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COMMUNICATIONS Burke BE, Fahn S, Mayeux R, Weinberg H, Louis K, Willner JH. Neuroleptic malignant syndrome caused by dopamine-depleting drugs in a patient with Huntington disease. Neurology 1981;31: 1022-1026. Talman WT, Florek G, Bullard DE. A hyperthermic syndrome in two subjects with acute hydrocephalus. Arch Neurol 1988;45: 1037-1040. Cox B. Kenvin R, Lee TF. Dopamine receptor in the central thermorequlatory pathways of the rat. J Physiol (Lond) 1978 ;282:47 1-483.
Levodopa-Associated Hemifacial Dystonia To the Editor: Dystonia associated with the treatment of Parkinson’s disease was noted soon after the introduction of levodopa therapy (1). As in idiopathic dystonia, levodopaassociated dystonia may be either focal, segmental, or generalized, and may involve any part of the body. Bilateral or hemidystonia involving unilateral limbs is common. We report a relatively uncommon case of isolated hemifacial dystonia as a complication of treatment with levodopa.
Case Report A 67-year-old right-handed woman developed symptoms of Parkinson’s disease 4 years ago, beginning with dragging of her left foot. She was begun on carbidopal levodopa (Sinemet) 25/100 and did well until 6 months ago, when bradykinesia, postural instability, and tremor progressed. At this time, she also developed hoarseness and a “spasm” of the left side of her face. Her levodopa dosage was increased to 200 mg three times daily. On this regimen, the parkinsonism improved slightly but the hemifacial movements remained. The abnormal movement is a slow, protracted, dystonic spasm of the left side of her face. It begins shortly after the morning dose of levodopa and lasts until midnight. If she skips her medication for a day or two, or if she awakens in the middle of the night (6 to 8 h after her last levodopa dose), the dystonia disappears. The dystonic movement causes her great discomfort but is not painful. It does not interfere with speech, swallowing, or vision. No other body parts, either cranial, limb, or trunk, are involved, nor does it involve the right side. Although her parkinsonism began on the left, examination revealed bilaterally symmetric rigidity and bradykinesia. When the dystonia abates, there is no facial weakness or synkinesis. She has no history of Bell’s palsy. A magnetic resonance imaging scan demonstrated diminished signal intensity of the substantia nigra, putamen, red nuclei, and dentate nuclei, suggesting increased fenitin deposition. There was no abnormality in the region of the facial nerve nucleus. Low-dose bromocriptine, carbamazepine, and deprenyl were of no benefit. An increase in Sinemet to 251250 three times daily caused worsening of the facial movement and new bilateral leg chorea that interfered with walking. Her levodopa dosage was reduced back to 200 mg each time, with subsequent resolution of the chorea
and some improvement in the hemifacial dystonia but with a diminution of her motor performance.
Discussion Levodopa-associated d ystonic reactions in Parkinson’s disease occur during trough, peak dose, or rising or falling plasma levodopa concentrations ( 2 ) . Off-period dystonia most commonly affects the feet (3), whereas peak-doseassociated dystonia usually involves the face symmetrically (2,3). Focal lesions (e.g., infarct, hemorrhage, or tumor) may cause secondary dystonia involving half of the face, but idiopathic or levodopa-induced facial dystonia is usually bilateral. Further, levodopa-induced hemidystonia of the limbs is common, while isolated unilateral facial dystonia in Parkinson’s disease is rarely noted. The explanation for this is speculative. As there is (a) a somatotopic rostral-to-caudal gradient of leg-to-face representation in the primate putamen (4), (b) an increasing gradient of dopamine depletion in the same distribution (3, and (c) somatotopically distinct simultaneous dyskinesias and parkinsonism in some patients, usually seen with a face-to-leg gradient of dyskinesia to parkinsonism (6), it is conceivable that the face-related receptors in the caudal putamen, particularly on the side of initial parkinsonian symptoms, might be more sensitive to dopamine stimulation than the rostravleg area, resulting in the observed phenomenon. It is possible that this pattern of dyskinesia is more common than is usually realized.
Legend to the Videotape A 67-year-old woman with Parkinson’s disease demonstrating pure left hemifacial dystonia associated with levodopa. Margery H. Mark Jacob I. Sage Department of Neurology University of Medicine a n d Dentistry of N e w Jersey Robert W o o d Johnson Medical School N e w Brunswick, N e w Jersey
References 1. Muenter MD, Sharpless NS, Tyce GM, Darley FL. Patterns
of dystonia (“I-GI” and “D-I-D”) in response to L-dopa therapy for Parkinson’s disease. Mayo Clin Proc 1977;52: 163- 174. 2. McHale DM, Sage JI, Sonsalla PK, Vitagliano D. Complex
dystonia of Parkinson’s disease: clinical features and relation to plasma levodopa profile. Clin Neuropharrnacol 1990; 13: 16k170. 3. Poewe WH, Lees AJ, Stern GM. Dystonia in Parkinson’s
disease: clinical and pharmacological features. Ann Neurol 1988;23:73-78. 4. Crutcher MD, DeLong MR. Single cell studies of the primate
putamen. I. Functional organization. Exp Brain Res 1984;53:233-234.
5 . Hornykiewicz 0 , Kish SJ. Biochemical pathophysiology of Parkinson’s disease. Adv Neurol 1986;45: 19-34. 6. Fahn S. Fluctuations of disability in Parkinson’s disease: pathophysiology. In: Marsden CD, Fahn S, eds. Movement disorders. London: Butterworth, 1982:123-145.
