Movement Disorders Vol. 6 , No. 1, 1991, pp. 85-86 0 1991 Movement Disorder Society
Brief Report
Paroxysmal Dystonic Head Tremor Andrew J . Hughes, Andrew J. Lees, and *C. David Marsden Department of Neurology, University College and Middlesex Hospitals, School of Medicine, and *University Department of Neurology, Institute of Neurology, The National Hospital for Nervous Diseases, Queen Square, London, England
Summary: Two patients with paroxysmal attacks of “no-no’’ direction head tremor and mild torticollis are described. One of them had the characteristicfeatures of paroxysmal dystonic choreoathetosis and responded well to clonazepam. Key Words: Head tremorParoxysmal dystonia-Torticollis-Choreoathetosis-Clonazepam.
mittent sternomastoid contractions were clearly visible. The frequency varied between 4 and 8 Hz. A left or right predominance of direction could at times be seen (see video segment 1). There was no impairment of consciousness. Tremor recording with surface electrodes over the splenius capitis and sternomastoid muscles showed a 5.56.0-Hz tremor. A computed tomography (CT) scan was normal, as was an electroencephalogram (EEG) during and between attacks. Results of routine blood tests including copper and caeruloplasmin levels were normal. Treatment with clonazepam (1 mg bd) has led to a marked decrease in the frequency and duration of attacks.
Isolated tremor of the head and trunk has recently been described as the sole or initial manifestation of focal dystonia (1). This is especially the case in slow-frequency (2-5 Hz) head tremor in a “no-no” direction (1). We now present two patients with paroxysmal attacks of isolated ‘‘no-no ,” dystonic head tremor.
CASE REPORTS
Case 1 A 26-year-old policeman was referred because of 8 years of paroxysmal involuntary head movements. There was no other past or family history of tremor, dystonia, or parkinsonism. Since late adolescence he had experienced episodes of head tremor lasting from several minutes to several hours (usually approximately 30 min), which began and ended abruptly, occasionally preceded by transient light-headedness. The attacks were commonly precipitated by tiredness, anxiety, fasting, alcohol, or eating after a period of fasting. Occasionally he could curtail an attack by lying down and relaxing. They had gradually increased in frequency and were occurring between two and three times per week at the time of presentation. Treatment with diazepam, carbamazepine, and propranolol had been ineffective. On three occasions in hospital, we were able to precipitate head tremor by fasting followed by ingestion of carbohydrate and alcohol. During an attack, he developed a jerky predominately “no-no” head tremor with mild laterocollis and retrocollis. Inter-
Case 2 A 30-year-old farm worker was referred for assessment of 10 years of intermittent head tremor. There was no family or previous history of neurological disease. At the age of 20 he had a right orchidectomy for testicular teratorna followed by radiotherapy and intensive chemotherapy. Approximately 3 months after treatment he noticed an intermittent head tremor occurring every few weeks. It had been refractory to treatment with carbamazepine, clonazepam, diazepam, acetazolamide, and benzhexol and had increased in frequency for 10 years and was now occurring on most days. Between attacks, he was asymptomatic and his general health was excellent. There were no clear precipitating factors. Each episode would last from a few minutes to approximately half an hour, during which there was a predominantly side-to-side, “no-no” head tremor of -7-8 Hz, with clear, intermittent, vigorous contractions of sternomastoid muscles and slight torticollis and retrocollis. Standing led to spread of the tremor to both upper limbs, although on sitting or lying
A videotape segment accompanies this article. Address correspondence and reprint requests to Dr. A. J. Lees, Department of Neurology, The Middlesex Hospital, London W1N 8AA, England.
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the tremor was restricted to his head and neck (see video segment 2). Occasionally he could almost stop the tremor by putting his hands behind his neck and pulling his head forward forcefully. During episodes he was alert and fully able to converse, with no other dystonic or parkinsonian features. All investigations, including CT scan, EEG during an attack and interictally, and results of routine blood tests including assessment of copper and caeruloplasmin levels, were normal. Electromyographic recording during an attack was not able to be performed.
between attacks, and the upper-limb tremor in case 2 during attacks was mild. The clear paroxysmal nature, the distribution, the “no-no” form, the associated neck dystonia, and the lack of response to propranolol make it improbable that these were essential head tremor variants. We think these cases broaden the described spectrum of dystonic head tremors.
DISCUSSION
SEGMENT 1. Case I . An attack of jerky “no-no” head tremor with mild torticollis precipitated by fasting and ingestion of alcohol. SEGMENT 2. Case 2. This segment shows a resting “no-no” head tremor of -7-8 Hz with mild torticollis during video-EEG monitoring.
