Movement Disorders Vol. 13, No. I , 1998, pp. 155-1.57 0 1998 Movement Disorder Society

Brief Report

Geste Device in Tardive Dystonia With Retrocollis and Opisthotonic Posturing

*"yaul Krack, f Sybilla Schneider, and "Gunther Deuschl

Departments of Neurology, *University of Kiel, Kiel; f University of Giessen, Giessen; and $University of Freiburg, Freiburg, Germany

Summary: Retrocollis and opisthotonic posturing are typical clinical features of tardive dystonia and may be extremely dis- abling. We have treated five such patients with a custom-made mechanical device that delivers constant contact to the occiput and shoulders. All patients experienced relief and their walking

improved. This geste device, working like a geste antugonis- tiyue, may be useful for the management of some patients with severe axial dystonia with retrocollis and back arching. Key Words: Tardive dystonia-Retrocollis-Opisthotonic postur- ing-Antagonistic gesture.

Approximately 50% of patients with tardive dystonia include retrocollis,' whereas pure retrocollis is found in only 1% of patients with idiopathic cervical dystonia.' If the trunk is involved, most of the patients (87%) have back-arching opisthotonus. The combination of retrocol- lis and back arching is most often found in tardive dys- tonia.' Severe axial dystonia is generally worse during movement, especially during walking, and these patients tend to lie down on their back, sit in a chair to rest their head and shoulders against its back, or stand with their head and shoulders leaning against a wall. Forster3 has described a chair with a support for both head and trunk, which he used to treat severe generalized athetosis in children, emphasizing the value of mechanical pressure and sensory input. Mechanical control of focal dystonia has also been attempted, but generally with little suc- C ~ S S . ~ The severe disability of patients with back arching and their spontaneous "geste behavior" prompted us to develop a device that delivers a sensory stimulus to the head and shoulders and provides mechanical resistance against involuntary movement. -~ ~~~

A Videotape accompanies this article. Received December 18, 1995; revisions received May 28, 1996, and

June 8, 1997. Accepted June 17, 1997. Address correspondence and reprint requests to Dr. C. Deuschl at

Neurologische Klinik der Christian Albrechts Universitat zu Kid, Ni- emannsweg 147, 24105 Kiel, Germany.

CASE REPORTS

Case 1

This 20-year-old schizophrenic man was treated with 100 mg/month haloperidol decanoate from ages 16 to 19. The treatment with haloperidol was stopped after a tar- dive dystonia with prominent retrocollis and back arch- ing developed. At his worst (not shown on the video- tape), he had severe pain and was almost unable to walk; his head was tipped back so that he was constantly look- ing at the ceiling, so he could hardly leave his bed. Apart from lying down, he found relief when leaning his head and shoulders against the back of a seat or against a wall. After the treatment with haloperidol was stopped, tardive dystonia gradually improved over a 3-year period, his paranoid symptoms being successfully treated with a low dose of clozapine (100 mgldayj. Twice, for psychiatric reasons, the clozapine dosage was increased to 400 mg/ day with concurrent worsening of dystonia. Treatment with 15 mg trihexyphenidyl, 75 mg tetrabenazine, and 6 mg clonazepam was mildly effective. Botulinum toxin (BTX) injections of up to 2000 units (U) of DysportB in his neck muscles were also mildly effective. His walking was moderately improved when he used the geste device shown in the videotape. He still used his geste device after almost complete (spontaneous) remission, when he

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156 P. KRACK ET AL.

complained of some residual stiffness in his neck, with- out visible retrocollis or back arching.

Case 2 This 63-year-old woman developed a blepharospasm

at the age of 50 after treatment with haloperidol for 6 weeks. The dystonia evolved into Meige’s syndrome with an additional retrocollis and finally back arching. There was marked improvement in her neck and lower back pain when she was lying down or when her head and shoulders were supported while sitting or standing. No change was noticed while she was taking daily doses of 4 mg pimozide and 15 mg trihexyphenidyl. Bilateral BTX injections into both splenii and the paravertebral thoracolumbar muscles, with total doses up to 1200 U DysportO, were mildly effective. A geste device with support of her head and shoulders was mildly effective.

