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BRIEF COMMUNICATIONS
but increasing the dosage of levodopa only worsened the head turning. At 39, she underwent a successful left stereotactic thalamotomy. However, her gait continued to deteriorate. Several attempts over the next 13 years to introduce Sinemet, Madopar, and selegiline at therapeutic doses were unsuccessful because of an increase in “no-no” head turning, which was associated with nausea and vomiting. On admission, she was taking two-hourly doses of plain levodopa totalling 1,375 mg/day, benzhexol 5 mg t.d.s., and amitriptyline 25 mg nocte. On this regime, her “on” periods were accompanied by neck dyskinesias with nausea, culminating in vomiting. In the “off” state, her King’s College Hospital disability score was 50/117 (moderately severe), Hoehn and Yahr stage IV. One tablet of Sinemet 275 after an overnight fast turned her “on,” without nausea, after 60 min. However, after a further 45 min, she developed “no-no” head turning with nausea, and vomited. We were uncertain whether the nausea and vomiting were secondary to the levodopa per se or to the levodopainduced head turning. We, therefore, gave domperidone 50 mg t.d.s. and rechallenged with levodopa 1 g. Nausea and vomiting, associated with “no-no” head turning, occurred 55 min after turning “on.” Domperidone was continued. The next morning, after 4 mg of apomorphine s / ~ , she came “on” after 15 rnin and developed “no-no” head turning with nausea 10 min later. The amplitude of head turning was too great for her to maintain central visual fixation. She was instructed to rate her nausea on an analogue scale of 0-10. On three occasions when her eyes were covered up with patches, the nausea consistently increased (score of 7-8), then decreased (score of 2 4 , when they were removed. The following morning after the same dose of apomorphine, head turning again began 10 min after turning “on.” When she felt nauseated enough to vomit, her head was held to stop it from turning, and the nausea rapidly diminished. The nausea and vomiting suffered by this patient appeared to be caused by vestibular stimulation secondary to head turning and was not affected by pretreatment with domperidone. A neurootological assessment was normal with, in particular, normal caloric testing bilaterally and normal vestibuloocular reflex. She was given a vestibular sedative (promethazine) and began bromocriptine in progressively increasing doses up to 27.5 mglday. Domperidone, benzhexol, and amitriptyline were continued, and the levodopa was reduced by 18%. On this regime, her “on” period disability improved, the head turning in the “on” period was dramatically reduced and often absent, and her nausea and vomiting disappeared. This state of affairs persisted at review 2 months later. The mechanism of nausea and vomiting at the time of head-turning dyskinesia in this patient is uncertain. Vestibular stimulation alone can, if sufficiently severe, cause motion sickness (1). However, the vestibuloocular reflex is able to compensate effectively for head movements over a frequency range of up to 7 Hz (2) and may explain the clinical observation that patients with torticollis do not complain of nausea during head turning. In our patient, the amplitude of “no-no” head turning was too great for this reflex to maintain constant central fixation.
Movement Disorders, Vol. 6 , No. 1 , 1991
However, brief attempts at visual fixation on the stationary surround may have been enough to depress the stimulus to vomit, for when her eyes were covered the nausea increased. Although de novo bromocriptine monotherapy rarely induces dyskinesias, preexisting levodopa dyskinesias tend also to occur when a therapeutic dose of bromocriptine is added to, or partially, or even completely, replaces (3) levodopa. However, in some of these patients, the seventy of dyskinesias may be considerably less with bromocriptine, hinting at the possible importance of D1 receptor stimulation in their genesis. This case demonstrates that nausea and vomiting on dopaminergic therapy may not always be directly due to dopamine receptor stimulation. Instead, they may occasionally be secondary to a form of motion sickness caused by dyskinetic neck movements.
M. J. Steiger N. P. Quinn C. D. Marsden Department of Clinical Neurology Institute of Neurology London, England
References 1. Money KE. Motion sickness. Physiol Rev 1970;50:1-39. 2. Donaghy M. The cat’s vestibule-ocular reflex. J . Physiol 1980;300:337-5 1. 3. Quinn N, Illas A, Lhermitte F, Agid Y. Bromocriptine and domperidone in the treatment of Parkinson disease. Neurology 198 1 ;3 1 :662-7.
