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Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
de caso
Obsessive-Compulsive Disorder Symptoms in
Huntington’s Disease: A Case Report
Juan Carlos Molano-Eslava
Ángela Iragorri-Cucalón
Gonzalo Ucrós-Rodríguez
Carolina Bonilla-Jácome
Santiago Tovar-Perdomo
David V. Herin
Luis Orozco-Cabal
: Few cases of obsessive-compulsive disorder (OCD) symptoms preceding the
clinical onset of Huntington Disease (HD) or during later stages of the disease have been
reported in the literature, but the nature of this association and its neurobiological mecha-
nisms have not been well-investigated.
: To review the scienti
c literature regarding
OCD symptoms in patients with HD and describe a case study from our clinic.
Extensive literature searches were performed to identify reports of patients with concurrent
HD and OCD symptoms. Results: Recent studies and the current case report suggest that
OCD symptoms may predate or coincide with motor, affective or behavioral symptoms in
patients with HD. The development of OCD and HD symptoms may involve structural and
functional changes affecting the orbital and medial prefrontal cortex, ventromedial caudate
nucleus, and pallidal sites.
: Some patients with HD develop symptoms asso-
ciated with OCD. Progressive and differential neuropathological changes in the ventrome-
dial caudate nucleus and related neural circuits may underlie this association. No speci
treatment strategy has been developed to treat these patients; however some medications
attenuate associated symptoms. Further testing is needed to determine the neurobiological
mechanisms of these disorders.
Key words:
Obsessive-compulsive disorder, Huntington disease.
Médico psiquiatra. Hospital Universitario Fundación Santa Fe de Bogotá, Bogotá, Co-
lombia. Facultad de Medicina, Universidad de los Andes, Bogotá, Colombia.
Médica neuróloga. Clínica de Memoria, Clínica La Inmaculada, Bogotá, Colombia.
Médico del Hospital Universitario Fundación Santa Fe de Bogotá, Colombia. Facultad
de Medicina, Universidad de los Andes, Bogotá, Colombia.
Médica. Western Psychiatric Institute and Clinic. Pittsburg, PA, Estados Unidos.
Médico graduado de la Facultad de Medicina, Universidad de los Andes. Bogotá, Co-
PhD, Department of Psychiatry, University of Texas Health Science Center at Houston,
Texas, Estados Unidos.
Médico. PhD en Neurociencias. Facultad de Medicina, Universidad de los Andes, Bo-
gotá, Colombia.
Obsessive-Compulsive Disorder Symptoms in Huntington’s Disease: A Case Report
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
Título: Síntomas del trastorno obsesivo-
compulsivo en la enfermedad de Hunting-
ton: reporte de caso
: Algunos reportes de caso
indican que pacientes con enfermedad de
Huntington (EH) pueden presentar sínto-
mas obsesivo-compulsivos (TOC) antes del
desarrollo de la enfermedad y durante ésta,
pero no se ha estudiado la naturaleza de esta
asociación y sus mecanismos neurobiológi-
: Revisar la literatura cientí
acerca de la asociación entre EH y síntomas
TOC y reportar el caso de un paciente con es-
tas condiciones.
: Búsqueda selectiva
de literatura relevante.
: Estudios
recientes y el caso aquí reportado sugieren
que los síntomas TOC pueden presentarse
antes de la EH y durante ésta. El desarrollo
concurrente de estas patologías puede estar
mediado por cambios estructurales y funcio-
nales de la corteza prefrontal orbital y medial,
región ventromedial del núcleo caudado y
regiones palidales.
: Algunos
pacientes con EH desarrollan síntomas de
TOC. Cambios neuropatológicos progresivos
y diferenciales en el caudado ventromedial
y circuitos dependientes pueden mediar
esta asociación. No se ha desarrollado una
estrategia terapéutica para el tratamiento
de estos pacientes; sin embargo, algunos
medicamentos parecen ofrecer mejoría sin-
tomática parcial a los sujetos afectados. Se
requieren mayores estudios acerca de los
mecanismos neuropatológicos involucrados
en esta asociación.
Palabras clave:
trastorno obsesivo-convul-
sivo, enfermedad de Huntington.
