chorea as a presenting feature of variant creutzfeldt-jakob disease

Clinical/Scientific Notes

Coping Strategies for Visual Hallucinations inParkinson’s Disease

Nico J. Diederich, MD,1,2* Vannina Pieri, PhD,1

and Christopher G. Goetz, MD2

1Department of Neuroscience, Centre Hospitalier deLuxembourg, Luxembourg

2Department of Neurological Sciences, Rush-Presbyterian-St.Luke’s Medical Center, Chicago, Illinois, USA

Abstract: We assessed the use of coping strategies in Parkin-son’s disease patients with visual hallucinations, using a semi-structured questionnaire. We found that 36 of our 46 Parkin-son’s disease subjects with hallucinations (78%) used copingstrategies: cognitive techniques in 69%; interactive techniquesin 62%; and visual techniques in 33%. © 2003 MovementDisorder Society

Key words: coping; Parkinson’s disease; hallucination

In the course of Parkinson’s disease (PD), visual hallucinations(VH) are persistent and progressive.1 These intrusions mayheighten anxiety levels, disrupt daily life activities, and dimin-ish self-esteem, thereby seriously compromising both the pa-tient’s and caregiver’s quality of life. There is controversy onhow best to treat hallucinations. To date, pharmacologicalinterventions have been the only therapies tested with evi-dence-based methods. The prevalence and impact of patient-driven coping strategies have never been assessed. Such strat-egies are used frequently and efficiently by schizophrenicpatients to abate auditory hallucinations.2–4 We, therefore,evaluated whether such coping strategies are also used by PDpatients and, if so, which type of coping strategy predominates.

Patients and Methods

Eighty-eight patients with idiopathic Parkinson’s disease(PD), defined according to the criteria of Litvan and col-leagues,5 without other neurological disease and a visual acuityof at least 0.6 Snellen fraction in the best-performing eye, gavetheir informed consent to participate in a semi-structured inter-view to determine the presence of visual hallucinations. Ofthese, 46 reported visual hallucinations, defined as “seeingsomething before your eyes that does not exist” and occurringat least once during wakefulness for the last 2 months. The

phenomenology and the conditions of appearance of thesehallucinations have been previously described.6,7 All the pa-tients agreed to further questions on coping strategies. For thatpurpose, we designed a 9-point questionnaire, based on previ-ous open-interview answers by other hallucinating patients.Following the current classification in action-oriented and in-trapsychic coping strategies,8 we stratified potentially usefulcoping strategies for reduction of the frequency and severity ofvisual hallucinations into three categories (three questionseach): 1) visual techniques, including better focusing on thehallucinatory object, focusing at another object, or lookingaway from the hallucination; 2) cognitive techniques, related tothe patient’s self-initiated reactions that do not involve otherpeople, specifically turning the lights on, consciously notingthat the hallucinations are not real or purposefully reassuringoneself that they will resolve shortly; and 3) interactive tech-niques, relying on discussions with family and other caregiversto gain comfort and reassurance as well as verification of thenon reality of the hallucinations. Patients were assessed byMini-Mental Status Examination (MMSE) and Unified Parkin-son’s Disease Rating Scale (UPDRS) with subscores on motorand mental disabilities as well as Activities of Daily Living(ADL). We asked if hallucinations were bothersome, and ifthey specifically caused depressive feelings. We used descrip-tive statistics as well as the t test or �2 test, when appropriate,to compare subjects who used or did not use coping strategiesand to compare demented versus nondemented groups. Demen-tia was defined as a score under 26 on the MMSE.

Results

The hallucinating PD patients were 20 women and 26 men,with a mean age of 68 � 7 years; a mean PD duration of 9.5 �4.0 years; a mean Hoehn and Yahr stage of 3.0 � 2.3; a meanUPDRS mental score of 5.0 � 1.9; a mean UPDRS motor scoreof 22 � 10; a mean ADL score of 16 � 7; and a mean MMSEscore of 26 � 3. These demographic features were not statis-tically different from the excluded group of 42 patients withouthallucinations. Nine patients were demented (MMSE scorebetween 18 and 23). Seventy-eight percent of the patients usedat least one coping strategy. Cognitive techniques were themost frequently used (69%), followed by interactive techniques(62%), and visual techniques (33%). Among the 9 dementedpatients, despite their cognitive impairment, the most fre-quently used coping strategy was again cognitive (45%), fol-lowed by interactive and visual (both 33%). These percentageswere not statistically different from those obtained in non-demented patients (t test, P � 0.05). There was no differencebetween patients who used or did not use coping strategies interms of age, duration of the disease, mental, motor or ADLsubscores of the UPDRS (t test, P � 0.05). Subjects withoutcoping strategies reported visual hallucinations more frequentlyas bothersome or depressing compared with subjects with cop-ing strategies (60 vs. 39%), but this difference was not statis-tically different (�2 test, P � 0.05).