Essential Tremor Associated with Paroxysmal Kinesigenic Dystonia Dear Editor: We wish to record an unusual association of essential tremor (ET) with paroxysmal kinesigenic dystonia (PKD). A 25-year-old male medical student developed a postural tremor of the arms at the age of 18 years, which gradually increased for a year, but subsequently has remained unchanged. Three years later he developed classic PKD, precipitated by abrupt movements, sounds, or anxiety. Each attack would last for -20-30 s , characterised by painful stiffness of the left upper limb, which would then assume a dystonic posture followed by extension and, at times, lateral flexion of the neck, pouting of the lips, and dystonic posturing of the right upper limb, with no alteration of consciousness. The attacks occurred five to six times per day. We witnessed such an attack during clinical interview, when he was asked to get up and undress. Previous physicians had tried a variety of drugs including phenytoin, propanolol, chlordiazepoxide, amitriptyline, and haloperidol, but we do not know the doses or duration of such therapy. He had even been submitted to electroconvulsive treatment. Previous presumptive diagnoses had included epilepsy, hysteria, and a psychosis. Drug therapy had made him dull and de-
BRIEF COMMUNICATIONS pressed, so he had rejected all medications for the previous year. There was no family history of neurological disease. He did not use alcohol. Examination revealed no neurological abnormality, other than tremor satisfying all the criteria for ET, without any evidence of cerebellar, extrapyramidal, pyramidal, or sensory dysfunction. There was a 7-8-Hz tremor of the outstretched arms, with no tremor at rest or worsening on movement; there was no tremor of the head, jaw, trunk, or legs. Computed tomography (CT) scan of head and repeated electroencephalograms (EEGs) were normal, as were serum ceruloplasmin, copper, and thyroid hormone estimations. Phenytoin was suggested as a single drug, but he refused it, for it had not provided benefit when tried initially. He chose to do without drugs as he had learned to live with his malady.
93
To our knowledge, ET and PKD have not been reported before in a single patient. This may be a chance association, but, if not, the discovery of the gene location for one of these dominantly inherited diseases may point to where to look for that responsible for the other.
K. R. Nair R. Bhaskaran Department of Neurology Medical College Hospital Trivandrum, India C. D. Marsden University Department of Clinical Neurology Institute of Neurology The National Hospitai for Neurology and Neurosurgery London, England
Announcement
The 10th International Symposium on Parkinson's Disease will convene at the Keio Plaza Hotel in Tokyo, Japan on October 27-30, 1991. For more information contact the secretariat % Japan Convention Services, Inc., Nippon Press Center Building, 2-2-1 Uchisaiwai-cho, Chiyoda-ku, Tokyo 100 Japan; telephone 81 3 508 1213, fax 81 3 508 0820. This symposium is presented under the auspices of the World Federation of Neurology, Research Committee on Extrapyramidal Disease.
Movement Disorders, Vol. 6 , No. 1, 1991
BRIEF COMMUNICATIONS
but increasing the dosage of levodopa only worsened the head turning. At 39, she underwent a successful left stereotactic thalamotomy. However, her gait continued to deteriorate. Several attempts over the next 13 years to introduce Sinemet, Madopar, and selegiline at therapeutic doses were unsuccessful because of an increase in “no-no” head turning, which was associated with nausea and vomiting. On admission, she was taking two-hourly doses of plain levodopa totalling 1,375 mg/day, benzhexol 5 mg t.d.s., and amitriptyline 25 mg nocte. On this regime, her “on” periods were accompanied by neck dyskinesias with nausea, culminating in vomiting. In the “off” state, her King’s College Hospital disability score was 50/117 (moderately severe), Hoehn and Yahr stage IV. One tablet of Sinemet 275 after an overnight fast turned her “on,” without nausea, after 60 min. However, after a further 45 min, she developed “no-no” head turning with nausea, and vomited. We were uncertain whether the nausea and vomiting were secondary to the levodopa per se or to the levodopainduced head turning. We, therefore, gave domperidone 50 mg t.d.s. and rechallenged with levodopa 1 g. Nausea and vomiting, associated with “no-no” head turning, occurred 55 min after turning “on.” Domperidone was continued. The next morning, after 4 mg of apomorphine s / ~ , she came “on” after 15 rnin and developed “no-no” head turning with nausea 10 min later. The amplitude of head turning was too great for her to maintain central visual fixation. She was instructed to rate her nausea on an analogue scale of 0-10. On three occasions when her eyes were covered up with patches, the nausea consistently increased (score of 7-8), then decreased (score of 2 4 , when they were removed. The following morning after the same dose of apomorphine, head turning again began 10 min after turning “on.” When she felt nauseated enough to vomit, her head was held to stop it from turning, and the nausea rapidly diminished. The nausea and vomiting suffered by this patient appeared to be caused by vestibular stimulation secondary to head turning and was not affected by pretreatment with domperidone. A neurootological assessment was normal with, in particular, normal caloric testing bilaterally and normal vestibuloocular reflex. She was given a vestibular sedative (promethazine) and began bromocriptine in progressively increasing doses up to 27.5 mglday. Domperidone, benzhexol, and amitriptyline were continued, and the levodopa was reduced by 18%. On this regime, her “on” period disability improved, the head turning in the “on” period was dramatically reduced and often absent, and her nausea and vomiting disappeared. This state of affairs persisted at review 2 months later. The mechanism of nausea and vomiting at the time of head-turning dyskinesia in this patient is uncertain. Vestibular stimulation alone can, if sufficiently severe, cause motion sickness (1). However, the vestibuloocular reflex is able to compensate effectively for head movements over a frequency range of up to 7 Hz (2) and may explain the clinical observation that patients with torticollis do not complain of nausea during head turning. In our patient, the amplitude of “no-no” head turning was too great for this reflex to maintain constant central fixation.
