Movement Disorders Vol. 5 , No. 1, 1990, pp. 44-46 0 1990 Movement Disorder Society
Diurnal Variability in Cranial Dystonia Pasquale Montagna, Gaetano Procaccianti, Alessandra Lugaresi, Marco Zucconi, and Elio Lugaresi Institute of Neurology, University of Bologna, Bologna, Italy
Summary: Eleven patients with cranial dystonia were investigated for diurnal variations in disability by means of video recordings. Disability increased significantly from morning to evening. The increase was not related to changes in vigilance levels assessed by dynamic electroencephalogram. Cranial movement disorders display diurnal fluctuations that are probably related to endogenous circadian rhythms. Key Words: Cranial dystonia-BlepharospasmParaspasm-Circadian rhythms.
had isolated BS or blepharoclonus, in six BS was associated with dyskinesia of the face, mouth, or other body segments, and one had prominent tongue dystonia. Seven patients reported that symptoms worsened during the course of the day, and two had remarkably symptom-free periods for some hours in the morning (Table 1). These periods had progressively shortened with increasing duration of the disease. Other neurological disturbances were absent and investigation, including brain computed tomography, was normal. The patients were asked to stop all medications at least 1 week before admission to the hospital. Video recordings of cranial movements were performed. Patients were allowed to relax as fully as possible while sitting before the camera (“rest” condition). Thereafter, they were requested to try to stop the abnormal movements for 30 s (“effort” conditiop). The patients’ position and dress, recording room, 11g,,2:g conditions, and operator were kept rigorously the same throughout the sessions, which could be identified only by numbers on cards placed near the patients. The recordings were obtained on two different days for each patient, at hourly intervals from 8:OO AM to 8:OO PM, totaling 26 recordings for each patient. Patients also kept a diary of daily activities, including times of sleep and meals. In between recordings, they remained free to move about and do what they pleased. After random mix-
Patients with cranial dystonia, in particular blepharospasm (BS), often report worsening disability during evening hours, whereas symptomfree periods may occur in the morning soon after awakening. Similar diurnal fluctuations of disability have been noted in other movement disorders. A “sleep benefit,” i.e., improvement of symptoms after a night’s sleep, may be reported by parkinsonian patients (l), and Dopa-responsive dystonia is characterized by marked diurnal fluctuations, with worsening in the evening (2). Sleep benefit has also been found in idiopathic dystonia, independent of site of onset (3). Disability in movement disorders, however, may vary widely (4) depending on environmental, especially emotional, factors; and patients’ self-reports may be unreliable. Quantitative measurements of variability have not been performed in cranial dystonia. We therefore quantitatively investigated diurnal fluctuations of disability in cranial dystonia by means of video recordings. Preliminary findings have already been reported (5). MATERIALS AND METHODS
Eleven patients, 4 men and 7 women, age range 42-75 years, participated in the study. Four patients Address correspondence and reprint requests to Dr. P. Montagna, Institute of Neurology, Via U.Foscolo 7, 40123 Bologna, Italy.
44
VARIABILITY IN FACIAL D YSKZNESIA
45
TABLE 1. Clinicalfeatures of patients Patient no.
Sex
Age (yr)
1
M
2 3 4 5 6 7 8 9
F F F F F F
54 65 55 69 42 75 55 69 65 60 49
10
M M M
11
F
+
=
mild;
++
=
moderate;
Duration of symptoms (yr)
2 8 10
2 3 2 2 3 6 5 2
+++
=
BS
Facehouth
Tongue
Jaw
+++ ++ ++ + ++ + + ++ + +
+ + + + + + +
+
+
+++
+
+
+
Neck
+ +
Sleep benefit
Arms
+ + ++ + + ++
+ + +
marked. BS, blepharospasm.
ing by the operator, the 26 videoclips were shown to three independent observers (P.M., A.L., G.P.) blind to the hour of recording. Movements were quantitated in preset, stepwise, arbitrary units, and values from the three observers were pooled together and finally listed according to the time of day. For statistical analysis, because the data were ordinal, we used nonparametric statistics (Friedman’s two-way analysis of variance). In five patients, vigilance levels were monitored by dynamic polysomnography [electroencephalogram (EEG): C,A,, C,A,, P,O,, P,O,, EOG, and chin electromyography] performed simultaneously to the video recordings. The EEG recorded during the periods of video recording were then scored for vigilance level (by M.Z.) according to a three-step preset rating scale (drowsiness, relaxed, and active wakefulness). Direct assessment of vigilance level, i.e., by reaction times, was impossible because it would have meant altering the neuropsychological state of the patients.
