Movement Disorders Vol. 5 , No. 1, 1990, pp. 8-14 0 1990 Movement Disorder Society
The Effect of Nocturnal Physiological Sleep on Various Movement Disorders Rosalia Silvestri, *Pietro De Domenico, Antonio E. Di Rosa, Placido Bramanti, Salvatore Serra, and Raoul Di Perri Institute of Neurological and Neurosurgical Sciences, Clinica Neurologica I , University of Messina, and *Mental Heulth Service, Messina, Italy
Summary: Thirty-one subjects affected by different movement disorders underwent polygraphic and videotape monitoring during nocturnal sleep, to assess movement patterns during the night. It was possible to distinguish two categories of disorders according to their pattern of movements. In the largest group (Meige’s syndrome, blepharospasm, amyotrophic choreoacanthocytosis, Tourette syndrome, tonic foot, hemiballism) abnormal movements were still present during sleep, but decreased in frequency and amplitude in all stages. The second group presented three syndromes (nocturnal paroxysmal dystonia, nocturnal myoclonus, restless legs syndrome), in which light nonrapid-eye-movement sleep induced a strong activation of abnormal movements, whereas rapid-eye-movement sleep suppressed them. Key Words: Hypnic modulation-Nocturnal movement disorders-Nocturnal polygraphic recordings.
acteristics as in waking. These authors also observed that myoclonus due to lesions in the lower brainstem and/or in the spinal cord increases remarkably during light non-rapid-eye-movement (NREM) sleep, becoming minimal in the rapid eye movement (REM) stage. Sleep patterns of rarer movement disorders have received less attention, although recently several syndromes (i.e., nocturnal paroxysmal dystonia, restless legs syndrome, and nocturnal myoclonus), in which abnormal movements occur only during sleep, have been investigated polygraphically (5,6). The aim of our study was to observe movement patterns during nocturnal sleep in patients affected by various uncommon movement disorders.
Differing reports exist regarding the pattern of extrapyramidal hyperkinesias during sleep. Classically, parkinsonian tremor, athetoid and choreiform movements, and several tics are believed to disappear completely during nocturnal sleep, whereas hemiballism only decreases (1). Subsequently Tassinari et al. (2,3) observed that abnormal movements usually do disappear during sleep, but that parkinsonian and choreoathetoid “tremors” may transiently reappear under three circumstances, i.e., awakening, body movements even if not associated with arousals, and simple arousals. Mano et al. (4), on the basis of clinical observation, reported that involuntary movements (such as ballism, choreoathetosis, dystonia, and myoclonus) due to lesions in the cerebral cortex, basal ganglia, and upper brainstem decrease considerably during sleep. They may, however, be found in any sleep stage, with the same electromyogram (EMG) char-
PATIENTS AND METHODS Thirty-one subjects affected by different movement disorders were studied.
Meige’s Syndrome The diagnosis was made on the basis of the characteristic bilateral involuntary facial spasms involv-
Address correspondence and reprint requests to Dr. R . Silvestri at Clinica Neurologica I. Policlinico Universitario. 98013. Contesse, Messina, Italy.