Case 3 This 50-year-old man developed involuntary move-

ments of his abdominal muscles after neuroleptic anal- gesia; this condition evolved into truncal torsion after 2 weeks and, after several months, into moderate painful retrocollis and back arching with lower back pain. There was no improvement on 10 mg trihexyphenydil per day. BTX injections (800 U Dysport@ into his neck and 400 U into his lumbar paravertebral muscles) led to mild improvement. With the aid of a geste device supporting his head and shoulders, moderate improvement was noted. The combination of the geste device and BTX injections markedly reduced his symptoms. A gradual improvement was observed over 8 months before spon- taneous remission.

Case 4 After 5 years of fluphenazine decanoate, this 43-year-

old schizophrenic man developed tardive axial dystonia with orolingual and pharyngeal involvement as well as retrocollis and back arching. No change was noted after 1 year without the use of neuroleptics. On treatment with 1.5 mg trihexyphenydil, 1.12.5 mg reserpine, and 50 mg clozapine, his psychosis improved, but the dystonia re- mained unchanged. Treatment with BTX injections of up to 1500 U DysportO into his splenii and his paravertebral neck muscles had no effect, whereas a geste device sup- porting his head and shoulders was moderately effective.

Case 5 After this 13-year-old girl had surgery on a large cra-

niopharyngioma, she showed aggressive tendencies. At age 26, after >2 years on classic neuroleptics (haloperi- do1 and flupenthixol), she developed tardive orofacial and pharyngeal dystonia, retrocollis, and back arching.

BTX injections of up to 1000 U Dysport@ into her sple- nii and her paravertebral cervical muscles was mildly effective. She had a very efficacious antagonistic gesture and, when sitting or standing, was always leaning her head and shoulders against the back of a seat or against a wall. A corset with a dorsal support for her head and her shoulders was moderately effective, although rarely used because it caused her some discomfort when she walked long distances.

Case 6 After a perinatal hypoxia, this boy had delayed motor

development. At age 17, the oligophrenic boy was started on a treatment with the atypical neuroleptic zot- epine to reduce his aggressive behavior. At the age of 26, he developed severe permanent retrocollis and intermit- tent opisthotonic trunk spasms. To look straight ahead, he had to bend his trunk forward to compensate for the retrocollis, which was possible because his opisthotonic spasms were not too frequent. The use of zotepine was stopped. Anticholinergics were of no benefit, and BTX injections were of little benefit. During 6 months, the patient had major benefit from a corset supporting his head and shoulders. Thereafter, his dystonia gradually improved to remission over 3 years.

THE GESTE DEVICE The geste device, which is shown in the videotape, is

made with standard orthopedic materials and was de- signed with standard orthopedic techniques in mind. It consists of a corset or cuirass to which is fixed a head- rest. It is essential that patients have mechanical support for both their shoulders and occiput. For the device to transmit the back-pulling forces to the chest wall without being deformed, its material must be strong and robust.

DISCUSSION The mechanisms underlying antagonistic gestures are

still poorly understood. Such a gesture may be “ridicu- lously weak,”5 that is, disproportionate to its purely me- chanical effect, in the case of true “geste antagoniste” opposing the involuntary movement. In some patients with torticollis, sensory stimulation is sufficient to im- prove dystonia. This is most obvious in the “geste para- doxal” where the fingers slightly touch the chin on the side opposed to the rotation of the head.6 Occasionally, the mere elevation of the arm without touching the head may ~uff ice .~ Thus, sensory information or even the pos- tural pattern of moving the arm may underlie this effect. Voluntary movement in the direction opposite to the dys- tonic movement may be aided when performed against resistance; in this case, the gesture is called a counter-

Movement Disorders, V d . 13, No. 1, 1998

GESTE DEVICE IN DYSTONIC BACK ARCHING 157

pressure phenomenon (Gegendru~kphanomen).~.” Clas- sic antagonistic gestures can be clearly separated from counterpressure because the electromyographic activity is reduced in the first condition and increased in the s c ~ o n d . ~ ~ ’ ~ Whereas the classic antagonistic gesture ap- pears to inhibit dystonic activity, the counterpressure phenomenon may help to overcome dystonic inhibition. Our mechanical device seems to operate on the basis of the physiologic effect of a classic antagonistic gesture.