The paroxysmal dyskinesias can be classified into two subgroups, although intermediate (2,3) and hypnogenic forms (43) have also been described. Paroxysmal kinesogenic choreoathetosis (2,6), which may be familial or sporadic, is the most common form, and is characterised by frequent, brief attacks (less than 5 min) of dystonia or chorea precipitated by sudden movement. In contrast, paroxysma1 dystonic choreoathetosis (PDC) (2,7-9) is precipitated by alcohol, coffee, fatigue, fasting, stress, and food, with less frequent attacks (up to three per day) lasting minutes to several hours, and may be controlled by clonazepam, oxazepam, or acetazolamide (2,10,11). PDC may be predominantly dystonic or choreic, even in different members of the same kindred (2), may be focal or generalized (2,7,1l), can include torticollis as a manifestation (7), and has been described with tremor of the limbs (12) although not of the head. The pathophysiology of PDC remains unknown. We believe our first case may be a sporadic form of PDC, with dystonic head tremor and torticollis being the only manifestations. The precipitants, frequency, and duration of attacks were typical of PDC as were the patient’s age of onset of symptoms, the normal ictal EEG, and the response to clonazepam. Torticollis, although not in isolation, was described in the original case of Mount and Reback (7), as was the observation that attacks could be attenuated by lying down. Our second case has none of the usual precipitants of PDC, although the duration and frequency of attacks are the same. The clear paroxysms, the clinical characteristics of the head tremor, and the mild retrocollis and torticollis during attacks have led us to label it paroxysmal dystonic head tremor, although further classification is not possible. It remains refractory to all drug treatment. Its relationship to treatment for his teratoma is uncertain, but at the time of assessment the tumour had been in full remission for 10 years. Essential tremor is common in patients with dystonia and their relatives (13). However, with essential tremor, head or trunk involvement is usually seen in the later stages when it is usually overshadowed by upper limb involvement (14,15). Neither of our cases had any tremor
Movement Disorders, Vol. 6 , No. I , 1991
Acknowledgment: A.J.H. was supported by a grant from the United Kingdom Parkinson’s Disease Society.
LEGENDS TO VIDEOTAPE
REFERENCES 1. Rivest J, Marsden CD. Trunk and head tremor as isolated
manifestations of dystonia. Movement Disorders 1990;5:6065. 2. Lance JW. Familial paroxysmal dystonic choreoathetosis and its differentiation from related syndromes. Ann Neurol 1977;2:285-293. 3. Plant GT, Williams AC, Earl CG, Marsden CD. Familial paroxysmal dystonia induced by exercise. J Neurol Neurosurg Psychiutry 1984;47:275-279. 4. Lugaresi E, Cirignotta F , Montagna P. Nocturnal paroxysmal dystonia. J Neurol Neurosurg Psychiatry 1986;49:375380. 5. Lehkuniec E , Micheli F, De Arbelaiz R, Torres M, Paradiso G. Concurrent hypnogenic and reflex paroxysmal dystonia. Movement Disorders 1988;3:290-294. 6. Kertesz A. Paroxysmal kinesigenic choreoathetosis. Neurology 1967;17:680-689. 7. Mount LA, Reback S . Familial paroxysmal choreoathetosis. Arch Neurol Psychiatry (Chicago) 1940;44:841-847. 8 Richards RN, Barnett HJM. Paroxysmal dystonic choreoathetosis: a family study and a review of the literature. Neurology 1968 ;18:461469. 9. Forssman H. Hereditary disorder characterized by attacks of muscular contractions, induced by alcohol amongst other factors. Acta Med Scund 1961 ;170:517-533. 10. Kurlan R, Shoulson I. Familial paroxysmal dystonic choreoathetosis and response to alternate day oxazepam therapy. Ann Neurol 1983 ;13 :456457. 1 1 . Mayeux R, Fahn S. Paroxysmal dystonic choreoathetosis in a patient with familial ataxia. Neurology 1982;32:1184-1186. 12. Bressman SB, Fahn S, Burke RE. In: Fahn S , Marsden CD, Calne DB, eds. Dystonia 2. New York: Raven Press, 1988:403413. (Advances in neurology; vol50). 13. Fahn S. Atypical tremors, rare tremors, and unclassified tremors. In: Findley LJ, Capildeo R, eds. Movement disorders: tremor. London: MacMillan, 1984:431-443. 14. Critchley E. Clinical manifestations of essential tremor. J Neurol Neurosurg Psychiatry 1972;35:365-372. 15. Martinelli P. Different clinical features of essential tremor: a 200 patient study. Arch Neurol Scand 1987;75:106-1 1 1 .