*Correspondence to: Nico J. Diederich, MD, Department of Neuro-science, Centre Hospitalier de Luxembourg, 5, Rue Barble, L-1210-Luxembourg. E-mail: [email protected]

Received 14 May 2002; Revised 4 December 2002; Accepted 24December 2002

Movement DisordersVol. 18, No. 7, 2003, pp. 831–838© 2003 Movement Disorder Society

831

Discussion

Coping strategies have only occasionally been explored inrelation to parkinsonian motor disability9–12 and never beforein relation to hallucinations. Based on the efficiency of copingstrategies in schizophrenic patients with auditory hallucina-tions,2–4 we focused on coping strategies used by PD patientswith chronic visual hallucinations. Our data show that withoutany instructions given by the treating neurologists, patientsfrequently use self-driven coping strategies for dealing withthese aberrant perceptions. Although the hallucinations werevisual, the patients relied more predictably on cognitive andinteractive coping strategies than on visual ones. This finding issomewhat in contrast to schizophrenic patients, in whom themost successful cluster of coping strategies consists of com-peting auditory stimulation, for example, by the use of ear-plugs, by singing or reading aloud. Possibly because of theirthought disorder, cognitive responses like concentrating onsomething else and interactive responses like talking to some-one else were less successful among schizophrenic patients.2Cognitive coping strategies predominated even among de-mented PD subjects. In this exploratory study, we restricted ourquestions to three types of coping strategies, but having estab-lished that patients utilize strategies to abate hallucinations, weare interested in other methods as well. Thus, an open-endedinterview technique would possibly identify other ways that PDpatients use to abate their hallucinations. We also restricted ourstudy sample to patients who recognized that they had hallu-cinated and voluntarily affirmed their hallucinations whenquestioned. It is possible that subjects without insight or thosewhose hallucinations are part of a delusional state, would usecoping strategies less often. A larger study that covers thegamut of hallucination-related disorders would be needed toanswer this question.

Although we did not assess patient’s perception of the suc-cess of these coping strategies, those who did not use themfound the hallucinations more frequently to be emotionallytroublesome. The small sample size precludes firm conclusionsin this issue, but prompts further research. This observationsuggests that utilization of a coping strategy may be actuallytherapeutic and reassuring to patients. Because there are stan-dardized rating scales for visual hallucinations in PD, a clinicaltrial of coping strategies as a treatment of visual hallucinationscould be feasibly conducted and even compared against cur-rently advocated pharmacological interventions.

We did not examine the interaction of depression and hallu-cinations, but the fact that hallucinations provoked depressivefeelings in 23 of 46 patients at least suggests that hallucinationsmay enhance affective distress. Other studies have reporteddepression as a risk factor for hallucinations,13,14 but the topicof hallucinations as a risk factor for depression has not been aresearch focus. The results of our limited study prompt thedesign of a larger and more comprehensive examination ofthese interactions.

Acknowledgments: This study was supported by the FondationThink (Luxembourg) and the United Parkinson Disease Foundation(USA).

References

1. Goetz CG, Leurgans S, Pappert EJ, Raman R, Stemer AB. Pro-spective longitudinal assessment of hallucinations in Parkinson’sdisease. Neurol 2001;57:2078–2082.

2. Carter DM, Mackinnon A, Copolov DL. Patients’ strategies forcoping with auditory hallucinations. J Nerv Ment Dis 1996;184:159–164.

3. Haddock G, Slade PD, Bentall RP, Reid D, Faragher EB. Acomparison of the long-term effectiveness of distraction and fo-cusing in the treatment of auditory hallucinations. Br J Med Psy-chol 1998;71:339–349.

4. Wiersma D, Jenner JA, van de Willige G, Spakman M, NienhuisFJ. Cognitive behaviour therapy with coping training for persistentauditory hallucinations in schizophrenia: a naturalistic follow-upstudy of the durability of effects. Acta Psychiatr Scand 2001;103:393–399.

5. Litvan I, MacIntyre A, Goetz CG, Wenning GK, Jellinger K,Verny M, Bartko JJ, Jankovic J, McKee A, Brandel JP, ChaudhuriKR, Lai EC, D’Olhaberriague L, Pearce RK, Agid Y. Accuracy ofthe clinical diagnoses of Lewy-Body disease, Parkinson’s diseaseand dementia with Lewy-Bodies. Arch Neurol 1998;55:969–978.

6. Diederich NJ, Pieri V, Goetz CG. Die visuellen Halluzinationendes Parkinson-Patienten und das Charles Bonnet-Syndrom. Einephanomenologische und pathogenetische Gegenuberstellung.Fortschr Neurol Psychiat 2000;68:129–136.

7. Diederich NJ, Pieri V, Pappert E, Goetz CG. Are there similaritiesbetween the visual hallucinations in Parkinson’s disease and theCharles Bonnet-syndrome? Parkinson Relat Disord 1999;5(Suppl.2):50.

8. Lazarus RS, Launier R. Stress related transactions between personand environment. In: Pearvin LA, Lewis M, editors. Perspectivesin international psychology. New York: Plenum Press; 1978. p287–327.

9. Frazier LD. Coping with disease-related stressors in Parkinson’sdisease. Gerontologist 2000;40:53–62.

10. Haltenhof H, Krakow K, Zofel P, Ulm G, Buhler KE. Krankheits-verarbeitung bei Morbus Parkinson [Coping behaviors in Parkin-son’s disease]. Nervenarzt 2000;71:275–281.