8
MOVEMENT DISORDERS DURING SLEEP
ing eyes and mouth, their chronicity, and EMG findings in the muscles innervated by the facial nerve. There were six subjects-four women and two men, mean age 58.8 years. Blepharospasm Blepharospasm is an involuntary, spasmodic, and persistent contraction of the orbicularis oculi. This patient was a 60-year-old man. Amyotrophic Choreoacanthocytosis(ACA) This is a rare inherited disease of adult onset, characterized by normal lipoproteinemic acanthocytosis and a dyskinesia consisting of mainly proximal chorea complicated by biting of the lips and tongue, vocalization, and neurogenic muscular atrophy (7,8). There were six subjects-five men and one woman, who were members of two families, mean age 41. I years. Marinesco-Sjogren’s Syndrome This is a very uncommon disease, transmitted by an autonomic recessive trait, characterized by cataract, cerebellar ataxia, mental retardation, and frequent, generalized myoclonic jerks (9,lO). There were three subjects, all men, mean age 24 years. Gilles de la Tourette’s Syndrome (TS) Diagnosis was made according to the DSM I11 (1980) (11) criteria. There were four patients, all men, mean age 19.7 years. Vascular Hemiballism This disorder consists of ballic hyperkinesias involving the right inferior limb. The patient was a 78-year-old woman. Tonic Foot Syndrome This syndrome involves involuntary, prolonged, episodic contraction of the right foot muscles, inducing a plantar flexion of the toes. The patient was a 72-year-old woman. Familial Idiopathic Orobuccal Dyskinesias The patient studied was a 67-year-old woman, presenting with a 2-year history of abnormal involuntary movements involving her tongue and lips. Identical symptoms had previously affected older members of her family. Nocturnal Paroxysmal Dystonia (NPD) These patients had recurrent plurinocturnal episodes consisting of dystonic attacks, tonic attacks, and abnormal sleep behavior (5). There were four
patients-three years.
9
men and one woman, mean age 21.5
Nocturnal Myoclonus There were three subjects, all men, mean age 68 years. Restless Legs Syndrome (RLS) The patient was a 65-year-old woman. Methods All patients were recorded for at least two consecutive nights, after 1 night adapting to the laboratory conditions. None of them had received any type of drug that might have interfered with their nocturnal sleep for the 2 weeks prior. Sleep recordings included: a minimum of two electroencephalogram (EEG) monopolar leads (C3-A2; 0l-A2), electrooculogram (EOG), submental, right and left anterior tibialis EMGs, and electrocardiogram with the aid of surface electrodes. Nasal and/or oral airflows were recorded by means of thermistors, while thoracoabdominal respirograms were detected by mercury-filled strain gauges. Other muscle EMGs were added to this standard montage in relation to the different topographic distribution of the abnormal movements. The gain of the four variables used to define sleep stages were fixed according to the standards of Rechtschaffen and Kales (12) (EEG and EOG 50 kV/7.5 mm; submental EMG 20 kV/cm). The gains of the other EMG channels were adjusted during wakefulness to obtain the best records. A more detailed study of the abnormal movements was achieved using all-night videotape recording (with split-screen presentation of the patient and the EEG), which allowed accurate observation of temporal, morphological, and topographical aspects of the hyperkinesias. The video recording was analyzed by at least two observors (blinded), to detect the occurrence of movements during the night and their distribution in different sleep stages. The same procedure was used to score nocturnal EEG recordings, according to Rechtschaffen and Kales’ criteria (12), on a basis of 30-s epochs. RESULTS Meige’s Syndrome Movements were present in each patient in all sleep stages. Sporadically the EEG showed brief periods (1-2 s) of desynchronization coinciding with
Movement Disorders, Vol. 5 , N o . I , 1990
R . SILVESTRI ET A L .
I0
the end of muscular contractions, without reaching a clear awake pattern. No obvious differences were observed in the incidence of dyskinesias in different sleep stages, although a smaller number of movements were seen in delta sleep. Frequency and duration of the spasms showed a clear-cut decrease during all sleep stages.
ing all sleep stages. A periodic, persistent vocalization was present during REM.
Blepharospasm
TS
Spasms of the orbicularis oculi were observed during stages 1 , 2, 3, and REM (stage 4 was not recorded). Their frequency and duration were considerably decreased in comparison with wakefulness. Their mean duration during sleep was 3-4 s, against 25 s during wakefulness. Mean frequency of spasms during sleep was one every 5-6 rnin against one every 2 min during wakefulness.
Movements were present in all sleep stages. However, they were prominent during stages 1 and 2, appeared only sporadically in delta sleep and were poorly defined, and were very brief and almost absent during REM (Fig. l).
