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CASE REPORTS
Year : 2020  |  Volume : 3  |  Issue : 2  |  Page : 115-117

Ocular mutilation associated with sensory phenomena in Tourette’s syndrome


Department of Psychiatry, OCD Clinic, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India

Date of Submission16-Mar-2020
Date of Decision28-Apr-2020
Date of Acceptance08-Jun-2020
Date of Web Publication28-Jul-2020

Correspondence Address:
Dr. Abel Thamby
Department of Psychiatry, OCD Clinic, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AOMD.AOMD_12_20

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  Abstract 

Self-injurious behaviors (SIBs) have been known to occur with Tourette’s syndrome (TS). We present two cases with ocular self-mutilation associated with TS. The first case had significant impulsivity and emotional dysregulation and was found to be resistant to all forms of treatment. The second case had an onset in adulthood with good response to pharmacotherapy. The SIB in both cases was associated with the underlying sensory phenomenon (SP) which may have treatment implications.

Keywords: Ocular mutilation, Tourette’s syndrome, self-injurious behavior, sensory phenomenon


How to cite this article:
Ray S, Thamby A, Kishore C, Pathak H, Arumugham SS, Narayanaswamy JC. Ocular mutilation associated with sensory phenomena in Tourette’s syndrome. Ann Mov Disord 2020;3:115-7

How to cite this URL:
Ray S, Thamby A, Kishore C, Pathak H, Arumugham SS, Narayanaswamy JC. Ocular mutilation associated with sensory phenomena in Tourette’s syndrome. Ann Mov Disord [serial online] 2020 [cited 2020 Oct 24];3:115-7. Available from: https://www.aomd.in/text.asp?2020/3/2/115/291075




  Introduction Top


Self-injurious behaviors (SIBs) are acts of deliberate self-harm without any intent to die. The most common function served by SIBs seems to be effective regulation of emotions with high arousal such as anger, tension, and anxiety.[1] Sensory phenomenon (SP), commonly described in tics and early-onset obsessive-compulsive disorder (OCD), is seldom described as psychopathology underlying SIBs. We describe two cases of Tourette’s syndrome (TS) one with comorbid OCD, where SP was related to grievous ocular mutilation.

Case report 1

A 26-year-old gentleman was referred for the treatment of TS associated with ocular self-mutilation. He had impulsivity, hyperactivity, and inattention in early childhood. By the age of 10, he developed motor tics in the form of shoulder jerks, blinking of eyes, and jumping movements. In the next few years, he developed vocal tics along with obsessive-compulsive symptoms. He also developed significant emotional dysregulation, low frustration tolerance, impulsivity, increased interpersonal sensitivity, and transient stress-related dissociative symptoms by adolescence. He was diagnosed with TS, OCD, and borderline personality disorder (as per the Structured Clinical Interview-II for DSM-IV).

At the age of 19, he developed strong urges to touch his eyeballs and put pressure on them with his fingers until it felt “just right.” He perceived these acts to be deliberate and under his control unlike traditionally defined motor tics, which are rapid and often perceived as involuntary. Despite causing severe pain and trauma, he was overpowered by strong urges to continue these acts till he felt “satisfied,” which resulted in a sensation of “relief” and “satisfaction.” He was evaluated with University of Sao Paulo Sensory Phenomenon (USP-SP) scale in which the patient endorsed most of the SP with the severity of 15/15.

Over the years, he underwent multiple surgeries for traumatic cataract and retinal detachment. There was progressive deterioration in his eyesight, with only perception of light persisting in his better eye at present.

He had been treated with adequate trials of serotonin reuptake inhibitors (fluoxetine, escitalopram, sertraline, fluvoxamine, clomipramine, and desvenlafaxine), antipsychotics (risperidone, haloperidol, fluphenazine, ziprasidone, olanzapine, chlorpromazine, quetiapine, and clozapine), mood stabilizers (lithium, divalproate, and carbamazepine), and clonidine. Most of these treatments had been combined with habit reversal techniques, cognitive behavior therapy, and repetitive transcranial magnetic stimulation without any significant benefit.

Due to treatment resistance and severe unremitting illness, deep brain stimulation/ablative neurosurgery was considered. However, as the severe personality disorder might render him noncompliant to surgical precautions/postoperative follow-up, he was not considered fit at this point for surgical treatment.

Case report 2

A 27-year-old gentleman presented with a 2-year history of vocal and motor tics. His motor tics manifested as repeated biting of tongue and scratching of eyes following an intense urge. The urge would peak but transiently reduce immediately after performing the motor acts. Even though it resulted in painful corneal abrasion and opacification, he could not overcome the urge. He also had four episodes of generalized tonic-clonic seizures, 7 months before the current hospital visit, for which he was on tablet levetiracetam. He was treated previously with amitriptyline, citalopram, fluvoxamine, clomipramine, fluoxetine, oxcarbazepine, and eslicarbazepine without significant improvement. He was diagnosed with TS and Yale Global Tic Severity Score (YGTSS) was 71/100 at the time of hospitalization. Magnetic resonance imaging (MRI) of his brain was found to be normal.