11. Herrmann M, Curio N, Petz T, Synowitz H, Wagner S, Bertels C,Wallesch CW. Coping with illness after brain diseases. A compar-ison between patients with malignant brain tumors, stroke, Parkin-son’s disease and traumatic brain injury. Disabil Rehabil 2000;22:539–546.

12. Krakow K, Haltenhof H, Buhler KE. Coping with Parkinson’sdisease and refractory epilepsy. A comparative study. J Nerv MentDis 1999;187:503–508.

13. Fenelon G, Mahieux F, Huon R, Ziegler M. Hallucinations inParkinson’s disease. Prevalence, phenomenology and risk factors.Brain 2000;123:733–745.

14. Sanchez-Ramos JR, Ortoll R, Paulson GW. Visual hallucinationsassociated with Parkinson’s disease. Arch Neurol 1996;53:1265–1268.

832 CLINICAL/SCIENTIFIC NOTES

Movement Disorders, Vol. 18, No. 7, 2003

An Artist’s View of Drug-Induced Hallucinosis

Georg Ebersbach, MD*

Fachkrankenhaus fur Bewegungsstorungen/Parkinson,Beelitz-Heilstatten, Germany

Abstract: The occurrence of hallucinations in patients withParkinson’s disease is often related to antiparkinsonian therapy,but other factors such as cognitive decline and sleep-cycledisturbances are thought to contribute. We describe an artistwho depicted her visual hallucinations experienced in thecourse of Parkinson’s disease. © 2003 Movement DisorderSociety

Key words: Parkinson’s disease; hallucinations; amantadine

Case Report

The artist, a 61-year-old German woman living in southernFrance, has suffered from Parkinson’s disease since 1986.Pharmacotherapy was started in 1988 with excellent success. In1997, response fluctuations and hyperkinesias began and led torepeated readjustments of therapy. During an inpatient stay atthe Movement Disorder Clinic in Beelitz-Heilstatten, amanta-dine-sulfate was introduced in August of 2000 due to severepeak-dose chorea; the patient also was treated with pergolide (4mg/day), levodopa/benserazide (600/150 mg/day) and entaca-pone (1,000 mg/day). Amantadine-sulfate was held at constantdose (350 mg/day) until May 2002 when she returned from herhome in France. Subsequently she reported recurrent halluci-nations consisting of faces, bodies, and figures silhouettingagainst trees, stones, and other backgrounds with structuredsurface. Often this was preceded by blurred (and then misin-terpreted) perception of the real visual material. Extensiveneuropsychological assessment did not disclose evidence forcognitive or behavioural disturbances, and insight into thehallucinatory nature of the visual phenomena was maintainedwithout need for reality-testing manoeuvres; confrontation test-ing of visual fields and assessment of visual acuity with Snellencharts did not disclose disturbances of vision.

To depict her hallucinations, the patient created a series ofcoloured photographs reflecting the visual sensations experi-enced under the influence of antiparkinsonian therapy (Figs.1–3). Because reduction of amantadine-sulfate was followed byrecurrence of peak-dose chorea, the patient was started onclozapine (12.5 mg/day), which led to complete cessation ofhallucinations.

Discussion

Hallucinations are a frequent phenomenon in Parkin-son’s disease and are positively related to disease dura-

tion and cognitive disorders.1,2 Complex visual halluci-nations have specific content and defined forms such asfaces, objects, or animals. In patients without dementia,insight is often maintained, and initial illusions (misin-terpretation of real external stimuli) may herald complexvisual hallucinations as was the case in the present pa-

*Correspondence to: Georg Ebersbach, M.D., Fachkrankenhaus furBewegungsstorungen/Parkinson, Paracelsusring 6a, 14547 Beelitz-Heilstatten, Germany. E-mail: [email protected]

Received 21 July 2002; Revised 4 December 2002; Accepted 18December 2002

FIG. 2. Tree bark, coloured photograph.

FIG. 1. Stone, coloured photograph.

CLINICAL/SCIENTIFIC NOTES 833


tient. The artwork created by this patient allows theappreciation of the nonthreatening, expressive, and plas-tic character that is a frequent feature of hallucinationsassociated with Parkinson’s disease drug treatment.

References

1. Fenelon G, Mahieux F, Huon R, Ziegler M. Hallucinations inParkinson’s disease: prevalence, phenomenology and risk factors.Brain 2000;123:733–745.

2. Barnes J, David AS. Visual hallucinations in Parkinson’s disease: areview and phenomenological survey. J Neurol Neurosurg Psychi-atry 2001;70:727–733.

