hypersexual disorder - increased of sexual arousal - hypersexuality - movement disorders
transtorno hipersexual - exacerbação do impulso sexual - hipersexualidade - distúrbios
de movimento
Several neurological disorders, such as epilepsy, stroke, multiple sclerosis, dementia,
and Parkinson`s disease (PD), are commonly associated with sexual dysfunction[1]. The DSM-5 diagnostic criteria recognize different forms of sexual dysfunction,
including disorders related to sexual desire, arousal, and orgasm[2]. Paraphilias represent a separate group of disorders, including exhibitionism, fetichism,
frotteurim, and pedophilia, among others[2]. Hypersexual disorder (HSD) is proposed as a new psychiatric disorder, defined primarily
as a nonparaphilic sexual desire disorder with an impulsivity component[3].
The purpose of this study is to present a case series of increased sexual arousal
(ISA) in a group of patients with movement disorders (MD).
METHOD
Fifteen patients with different MD were prospectively evaluated over a 5-year period
in the Movement Disorders Unit of Hospital de Clínicas, Federal University of Paraná.
Patients were regularly questioned at each visit for symptoms related to sexual disorders,
and those who agreed to participate in the study signed the consent form. The study
was approved by the Ethics Committee of the Federal University of Paraná.
The MD were classified as either PD or hyperkinetic disorders, including Huntington´s
disease (HD), Tourette`s syndrome (TS), and spinocerebellar ataxia type 3 (SCA3).
Diagnostic criteria for PD were based on the Queen Square Brain Bank. Huntington’s
disease and SCA3 patients had genetic diagnostic confirmation. Tourette’s syndrome
diagnosis was made according to DSM-5[2] criteria. Hypersexual disorder and paraphylias were diagnosed according to Kafka[3] and DSM-5 diagnostic criteria, respectively.
A total of 15 cases of ISA were identified, 13 of which were males. Mean age of patients
in the group with PD (7 patients) was 66.7 years, and 39.6 years in the group of hyperkinetic
disorders (4 patients with SCA3, 2 patients with HD, and 2 patients with TS).
RESULTS
We identified seven PD patients (six males) with different forms of HSD (excessive
masturbation, obsessive anal intercourse preference, excessive sexual intercourse)
and paraphylias (exhibitionism, and pedophilia). Dysfunctions resulted from dopaminergic
agonists (DA) use (high doses of pramipexole in four of seven patients), levodopa
abuse (dopamine dysregulation syndrome in one case), and after deep brain stimulation
(DBS) (bilateral subthalamic nucleous in two cases).
In the group of hyperkinetic disorders, there were two patients with HD (one female
patient with excessive sexual intercourse associated with mood disorder that started
before the onset of motor symptoms, and one male patient, with excessive sexual intercourse,
developed during follow-up). The first patient was treated with clonazepam 1mg/day
with improvement and the second with olanzapine 10mg/day, with partial improvement.
Two patients with TS developed severe HSD [one with excessive masturbation and one
with sexual promiscuity, who later developed acquired immunodeficiency syndrome (AIDS)].
Patients received flufenazine 1.5mg/day plus clonazepam 0.5mg/day, and haloperidol
7.5mg/day, respectively. Four patients, from two families with SCA3, presented with
HSD (excessive sexual intercourse) after beginning of gait ataxia. The patients presented
with cerebellar phenotype of SCA3 and were not taking any dopaminergic medication.
One patient was treated with buspirone 15mg/day, with improvement of symptoms, and
the other one, which also showed improvement of the disorder, conducted monitoring
in a psychotherapy service during an extended period. Clinical data of these patients
are described in [table 1].
Table 1
Increased sexual arousal in a group of movement disorders patients.
Patient
|
Age/Gender
|
Disease
|
Scales
|
Treatment
|
ISA
|
Follow-up
|
1
|
67/M
|
PD
|
HY = II
|
DA
|
Annal intercourse
|
Improvement
|
2
|
57/M
|
PD
|
HY = II
|
Levodopa (high doses)
|
Excessive sexual intercourse
|
Partial improvement
|
3
|
58/M
|
PD
|
HY = II
|
DA
|
Excessive masturbation
|
Improvement
|
4
|
65/M
|
PD
|
HY = III
|
DBS
|
Excessive sexual intercourse
|
Improvement
|
5
|
64/M
|
PD
|
HY = III
|
DBS
|
Excessive sexual intercourse
|
NR
|
6
|
81/F
|
PD
|
HY = III
|
DA
|
Exibitionism
|
Improvement
|
7
|
75/M
|
PD
|
HY = III
|
DA
|
Pedophilia
|
Improvement
|
8
|
58/F
|
HD
|
YGTSS (intensity subscale) = 3
|
Clonazepam
|
Excessive sexual intercourse
|
Improvement
|
9
|
55/M
|
HD
|
YGTSS (intensity subscale) = 4
|
Olanzapine
|
Excessive sexual intercourse
|
Partial improvement
|
10
|
38/M
|
TS
|
UHDRS (motor) = 20
|
Flufenazine, Clonazepam
|
Sexual promiscuity
|
AIDS
|
11
|
22/M
|
TS
|
UHDRS (motor) = 15
|
Haloperidol
|
Excessive masturbation
|
Sudden death
|
12
|
35/M
|
SCA3
|
SARA = 11
|
Buspirone
|
Excessive sexual intercourse
|
Improvement
|
13
|
34/M
|
SCA3
|
SARA = 7
|
-
|
Excessive sexual intercourse
|
NR
|
14
|
36/M
|
SCA3
|
SARA = 13
|
-
|
Excessive sexual intercourse
|
NR
|
15
|
39/M
|
SCA3
|
SARA = 7
|
-
|
Excessive sexual intercourse
|
Improvement
|
M: Male; F: Female; PD: Parkinson´s Disease; HD: Huntington´s disease; TS: Tourette´s
syndrome; SCA3: Spinocerebellar Ataxia type 3; HY: Hoehn&Yahr Scale; YGTSS: Yale Global
Tourete´s Syndrome Scale; UHDRS: Unified Huntington´s Disease Rating Scale; SARA:
Scale for the Assessment and Rating of Ataxia; DA: Dopaminergic agonist; DBS: Deep
Brain Stimulation; ISA: Increased Sexual Arousal; NR: not reported; AIDS: Acquired
Immune Deficiency Syndrome.