Huntington’s disease (HD) is an
autosomal, dominantly inherited,
neurodegenerative disorder which
manifests during middle adult life
(40´s). This disorder affects 4-8
individuals per 100000 people in
European populations, whereas in
Japan, less than 1 per 100000 in-
dividuals have this disorder (1). The
diagnostic hallmark of HD includes
cognitive de
cits, mood alterations
and motor disturbances (2) such as
chorea and other motor disorders
(dystonia, dysathria, gait disturban-
ces). However, behavioral problems
and neuropsychiatric conditions
are also often present (3,4), and ac-
cumulating evidence suggests that
obsessive compulsive disorder (OCD)
symptoms may precede the clinical
onset of HD or emerge during the
later stages of the disease. Unfortu-
nately the mechanisms underlying
these comorbid disorders and treat-
ments for the dual condition have
not been well-investigated (3).
Thus, the goal of this article is
to review the scienti
c literature
on OCD symptoms in patients with
HD and describe a case study from
a patient presenting to our clinic.
Additionally, we will explore the po-
tential neurobiological mechanisms
underlying this association and dis-
cuss the medications used to treat
these comorbid conditions.
HD Pathophysiology
HD is caused by expanded CAG
repeats in the 5´ region of the hun-
tingtin gene located on chromosome
4p16.3 (5-7). Wild-type chromo-
somes contain between 6-34 CAG
repeats, while HD chromosomes
Molano-Eslava J., Iragorri-Cucalón Á., Ucrós-Rodríguez G., Bonilla-Jácome C., Tovar-Perdomo S.,
et al
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
contain 36-121 repeats (8). Signi-
ficant positive associations have
been described between the repeat
length and various clinical features
of HD, such as age of onset, disease
severity and age of death (9).
Huntingtin is a 3136 amino acid
protein that is extensively expressed
in the mammalian brain, particu-
larly in neuronal cell bodies and
dendrites of large neurons (10,11).
Numerous proteins have been
shown to associate with the N-ter-
minal polyglutamine fragment and
C-terminal HEAT repeats of hun-
tingtin, suggesting that it may act
as a scaffolding protein in multiple
signaling pathways (12,13) inclu-
ding those involved in neurogenesis
and cellular processes necessary to
maintain cellular viability (14-17).
Some researchers hypothesize that
increased polyglutamine fragments
in mutant huntingtin may alter pro-
tein-protein interactions, leading to
selective neuronal dysfunction and
neurodegeneration (11,18). The me-
chanisms underlying neural dege-
neration are unknown (19), however
polyglutamine fragment-induced
toxicity, huntingtin aggregation,
transcription factor alterations, ab-
normal axonal transport, mitochon-
drial dysfunction, and activation of
apoptosis may be involved (20,21).
This neurodegeneration has
been localized to fronto-striatal
systems (22). For example, there is
marked and selective neuronal dea-
th with astrogliosis in the caudate
nucleus, putamen and deep layers
(III, IV, and VI) of the cortex (23).
Of striatal neurons, medium spiny
efferent neurons (GABA-ergic) are
primarily affected in HD (24,25).
Neuropsychiatric and behavioral
Changes in Patients with HD
Recent reports suggest that
behavioral problems and neurop-
sychiatric conditions are often
present in patients with HD (4). For
example, Craufurd et al. (26) de-
monstrated that loss of energy and
initiative, poor perseverance and
quality of work, impaired judgment,
poor self-care and emotional blun-
ting are often present in HD patients.
In addition, depression, apathy and
irritability are some major affecti-
ve symptoms found in those with
HD. In fact, major depression and
intermittent explosive disorder oc-
cur in >30% of these patients (27).
Furthermore, HD patients exhibit
executive dysfunction and progres-
sive cognitive decline, resulting in in-
creased functional impairment after
controlling for motor disturbances,
(28). These cognitive and behavioral
symptoms usually emerge following
changes in cortical architecture (29),
but may also predate the onset of
motor symptoms. The mechanisms
causing early or late non-motor
symptoms remain unknown.