Tongue Tremor in a Patient with ComaAfter Electrical Injury

Katia Lin, MD, Jaime Lin, MD, Elcio Juliato Piovesan, MD,Francisco M.B. Germiniani, MD, Helio A.G. Teive, MD,*

and Lineu Cesar Werneck, MD, PhD

Movement Disorders Unit, Neurology Service, Department ofInternal Medicine, Hospital de Clınicas, Federal University

of Parana, Curitiba, Brazil

Abstract: We report on the case of a patient with transienttongue tremor and coma after electrical injury, probably due toa reversible brainstem dysfunction. We then reviewed the dif-ferential diagnosis of abnormal involuntary movements of thetongue as well as movement disorders related to electrocution.© 2003 Movement Disorder Society

Key words: tongue tremor; electrical injury; tongue hyperki-nesia; lingual tremor

Electrical injury may produce several different types of centralor peripheral nervous system complications, either acutely orwith a delay of several weeks or months after the initial injury.1Critchley divided neurological sequelae of electrical injury intofour major groups: cerebral effects (loss of consciousness,seizures, cognitive impairment, hemiplegia, and striatal syn-dromes), spinal effects (acute paraplegia and delayed progres-sive myelopathy with muscular atrophy), peripheral nerve in-juries (mononeuropathies and reflex sympathetic dystrophy),and hysterical manifestations (blindness, deafness, loss ofspeech, and fugue states).2 In addition, movement disordersafter electrical injury, predominantly dystonia, have been de-scribed in a few cases.1 We report on the case of a patient whodeveloped a transient tremor involving exclusively the tongue,without any clear evidence of a previous pathology save for asingle episode of electrical shock.

Case Report

A 27-year-old man, who worked as a metallurgist, acciden-tally incurred an electrical injury and was immediately taken tothe emergency room. He was holding a piece of 2,000-V solderequipment in his right hand when the electrical circuitry short-circuited. The electrical discharge entered through his righthand and exited through two different wounds, one in his rightneck and the other over the right temporal area.

A videotape accompanies this article.*Correspondence to: Helio A.G. Teive, Rua General Carneiro 1103/

102, Centro Curitiba, Parana, CEP 80.060-150 Brazil.E-mail: [email protected]

Received 3 April 2002; Revised 4 November 2002; Accepted 12December 2002

FIG. 3. Marble, coloured photograph.



The patient had burn lesions at the fingertips of the righthand as well as on the right side of his neck and on the rightzygomatic and temporal areas. The tongue had no burns orother lesions. There was no history of head trauma, otherprevious central nervous system (CNS) compromise, systemicdiseases, nor exposure to neuroleptic drugs. Family history ofmovement disorders was also negative.

He was admitted 45 minutes after the electrical injury in acomatose state (Glasgow Coma Scale score � 6), exhibitingresponse only to painful stimulation. Other than the coma,continuous, rhythmic tongue movements, characterized by a2-Hz tremor were found during neurological examination.There was no movement disorder involving the soft palate (seeVideo) and there was no relation between the tongue tremorand the patient’s reaction to pain.

Neuroimaging study with both cranial computed tomographyscan (CT) and magnetic resonance image (MRI) were normal.A complete work-up with whole-blood count and biochemistrywere also normal. Thirty-six hours after admission, he startedrecovering consciousness and after 48 hours he was wideawake and no longer exhibited the aforementioned involuntarytongue movements.

Discussion

Involuntary tongue movements are a rare and poorly under-stood group of movement disorders. Several abnormal invol-untary movements may appear in the tongue, such as tremor,fibrillations, fasciculations, myokimia, and dyskinesias.Tongue tremors occur occasionally in parkinsonism, whereasnonrhythmic movements are seen frequently with chorea ortardive dyskinesias.3,4 Episodic or paroxysmal rhythmic lingualmovements have been reported in association with chronicepilepsy as well as after head trauma.3,5 In addition, continuousrhythmic nonepileptic lingual myoclonus is a rare disorderusually described in association with similar palatal, ocular,facial, diaphragmatic or shoulder movements (rhythmic palatalmyoclonus).5 Another cause of tongue tremor is cerebrovascu-lar disease. Postert and colleagues3 described a case of episodicundulating hyperkinesias of the tongue associated with brain-stem ischemia. As a whole, movement disorders occurringduring coma, particularly those involving the tongue, are anuncommon condition. Misra and Kalita described movementdisorders occurring in 14 of 17 Japanese patients with enceph-alitis and only 3 of them had tongue dyskinesia.6

Although several different types of movement disorders afterelectrocution have been reported, including torticollis, limb andlingual dystonia, and parkinsonism,1,7–10 no other reports ofelectrical injury leading to isolated rhythmic lingual tremorcould be found in the current literature.

Electric contact injury is characterized by the existence of“entrance and exit” wounds on the skin as a result of a passingelectrical current.11 Little is known about the pathway thatelectricity takes once it enters the body after peripheral contact,even in those cases where there are apparent hand to hand orhand to foot injuries.12 Usually, tissue damage is more severe inthe entrance site, and deep tissue layers are more extensivelyaffected than the skin surface.11 In fact, in several large series,the nervous system is most often the site of major complica-tions,13 with animal studies suggesting that nerves and musclesare frequently damaged by high electrical voltage and thatcomplications often present themselves as late sequelae. Dam-age occurs as a result of tissue sensitivity to thermal injury and

electroporation, a term describing nonthermal damage to cellmembranes as a result of electricity.14

