BARONE, Paolo

Carmine Vitale

Dario Grossi

Luigi Di Maio

Nina Fragassi

PELLECCHIA, MARIA TERESA

Teresa Cuomo

English

Archivio della Ricerca - Università di Salerno

Unawareness of dyskinesias inParkinson’s and Huntington’s diseasesC. Vitale • M.T. Pellecchia • D. Grossi • N. FragassiT. Cuomo • L. Di Maio • P. Barone ()Department of Neurological Sciences, University Federico II,Naples, ItalyAbstract We performed a clinical study to evaluate theunawareness of dyskinesias in patients affected byParkinson’s disease (PD) and Huntington’s disease (HD).Thirteen PD patients with levodopa-induced dyskinesias and9 HD patients were enrolled. Patients were asked to evaluatethe presence of dyskinesias while performing specific motortasks. The Abnormal Involuntary Movement Scale (AIMS)and Goetz dyskinesia rating scale were administered to deter-mine the severity of dyskinesias. The Unified Parkinson’s dis-ease rating scale (UPDRS) and Unified Huntington’s DiseaseRating Scale (UHDRS) were used in PD and HD patients,respectively. In PD we found a significant negative relation-ship between unawareness score at standing and AIMS scoreand between unawareness score at hand pronation-supinationand AIMS score for upper limbs. In HD we found a signifi-cant positive relationship between total unawareness scoreand UHDRS score and between total unawareness score anddisease duration. In PD the unawareness seems to be inverse-ly related with severity of dyskinesias, while in HD it isdirectly related to disease duration and severity. Patients with Huntington’s disease (HD) rarely complain oftheir dyskinesias, possibly due to an impaired subjectiveexperience of chorea [1]. A similar phenomenon is common-ly observed in patients with Parkinson’s disease (PD), whomay appear oblivious to their drug-induced dyskinesias.Although the phenomenon is well recognized clinically, itsbasis has not been systematically  investigated. The aim ofour study was to evaluate the unawareness of dyskinesias inPD as compared with HD patients, by means of a subjectivequestionnaire pertaining to the direct experience of involun-tary movements while performing specific motor tasks.Subjective reports were examined in relation to objectivemeasures of neurological status. Thirteen PD patients (median age, 60.1 years; range, 49-77 years) with motor fluctuations and levodopa-induceddyskinesias and 9 patients with HD (median age, 58.5 years;range, 31-71) confirmed by molecular analysis participatedin the study. The median duration of illness was 11.5 years(range, 6-20 years) in PD patients and 7.7 years (range, 5-10years) in HD patients. Cognitive status was assessed by theMini-Mental State Examination (MMSE) [2]. Only patientswith an MMSE score equal to or higher than 23 wereenrolled in the study. In all patients involuntary movementswere evident both to the clinician and to the patient’s care-giver. Patients were asked to subjectively evaluate the pres-ence of dyskinesias while performing specific motor tasks(standing, gait, finger-tapping, hand pronation-supination).A score of unawareness ranging from 0 (full awareness) to 2(full unawareness) was attributed during each motor task.YES(0)YES(1)Due to weakness ? (1)Due to slowness? (1)Due to involuntary movements ? (0)NO(0)If not , why?NO(1)If not , do you think that the motor task was correctly performed ?Did you experienceinvoluntary movementswhile performing  motor tasks?Tasks:1) Standing2) Gait3) Finger- tapping4) Hand pronation -supinationFig. 1 Subjective questionnaire of dyskinesia awareness. Scores ranged from 0 (full awareness) to 2 (full unawareness)  Neurol Sci (2001) 22:105-106 © Springer-Verlag 2001Thus, unawareness total score ranged from 0 (full aware-ness) to 8 (full unawareness) (Fig. 1). Objective rating ofdyskinesias was obtained using the Abnormal InvoluntaryMovement Scale (AIMS) [3] and the Goetz dyskinesia ratingscale [4] in all patients. We used the Unified Parkinson’sDisease Rating Scale (UPDRS) [5] and the UnifiedHuntington’s Disease Rating Scale (UHDRS) [6] to assessillness severity. Spearman’s r correlation coefficient wasused to examine the relationship between subjective report-ed data and objective measures of disability.Five PD patients were completely unaware of their dys-kinesias, 4 were partially