Muvement Disorders, Vol. 6, No.4, I991
COMMUNICATIONS Burke BE, Fahn S, Mayeux R, Weinberg H, Louis K, Willner JH. Neuroleptic malignant syndrome caused by dopamine-depleting drugs in a patient with Huntington disease. Neurology 1981;31: 1022-1026. Talman WT, Florek G, Bullard DE. A hyperthermic syndrome in two subjects with acute hydrocephalus. Arch Neurol 1988;45: 1037-1040. Cox B. Kenvin R, Lee TF. Dopamine receptor in the central thermorequlatory pathways of the rat. J Physiol (Lond) 1978 ;282:47 1-483.
Levodopa-Associated Hemifacial Dystonia To the Editor: Dystonia associated with the treatment of Parkinson’s disease was noted soon after the introduction of levodopa therapy (1). As in idiopathic dystonia, levodopaassociated dystonia may be either focal, segmental, or generalized, and may involve any part of the body. Bilateral or hemidystonia involving unilateral limbs is common. We report a relatively uncommon case of isolated hemifacial dystonia as a complication of treatment with levodopa.
Case Report A 67-year-old right-handed woman developed symptoms of Parkinson’s disease 4 years ago, beginning with dragging of her left foot. She was begun on carbidopal levodopa (Sinemet) 25/100 and did well until 6 months ago, when bradykinesia, postural instability, and tremor progressed. At this time, she also developed hoarseness and a “spasm” of the left side of her face. Her levodopa dosage was increased to 200 mg three times daily. On this regimen, the parkinsonism improved slightly but the hemifacial movements remained. The abnormal movement is a slow, protracted, dystonic spasm of the left side of her face. It begins shortly after the morning dose of levodopa and lasts until midnight. If she skips her medication for a day or two, or if she awakens in the middle of the night (6 to 8 h after her last levodopa dose), the dystonia disappears. The dystonic movement causes her great discomfort but is not painful. It does not interfere with speech, swallowing, or vision. No other body parts, either cranial, limb, or trunk, are involved, nor does it involve the right side. Although her parkinsonism began on the left, examination revealed bilaterally symmetric rigidity and bradykinesia. When the dystonia abates, there is no facial weakness or synkinesis. She has no history of Bell’s palsy. A magnetic resonance imaging scan demonstrated diminished signal intensity of the substantia nigra, putamen, red nuclei, and dentate nuclei, suggesting increased fenitin deposition. There was no abnormality in the region of the facial nerve nucleus. Low-dose bromocriptine, carbamazepine, and deprenyl were of no benefit. An increase in Sinemet to 251250 three times daily caused worsening of the facial movement and new bilateral leg chorea that interfered with walking. Her levodopa dosage was reduced back to 200 mg each time, with subsequent resolution of the chorea
and some improvement in the hemifacial dystonia but with a diminution of her motor performance.
Discussion Levodopa-associated d ystonic reactions in Parkinson’s disease occur during trough, peak dose, or rising or falling plasma levodopa concentrations ( 2 ) . Off-period dystonia most commonly affects the feet (3), whereas peak-doseassociated dystonia usually involves the face symmetrically (2,3). Focal lesions (e.g., infarct, hemorrhage, or tumor) may cause secondary dystonia involving half of the face, but idiopathic or levodopa-induced facial dystonia is usually bilateral. Further, levodopa-induced hemidystonia of the limbs is common, while isolated unilateral facial dystonia in Parkinson’s disease is rarely noted. The explanation for this is speculative. As there is (a) a somatotopic rostral-to-caudal gradient of leg-to-face representation in the primate putamen (4), (b) an increasing gradient of dopamine depletion in the same distribution (3, and (c) somatotopically distinct simultaneous dyskinesias and parkinsonism in some patients, usually seen with a face-to-leg gradient of dyskinesia to parkinsonism (6), it is conceivable that the face-related receptors in the caudal putamen, particularly on the side of initial parkinsonian symptoms, might be more sensitive to dopamine stimulation than the rostravleg area, resulting in the observed phenomenon. It is possible that this pattern of dyskinesia is more common than is usually realized.
Legend to the Videotape A 67-year-old woman with Parkinson’s disease demonstrating pure left hemifacial dystonia associated with levodopa. Margery H. Mark Jacob I. Sage Department of Neurology University of Medicine a n d Dentistry of N e w Jersey Robert W o o d Johnson Medical School N e w Brunswick, N e w Jersey
References 1. Muenter MD, Sharpless NS, Tyce GM, Darley FL. Patterns
of dystonia (“I-GI” and “D-I-D”) in response to L-dopa therapy for Parkinson’s disease. Mayo Clin Proc 1977;52: 163- 174. 2. McHale DM, Sage JI, Sonsalla PK, Vitagliano D. Complex
dystonia of Parkinson’s disease: clinical features and relation to plasma levodopa profile. Clin Neuropharrnacol 1990; 13: 16k170. 3. Poewe WH, Lees AJ, Stern GM. Dystonia in Parkinson’s
disease: clinical and pharmacological features. Ann Neurol 1988;23:73-78. 4. Crutcher MD, DeLong MR. Single cell studies of the primate
putamen. I. Functional organization. Exp Brain Res 1984;53:233-234.
5 . Hornykiewicz 0 , Kish SJ. Biochemical pathophysiology of Parkinson’s disease. Adv Neurol 1986;45: 19-34. 6. Fahn S. Fluctuations of disability in Parkinson’s disease: pathophysiology. In: Marsden CD, Fahn S, eds. Movement disorders. London: Butterworth, 1982:123-145.
Muvement Disorders, Vol. 6, No.4, I991