OCD Symptoms in Patients with
In contrast, the relationship
between OCD and HD has been
Obsessive-Compulsive Disorder Symptoms in Huntington’s Disease: A Case Report
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
little-investigated, despite the fact
that both diseases are associated
with striatal dysfunction (30) and
that the number of case reports of
obsessive-compulsive symptoms
either preceding the clinical onset of
HD or during later stages of the di-
sease is increasing (31). For example,
Dewhurst et al. (32) reported “obses-
sional features” in 7 of 102 patients
at onset of HD. Twenty years later,
Tonkonogy and Barrera (33) des-
cribed a patient with obsessive and
compulsive symptoms, namely ideas
of contamination and compulsive
hand washing, associated with affec-
tive disturbances and cognitive decli-
ne characteristic of HD. Additionally,
Cummings and Cunningham (34)
described two unrelated HD patients
with late onset OCD symptoms (i.e.,
compulsions of cleaning). De Marchi
et al. (35) described a pedigree in
which three cases of OCD and two
cases of pathological gambling were
ed prior to clinical onset of
HD, and Scicutella (36) reported a
72-year-old patient who developed
HD and OCD. More recently, Patzold
and Brune (37) described a 42-year-
old woman successfully treated with
sertraline for obsessive thoughts
which emerged 10 years after the
onset of HD. Furthermore, Beglinger
et al. (38) demonstrated that the
probability of obsessive-compulsive
symptoms increased with severity of
HD, such that obsessions and com-
pulsions were three times greater in
patients with motor symptoms than
in patients at risk with no motor
Our group was recently con-
sulted in a case of a 45-year-old
woman who met diagnostic criteria
for HD and OCD (39). The patient
sought medical attention for con-
ciliation insomnia, hyporexia, 4kg
weight loss, emotional liability,
anxiety and obsessive-compulsive
symptoms. Speci
cally, she repor-
ted being constantly worried about
contamination, washing her hands
and teeth repeatedly throughout the
day (20-30 times a day) and spen-
ding great part of her day cleaning
her body, during the past 18 mon-
ths. She also reported trying not
to touch light switches, money or
any surface that could be touched
by someone else. In addition, the
patient reported having dif
concentrating, remembering recent
events and performing mathemati-
cal operations to the point she had
to quit her job a year ago. She also
reported mild involuntary choreoa-
thetoid movements of the upper
extremities and dif
culty walking
during the past 6 months. Her de-
ceased mother was diagnosed with
HD at age 42. Similarly, her sister,
now 58, was diagnosed with HD
during her early forties.
The neurological examination
on admission was remarkable for
choreoathetoid movements of the
right upper limb, head tilting to
the left side, and a wide-based,
unsteady gait. On the mental state
examination, the patient was alert,
oriented in time, place and person;
although hypoprosexic. There was
no evidence of hallucinations or
Molano-Eslava J., Iragorri-Cucalón Á., Ucrós-Rodríguez G., Bonilla-Jácome C., Tovar-Perdomo S.,
et al
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
delusions. She exhibited short-term
memory de
cits; long-term memory
without alterations. Her intelligence
was above average but she had dis-
calculia. She spoke slowly and with
a low tone. Her thought process was
slow too and had recurrent ideas
of cleanliness or fear of becoming
infected or contaminated. Her mood
was dysphoric, with depressive
affect and severe anxiety. She felt
sad when she thought about the
possibility of having HD. The patient
was evaluated by a dermatologist
who diagnosed irritative dermatitis
probably due to her compulsive
CBC count with differential,
glycemia, hepatic and renal func-
tion tests, TSH levels, VDRL and
urinalysis were normal. Magnetic
Resonance was performed which
revealed increased subarachnoid
space surrounding cortical sulci
and gyri, suggesting cortical atro-
phy. PET scan with [18F] Fluo-
rodeoxyglucose (FDG) revealed a
marked, symmetrical and bilateral,
reduction of FDG uptake in the
caudate. Neuropsychological tes-
ting (Barcelona diagnostic tests,
gure y Wisconsin
Card Sorting Test) revealed abnor-
mal attention, short-term memory
cits, discalculia, and de
cits in
task planning and execution, all of
which suggest cognitive de
cits of
subcortical origin compatible with
HD. The patient was diagnosed
with OCD, possible HD and mayor
depression. HD was con
rmed by
genetic testing which revealed 44
CAG repeats compared with 25
repeats of the healthy allele. The
patient was started on olanzapine
2,5mg PO BID and paroxetine 40
mg PO per day. After 4 weeks of
treatment, obsessional thoughts de-
creased signi
cantly and the affect
improved. Motor symptoms remain
the same.