He was initiated on tablet risperidone gradually increased up to 6 mg per day. The YGTSS score was 42/100 at the time of discharge after 6 weeks of treatment.


  Discussion Top


TS is a neurodevelopmental disorder that is characterized by presence of chronic vocal and motor tics with onset before the age of 18 years.[2] However, onset of tics after the age of 18 years has been reported in literature which was the case with our second patient.[3]

The prevalence of SIB and severe SIB in patients with TS was found to be 30%–60% and 4%, respectively.[4],[5],[6] SIB reported in TS range from skin picking, self-hitting, dental extraction, lip biting, and tongue biting to poking of eyes.[7],[8],[9] It also have been reported to result in grievous ophthalmological complications such as retinal detachment, orbital hemorrhage, subluxation of lens, and even severe vision loss, which was observed in our patients.[7],[8] Comorbidities in TS may play a crucial role in determining SIB and its severity, especially obsessive-compulsive symptoms, attention-deficit/hyperactivity disorder (ADHD), and hostility.[6],[7] For example, it has been seen that mild–moderate severity of SIB correlates with obsessive-compulsive symptoms, whereas severe SIB is associated with impulsivity and affective dysregulation.[4]

Although the above patients reported these behaviors to be deliberate and under their control, they were preceded by strong urges, with a sense of relief following the acts. The psychopathology behind such SIB has been variously conceptualized as complex tics, compulsions, and stereotypies.[4],[7] The role of SP leading to SIB is also an important phenomenological consideration. In both the cases described above, there was a host of SP especially urges and “just right” phenomenon. As SP is found in both tics and compulsions, it is often clinically difficult to differentiate whether the repetitive behavior is a tic or a compulsion. Although neither OCD-specific treatments nor tic-specific treatments showed a significant change in symptomology in the former patient, the later patient improved after treatment with risperidone and not selective serotonin-reuptake inhibitor (SSRI), suggestive of a tic-related psychopathology.

Dysregulation of the endorphin system may be related to SIB in TS, with the repeated release of endorphins following SIB reinforcing the behavior.[4] Such a dysregulation of opioid system might alter skin temperature or nociceptive sensation, which might accentuate sensory phenomena.[4] In addition, opioid system indirectly modulates the dopaminergic system, which is strongly implicated in the pathophysiology of TS.[10]

This case series brings to attention the important issue of severe SIBs in the context of SP. Although SP is seen in the majority of patients with TS,[10] SIB is seen only in a subset of these subjects. As both phenomena are known to occur at the same location as tics, there is a need to study the interplay of these complex phenomena in TS.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Klonsky ED The functions of deliberate self-injury: A review of the evidence. Clin Psychol Rev2007;27:226-39.  Back to cited text no. 1
    
2.
Battle DE Diagnostic and statistical manual of mental disorders (DSM). Codas 2013;25:191-2.  Back to cited text no. 2
    
3.
Jankovic J, Gelineau-Kattner R, Davidson A Tourette’s syndrome in adults. Mov Disord 2010;25:2171-5.  Back to cited text no. 3
    
4.
Mathews CA, Waller J, Glidden D, Lowe TL, Herrera LD, Budman CL, et al. Self injurious behaviour in Tourette syndrome: Correlates with impulsivity and impulse control. J Neurol Neurosurg Psychiatry 2004;75:1149-55.  Back to cited text no. 4
    
5.
Cath DC, Spinhoven P, Hoogduin CA, Landman AD, van Woerkom TC, van de Wetering BJ, et al. Repetitive behaviors in Tourette’s syndrome and OCD with and without tics: What are the differences? Psychiatry Res 2001;101:171-85.  Back to cited text no. 5
    
6.
Freeman RD, Fast DK, Burd L, Kerbeshian J, Robertson MM, Sandor P An international perspective on Tourette syndrome: Selected findings from 3,500 individuals in 22 countries. Dev Med Child Neurol 2000;42:436-47.  Back to cited text no. 6
    
7.
Robertson MM, Trimble MR, Lees AJ Self-injurious behaviour and the Gilles De La Tourette syndrome: A clinical study and review of the literature. Psychol Med 1989;19: 611-25.  Back to cited text no. 7
    
8.
Lim S, Rezai KA, Abrams GW, Eliott D Self-induced, bilateral retinal detachment in Tourette syndrome. Arch Ophthalmol 2004;122:930-1.  Back to cited text no. 8
    
9.
Fornaro M, Maremmani AG, Colicchio MG, Romano A, Fornaro S, Rizzato S, et al. A case of severe oral self-injurious Tourette’s syndrome alleviated by Pregabalin. Gen Hosp Psychiatry 2012;34:321.e1-4.  Back to cited text no. 9
    
10.
Conceição M, Gabriela A, Ferrão Y Sensory phenomena in obsessive ‐ compulsive related disorders. In: Jonathan S. Abramowitz, Dean Mckay EAS, editors. The Wiley Handbook of Obsessive Compulsive Disorders. Chichester, UK: John Wiley & Sons; 2017. pp. 1119-32.  Back to cited text no. 10
    




 

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