ditions). Analysis of the polysomnography showed that all five patients investigated were in the state of active wakefulness (parameters: EEG rapid activity, posterior alpha, ocular movements, and movement artifacts) throughout the 26 video-recording sessions. No particular relationship was noted with daily activities; patients commonly took their meals between noon and 1:OO PM and 5:OO PM and 6:OO PM. DISCUSSION Quantitative assessment of disability in cranial dystonia substantiated the clinical impression and patients’ claims that abnormal movements worsened during the day, peaking in the evening. Vigilance levels are an important factor of variability of movement disorders as exemplified by the attenuation or complete disappearance of cranial dystonia during sleep (6). Vigilance shows circadian fluctua-
RESULTS In the entire group of patients, disability scores increased significantly from 8:OO AM to 8:OO PM, both when considering “rest” (p < 0.01) and “effort” (p < 0.05) conditions separately, or when pooling “rest” and “effort” data together (p < 0.001). The trend to increasing mean disability was progressive from 8:OO AM, with a maximum at 7:OO PM. Disability moreover fell somewhat at 2:OO PM and 6:OO PM (Fig. 1). The trend was similar for the 2 days of recording but, when analyzing the 2 days separately, significance was attained only for the first day (p < 0.05 for both “rest” or “effort” con-
7
9
11
13
15
17
19
21
TIME (hours)
FIG. 1. Mean disability scores (2SD) from 8:OO AM to 8:OO PM for the whole group of patients (2-day pooled values).
Movement Disorders, Vol. 5 , N o . 1 , 1990
46
P . MONTAGNA ET AL.
tions, with increased sleepiness in the afternoon (7). The trend to increasing cranial dystonia disability in the evening, however, did not seem to be related to systematic variations in vigilance levels because no EEG changes were detected on dynamic polysomnography during the video-recording sessions. Moreover, we observed increasing cranial dystonia disability in the afternoon, a time of diminished vigilance, when decreased cranial dystonia intensity should be expected. Cranial dystonias thus follow a pattern common to Dopa-responsive and idiopathic dystonia and to Parkinson’s disease (1--3), that of progressive worsening during the day, a time of psychomotor activity, and improvement after sleep, a time of physical rest. Acknowledgment: We are grateful to Mr. Carlo Grassi for the video-recording sessions and to Ms. A. Pollini for typing the manuscript.
Movement Disorders, Vol. 5 , No. 1 , 1990
REFERENCES 1. Marsden CD, Parkes JD, Quinn N. Fluctuations of disability
2. 3. 4.
5, 6.
7.
in Parkinson’s disease-clinical aspects. In: Marsden CD, Fahn S , eds. Movement disorders. London: Butterworth, 1981:96122. Segawa M, Hosaka A, Miyagawa F, Nomura Y, Imai H. Hereditary progressive dystonia with marked diurnal fluctuation. Adv Neurol 1976;l4:2 15-33. Nygaard TG, Fahn S. Variability of symptoms in idiopathic dystonia [abstract]. Neurology 1988;38(suppl 1):419. Cleeves L, Findley LJ. Variability in amplitude of essential tremor. J Neurol Neurosurg Psychiatty 1987;50:704-8. Montagna P, Lugaresi A, Procaccianti G , Bizzi A, Martinelli P. Diurnal fluctuations of disability in facial dyskinesia. Abstract presented at ISMD Congress, Rome, 1988. Sforza E, Defazio G , Santostasi R. Polysomnography in blepharospasm and oromandibular dystonia. In: Koella WP, Obal S , Schultz H, Wiser P, eds. Sleep 86. Stuttgart: Fischer Verlag, 1988:460-1. Richardson GS, Carskadon MA, Flagg W, Van den Hoed J, Dement WC, Mitler MM. Excessive daytime sleepiness in man: multiple sleep latency measurement in narcoleptic and control subjects. Electroencephalogr Clin Neurophysiol 1978;45:621-7.