Neurobiological Mechanisms
Underlying the Relationship
between OCD Symptoms and HD
Functional and structural ima-
ging studies have consistently de-
monstrated that HD patients exhibit
frontal and striatal hypometabolism
along with thalamic hypermetabo-
lism (40,41). Together the studies
suggest HD is not exclusively con
ned to the striatum, but also affects
striatum-elated structures, such
as the frontal cortex. Alexander et
al. (42) proposed
ve segregated
circuits between the basal gan-
glia and selected frontal cortical
areas. Speci
cally, neuropsychiatric
symptoms (cognitive, affective and
behavioral) in HD patients have
been attributed to orbitofrontal-,
anterior cingulate- and lateral pre-
frontal-striatal circuit dysfunction
(22), possibly caused by basal gan-
glia and cortical neurodegeneration
or developmental alterations, which
lead to reduced basal ganglia output
to the frontal cortex and further
frontal dysfunction (43-45).
In addition, alterations in neu-
rotransmitter systems may contri-
bute to functional and structural
Obsessive-Compulsive Disorder Symptoms in Huntington’s Disease: A Case Report
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
changes within these circuits. For
example, numerous post-mortem
studies and in vivo studies using
PET scan have demonstrated de-
creased binding for D1 and D2
receptor-ligands in the striatum
and frontal cortex of patients with
clinical HD (46-50). Decreased bin-
ding for opiate receptors (44) and
the benzodiazepine site on GABAA
receptors (51) have also been docu-
mented in the striatum and medial
prefrontal cortex and caudate nu-
cleus, respectively.
In contrast, numerous func-
tional imaging studies showing in-
creased activity in the orbitofrontal
cortex, anterior cingulate, caudate
nucleus and thalamus in patients
with OCD (reviewed by Baxter et al.)
(52). Furthermore, structural chan-
ges of corticolimbic regions of the
frontal lobes (orbitofrontal and me-
dial prefrontal cortex), ventromedial
caudate nucleus and pallidal sites
have been associated with the deve-
lopment of obsessive and compulsi-
ve symptoms in humans (34). These
ndings suggest that dysfunction of
fronto-subcortical circuits plays a
cant role in OCD, similar to
HD. For example, Baxter et al. (52)
suggested that overactivity of the or-
bitofrontal cortex and ventromedial
caudate along with hypoactivity of
lateral prefrontal cortex would tend
to disinhibit the thalamus via its
predominant direct pathway tone,
leading to compulsive behaviors.
Accordingly, recovery from OCD
after treatment with selective sero-
tonin reuptake inhibitors (SSRI) or
tricyclic antidepressants is asso-
ciated with lateralized or bilateral
reductions in orbitofrontal cortex,
anterior cingulate and caudate nu-
cleus activity (53,54).
In addition to striatal degene-
ration, dysfunction of neuropep-
tide systems may be involved in
co-occurrence of HD and OCD. For
example, HD and OCD patients ex-
hibit increased somatostatin levels
and immunoreactivity in selective
CNS regions (55,56). Furthermore,
chronic administration of serotonin
reuptake inhibitors that effectively
treats OCD symptoms decreases
somatostatin contents in selective
brain regions (57). Thus, multiple
mechanisms are likely involved in
the presentation of OCD symptoms
in HD patients.
However, as stated by Patzold
and Brüne (37): “whereas the link of
OCD and HD is intuitively obvious,
the clinical and pathophysiologic
association between the two disor-
ders is still obscure to some extent.”
cally, the neural mechanisms
underlying the development of OCD
in patients with HD remain un-
clear. It is possible that differential
neurodegeneration in the caudate
nucleus, frontal cortex or other
structures within fronto-subcorti-
cal circuits may explain differential
neuropsychiatric symptoms in
these patients. For example, exe-
cutive dysfunction in early stages of
Huntington’s disease is speci
associated with striatal and insular
atrophy (58). Similarly, progression
in neuropsychiatric symptoms of
Molano-Eslava J., Iragorri-Cucalón Á., Ucrós-Rodríguez G., Bonilla-Jácome C., Tovar-Perdomo S.,
et al
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
HD is correlated with differential
dopaminergic dysfunction in HD
patients (59). Based on the above it
is possible to hypothesize that diffe-
rential and progressive neuropatho-
logic changes, likely involving the
ventromedial caudate or associated
prefrontal subregions and modula-
tory neurotransmitter systems, are
responsible for the development of
OCD symptoms in patients with HD.
In fact, progression of HD severity
has been linked to the development
of OCD symptoms in these patients.
In a recent study, Beglinger et al.