ACA Dyskinesias persisted in the same muscular groups in sleep as in wakefulness. However, their amplitude, duration, and frequency decreased dur-
Marinesco-Sjogren Syndrome Short, asynchronous, arrhythmic muscular jerks (
The Effect of Nocturnal Physiological Sleep on Various Movement Disorders Rosalia Silvestri, *Pietro De Domenico, Antonio E. Di Rosa, Placido Bramanti, Salvatore Serra, and Raoul Di Perri Institute of Neurological and Neurosurgical Sciences, Clinica Neurologica I , University of Messina, and *Mental Heulth Service, Messina, Italy
Summary: Thirty-one subjects affected by different movement disorders underwent polygraphic and videotape monitoring during nocturnal sleep, to assess movement patterns during the night. It was possible to distinguish two categories of disorders according to their pattern of movements. In the largest group (Meige’s syndrome, blepharospasm, amyotrophic choreoacanthocytosis, Tourette syndrome, tonic foot, hemiballism) abnormal movements were still present during sleep, but decreased in frequency and amplitude in all stages. The second group presented three syndromes (nocturnal paroxysmal dystonia, nocturnal myoclonus, restless legs syndrome), in which light nonrapid-eye-movement sleep induced a strong activation of abnormal movements, whereas rapid-eye-movement sleep suppressed them. Key Words: Hypnic modulation-Nocturnal movement disorders-Nocturnal polygraphic recordings.
acteristics as in waking. These authors also observed that myoclonus due to lesions in the lower brainstem and/or in the spinal cord increases remarkably during light non-rapid-eye-movement (NREM) sleep, becoming minimal in the rapid eye movement (REM) stage. Sleep patterns of rarer movement disorders have received less attention, although recently several syndromes (i.e., nocturnal paroxysmal dystonia, restless legs syndrome, and nocturnal myoclonus), in which abnormal movements occur only during sleep, have been investigated polygraphically (5,6). The aim of our study was to observe movement patterns during nocturnal sleep in patients affected by various uncommon movement disorders.
Differing reports exist regarding the pattern of extrapyramidal hyperkinesias during sleep. Classically, parkinsonian tremor, athetoid and choreiform movements, and several tics are believed to disappear completely during nocturnal sleep, whereas hemiballism only decreases (1). Subsequently Tassinari et al. (2,3) observed that abnormal movements usually do disappear during sleep, but that parkinsonian and choreoathetoid “tremors” may transiently reappear under three circumstances, i.e., awakening, body movements even if not associated with arousals, and simple arousals. Mano et al. (4), on the basis of clinical observation, reported that involuntary movements (such as ballism, choreoathetosis, dystonia, and myoclonus) due to lesions in the cerebral cortex, basal ganglia, and upper brainstem decrease considerably during sleep. They may, however, be found in any sleep stage, with the same electromyogram (EMG) char-
PATIENTS AND METHODS Thirty-one subjects affected by different movement disorders were studied.
Meige’s Syndrome The diagnosis was made on the basis of the characteristic bilateral involuntary facial spasms involv-
Address correspondence and reprint requests to Dr. R . Silvestri at Clinica Neurologica I. Policlinico Universitario. 98013. Contesse, Messina, Italy.