Histopathologically, electrical injuries may result in changesthat include focal petechial hemorrhages throughout the brainand medulla, chromatolysis of medullary cells, dilatation of theperivascular spaces, fragmentation of peripheral nerves, andballooning of myelin sheaths. Unfortunately, it is impossible todifferentiate tissue changes due to electrical current damagefrom those that might have been caused by necrosis from heat,sepsis, or associated mechanical trauma.2

Electricity also causes coagulation necrosis and, althoughpermanent damage to peripheral nerves generally does notextend beyond the area of local tissue damage, fibrosis and scarformation in and around the nerve can lead to compression anddecreased conductance.13

The tongue is a functionally prominent part of the oral-pharyngeal system, receiving its innervation from the lowerbrainstem.3 Experimental studies with stimulation of an areamedial to the inferior olive and close to the adjacent hypoglos-sal nucleus in cats produced rhythmic movements of the pos-terior part of the tongue.15 The inferior olivary nucleus receivesprojections from the ipsilateral red nucleus through the centraltegmental tract, which is probably the main affected area inmovement disorders of the tongue.3

In our case, clinical examination suggested brainstem struc-tures to be the origin of the movement disorder, althoughimaging techniques failed to reveal any brainstem lesion at theinferior olivary or adjacent areas.

Conversely, one may even wonder if this tongue tremorcould not be a movement disorder due to injury of the periph-eral nervous system. Proposed criteria for a peripherally in-duced movement disorder are 1) sufficient severity of theinjury, 2) anatomic relationship between the injury site and thesite of onset of the movement disorder, and 3) a latent period ofless than 1 year between the injury and the subsequent dis-ease.16 Our patient successfully fulfilled these criteria, but thereis no reason to consider peripheral nerve damage as a cause inthis case. In conclusion, our patient developed an isolatedtongue tremor involving mainly the anterior portion of thetongue, probably due a transient brainstem dysfunction second-ary to an electrical injury.

Legend to the Video

Comatose patient exhibits a continuous rhythmic hyperki-netic movement of the tongue, resembling a tremor.

References

1. Tarsy D, Sudarsky L, Charness ME. Limb dystonia followingelectrical injury. Mov Disord 1994;9:230–232.

2. Critchley M. Neurological effects of lightning and of electricity.Lancet 1934;1:68–72.

3. Postert T, Amoiridis G, Pohlau D, Hoffmann V, Przuntek H.Episodic undulating hyperkinesias of the tongue associated withbrainstem ischemia. Mov Disord 1997;12:619–621.

4. Keane JR. Galloping tongue: post-traumatic, episodic, rhythmicmovements. Neurology 1984;34:251–252.

5. Gobernado JM, Galarreta M, DeBlas G, Jimenez-Escrig A, Her-nandez A. Isolated continuous rhythmic lingual myoclonus. MovDisord 1992;7:367–369.

6. Misra UK, Kalita J. Movement disorders in Japanese encephalitis.J Neurol 1997;244:299–303.

7. Colosimo C, Kocen RS, Powell M, Lees AJ. Torticollis afterelectrocution. Mov Disord 1993;8:117–118.



8. Boonkongchuen P, Lees A. Case of torticollis following electricalinjury. Mov Disord 1996;11:109–110.

9. Ondo W. Lingual dystonia following electrical injury. Mov Disord1997;12:253.

10. Morris HR, Moriabadi NF, Lees AJ, Dick DJ, Moriabadi NF.Parkinsonism following electrical injury to the hand. Mov Disord1998;13:600–602.

11. Yan GR, Li M, Wu Y. A clinical analysis of 836 cases with electricinjury. Ann NY Acad Sci 1999;888:88–95.

12. Pliskin N, Fink J, Malina A, Moran S, Kelley K, Capelli-Schellpfeffer M, Lee R. The neuropsychological effects of electri-cal injury: new insights. Ann NY Acad Sci 1999;888:140–149.

13. Grube BJ, Heimbach DM, Engrav LH, Copass MK. Neurologicconsequences of electrical burns. J Trauma 1990;30:254–258.

14. Chen W, Lee RC. Evidence for electrical shock-induced confor-mational damage of voltage-gated ionic channels. Ann NY AcadSci 1994;720:124–135.

15. Bender MB, Nathanson M, Gordon GG. Myoclonus of muscle ofthe eye, face and throat. Arch Neurol Psychiatry 1952;67:44–58.

16. Jankovic J. Post-traumatic movement disorders: central and pe-ripheral mechanisms. Neurology 1994;44:2006–2014.

Episodic Focal Lingual Dystonic Spasms

Mark Edwards, MRCP,1 Geoffrey Schott, FRCP,2

and Kailash Bhatia, FRCP1*

1Sobell Department of Movement Neuroscienceand Movement Disorders, Institute of Neurology,

Queen Square, London, United Kingdom2National Hospital for Neurology and Neurosurgery,

Queen Square, London, United Kingdom

Abstract: We present the clinical history of a woman withidiopathic episodic focal lingual dystonic spasms. Although dys-tonic spasms of the tongue have been reported as a primarypersistent phenomenon and as a feature of secondary dystonias,primary episodic lingual dystonias are rare, tend to be unilateral,and in contrast to most other paroxysmal movement disorders, donot appear responsive to anti-epileptic drugs. © 2003 MovementDisorder Society

Key words: dystonia; tongue; paroxysmal

Dystonic involvement of the tongue is a well-recognised fea-ture of secondary dystonias. However, episodic dystonia of thetongue is only rarely reported.1,2 We present the case of a33-year-old woman who developed episodic dystonic spasms

of her tongue. Results of investigations, including an electro-encephalogram (EEG) during an attack, inter-ictal electromyo-graphic (EMG) sampling of the tongue and magnetic resonanceimaging (MRI) of the brain and cervical spine, were normal.

