Movement Disorders Vol. 5 , No. 1, 1990, pp. 89-91 0 1990 Movement Disorder Society

Letters to the Editor been diagnosed slightly, but not statistically significantly, more frequently in patients with blepharospasm than in controls, but the prevalence in both groups was very low. Thus, thyrotoxicosis occurred in only 3.4% and myxoedema in only 1.5% of our entire series of 264 patients with blepharospasm. Therefore, a strong link between blepharospasm and thyroid dysfunction seems unlikely, and the reported association may be caused by the female preponderance of both diseases. F. Grandas Servicio de Neurologia Hospital General Gregorio Maranon Madrid, Spain

To the Editor:

The cause of blepharospasm is unknown in most cases. However, immune-mediated mechanisms have been suggested, based on the presence of various autoimmune disorders in a number of patients with blepharospasm (1-5). In particular, blepharospasm has been reported to be associated with thyroid abnormalities (2,5). We have compared the prevalence of known thyroid disorders in a series of 264 patients (170 women and 94 men) with blepharospasm (mean age f 1 SD, 55.8 f 12.5 years; range 11-81 years) with that in a general population community survey of 2,779 individuals (1,494 women and 1,285 men) conducted in the U.K. (mean age 2 1 SD, 47.1 f 16.4 years; range 18-75 years) (6). Seventeen of the patients with blepharospasm (6.4%) had some thyroid disorder diagnosed from several months to 20 years before the onset of their cranial dystonia. Seven women (4.1%) and two men (2.1%) with blepharospasm had been diagnosed as thyrotoxic, as compared to 2.3% of women and 0.23% of men in the community sample. This difference in women did not reach statistical significance using x2 analysis; the very small numbers of men affected did not allow for statistical analysis. Four women (2.4%) with blepharospasm had been diagnose& and treated for myxoedema, as compared to 1.5% of women in the community sample, a difference that again was not statistically significant. No men with blepharospasm had myxoedema. Two women (1.2%) with blepharospasm (but no men) had thyroid nodules, as compared to 5.3% of women in the community survey. One woman (0.6%) with blepharospasm (but no men) had a goitre, as compared to 6.9% of women with obvious (palpable and visible) goitre in the community sample. One man (1, 1%) with blepharospasm (but no women) had a thyroid carcinoma, but there were no cases in the community sample. The higher prevalence of thyroid nodules and goitre in the community sample is probably because all subjects in that survey had their thyroid gland specifically palpated, whereas in our blepharospasm sample this was not routinely done. On the other hand, the figures for the prevalence of thyrotoxicosis and myxoedema should be comparable, as the community figures cited are for those previously diagnosed and treated, and ignores the detection of new cases by specific endocrine screening in the course of the study. Because the mean age of the blepharospasm group is 8.7 years greater than that of the subjects in the community survey, the figure for previously diagnosed and treated thyroid disease in the latter may be artificially low because of unequal patient ages. If one could correct for this difference, it would in fact reduce any difference between the two groups. We conclude that thyroid disease occurs no more frequently in patients with blepharospasm than in the community in general. Thyrotoxicosis and myxoedema had

N. Quinn, J. Elston, C . D. Marsden University Department of Clinical Neurology Institute of Neurology The National Hospital for Nervous Diseases London, England

REFERENCES 1 . Ashizawa T, Patten BM, Jankovic J. Meige’s syndrome. South Med J 1980;73:863-6. 2. Jankovic J, Ford J. Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings. Ann Neurol 1983;13:402-11. 3. Jankovic J, Patten BM. Blepharospasm and autoimmunediseases. Movement Disorders 1987;2:15M3. 4. Kurlan R, Jankovic J, Rubin A, Patten B, Griggs R, Shoulson 1. Coexistent Meige’s syndrome and myasthenia gravis. Arch Neurol 1987;44:1057-60. 5 . Nutt JG, Carter J, DeGarmo P, Hammerstad JP. Meige syndrome and thyroid dysfunction. Neurology 1984;34(suppl 1):222. 6 . Tunbridge WM, Evered DC, Hall R, et al. The spectrum of thyroid disease in a community: the Whickham survey. Clin Endocrinol 1977;7:481-93.