(38) demonstrated that the probabi-
lity of HD patients having obsessio-
ns and compulsions increased with
both greater diagnostic certainty
and greater functional impair-
ment. A future post-mortem study
characterizing the progression of
neuropathological changes within
fronto-subcortical circuits would
allow us to test this hypothesis.
Similarly, we lack evidence
about the right treatment for pa-
tients with OCD symptoms and
HD. Previous reports suggest that
patients with HD and behavioral
or affective symptoms bene
t from
SSRI treatment. Ranen et al. (60)
successfully treated two consecu-
tive cases of genetically con
Huntington’s disease in which se-
vere irritability and aggressiveness
required inpatient admission. Patel
et al. (61) administered
an SSRI, and L-deprenyl in a 19-
year-old female with Huntington’s
disease with signi
cant improve-
ment of affective, behavioral, and
motor function. Interestingly, there
is only one published report of a pa-
tient with HD and OCD symptoms
treated with sertraline (37). Olan-
zapine, an atypical antipsychotic,
has been used to control motor
and behavioral disturbances in
patients with single-diagnosis HD
(62). In addition, olanzapine has
been used successfully in patients
with severe OCD alone (63) or with
schizophrenia (64), despite reports
of atypical antipsychotics evoking
OCD-like symptoms (65,66). Undo-
ubtedly, clinical studies are needed
to determine the effectiveness in
the treatment of these co-morbid
OCD symptoms may precede
or coincide with motor, affective or
behavioral symptoms in patients
with HD. Both diseases are inva-
riably associated with dysfunction
of striatal neural circuits such as
prefrontal-striatal circuits. Multiple
mechanisms have been proposed to
account for striatal degeneration
in HD and related circuit dysfunc-
tion. Speci
cally, structural and
functional changes affecting orbi-
tal and medial prefrontal cortex,
ventromedial caudate nucleus and
pallidal sites have been suggested
to play a role in the development
OCD symptoms in HD. Anecdotal
evidence suggest that SSRIs alone
or in combination with atypical
antipsychotics like olanzapine may
be useful for these patients. Howe-
Obsessive-Compulsive Disorder Symptoms in Huntington’s Disease: A Case Report
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
ver, these hypotheses need further
Supported by SEMILLA grant
from Los Andes University (LO-C) and
U.S. National Institute on Drug Abuse
DA023548 (DVH).
Harper PS. The epidemiology of
Huntington’s disease. Hum Genet,
Vonsattel JP, DiFiglia M. Huntington’s
disease. J Neuropathol Exp Neurol.
Cummings JL. Behavioral and
psychiatric symptoms associated
with Huntington’s disease. Adv Neurol.
Paulsen JS, Ready RE, Hamilton JM,
Mega S, Cummings JL. Neuropsychia-
tric aspects of Huntington’s disea-
se. J Neurol Neurosurg Psychiatry.
Gusella JF, Wexler NS, Conneally PM,
Naylor SL, Anderson MA, Tanzi RE, et
al. A polymorphic DNA marker gene-
tically linked to Huntington’s disease.
Nature. 1983;306(5940):234-8.
The Huntington’s Disease Collabo-
rative Research Group. A novel gene
containing a trinucleotide repeat
that is expanded and unstable on
Huntington’s disease chromosomes.
Cell. 1993;72(6):971-83.
MacDonald ME, Novelletto A, Lin C,
Tagle D, Barnes G, Bates G, et al. The
Huntington’s disease candidate region
exhibits many different haplotypes. Nat
Genet. 1992;1(2):99-103.
Read AP. Huntington’s disease: testing
the test. Nat Genet. 1993;4:329-30.
Andrew SE, Goldberg YP, Kremer B,
Telenius H, Theilmann J, Adam S, et al.
The relationship between trinucleotide
(CAG) repeat length and clinical featu-
res of Huntington’s disease. Nat Genet.
10. Strong TV, Tagle DA, Valdés JM, El-
mer LW, Boehm K, Swaroop M, et al.
Widespread expression of the human
and rat Huntington’s disease gene
in brain and nonneural tissues. Nat
Genet. 1993;5(3):259-65.
11. Trottier Y, Devys D, Imbert G, Saudou
F, An I, Lutz Y, et al. Cellular localization
of the Huntington’s disease protein and
discrimination of the normal and mutated
form. Nat Genet. 1995;10(1):104-10.
12. Andrade MA, Bork,P. HEAT repeats in
the Huntington’s disease protein. Nat
Genet. 1995;11(2):115-6.