8
MOVEMENT DISORDERS DURING SLEEP
ing eyes and mouth, their chronicity, and EMG findings in the muscles innervated by the facial nerve. There were six subjects-four women and two men, mean age 58.8 years. Blepharospasm Blepharospasm is an involuntary, spasmodic, and persistent contraction of the orbicularis oculi. This patient was a 60-year-old man. Amyotrophic Choreoacanthocytosis(ACA) This is a rare inherited disease of adult onset, characterized by normal lipoproteinemic acanthocytosis and a dyskinesia consisting of mainly proximal chorea complicated by biting of the lips and tongue, vocalization, and neurogenic muscular atrophy (7,8). There were six subjects-five men and one woman, who were members of two families, mean age 41. I years. Marinesco-Sjogren’s Syndrome This is a very uncommon disease, transmitted by an autonomic recessive trait, characterized by cataract, cerebellar ataxia, mental retardation, and frequent, generalized myoclonic jerks (9,lO). There were three subjects, all men, mean age 24 years. Gilles de la Tourette’s Syndrome (TS) Diagnosis was made according to the DSM I11 (1980) (11) criteria. There were four patients, all men, mean age 19.7 years. Vascular Hemiballism This disorder consists of ballic hyperkinesias involving the right inferior limb. The patient was a 78-year-old woman. Tonic Foot Syndrome This syndrome involves involuntary, prolonged, episodic contraction of the right foot muscles, inducing a plantar flexion of the toes. The patient was a 72-year-old woman. Familial Idiopathic Orobuccal Dyskinesias The patient studied was a 67-year-old woman, presenting with a 2-year history of abnormal involuntary movements involving her tongue and lips. Identical symptoms had previously affected older members of her family. Nocturnal Paroxysmal Dystonia (NPD) These patients had recurrent plurinocturnal episodes consisting of dystonic attacks, tonic attacks, and abnormal sleep behavior (5). There were four
patients-three years.
9
men and one woman, mean age 21.5
Nocturnal Myoclonus There were three subjects, all men, mean age 68 years. Restless Legs Syndrome (RLS) The patient was a 65-year-old woman. Methods All patients were recorded for at least two consecutive nights, after 1 night adapting to the laboratory conditions. None of them had received any type of drug that might have interfered with their nocturnal sleep for the 2 weeks prior. Sleep recordings included: a minimum of two electroencephalogram (EEG) monopolar leads (C3-A2; 0l-A2), electrooculogram (EOG), submental, right and left anterior tibialis EMGs, and electrocardiogram with the aid of surface electrodes. Nasal and/or oral airflows were recorded by means of thermistors, while thoracoabdominal respirograms were detected by mercury-filled strain gauges. Other muscle EMGs were added to this standard montage in relation to the different topographic distribution of the abnormal movements. The gain of the four variables used to define sleep stages were fixed according to the standards of Rechtschaffen and Kales (12) (EEG and EOG 50 kV/7.5 mm; submental EMG 20 kV/cm). The gains of the other EMG channels were adjusted during wakefulness to obtain the best records. A more detailed study of the abnormal movements was achieved using all-night videotape recording (with split-screen presentation of the patient and the EEG), which allowed accurate observation of temporal, morphological, and topographical aspects of the hyperkinesias. The video recording was analyzed by at least two observors (blinded), to detect the occurrence of movements during the night and their distribution in different sleep stages. The same procedure was used to score nocturnal EEG recordings, according to Rechtschaffen and Kales’ criteria (12), on a basis of 30-s epochs. RESULTS Meige’s Syndrome Movements were present in each patient in all sleep stages. Sporadically the EEG showed brief periods (1-2 s) of desynchronization coinciding with
Movement Disorders, Vol. 5 , N o . I , 1990
R . SILVESTRI ET A L .
I0
the end of muscular contractions, without reaching a clear awake pattern. No obvious differences were observed in the incidence of dyskinesias in different sleep stages, although a smaller number of movements were seen in delta sleep. Frequency and duration of the spasms showed a clear-cut decrease during all sleep stages.
ing all sleep stages. A periodic, persistent vocalization was present during REM.
Blepharospasm
TS
Spasms of the orbicularis oculi were observed during stages 1 , 2, 3, and REM (stage 4 was not recorded). Their frequency and duration were considerably decreased in comparison with wakefulness. Their mean duration during sleep was 3-4 s, against 25 s during wakefulness. Mean frequency of spasms during sleep was one every 5-6 rnin against one every 2 min during wakefulness.
Movements were present in all sleep stages. However, they were prominent during stages 1 and 2, appeared only sporadically in delta sleep and were poorly defined, and were very brief and almost absent during REM (Fig. l).
ACA Dyskinesias persisted in the same muscular groups in sleep as in wakefulness. However, their amplitude, duration, and frequency decreased dur-
Marinesco-Sjogren Syndrome Short, asynchronous, arrhythmic muscular jerks (