Case Report

A 33-year-old Caucasian woman presented with a 2-monthhistory of tightening of her tongue, predominantly affecting theleft side. The first episode occurred during a stressful argument,but subsequent episodes of tongue spasm had no clear precip-itants. Within 6 months of the first spasm, episodes of spasmwere occurring over 100 times per day. During episodes oftongue spasm, the patient had difficulty in articulation andswallowing. On occasion, she would experience bilateral painand tightness in her neck during episodes of tongue spasm.

She was on no medication, and her only previous medicationhad been the oral contraceptive pill. There was no previoushistory of neurological illness. She had a past history of sinus-itis, endometriosis, and mild postnatal depression after the birthof her first child for which she required no medication. Theonly family history of note was of multiple sclerosis in apaternal aunt.

On examination she had episodic spasm mainly affecting theleft side of her tongue. Each attack would last between 30seconds and 2 minutes. The left side of her tongue wouldbecome puckered and ridged, with occasional bulging. Cranialnerve examination was otherwise unremarkable. She had a mildbilateral postural tremor of the arms. Her gait was normal apartfrom a mild abnormal posturing of the right hand, which tendedto pull back and curl on walking. The remainder of the neuro-logical and general examination was normal.

Magnetic resonance imaging of the brain and cervical spinewas normal. EEG recorded during an episode of tongue spasmwas normal. Inter-ictal EMG sampling of the tongue was nor-mal and showed no evidence of denervation or myokymia.Tests of copper and ceruloplasmin, a fresh blood smear foracanthocytes, an autoimmune screen, and syphilis serologywere normal or negative. Results of her cerebrospinal fluidexamination were normal, with no evidence of oligoclonalbands.

A trial of treatment with carbamazepine was ineffective.Currently, the patient is on no medication, and the tonguespasms together with occasional tightening of the neck musclescontinue as before. The patient has declined further trials ofmedication at present.

Discussion

Paroxysmal movement disorders such as paroxysmal kine-sogenic dyskinesias and episodic ataxias are well described andusually involve the limbs. Spasms of the tongue are not re-ported to occur in these syndromes.3 Episodic or paroxysmalmovement disorders involving the tongue are rare.1–3 Lees andcolleagues reported two cases of primary paroxysmal spasms ofthe tongue in 1986.1 In both of these cases, the spasm of thetongue was unilateral, and tonic contraction of the tongue lastedbetween 10 and 60 seconds. In 1 patient, the attacks weretriggered by speech, eating, and also general physical exertion,whereas in the other, stress was an occasional trigger. No othersigns were found on physical examination, and imaging of bothpatients was uninformative. There are similarities between ourcase and those of Lees and coworkers. The only other reported

A videotape accompanies this article.*Correspondence to: Bhatia, Sobell, MRCP, Department of Move-

ment, Neuroscience and Movement Disorders, Institute of Neurology,Queen Square, London, WC1N 3BG United Kingdom.E-mail: [email protected]

Received 16 July 2002; Revised 5 October 2002; Accepted 18December 2002



case of episodic lingual spasm is of an 84-year-old individualwith unilateral episodic lingual spasm in the presence of anipsilateral parotid adenoma.2 The authors reporting this casesuggested that the spasm might be due to dystonia triggered byperipheral trauma, although it may also be possible that localspread of the tumour was the cause. This case, thus, is differentfrom our case and from those of Lees and colleagues.

There has been one report of primary persistent focal lingualdystonia that occurred spontaneously in a 32-year-old man.4

The dystonia was made worse by speaking but not by eating ordrinking. Although the intensity of the dystonia fluctuated, itdid not disappear fully at any time during wakefulness, differ-entiating this case from the episodic lingual dystonia seen inour own patient. Past history was significant only for righttemporomandibular arthritis 10 years previously. There was nohistory of neuroleptic drug use. Full investigation, includingMRI of the brain and cervical cord and EEG revealed noabnormality. The dystonia was improved by the use of trihexy-phenidyl, and after 5 months, this treatment was withdrawn,with no return of the dystonia.

Rarely, isolated dystonia involving the tongue occurring as asecondary phenomenon has been reported. Keane reported twopatients with tongue spasm following severe head injury.5 Onepatient had suffered brain injury affecting the right cerebellarhemisphere and left temporal lobe, and the other had a smallsubdural haematoma and a “hangman’s” fracture of the cervicalspine. Significant eye movement abnormalities in both patientssuggested that pontine damage had also occurred. Rhythmicepisodic spasms of the tongue were noted in both patients asthey recovered from coma, and persisted during further recov-ery. The phenomenon lasted approximately 2 months in 1patient and 4 months in the other. No EEG changes were notedduring attacks.