Brief Report
Paroxysmal Dystonic Head Tremor Andrew J . Hughes, Andrew J. Lees, and *C. David Marsden Department of Neurology, University College and Middlesex Hospitals, School of Medicine, and *University Department of Neurology, Institute of Neurology, The National Hospital for Nervous Diseases, Queen Square, London, England
Summary: Two patients with paroxysmal attacks of “no-no’’ direction head tremor and mild torticollis are described. One of them had the characteristicfeatures of paroxysmal dystonic choreoathetosis and responded well to clonazepam. Key Words: Head tremorParoxysmal dystonia-Torticollis-Choreoathetosis-Clonazepam.
mittent sternomastoid contractions were clearly visible. The frequency varied between 4 and 8 Hz. A left or right predominance of direction could at times be seen (see video segment 1). There was no impairment of consciousness. Tremor recording with surface electrodes over the splenius capitis and sternomastoid muscles showed a 5.56.0-Hz tremor. A computed tomography (CT) scan was normal, as was an electroencephalogram (EEG) during and between attacks. Results of routine blood tests including copper and caeruloplasmin levels were normal. Treatment with clonazepam (1 mg bd) has led to a marked decrease in the frequency and duration of attacks.
Isolated tremor of the head and trunk has recently been described as the sole or initial manifestation of focal dystonia (1). This is especially the case in slow-frequency (2-5 Hz) head tremor in a “no-no” direction (1). We now present two patients with paroxysmal attacks of isolated ‘‘no-no ,” dystonic head tremor.
CASE REPORTS
Case 1 A 26-year-old policeman was referred because of 8 years of paroxysmal involuntary head movements. There was no other past or family history of tremor, dystonia, or parkinsonism. Since late adolescence he had experienced episodes of head tremor lasting from several minutes to several hours (usually approximately 30 min), which began and ended abruptly, occasionally preceded by transient light-headedness. The attacks were commonly precipitated by tiredness, anxiety, fasting, alcohol, or eating after a period of fasting. Occasionally he could curtail an attack by lying down and relaxing. They had gradually increased in frequency and were occurring between two and three times per week at the time of presentation. Treatment with diazepam, carbamazepine, and propranolol had been ineffective. On three occasions in hospital, we were able to precipitate head tremor by fasting followed by ingestion of carbohydrate and alcohol. During an attack, he developed a jerky predominately “no-no” head tremor with mild laterocollis and retrocollis. Inter-
Case 2 A 30-year-old farm worker was referred for assessment of 10 years of intermittent head tremor. There was no family or previous history of neurological disease. At the age of 20 he had a right orchidectomy for testicular teratorna followed by radiotherapy and intensive chemotherapy. Approximately 3 months after treatment he noticed an intermittent head tremor occurring every few weeks. It had been refractory to treatment with carbamazepine, clonazepam, diazepam, acetazolamide, and benzhexol and had increased in frequency for 10 years and was now occurring on most days. Between attacks, he was asymptomatic and his general health was excellent. There were no clear precipitating factors. Each episode would last from a few minutes to approximately half an hour, during which there was a predominantly side-to-side, “no-no” head tremor of -7-8 Hz, with clear, intermittent, vigorous contractions of sternomastoid muscles and slight torticollis and retrocollis. Standing led to spread of the tremor to both upper limbs, although on sitting or lying
A videotape segment accompanies this article. Address correspondence and reprint requests to Dr. A. J. Lees, Department of Neurology, The Middlesex Hospital, London W1N 8AA, England.
85
A . J . HUGHES ET AL.
86
the tremor was restricted to his head and neck (see video segment 2). Occasionally he could almost stop the tremor by putting his hands behind his neck and pulling his head forward forcefully. During episodes he was alert and fully able to converse, with no other dystonic or parkinsonian features. All investigations, including CT scan, EEG during an attack and interictally, and results of routine blood tests including assessment of copper and caeruloplasmin levels, were normal. Electromyographic recording during an attack was not able to be performed.
between attacks, and the upper-limb tremor in case 2 during attacks was mild. The clear paroxysmal nature, the distribution, the “no-no” form, the associated neck dystonia, and the lack of response to propranolol make it improbable that these were essential head tremor variants. We think these cases broaden the described spectrum of dystonic head tremors.
DISCUSSION
SEGMENT 1. Case I . An attack of jerky “no-no” head tremor with mild torticollis precipitated by fasting and ingestion of alcohol. SEGMENT 2. Case 2. This segment shows a resting “no-no” head tremor of -7-8 Hz with mild torticollis during video-EEG monitoring.