Movement Disorders, Vol. 6 , No. 1 , 1991
However, brief attempts at visual fixation on the stationary surround may have been enough to depress the stimulus to vomit, for when her eyes were covered the nausea increased. Although de novo bromocriptine monotherapy rarely induces dyskinesias, preexisting levodopa dyskinesias tend also to occur when a therapeutic dose of bromocriptine is added to, or partially, or even completely, replaces (3) levodopa. However, in some of these patients, the seventy of dyskinesias may be considerably less with bromocriptine, hinting at the possible importance of D1 receptor stimulation in their genesis. This case demonstrates that nausea and vomiting on dopaminergic therapy may not always be directly due to dopamine receptor stimulation. Instead, they may occasionally be secondary to a form of motion sickness caused by dyskinetic neck movements.
M. J. Steiger N. P. Quinn C. D. Marsden Department of Clinical Neurology Institute of Neurology London, England
References 1. Money KE. Motion sickness. Physiol Rev 1970;50:1-39. 2. Donaghy M. The cat’s vestibule-ocular reflex. J . Physiol 1980;300:337-5 1. 3. Quinn N, Illas A, Lhermitte F, Agid Y. Bromocriptine and domperidone in the treatment of Parkinson disease. Neurology 198 1 ;3 1 :662-7.
Essential Tremor Associated with Paroxysmal Kinesigenic Dystonia Dear Editor: We wish to record an unusual association of essential tremor (ET) with paroxysmal kinesigenic dystonia (PKD). A 25-year-old male medical student developed a postural tremor of the arms at the age of 18 years, which gradually increased for a year, but subsequently has remained unchanged. Three years later he developed classic PKD, precipitated by abrupt movements, sounds, or anxiety. Each attack would last for -20-30 s , characterised by painful stiffness of the left upper limb, which would then assume a dystonic posture followed by extension and, at times, lateral flexion of the neck, pouting of the lips, and dystonic posturing of the right upper limb, with no alteration of consciousness. The attacks occurred five to six times per day. We witnessed such an attack during clinical interview, when he was asked to get up and undress. Previous physicians had tried a variety of drugs including phenytoin, propanolol, chlordiazepoxide, amitriptyline, and haloperidol, but we do not know the doses or duration of such therapy. He had even been submitted to electroconvulsive treatment. Previous presumptive diagnoses had included epilepsy, hysteria, and a psychosis. Drug therapy had made him dull and de-
BRIEF COMMUNICATIONS pressed, so he had rejected all medications for the previous year. There was no family history of neurological disease. He did not use alcohol. Examination revealed no neurological abnormality, other than tremor satisfying all the criteria for ET, without any evidence of cerebellar, extrapyramidal, pyramidal, or sensory dysfunction. There was a 7-8-Hz tremor of the outstretched arms, with no tremor at rest or worsening on movement; there was no tremor of the head, jaw, trunk, or legs. Computed tomography (CT) scan of head and repeated electroencephalograms (EEGs) were normal, as were serum ceruloplasmin, copper, and thyroid hormone estimations. Phenytoin was suggested as a single drug, but he refused it, for it had not provided benefit when tried initially. He chose to do without drugs as he had learned to live with his malady.
93
To our knowledge, ET and PKD have not been reported before in a single patient. This may be a chance association, but, if not, the discovery of the gene location for one of these dominantly inherited diseases may point to where to look for that responsible for the other.
K. R. Nair R. Bhaskaran Department of Neurology Medical College Hospital Trivandrum, India C. D. Marsden University Department of Clinical Neurology Institute of Neurology The National Hospitai for Neurology and Neurosurgery London, England
Announcement
The 10th International Symposium on Parkinson's Disease will convene at the Keio Plaza Hotel in Tokyo, Japan on October 27-30, 1991. For more information contact the secretariat % Japan Convention Services, Inc., Nippon Press Center Building, 2-2-1 Uchisaiwai-cho, Chiyoda-ku, Tokyo 100 Japan; telephone 81 3 508 1213, fax 81 3 508 0820. This symposium is presented under the auspices of the World Federation of Neurology, Research Committee on Extrapyramidal Disease.
Movement Disorders, Vol. 6 , No. 1, 1991