aware and 4 were totally aware oftheir dyskinesias. We found a significant negative relation-ship between unawareness score at standing and total AIMSscore (r=-0.7; p<0.01), unawareness score at hand pronation-supination and AIMS score for upper limbs (r=-0.6; p<0.05),and unawareness score at standing and AIMS score for lowerlimbs (r=-0.6; p<0.05). We also found a trend to a significantnegative relationship between total unawareness score andtotal AIMS score (r=-0.5; p=0.07). Unawareness total scorewas not related with UPDRS score, Goetz dyskinesia ratingscale score and disease duration. Seven HD patients were completely unaware of theirdyskinesias, while 2 patients were totally aware. We found asignificant positive relationship between total unawarenessscore and UHDRS score (r=0.7, p<0.05) and between totalunawareness score and disease duration (r=0.9, p=0.001).There was no significant relationship between objectivemeasures of dyskinesias (AIMS and Goetz dyskinesia ratingscale) and unawareness total score. DiscussionLack of awareness of involuntary movements is commonlyobserved in clinical practice in both PD and HD patients.Snowden et al. [1] suggested that lack of awareness in HDpatients was not a consequence of patients’ cognitive impair-ment or a psychological defense against a debilitating dis-ease, but reflected an impaired subjective experience ofchorea. To our knowledge, failure to report dyskinesias hasnot been previously investigated in PD patients. Our resultsconfirm that both PD and HD patients frequently fail toreport their dyskinesias. We found that unawareness wasdirectly related with disease severity and duration in HDpatients, and inversely related with severity of dyskinesias in106PD patients. To explain these findings, different hypothesesmay be suggested. According to a physiopathologicalhypothesis, we suggest that dopaminergic overstimulation inPD, while inducing dyskinesias by acting on basal ganglia,may affect the awareness of involuntary movements by stim-ulating mesocorticolimbic pathways. Instead, it is well estab-lished that disease duration and severity in HD are associat-ed with frontal lobe lesions [7] that may explain the impairedawareness of dyskinesias in patients with long-term disease.We cannot exclude a psychodynamic explanation of ourresults. According to this hypothesis, dyskinesias in PD arethe expression of an overtreatment of bradykinesia, andpatients may fail to report them for the simple reason thatthey do not experience moving abnormally. In fact, PDpatients were more likely to report dyskinesias when inter-fering with a specific motor task. Instead, in HD dyskinesiasare the main clinical manifestation, so a psychologicaldefense mechanism of denial may explain the lack of aware-ness of involuntary movements in our patients. Both physiopathological and psychodynamic hypothesesrequire further investigations to highlight the precise mecha-nisms underlying the lack of awareness of involuntary move-ments in PD and HD.References1. Snowden JS, Craufurd D, Griffiths HL et al (1998) Awarenessof involuntary movements in Huntington disease. ArchNeurol 55:801-8052. Folstein MF, Folstein SE, McHugh PR (1975) “Mini MentalState” a practical method for grading the cognitive state ofpatients for the clinician. J Psychiat Res 12:189-198 3. Simpson GM, Lee JH, Houbok B et al (1979) Rating scale fortardive dyskinesia. Psychopharmacol 64:171-1794. Goetz CG, Stebbins GT, Shale HM et al (1994) Utility of anobjective dyskinesia rating scale for Parkinson’s disease:inter- and intrarater reliability assessment. Mov Disord9:390-3945. Fahn S, Elton RL, and Members of the UPDRS DevelopmentCommittee (1987) In: Fahn S, Marsden CD, Goldstein M et al(eds) Recents developments in Parkinson’s disease II.McMillan, New York, pp 153-1636. Huntington Study Group (1996) Unified Huntington’sDisease Rating Scale: reliability and consistency. Mov Disord11:136-1427. Folstein MF, Folstein SE, Brandt J (1990) Huntington’s dis-ease. In: Cummings JL (ed) Subcortical dementia. Oxford,New York

Unawareness of dyskinesias in Parkinson's and Huntington's diseases

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Unawareness of dyskinesias in Parkinson's and Huntington's diseases

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