Tongue dystonia has been reported in other conditions. Sec-ondary dystonic involvement of the tongue is well recognisedas part of generalised dystonia in disorders such as neuroacan-thocytosis. There is a curious disorder called the “neck–tonguesyndrome,” reported by Orrell and Marsden.6 This syndrome ischaracterised by pseudoathetoid movements of the tongue, painin the neck exacerbated by movement, and paresthesiae of theipsilateral side of the tongue. The syndrome is thought to arisefrom damage to lingual afferent fibres travelling in the hypo-glossal nerve to the C2 spinal roots. Most patients with thesyndrome have evidence of pathology at the atlanto-axial oratlanto-occipital joints. The patient reported by Orrell andMarsden developed pseudoathetoid movements of the tonguetogether with typical features of the neck–tongue syndromesecondary to a traumatic fracture of the right occipital condyle.

Wavelike high amplitude movements of the tongue duringsleep have been reported in three children with chronic epile-psy.7 These abnormal movements were associated with desyn-chronisation on the EEG and were thought to represent unusualsubcortical seizure activity.

In conclusion, we have reported on a case of episodic lingualdystonic spasm. Given the tightening of the neck muscles thatour patient sometimes experiences and the subtle dystonic signson limb examination with no abnormalities found on investi-gation, we would consider the disorder to be an unusual man-ifestation of idiopathic segmental dystonia.

Legends to the Video

Segment 1. Toward the end of an attack, some puckering canbe seen on the left side of the tongue.

Segment 2. Bulging of the tongue on the left is clearly seen.Segment 3. Puckering and bulging of the left side of the

tongue can be seen.

References

1. Lees AJ, Blau JN, Schon F. Paroxysmal hemiglossal twisting.Lancet 1986;2:812–813.

2. Micheli F, Scoricati MC, Caro I, Pikelny R. Focal paroxysmaldyskinesias secondary to peripheral lesions: a report of two cases[abstract]. Mov Disord 1998;13(Suppl. 2):196.

3. Bhatia KP. The paroxysmal dyskinesias. J Neurol 1999;246:149–155.4. Ishii K, Tamaoka A, Shoji S. A case of primary focal lingual

dystonia induced by speaking. Eur J Neurol 2001;8:507.5. Keane RJ. Galloping tongue: post-traumatic episodic, rhythmic

movements. Neurology 1984;34:251–252.6. Orrell RW, Marsden CD. The neck–tongue syndrome. J Neurol

Neurosurg Psychiatry 1994;57:348–352.7. Jabbari B, Coker SB. Paroxysmal rhythmic lingual movements and

chronic epilepsy. Neurology 1981;31:1364–1367.

Chorea as a Presenting Feature of VariantCreutzfeldt-Jakob Disease

David McKee, MRCP,* and Paul Talbot, MD, FRCP

Department of Neurology, Greater Manchester Centre forClinical Neurosciences, Manchester, United Kingdom

Abstract: We report on a patient with pathologically provenvariant Creutzfeld-Jakob disease in whom chorea was a pre-senting feature of the disease, and was unaccompanied by thetypical prodrome of psychiatric disturbance or sensory symp-toms. © 2003 Movement Disorder Society

Key words: Creutzfeldt-Jakob disease; chorea; movementdisorders

A 27-year-old man presented with a 2-month history of progres-sive neurological deterioration. He reported steadily worseningproblems with slurred speech, unsteadiness of gait, and “fidgeting”from the outset. His partner had also noticed that he had developedcontinuous involuntary movements of the limbs, head and neck,and there was a suggestion of cognitive dysfunction in that he hadbegun to engage in repetitive questioning. There were, however,no features to suggest psychiatric disturbance and no history ofsensory symptoms. He was otherwise fit and well with no past

*Correspondence to: Dr. D.H. McKee, Department of Neurology,Greater Manchester Centre for Clinical Neurosciences, Hope Hospital,Stott Lane, Salford M6 8HD, United Kingdom.E-mail: [email protected]

Received 17 July 2002; Revised 6 November 2002; Accepted 18November 2002



medical history of note and was taking no medications. There wasno family history of neurological disease.

On examination, the most striking abnormality was the pres-ence of continuous choreiform movements of all four limbs andthe neck. In addition he was mildly dysarthric and demon-strated mild bilateral limb ataxia and difficulty in performingtandem gait. The remainder of the neurological examinationwas entirely normal and there were no abnormalities on generalphysical examination.