Kazantsev A, Preisinger E, Dranovsky
A, Goldgaber D, Housman D. Insoluble
detergent-resistant aggregates form
between pathological and nonpatho-
logical lengths of polyglutamine in
mammalian cells. Proc Natl Acad Sci
USA. 1999;96(20):11404-9.
14. Hoogeveen AT, Willemsen R, Meyer
N, de Rooij KE, Roos RA, van Om-
men GJ, et al. Characterization and
localization of the Huntington disea-
se gene product. Hum Mol Genet.
15. Gutekunst CA, Levey AI, Heilman CJ,
Whaley WL, Yi H, Nash NR, et al. Iden-
tification and localization of Huntington
in brain and human lymphoblastoid
cell lines with anti-fusion protein
antibodies. Proc Natl Acad Sci USA.
Duyao MP, Auerbach AB, Ryan A, Per-
sichetti F, Barnes GT, McNeil SM, et al.
Inactivation of the mouse Huntington’s
disease gene homolog Hdh. Science.
Dragatsis I, Levine MS, Zeitlin S. Inac-
tivation of Hdh in the brain and testis
results in progressive neurodegene-
ration and sterility in mice. Nat Genet.
18. Orr HT, Zoghbi HY. Trinucleotide
repeat disorders. Ann Rev Neurosci.
19. Greenamyre JT. Huntington’s disea-
se: getting closer. Am J Psychiatry.
20. Walling HW, Baldassare, JJ, Westfall TC.
Molecular aspects of Huntington’s disea-
se. J Neurosci Res. 1998;54(3):301-8.
Molano-Eslava J., Iragorri-Cucalón Á., Ucrós-Rodríguez G., Bonilla-Jácome C., Tovar-Perdomo S.,
et al
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
21. Sawa A, Tomoda T, Bae BI. Me-
chanisms of neuronal cell death in
Huntington’s disease. Cytogenet Ge-
nome Res. 2003;100(1-4):287-95.
22. Lichter DG. Movement disorders. In: Li-
chter DG, Cummings JL, editors. Fron-
tal-subcortical circuits in psychiatric
and neurological disorders. New York:
Guilford Press; 2001. p. 260-313.
23. Vonsattel JP, Myers RH, Stevens, TJ,
Ferrante RJ, Bird ED, Richardson EP
Jr. Neuropathological classification of
Huntington’s disease. J Neuropathol
Exp Neurol. 1985;44(6):559-77.
24. Ferrante RJ, Kowall NW, Beal MF,
Richardson EP Jr, Bird ED, Martin JB.
Selective sparing of a class of striatal
neurons in Huntington’s disease.
Science. 1985;230(4725):561-3.
25. Albin RL, Reiner A, Anderson KD,
Dure ES 4th, Handelin B, Balfour R,
et al. Preferential loss of striato-ex-
ternal pallidal projection neurons in
presymptomatic Huntington’s disease.
Ann Neurol. 1992;31(4):425-30.
26. Craufurd D, Thompson JC, Snowden
JS. Behavioral changes in Huntington
Disease. Neuropsychiatry Neuropsychol
Behav Neurol. 2001;14(4):219-26.
27. Di Maio L, Squitieri F, Napolitano G,
Campanella G, Trofatter JA, Conneally
PM. Onset symptoms in 510 patients
with Huntington’s disease. J Med Ge-
net. 1993;30(4):289-92.
28. Rothlind JC, Bylsma FW, Peyser C,
Folstein SE, Brandt J. Cognitive and
motor correlates of everyday functio-
ning in early Huntington’s disease. J
Nerv Ment Dis. 1993;181(3):194-9.
29. Nopoulos P, Magnotta VA, Mikos A,
Paulson H, Andreasen NC, Paulsen
JS. Morphology of the cerebral cortex
in preclinical Huntington’s disease. Am
J Psychiatry. 2007;164(9):1428-34.
30. Modell JG, Mountz JM, Curtis GC, Gre-
den JF. Neurophysiologic dysfunction
in basal ganglia/limbic striatal and tha-
lamocortical circuits as a pathogenetic
mechanism of obsessive-compulsive
disorder. J Neuropsychiatry Clin Neu-
rosci. 1989;1(1):27-36.
31. Anderson KE, Louis ED, Stern Y,
Marder KS. Cognitive correlates of ob-
sessive and compulsive symptoms in
Huntington’s disease. Am J Psychiatry.