Diurnal Variability in Cranial Dystonia Pasquale Montagna, Gaetano Procaccianti, Alessandra Lugaresi, Marco Zucconi, and Elio Lugaresi Institute of Neurology, University of Bologna, Bologna, Italy
Summary: Eleven patients with cranial dystonia were investigated for diurnal variations in disability by means of video recordings. Disability increased significantly from morning to evening. The increase was not related to changes in vigilance levels assessed by dynamic electroencephalogram. Cranial movement disorders display diurnal fluctuations that are probably related to endogenous circadian rhythms. Key Words: Cranial dystonia-BlepharospasmParaspasm-Circadian rhythms.
had isolated BS or blepharoclonus, in six BS was associated with dyskinesia of the face, mouth, or other body segments, and one had prominent tongue dystonia. Seven patients reported that symptoms worsened during the course of the day, and two had remarkably symptom-free periods for some hours in the morning (Table 1). These periods had progressively shortened with increasing duration of the disease. Other neurological disturbances were absent and investigation, including brain computed tomography, was normal. The patients were asked to stop all medications at least 1 week before admission to the hospital. Video recordings of cranial movements were performed. Patients were allowed to relax as fully as possible while sitting before the camera (“rest” condition). Thereafter, they were requested to try to stop the abnormal movements for 30 s (“effort” conditiop). The patients’ position and dress, recording room, 11g,,2:g conditions, and operator were kept rigorously the same throughout the sessions, which could be identified only by numbers on cards placed near the patients. The recordings were obtained on two different days for each patient, at hourly intervals from 8:OO AM to 8:OO PM, totaling 26 recordings for each patient. Patients also kept a diary of daily activities, including times of sleep and meals. In between recordings, they remained free to move about and do what they pleased. After random mix-
Patients with cranial dystonia, in particular blepharospasm (BS), often report worsening disability during evening hours, whereas symptomfree periods may occur in the morning soon after awakening. Similar diurnal fluctuations of disability have been noted in other movement disorders. A “sleep benefit,” i.e., improvement of symptoms after a night’s sleep, may be reported by parkinsonian patients (l), and Dopa-responsive dystonia is characterized by marked diurnal fluctuations, with worsening in the evening (2). Sleep benefit has also been found in idiopathic dystonia, independent of site of onset (3). Disability in movement disorders, however, may vary widely (4) depending on environmental, especially emotional, factors; and patients’ self-reports may be unreliable. Quantitative measurements of variability have not been performed in cranial dystonia. We therefore quantitatively investigated diurnal fluctuations of disability in cranial dystonia by means of video recordings. Preliminary findings have already been reported (5). MATERIALS AND METHODS
Eleven patients, 4 men and 7 women, age range 42-75 years, participated in the study. Four patients Address correspondence and reprint requests to Dr. P. Montagna, Institute of Neurology, Via U.Foscolo 7, 40123 Bologna, Italy.
44
VARIABILITY IN FACIAL D YSKZNESIA
45
TABLE 1. Clinicalfeatures of patients Patient no.
Sex
Age (yr)
1
M
2 3 4 5 6 7 8 9
F F F F F F
54 65 55 69 42 75 55 69 65 60 49
10
M M M
11
F
+
=
mild;
++
=
moderate;
Duration of symptoms (yr)
2 8 10
2 3 2 2 3 6 5 2
+++
=
BS
Facehouth
Tongue
Jaw
+++ ++ ++ + ++ + + ++ + +
+ + + + + + +
+
+
+++
+
+
+
Neck
+ +
Sleep benefit
Arms
+ + ++ + + ++
+ + +
marked. BS, blepharospasm.
ing by the operator, the 26 videoclips were shown to three independent observers (P.M., A.L., G.P.) blind to the hour of recording. Movements were quantitated in preset, stepwise, arbitrary units, and values from the three observers were pooled together and finally listed according to the time of day. For statistical analysis, because the data were ordinal, we used nonparametric statistics (Friedman’s two-way analysis of variance). In five patients, vigilance levels were monitored by dynamic polysomnography [electroencephalogram (EEG): C,A,, C,A,, P,O,, P,O,, EOG, and chin electromyography] performed simultaneously to the video recordings. The EEG recorded during the periods of video recording were then scored for vigilance level (by M.Z.) according to a three-step preset rating scale (drowsiness, relaxed, and active wakefulness). Direct assessment of vigilance level, i.e., by reaction times, was impossible because it would have meant altering the neuropsychological state of the patients.