The paroxysmal dyskinesias can be classified into two subgroups, although intermediate (2,3) and hypnogenic forms (43) have also been described. Paroxysmal kinesogenic choreoathetosis (2,6), which may be familial or sporadic, is the most common form, and is characterised by frequent, brief attacks (less than 5 min) of dystonia or chorea precipitated by sudden movement. In contrast, paroxysma1 dystonic choreoathetosis (PDC) (2,7-9) is precipitated by alcohol, coffee, fatigue, fasting, stress, and food, with less frequent attacks (up to three per day) lasting minutes to several hours, and may be controlled by clonazepam, oxazepam, or acetazolamide (2,10,11). PDC may be predominantly dystonic or choreic, even in different members of the same kindred (2), may be focal or generalized (2,7,1l), can include torticollis as a manifestation (7), and has been described with tremor of the limbs (12) although not of the head. The pathophysiology of PDC remains unknown. We believe our first case may be a sporadic form of PDC, with dystonic head tremor and torticollis being the only manifestations. The precipitants, frequency, and duration of attacks were typical of PDC as were the patient’s age of onset of symptoms, the normal ictal EEG, and the response to clonazepam. Torticollis, although not in isolation, was described in the original case of Mount and Reback (7), as was the observation that attacks could be attenuated by lying down. Our second case has none of the usual precipitants of PDC, although the duration and frequency of attacks are the same. The clear paroxysms, the clinical characteristics of the head tremor, and the mild retrocollis and torticollis during attacks have led us to label it paroxysmal dystonic head tremor, although further classification is not possible. It remains refractory to all drug treatment. Its relationship to treatment for his teratoma is uncertain, but at the time of assessment the tumour had been in full remission for 10 years. Essential tremor is common in patients with dystonia and their relatives (13). However, with essential tremor, head or trunk involvement is usually seen in the later stages when it is usually overshadowed by upper limb involvement (14,15). Neither of our cases had any tremor
Movement Disorders, Vol. 6 , No. I , 1991
Acknowledgment: A.J.H. was supported by a grant from the United Kingdom Parkinson’s Disease Society.
LEGENDS TO VIDEOTAPE
REFERENCES 1. Rivest J, Marsden CD. Trunk and head tremor as isolated
manifestations of dystonia. Movement Disorders 1990;5:6065. 2. Lance JW. Familial paroxysmal dystonic choreoathetosis and its differentiation from related syndromes. Ann Neurol 1977;2:285-293. 3. Plant GT, Williams AC, Earl CG, Marsden CD. Familial paroxysmal dystonia induced by exercise. J Neurol Neurosurg Psychiutry 1984;47:275-279. 4. Lugaresi E, Cirignotta F , Montagna P. Nocturnal paroxysmal dystonia. J Neurol Neurosurg Psychiatry 1986;49:375380. 5. Lehkuniec E , Micheli F, De Arbelaiz R, Torres M, Paradiso G. Concurrent hypnogenic and reflex paroxysmal dystonia. Movement Disorders 1988;3:290-294. 6. Kertesz A. Paroxysmal kinesigenic choreoathetosis. Neurology 1967;17:680-689. 7. Mount LA, Reback S . Familial paroxysmal choreoathetosis. Arch Neurol Psychiatry (Chicago) 1940;44:841-847. 8 Richards RN, Barnett HJM. Paroxysmal dystonic choreoathetosis: a family study and a review of the literature. Neurology 1968 ;18:461469. 9. Forssman H. Hereditary disorder characterized by attacks of muscular contractions, induced by alcohol amongst other factors. Acta Med Scund 1961 ;170:517-533. 10. Kurlan R, Shoulson I. Familial paroxysmal dystonic choreoathetosis and response to alternate day oxazepam therapy. Ann Neurol 1983 ;13 :456457. 1 1 . Mayeux R, Fahn S. Paroxysmal dystonic choreoathetosis in a patient with familial ataxia. Neurology 1982;32:1184-1186. 12. Bressman SB, Fahn S, Burke RE. In: Fahn S , Marsden CD, Calne DB, eds. Dystonia 2. New York: Raven Press, 1988:403413. (Advances in neurology; vol50). 13. Fahn S. Atypical tremors, rare tremors, and unclassified tremors. In: Findley LJ, Capildeo R, eds. Movement disorders: tremor. London: MacMillan, 1984:431-443. 14. Critchley E. Clinical manifestations of essential tremor. J Neurol Neurosurg Psychiatry 1972;35:365-372. 15. Martinelli P. Different clinical features of essential tremor: a 200 patient study. Arch Neurol Scand 1987;75:106-1 1 1 .