Three weeks later he was admitted to hospital for investigation.By this time considerable deterioration in his neurological condi-tion had occurred. His partner described increasing problems withhis short-term memory and in addition he had developed frequentnocturnal myoclonic jerks of the limbs. The patient complained ofabnormal right-sided sensory symptoms affecting the face, arm,and leg. On examination there was clear progression of his abnor-mal neurological signs, with more pronounced choreiform move-ments, and deterioration in his speech, coordination and balance.His ocular pursuit movements had become broken but there wereno other new neurological abnormalities. In particular, myoclonicjerks were not apparent during waking hours. Neuropsychologicalassessment revealed a picture of predominantly subcortical im-pairment, with a slowed rate of performance, retrieval deficits onmemory testing, perseveration, difficulty in shifting mental set andinterference errors. In addition he displayed evidence of focalcortical deficit in the form of perceptuo-spatial difficulties. Elec-troencephalogram (EEG) showed generalised slow wave activityin the delta range. MRI of the brain was carried out, and althoughthe images were degraded by motion artefact, on T2 weightingthey nonetheless clearly demonstrated bilateral symmetrical highsignal intensities in the pulvinar regions. Cerebrospinal fluid(CSF) was acellular with normal protein and glucoseconcentrations.

Because of the distinctive radiological appearances and de-spite the rather atypical presentation, a diagnosis of variantCreutzfeldt-Jakob disease (vCJD) was considered. Extensiveinvestigation failed to identify an alternative diagnosis and hewas referred to and assessed by the National CJD SurveillanceCentre. His condition continued to deteriorate steadily withdeath occurring approximately 8 months after the onset of hisfirst symptoms. By the time of death he met published researchcriteria for a clinical diagnosis of probable vCJD, althoughwithout the typical feature of early psychiatric symptoms (Ta-ble 1). Neuropathological examination of the brain at autopsyconfirmed the diagnosis of variant CJD. As with cases reportedpreviously, he was found to be homozygous for methionine atcodon 129 of the prion protein gene.

Discussion

The clinical features of variant CJD have been well de-scribed in the literature.1,2 The condition typically presents withpsychiatric disturbance often with unpleasant sensory distur-bances, followed by increasing cognitive decline and ataxia,progressing to akinetic mutism and death. Movement disorders,including chorea and myoclonus, are well recognised but havegenerally been reported as features of the later stages of thedisease. In a published series of 35 patients with pathologicallyconfirmed vCJD a total of 20 patients developed chorea at somepoint during the disease process, but none had chorea on initialpresentation.3 More recently, the case of a 28-year-old womanwho developed chorea as a prominent early feature of vCJDwas reported.4 In this case, the patient had presented with a ten

month history of behavioural disturbances and cognitive de-cline, and had been treated for an initial diagnosis of depres-sion. It was not until 4 months after her first psychiatricsymptoms, and following treatment with several antidepres-sants, that she developed chorea, which became subsequentlymore florid as her condition progressed.

In the case described, it is of note that the patient and his partnerreported chorea as one of the initial symptoms. Chorea was alsonoted on examination at the time of first presentation 2 monthsinto the history of the disease, and was significantly more floridseveral weeks later. Unlike the case reported previously, thispatient did not present with a prodrome of psychiatric disturbancetypical of vCJD and had been on no previous antidepressanttreatment. This is the first reported case of pathologically con-firmed vCJD where chorea was present from the outset and was aprominent presenting feature, as opposed to an early feature of thedisease. In conjunction with the previously reported case thisillustrates the point that vCJD may present in an atypical manner,an issue that is particularly relevant given the possibility of makingan antemortem pathological diagnosis by tonsillar biopsy,5 andearly diagnosis becoming increasingly important in the context oftreatment with potential disease-modifying agents. The possibilityof vCJD should be considered in the differential diagnosis ofchorea in younger patients.

References

1. Zeidler M, Stewart GE, Barraclough CR, et al. New variantCreutzfeldt-Jakob disease: neurological features and diagnostictests. Lancet 1997;350:903–907.

2. Zeidler M, Johnstone EC, Bamber RW, et al. New variantCreutzfeldt-Jakob disease: psychiatric features. Lancet 1997;350:908–910.

3. Will RG, Zeidler M, Stewart GE, et al. Diagnosis of New VariantCreutzfeldt-Jakob disease. Ann Neurol 2000;47:575–582.

4. Bowen J, Mitchell T, Pearce R, Quinn N. Chorea in New VariantCreutzfeldt-Jakob disease. Mov Disord 2000;15:1284–1285.

5. Hill AF, Butterworth RJ, Joiner S, et al. Investigation of variantCreutzfeldt-Jakob disease and other human prion diseases withtonsil biopsy samples. Lancet 1999;353:183–189.

TABLE 1. Diagnostic criteria for vCJD

Criterion

IA Progressive neuropsychiatric disorderB Duration of illness �6 mo.C Routine investigations do not suggest an alternative diagnosisD No history of potential iatrogenic exposure

IIA Early psychiatric symptomsB Persistent painful sensory symptomsC AtaxiaD Myoclonus or chorea or dystoniaE Dementia

IIIA EEG does not show the typical appearance of sporadic CJD (or

no EEG performed)B Bilateral pulvinar high signal on MRI scan

From Will et al.3Definite: IA and neuropathological confirmation of vCJD.Probable: I and 4/5 of II and IIIA and IIIB.Possible: I and 4/5 of II and IIIA.vCJD, variant Creutzfeld-Jakob disease; EEG, electroencephalo-

gram; MRI, magnetic resonance imaging.



chorea as a presenting feature of variant creutzfeldt-jakob disease

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