In general, most patients improved of HSD and paraphylias after reduction of dopaminergic
drugs doses (DA and levodopa), DBS parameters correction and use of typical and atypical
neuroleptics ([Table 2]).
Table 2
Dopaminergic adjustment in Parkinson’s disease patients with increased sexual arousal.
Patient
|
Initial treatment
|
Initial dose
|
Final dose
|
Follow-up
|
1
|
DA
|
Pramipexole 4mg/day
|
Levodopa 800mg/day + Entacapone 800mg/day
|
Improvement
|
2
|
Levodopa
|
Levodopa 1800mg/day
|
Levodopa 800mg/day
|
Partial improvement
|
3
|
DA
|
Pramipexole 4,5mg/day
|
Levodopa 800mg/day + Entacapone 800mg/day
|
Improvement
|
4
|
DBS
|
-
|
-
|
Improvement
|
5
|
DBS
|
-
|
-
|
NR
|
6
|
DA
|
Pramipexole 4mg/day
|
Levodopa 800mg/day + Entacapone 800mg/day
|
Improvement
|
7
|
DA
|
Pramipexole 5mg/day
|
Levodopa 800mg/day + Entacapone 800mg/day
|
Improvement
|
DA: Dopaminergic agonist; DBS: Deep Brain Stimulation; NR: not reported.
DISCUSSION
In patients with PD, non-motor symptoms including depression, anxiety, apathy, sleep
disorders, loss of libido, and erectile dysfunction are common. Sexual dysfunction
in PD contributes to poor quality of life[4]. Hypersexual disorders occur in patients with PD due to impulse control disorders
(ICD), a complication of dopaminergic therapy, particularly when DA and levodopa are
used. Therefore, an excessive stimulation of dopamine receptors may be the cause of
HSD in PD[4]. Rarely, HSD can be related to DBS in the subthalamic nucleus. In our case series
of patients with PD and ISA we observed these complications associated with DA use,
levodopa (typically in high doses), and DBS in the subthalamic nucleous. The most
common HSD were excessive sexual intercourse, excessive masturbation, and the unusual
preference for anal intercourse[5]. Two kinds of paraphylias were observed: exhibitionism and pedophilia. In general,
after dose reduction of dopaminergic drugs and DBS parameters changes, most patients
improved HSD and paraphylias.
On the other hand, in the group of patients with hyperkinetic disorders and ISA, the
most common abnormalities were related to the disease or a probable psychological
factor. Two patients with HD presented excessive sexual intercourse, in one of them,
previous to the development of the motor symptoms. In fact, symptoms of HSD were described
by Professor Américo Negrette, a Venezuelan neurologist, in high frequency, in his
seminal clinical contribution about HD[6]. Additionally, other publications about sexuality in HD, have demonstrated hypoactive
sexual disorders[7]. In conclusion, psychosocial factors or specific brain lesion could be the cause
of sexual dysfunction in patients with HD[8].
Two patients with TS presented with HSD, with excessive masturbation and sexual promiscuity.
In addition to the symptoms of motor and vocal tics, patients with TS also have comorbid
conditions, such as attention deficit hyperactivity disorder, obsessive-compulsive
disorder, impulse control disorder, and behavior disorder[9].
Four patients with SCA3 presented with HSD. This condition was described preferentially
after the confirmation of the disease by genetic tests. The main non-motor manifestations
of SCA3 are sleep disorders, cognitive and affective disturbances, psychiatric symptoms,
olfactory dysfunction, pain, cramps and fatigue, among others[10]. In general, there are no descriptions of sexual dysfunctions in the Brazilian population.
One possibility to explain HSD in patients with SCA3 is psychological factors, in
a Latin-American context. On the other hand, this disorder could also be explained
by a fronto-striatal dopaminergic dysfunction. A recent study with [123I]-FP-CIT SPECT showed asymmetrical involvement in striatum, greater in the parkinsonian
rather than the cerebellar SCA3 phenotype[11]. Braga-Neto et al.[12] confirmed significant DAT density reduction at the striatum level among SCA3 patients
compared to controls. However, no correlation was found between DAT densities with
psychiatric assessment scales or neuropsychological tests[12].
In conclusion, ISA in this group of Brazilian patients with MD had different etiologies,
predominantly related to the dopaminergic treatment or DBS procedure in the PD patients.
In the group of HD and TS, it was probably part of the background clinical picture.
In SCA3 patients, ISA was probably associated with cultural aspects.