32. Dewhurst K, Oliver J, Trick KL, McK-
night AL. Neuro-psychiatric aspects of
Huntington’s disease. Confin Neurol.
33. Tonkonogy J, Barreira P. Obsessive-
compulsive disorder and caudate-fron-
tal lesion. Neuropsychiatry Neuropsy-
chol Behav Neurol. 1989;2(3):203-9.
34. Cummings JL, Cunningham K.
Obsessive-compulsive disorder in
Huntington’s disease. Biol Psychiatry,
De Marchi N, Morris M, Mennella R, La
Pia S, Nestadt G. Association of ob-
sessive-compulsive disorder and pa-
thological gambling with Huntington’s
disease in an Italian pedigree: possible
association with Huntington’s disea-
se mutation. Acta Psychiatr Scand.
36. Scicutella A. Late-life obsessive-com-
pulsive disorder and Huntington’s
disease. J Neuropsychiatry Clin Neu-
rosci. 2000;12(2):288-9.
37. Patzold T, Brune M. Obsessive compul-
sive disorder in Huntington disease: a
case of isolated obsessions succes-
sfully treated with sertraline. Neurop-
sychiatry Neuropsychol Behav Neurol.
38. Beglinger LJ, Langbehn DR, Duff K,
Stierman L, Black DW, Nehl C, et al.
Probability of obsessive and compulsi-
ve symptoms in Huntington’s disease.
Biol Psychiatry. 2007;61(3):415-8.
Martin JB, Gusella JF. Huntington’s di-
sease: pathogenesis and management.
N Engl J Med. 1986;315(20):1267-76.
40. Young AB, Penney JB, Starosta-
Rubinstein S, Markel DS, Berent S,
Giordani B, et al. PET scan investiga-
tions of Huntington’s disease: cerebral
metabolic correlates of neurological
features and functional decline. Ann
Neurol. 1986;20:296-303.
41. Berent S, Giordani B, Lehtinen S,
Markel D, Penney JB, Buchtel HA,
et al. Positron emission tomographic
scan investigations of Huntington’s
disease: cerebral metabolic correla-
tes of cognitive function. Ann Neurol.
Obsessive-Compulsive Disorder Symptoms in Huntington’s Disease: A Case Report
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
Alexander GE, DeLong MR, Strick PL.
Parallel organization of functionally
segregated circuits linking basal gan-
glia and cortex. Annu Rev Neurosci.
43. Andrews TC, Brooks DJ. Advances in
the understanding of early Huntington’s
disease using the functional imaging
techniques of PET and SPET. Mol Med
Today. 1998;4(12):532-9.
Weeks RA, Ceballos-Baumann A, Pic-
cini P, Boecker H, Harding AE, Brooks
DJ. Cortical control of movement in
Huntington’s disease: a PET activation
study. Brain. 1997;120(Pt 9):1569-78.
45. Bartenstein P, Weindl A, Spiegel S,
Boecker H, Wenzel R, Ceballos-Bau-
mann A, et al. Central motor proces-
sing in Huntington’s disease. A PET
study. Brain, 1997; 120(Pt 9):1553-
46. Reisine TD, Fields JZ, Stern LZ, Jo-
hnson PC, Bird ED, Yamamura HI.
Alterations in dopaminergic receptors
in Huntington’s disease. Life Sci.
47. Cross A, Rossor M. Dopamine D-1 and
D-2 receptors in Huntington’s disease.
Eur J Pharmacol. 1983;88(2-3):223-9.
48. Sedvall G, Karlsson P, Lundin A,
Anvret M, Suhara T, Halldin C, et al.
Dopamine D1 receptor number-a
sensitive PET marker for early brain
degeneration in Huntington’s disease.
Eur Arch Psychiatry Clin Neurosci.
49. Antonini A, Leenders KL, Spiegel R,
Meier D, Vontobel P, Weigell-Weber
M, et al. Striatal glucose metabolism
and dopamine D2 receptor binding
in asymptomatic gene carriers and
patients with Huntington’s disease.
Brain. 1996;119(Pt 6):2085-95.
Ginovart N, Lundin A, Farde L, Halldin
C, Bäckman L, Swahn CG, et al. PET
study of the pre- and post-synaptic
dopaminergic markers for the neuro-
degenerative process in Huntington’s
disease. Brain. 1997;120(Pt 3):503-
51. Holthoff
VA, Koeppe RA, Frey KA,
Penney JB, Markel DS, Kuhl De, et al.
