. Bourgeois, “Paranoiddelusions and cognitive impairment suggesting Fahr’s disease,”Psychosomatics, vol. 46, no. 6, pp. 569–572, 2005.

[8] L. L. Brown, J. S. Schneider, and T. I. Lidsky, “Sensory andcognitive functions of the basal ganglia,” Current Opinion inNeurobiology, vol. 7, no. 2, pp. 157–163, 1997.

[9] A. A. Mufaddel, O. T. Osman, G. Al-Hassani, S. Al-Bedwawi,and M. J. Hashim, “Basal ganglia calcification in psychiatricinpatients: a case-control study,” Cognitive and Behavioral Neu-rology, vol. 29, no. 3, pp. 139–143, 2016.

[10] J. L. Cummings, L. F. Gosenfeld, J. P. Houlihan, and T. McCaf-frey, “Neuropsychiatric disturbances associated with idiopathiccalcification of the basal ganglia,” Biological Psychiatry, vol. 18,no. 5, pp. 591–601, 1983.

[11] E. D. Caine and I. Shoulson, “Psychiatric syndromes in Hunt-ington’s disease,” �e American Journal of Psychiatry, vol. 140,no. 6, pp. 728–733, 1983.

[12] B. Chabot, C. Roulland, and S. Dollfus, “Schizophrenia andfamilial idiopathic basal ganglia calcification: a case report,”Psychological Medicine, vol. 31, no. 4, pp. 741–747, 2001.

[13] P. Entezami, M. Raff, and S. Bonner, “Visual hallucinationssecondary to infarction of the caudate,” Journal of NeurologyNeurophysiology, vol. 6, no. 3, p. 298, 2015.

[14] M. Shirahama, J. Akiyoshi, Y. Ishitobi et al., “A young womanwith visual hallucinations, delusions of persecution and ahistory of performing arson with possible three-generationFahr disease,” Acta Psychiatrica Scandinavica, vol. 121, no. 1, pp.75–77, 2010.

[15] B. Pan, W. Liu, Q. Chen et al., “Idiopathic basal gangliacalcification presenting as schizophrenia-like psychosis and ob-sessive-compulsive symptoms: a case report,” Experimental and�erapeutic Medicine, vol. 10, no. 2, pp. 608–610, 2015.

[16] K. Kasuga, T. Konno, K. Saito, A. Ishihara, M. Nishizawa, andT. Ikeuchi, “A Japanese family with idiopathic basal gangliacalcification with novel SLC20A2 mutation presenting withlate-onset hallucination anddelusion,” Journal ofNeurology, vol.261, no. 1, pp. 242–244, 2014.

[17] G. Nicolas, O. Guillin, A. Borden, S. Bioux, R. Lefaucheur, andD. Hannequin, “Psychosis revealing familial idiopathic basalganglia calcification,”General Hospital Psychiatry, vol. 35, no. 5,2013.

[18] E. C. Lauterbach, T. E. Spears, M. J. Prewett, S. T. Price,J. G. Jackson, and A. D. Kirsh, “Neuropsychiatric disorders,myoclonus, and dystonia in calcification of basal ganglia path-ways,” Biological Psychiatry, vol. 35, no. 5, pp. 345–351, 1994.

[19] J. Flint and L. H. Goldstein, “Familial calcification of the basalganglia: A case report and reviewof the literature,”PsychologicalMedicine, vol. 22, no. 3, pp. 581–595, 1992.

[20] H. Forstl, B. Krumm, S. Eden, andK. Kohlmeyer, “Neurologicaldisorders in 166 patients with basal ganglia calcification: astatistical evaluation,” Journal of Neurology, vol. 239, no. 1, pp.36–38, 1992.

[21] A. F. Francis, “Familial basal ganglia calcification and schizo-phreniformpsychosis,”�eBritish Journal of Psychiatry, vol. 135,no. 4, pp. 360–362, 1979.

Case Reports in Neurological Medicine 11

[22] A. Vakaet, R. Rubens, J. de Reuck, and H. vander Eecken,“Intracranial bilateral symmetrical calcification on CT-scanning. A case report and a review of the literature,” ClinicalNeurology and Neurosurgery, vol. 87, no. 2, pp. 103–111, 1985.

[23] D. H. Geschwind, M. Loginov, and J. M. Stern, “Identificationof a locus on chromosome 14q for idiopathic basal ganglia cal-cification (Fahr disease),”American Journal of Human Genetics,vol. 65, no. 3, pp. 764–772, 1999.

[24] B. Roiter, G. Pigato, and G. Perugi, “Late-Onset mania in apatient with movement disorder and basal ganglia calcifica-tions: a challenge for diagnosis and treatment,” Case Reports inPsychiatry, vol. 2016, Article ID 1393982, 5 pages, 2016.

[25] J. M. Johnson, B. Legesse, J. A. Camprodon, E. Murray, andB. H. Price, “The clinical significance of bilateral basal gangliacalcification presenting with mania and delusions,”�e Journalof Neuropsychiatry and Clinical Neurosciences, vol. 25, no. 1, pp.68–71, 2013.

[26] M. P. Philpot and S. W. Lewis, “The psychopathology of basalganglia calcification,” Behavioural Neurology, vol. 2, no. 4,Article ID 643807, pp. 227–233, 1989.

[27] R. J. Trautner, J. L. Cummings, S. L. Read, and D. F. Benson,“Idiopathic basal ganglia calcification and organic mood dis-order,” �e American Journal of Psychiatry, vol. 145, no. 3, pp.350–353, 1988.

[28] S. Srivastava, M. S. Bhatia, V. Sharma, S. Mahajan, and G.Rajender, “Fahr’s disease: An incidental finding in a casepresenting with psychosis,” German Journal of Psychiatry, vol.13, no. 2, pp. 86–90, 2010.

[29] P. Konig, “Psychopathological alterations in cases of symmetri-cal basal ganglia sclerosis,” Biological Psychiatry, vol. 25, no. 4,pp. 459–468, 1989.

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