In 1885, the French neurologist Georges Gilles de la Tourette described9 patients with childhood-onset tics accompanied in some by uncontrollablenoises and utterances, as well as hyperactivity and obsessive-compulsive behavior.1 The current diagnosis of Tourette syndrome, accordingto the Diagnostic and Statistical Manual of Mental Disorders,Fourth Edition (DSM-IV), involves multiplemotor tics and at least 1 vocal tic, which occur many times a day, nearlyevery day, or intermittently for more than 1 year. Tics must begin beforeage 18 years.2 The average age of onsetis 7 years, and boys are more commonly affected than girls. Motor tics arecharacterized by involuntary movements such as facial grimacing, frequenteye blinking, blepharospasm, spitting, and arm jerking. Vocal tics often havean aggressive or sexual component, such as grunting, barking, echolalia, andcoprolalia (uncontrolled swearing). The condition often results in deleterioussocial consequences. We report a case of self-induced bilateral retinal detachmentin a young man with Tourette syndrome who was initially referred for monocularhyphema.
A 25-year-old white man was initially seen with a 1-week history offloaters and decreased vision in his left eye. The patient had been diagnosedwith Tourette syndrome at age 7 years, obsessive-compulsive disorder at age11 years, and depression at age 24 years. His motor tics involved excessiveblinking, blepharospasm, clapping, jabbing his fingers into his eyes, andpunching himself in the periorbital area. The patient was taking buspironehydrochloride (10 mg twice a day) and clomipramine hydrochloride (25 mg twicea day). On examination, the patient was alert and oriented, and he had noevidence of cognitive impairment. Visual acuity was 20/200 OD and hand motionOS. There was no afferent pupillary defect. Intraocular pressures were 18OD and 16 OS. Slitlamp examination findings of the right eye demonstratedpigment deposits on the corneal endothelium, moderate (2+) aqueous pigmentedcells, and posterior subcapsular cataract. The left eye had a less than 1-mmhyphema and many (4+) circulating red blood cells in the anterior chamber,as well as a dense posterior subcapsular cataract. Funduscopy results revealeda retinal dialysis from the 1:30 to the 4:30 clock position with a macula-on-retinaldetachment in the right eye. Vitreous hemorrhage was present centrally inthe left eye, and there were nasal and temporal giant retinal tears. The righteye was repaired with a scleral buckling procedure. The left eye underwentanterior segment washout, pars plana lensectomy, pars plana vitrectomy, endolaser,and silicone oil injection. Intraoperatively, the giant retinal tears werefound to extend from the 12:30 to the 4:30 clock position with 4 long radialextensions to the temporal macula and from the 6-o'clock to the 11-o'clockposition with 1 long radial extension to the optic disc. There was an additionalradially oriented posterior retinal break. Postoperatively, the retinas wereattached in both eyes. One month later, the left eye developed proliferativevitreoretinopathy with retinal detachment and underwent reoperation. At 6months, the retinas remained attached and the visual acuity was 20/100 OU.
Ophthalmic manifestations of Tourette syndrome include frequent blinkingand blepharospasm, gaze deviations and abnormal saccades, and accidental andself-inflicted ocular injuries.3- 5 Theretinal detachments in our patient were most likely the result of repeated,self-induced finger jabbing to the eyes since the patient had no other riskfactors for retinal detachment. In patients with retinal detachment, factorssuggesting a traumatic etiology typically include unilateral vitreoretinalfindings, retinal dialysis or giant retinal tear, and age younger than 40years.6,7 However, in patientswith self-induced or repeated trauma, the vitreoretinal pathologic featuresmay be bilateral, as demonstrated by our patient. To prevent further self-injury,patients should wear protective polycarbonate goggles, and they should bemonitored closely in conjunction with the psychiatry service. Treatment ofthe underlying disorder with behavior modification and pharmacotherapy isessential, and pharmacological agents that antagonize dopamine are most effectivein reducing the severity of motor and vocal tics.
The authors have no relevant financial interest in this article.
Corresponding author: Dean Eliott, MD, Kresge Eye Institute, WayneState University School of Medicine, 4717 St Antoine, Detroit, MI (e-mail: firstname.lastname@example.org).
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The Rational Clinical Examination: Evidence-Based Clinical Diagnosis
Original Article: Acute-Onset Floaters and Flashes: Is This Patient at Risk for Retinal Detachment?
The Rational Clinical Examination: Evidence-Based Clinical Diagnosis
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