Positron emission tomography mea-
sures of benzodiazepine receptors
in Huntington’s disease. Ann Neurol.
52. Baxter LR Jr. Functional imaging of
brain systems mediating obsessive-
compulsive disorder. In: Charney
DS, Nestler ES, Bunney BS, editors.
Neurobiology of mental ilness. New
York: Oxford University Press; 1999.
p. 534-45.
53. Baxter LR Jr, Mazziotta JC, Pahl JJ,
Grafton ST, St George-Hyslop P,
Haines JL, et al. Psychiatric, genetic,
and positron emission tomogra-
phic evaluation of persons at risk
for Huntington’s disease. Arch Gen
Psychiatry. 1992;49(2):148-54.
54. Schwartz JM, Stoessel PW, Baxter LR
Jr, Martin KM, Phelps ME. Systematic
changes in cerebral glucose metabolic
rate after successful behavior modifi-
cation treatment of obsessive-com-
pulsive disorder. Arch Gen Psychiatry.
55. Altemus M, Pigott T, L’Heureux F,
Davis CL, Rubinow DR, Murphy DL,
et al. CSF somatostatin in obsessive-
compulsive disorder. Am J Psychiatry.
Mazurek MF, Garside S, Beal MF. Cor-
tical peptide changes in Huntington’s
disease may be independent of
striatal degeneration. Ann Neurol.
Kakigi T, Maeda K, Kaneda H, Chihara
K. Repeated administration of antide-
pressant drugs reduces regional so-
matostatin concentrations in rat brain.
J Affect Disord. 1992; 25(4):215-20.
Peinemann A, Schuller S, Pohl C, Jahn
T, Weindl A, Kassubek J. Executive dys-
function in early stages of Huntington’s
disease is associated with striatal and
insular atrophy: a neuropsychological
and voxel-based morphometric study.
J Neurol Sci. 2005;239(1):11-9.
59. Pavese N, Andrews TC, Brooks DJ,
Ho AK, Rosser AE, Barker RA, et
al. Progressive striatal and cortical
dopamine receptor dysfunction in
Huntington’s disease: a PET study.
Brain. 2003;126(Pt 5):1127-35.
60. Ranen NG, Lipsey JR, Treisman G,
Ross CA. Sertraline in the treatment of
severe aggressiveness in Huntington’s
Molano-Eslava J., Iragorri-Cucalón Á., Ucrós-Rodríguez G., Bonilla-Jácome C., Tovar-Perdomo S.,
et al
Rev. Colomb. Psiquiat., vol. 37
No. 4 / 2008
disease. J Neuropsychiatry Clin Neu-
rosci. 1996;8(3):338-40.
Patel SV, Tariot PN, Asnis J. L-Deprenyl
augmentation of fluoxetine in a patient
with Huntington’s disease. Ann Clin
Psychiatry. 1996;8(1):23-6.
62. Laks J, Rocha M, Capitao C, Domin-
gues RC, Ladeia G, Lima M, et al.
Functional and motor response to low
dose olanzapine in huntington disea-
se: case report. Arq Neuropsiquiatr.
63. Marazziti D, Pallanti S. Effectiveness
of olanzapine treatment for severe
obsessive-compulsive disorder. Am J
Psychiatry. 1999;156(11):1834-5.
64. Poyurovsky M, Kriss V, Weisman G,
Faragian S, Kurs R, Schneidman M, et
al. Comparison of clinical characteris-
tics and comorbidity in schizophrenia
patients with and without obsessive-
compulsive disorder: schizophrenic
and OC symptoms in schizophrenia. J
Clin Psychiatry. 2003;64(11):1300-7.
65. Patel B, Tandon R. Development of ob-
sessive-compulsive symptoms during
clozapine treatment. Am J Psychiatry.
66. Morrison D, Clark D, Goldfarb E, Mc-
Coy L. Worsening of obsessive-com-
pulsive symptoms following treatment
with olanzapine. Am J Psychiatry.
Interest con
icts: None of the authors
reported interest con
icts in this article.
Received for assessment:
October 14, 2008
Accepted for publication:
November 20, 2008
Luis Felipe Orozco-Cabal
Neurociencias-Facultad de Medicina
Universidad de los Andes
Carrera 1ª
Nº 18A-10
Bogotá, Colombia