ditions). Analysis of the polysomnography showed that all five patients investigated were in the state of active wakefulness (parameters: EEG rapid activity, posterior alpha, ocular movements, and movement artifacts) throughout the 26 video-recording sessions. No particular relationship was noted with daily activities; patients commonly took their meals between noon and 1:OO PM and 5:OO PM and 6:OO PM. DISCUSSION Quantitative assessment of disability in cranial dystonia substantiated the clinical impression and patients’ claims that abnormal movements worsened during the day, peaking in the evening. Vigilance levels are an important factor of variability of movement disorders as exemplified by the attenuation or complete disappearance of cranial dystonia during sleep (6). Vigilance shows circadian fluctua-
RESULTS In the entire group of patients, disability scores increased significantly from 8:OO AM to 8:OO PM, both when considering “rest” (p < 0.01) and “effort” (p < 0.05) conditions separately, or when pooling “rest” and “effort” data together (p < 0.001). The trend to increasing mean disability was progressive from 8:OO AM, with a maximum at 7:OO PM. Disability moreover fell somewhat at 2:OO PM and 6:OO PM (Fig. 1). The trend was similar for the 2 days of recording but, when analyzing the 2 days separately, significance was attained only for the first day (p < 0.05 for both “rest” or “effort” con-
7
9
11
13
15
17
19
21
TIME (hours)
FIG. 1. Mean disability scores (2SD) from 8:OO AM to 8:OO PM for the whole group of patients (2-day pooled values).
Movement Disorders, Vol. 5 , N o . 1 , 1990
46
P . MONTAGNA ET AL.
tions, with increased sleepiness in the afternoon (7). The trend to increasing cranial dystonia disability in the evening, however, did not seem to be related to systematic variations in vigilance levels because no EEG changes were detected on dynamic polysomnography during the video-recording sessions. Moreover, we observed increasing cranial dystonia disability in the afternoon, a time of diminished vigilance, when decreased cranial dystonia intensity should be expected. Cranial dystonias thus follow a pattern common to Dopa-responsive and idiopathic dystonia and to Parkinson’s disease (1--3), that of progressive worsening during the day, a time of psychomotor activity, and improvement after sleep, a time of physical rest. Acknowledgment: We are grateful to Mr. Carlo Grassi for the video-recording sessions and to Ms. A. Pollini for typing the manuscript.
Movement Disorders, Vol. 5 , No. 1 , 1990
REFERENCES 1. Marsden CD, Parkes JD, Quinn N. Fluctuations of disability
2. 3. 4.
5, 6.
7.
in Parkinson’s disease-clinical aspects. In: Marsden CD, Fahn S , eds. Movement disorders. London: Butterworth, 1981:96122. Segawa M, Hosaka A, Miyagawa F, Nomura Y, Imai H. Hereditary progressive dystonia with marked diurnal fluctuation. Adv Neurol 1976;l4:2 15-33. Nygaard TG, Fahn S. Variability of symptoms in idiopathic dystonia [abstract]. Neurology 1988;38(suppl 1):419. Cleeves L, Findley LJ. Variability in amplitude of essential tremor. J Neurol Neurosurg Psychiatty 1987;50:704-8. Montagna P, Lugaresi A, Procaccianti G , Bizzi A, Martinelli P. Diurnal fluctuations of disability in facial dyskinesia. Abstract presented at ISMD Congress, Rome, 1988. Sforza E, Defazio G , Santostasi R. Polysomnography in blepharospasm and oromandibular dystonia. In: Koella WP, Obal S , Schultz H, Wiser P, eds. Sleep 86. Stuttgart: Fischer Verlag, 1988:460-1. Richardson GS, Carskadon MA, Flagg W, Van den Hoed J, Dement WC, Mitler MM. Excessive daytime sleepiness in man: multiple sleep latency measurement in narcoleptic and control subjects. Electroencephalogr Clin Neurophysiol 1978;45:621-7.