Truncal dystonia generally is aggravated by postural effort, such as sitting, standing, or walking.” All of our patients were severely disabled when walking, and all found spontaneous relief when leaning their head and shoulders against a wall while standing. With the aid of the geste device, all of the patients experienced func- tional improvement while walking. None experienced pain resulting from mechanical pressure. This argues against a pure mechanical mechanism because it seems improbable that an extreme opisthotonic posturing of a different etiology (for example, tetanus, meningism, or arc de cercle) could be treated with mechanical pressure.

In conclusion, a device delivering pressure at the oc- ciput and the shoulders may, in analogy to an antagonis- tic gesture, functionally improve sitting, standing, and walking for patients who suffer a combination of severe retrocollis and back arching. Classic neuroleptics should be immediately stopped or replaced by atypical neuro- leptics, such as clozapine, if further antipsychotic treat- ment is necessary. Spontaneous remission, however, is rare and may take several years to occur.’ Oral drugs such as anticholinergics, dopamine-depleting drugs, and baclofen as well as BTX injections into the neck muscles may be of benefit. Because none of these treatments is wholly satisfactory, different approaches have to be com-

bined to help the patients with these rare but disabling and long-lasting symptoms.

Legends to the Videotape Segment 1. This is patient 6, who has a severe per-

manent retrocollis. His intermittent back arching is not shown. To look straight ahead, the patient has to bend hi< trunk forward.

Segment 2. This patient is wearing a corset that pro- vides support to the back of his head and shoulders. He can now stand straight, which makes his walking much easier and enables him to use his arms.

REFERENCES 1 . Kang UJ, Burke RE, Fahn S. Natural history and treatment of

tardive dystonia. Mov Disord 1986;l: 193-208. 2. Rondot P, Marchand MP, Dellatolas G. Spasmodic torticollis: re-

view of 220,patients. Can J Neurol Sci 1991;18:143-151. 3. Forster 0. Ubungsbehandlung bei Nervenerkrankungen mit oder

ohne vorausgegangene Operationen. Z Phys Diat Ther 1913;17: 321-333, 403415.

4. Eldridge R. The torsion dystonias: literature review and genetic and clinical studies. Neurology 1970;2O(suppl): 1-78.

5. Brissaud E. LeFons sur les maladies nerveuses. Paris: Masson, 1895.

6. Rondot P, Bathien N, Zitgler M. Les mouvements anotmaux. Paris: Masson, 1988.

7. Deuschl C , Heinen F, Kleedorfer B, Wagner M, Lucking CH, Poewe W. Clinical and polymyographic investigation of spas- modic torticollis. J Neurol 1992;239:9-15.

8. Forster 0. Zur Analyse und Pathophysiologie der sui2ren Bewe- gungsstorungen. Z Ges Neurol Psychiatr 1921 ;13: 1-169.

9. Wartenberg R. Zur Klinik und Pathophysiologie der extrapyrami- dalen Bewegungsstorungen. Z Ges Neurol Psychiatr 1923;83:303- 354.

10. Podivinsky F. Torticollis. In: Vinken PJ, Bruyn GW, eds. Diseases of the basal ganglia. Amsterdam: Elsevier, 1969567-603 (Hand- book of neurology, vol 6).

11. Yanagisawa N, Goto A. Dystonia musculomm deformans: analysis with electromyography. J Neurol Sci 1971;13:3945.

Movement Disorders, Vol. 13, No. I , 1998