To the Editor: Congratulations to Burke et al. for an excellent survey of tardive akathisia (1). In their article they make reference to our original study on opioids in akathisia (2). Based on further patient observation (3), we now agree that opioids have little role to play in the treatment of tardive akathisia. However, the opioids do remain a useful treatment for acutely akathitic patients who, for psychiatric reasons, cannot have their neuroleptic therapy stopped (2,3). We have also informally tried opioid therapy in the movement disorders associated with Parkinson’s disease, essential tremor, idiopathic torsion dystonia, Tourette’s syndrome, essential myoclonus, tardive dyskinesia, and Huntington’s chorea and have found it to be of no clinical value in these situations. However, one patient taking Talwin (pentazocine) did have a worsening

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LETTERS TO THE EDITOR

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of Tourette’s syndrome after opiate withdrawal, in a manner similar to the patient reported by Lichter et al. (4). Opioid therapy in our experience is useful in the following clinical situations: (a) acute neuroleptic-induced akathisia (2,3); (b) idiopathic restless leg syndrome and its associated paresthesias, motor restlessness, resting myoclonus while awake, periodic movements in sleep and sleep disturbances (5,6); (c) periodic movements in sleep and sleep disturbances without restless legs (7); (d) uremic restless legs [ S . Chokroverty-two patients-unpublished observations; also see (8)l. Arthur S. Walters Wayne Hening Sudhansu Chokroverty D e p a r t m e n t of Neurology University of Medicine and Dentistry of New Jersey-Robert W o o d Johnson Medical School and L y o n s Veterans Administration Medical C e n t e r

REFERENCES 1. Burke RE, Kang UJ, Jankovic J, Miller LG, Fahn S. Tardive

akathisia: an analysis of clinical features and response to open therapeutic trials. Movement Disorders 1989;4:157-75. 2. Walters A, Hening W, Chokroverty S, Fahn S. Opioid responsiveness in patients with neuroleptic-induced akathisia. Movement Disorders 1986;1:119-27. 3. Walters AS, Hening WA. Opioids a better treatment for acute than tardive akathisia: possible role for the endogenous opiate system in neuroleptic-induced akathisia. Med Hypotheses 1989;28:1-2. 4. Lichter D, Majumdar L, Kurlan R. Opiate withdrawal unmasks Tourette’s syndrome. Clin Neurophurmucot 1988;

responsive to levodopa, as a different condition from Barbeau’s pure akinesia. “Nonresponsive to levodopa” is included in the definition of the syndrome in our cases. Ten years later Imai et al. (3) reported as follows, “To date, 20 cases are known, some followed for as long as 10 years; but no case has yet come to autopsy. L - D O ~[i.e., S L-threo-DOPS] has had mild-to-moderate effect on this condition.” Unfortunately, ‘‘L-DoPs” was written “LDopas” by printing error, and Quinn et al. have taken “L-Dopas” to mean “levodopa.” To clear up their misunderstanding, we would like to state again: Lthreo-DOPS has mild-to-moderate effect on some of our cases of pure akinesia or freezing symptom, which is nonresponsive to levodopa. A single case of Quinn et al. was responsive to levodopa with “on-off’ fluctuations. There was no rigidity in either the “on” or the “off” condition, and there was constant severe freezing of gait when “off,” and very rare, unpredictable freezing episodes when “on.” They did not mention anything about the washout of levodopa. Therefore, the question arises as to whether the rigidity of their case might be masked by levodopa and hypotonic freezing might be due to long-term levodopa therapy (5,6). In summary, the pure akinesia by Quinn et al. (1) would be the same as the pure akinesia of Barbeau (4)and different from that reported by us (2,3). Hisamasa Imai Hirotaro Narabayashi D e p a r t m e n t of N e u r o l o g y Juntendo University School of Medicine Tokyo, J a p a n

11:559-64. 5 . Walters A, Hening W, CBtC L, Fahn S. Dominantly inherited

restless legs with myoclonus and periodic movements of sleep: a syndrome related to the endogenous opiates? Adv Neurol 1986;43:309-19. 6. Hening WA, Walters A, Kavey N, Gidro-Frank S, CBtt L , Fahn S . Dyskinesias while awake and periodic movements in sleep in restless legs syndrome: treatment with opioids. Neurology 1986;36:1363-6. 7. Kavey N, Walters AS, Hening W, Gidro-Frank S. Opioid treatment of periodic movements in sleep in patients without restless legs. Neuropeptides 1988;ll:1814. 8. Eqawa I, Sugita Y, Teshima Y , et al. A polysomnographic study of restless legs syndrome in patients undergoing hemodialysis treatment. Sleep Res 1987;16:330.

To the Editor: Quinn et al. (1) reported a case of pure akinesia due to Lewy body Parkinson’s disease with pathology. They cited our papers on pure akinesia from Japan (2,3) and discussed the differences between their single case and our series of patients. Barbeau (4) first described 50 cases of pure akinesia without pgidity and tremor. “Pure” means, of course, “without rigidity and tremor. ” Barbeau’s cases of pure akinesia were responsive to levodopa and were called akinesia due to striatal dopamine deficiency. Narabayashi et al. ( 2 ) reported four cases of pure akinesia, i.e., freezing symptom alone, without rigidity and tremor and non-

Movement Disorders, Vol. 5 , N o . 1 , 1990

REFERENCES I . Quinn NP, Luthert P, Honavar M, Marsden CD. Pure akinesia due to Lewy body Parkinson’s disease: a case with pathology. Movement Disorders 1989;4:85-9. 2. Narabayashi H, Imai H, Yokochi M, Hirayama K, Nakamura R. Cases of pure akinesia without rigidity and tremor and with no effect by L-Dopa therapy. In: Birkmayer W, Hornykiewicz 0, eds. Advances in parkinsonism. Basle: Editiones Roche, 1976:335-42. 3. Imai H, Narabayashi H, Sakata E. “Pure akinesia” and the later added supranuclear ophthalmoplegia. Adv Neurol 1986;451207-12. 4. Barbeau A. Contribution of levodopa therapy to the neuropharmacology of akinesia. In: Siegfried J, ed. Parkinson’s disease. Bern: Hans Huber, 1972:151-74. 5. Ambani LM, Van Woert MH. Start hesitation: a side effect of long-term levodopa therapy. N Engl J Med 1973; 288: 11 13-5. 6. Barbeau A. Six years of high-level levodopa therapy in severely akinetic parkinsonian patients. Arch Neurol 1976; 33:333-8.

To the Editor:

We are grateful to Drs. Imai and Narabayashi for correcting the misunderstanding arising from the misprint in their article. Barbeau was of course the first to describe pure akinesia, which he found in 15% of his clinic population with Parkinson’s disease. He also emphasised

LETTERS TO THE EDITOR “that this subgroup of patients is in itself heterogeneous as regards duration and speed of development of the illness. Some patients have only minimal symptoms, others in the space of only a very few years have become completely invalid, still without developing tremor or rigidity.” The main point of our article was simply to put on record that this picture can indeed occur in pathologically proven cases of Lewy body Parkinson’s disease. However, we fully agree that the levodopa-unresponsive cases of the authors are likely to represent another disease, or diseases, perhaps multiple system atrophy or Steele-Richardson-Olszewskisyndrome. In this regard, it is worth drawing attention to a Japanese paper by Yuasa et al. (1) in which two of five cases of levodopaunresponsive pure akinesia came to autopsy. Both had pathologically proven progressive supranuclear palsy. Finally, in regard to our own patient, the lack of rigidity was confirmed in both fully developed “off’ periods and

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after overnight levodopa withdrawal, so we do not believe that this was due to masking by levodopa. As with a number of late features of parkinsonism, it is difficult to be certain whether freezing is due to progression of the underlying disease or to long-term levodopa therapy. We favour, as did Barbeau, the former. Niall Quinn C. David Marsden University Department of Clinical Neurology Institute of Neurology London, England

REFERENCE 1. Yuasa T, Mori S, Hayashi H, Takahashi H, Honma Y. Pro-

gressive supranuclear palsy with pure akinesia as an initial symptom. Neurol Med 1987;26:4&7.

Movement Disorders, Vol. 5, No. I , 1990