BRIEF COMMUNICATIONS Apraxia of Eyelid Opening Secondary to Right Hemisphere Infarction James C. Johnston, MD,' Daniel M. Rosenbaum, MD,I Carmela M. Picone, MD, and James C. Grotta, MD ~~ A variety of eyelid movement abnormalities have been attributed to lesions of the central nervous system. Apraxia of lid movements, and especially of lid opening, has received the least attention. We present 2 cases of lid opening apraxia and propose that this abnormality may be due to right hemisphere dysfunction. Johnston JC, Rosenbaum DM, Picone CM, Grotta JC. Apraxia of eyelid opening secondary to right hemisphere infarction. Ann Neurol 1989;25:622-624 Over the last century, a variety of eyelid movement abnormalities have been attributed to lesions of the central nervous system, including ptosis, motor impersistence, and apraxia of lid movements. T h e latter disorder has received the least attention. Apraxia of lid closure was first described by Lewandowsky {l] in 1907, but only a few cases have been reported since {2, 31. We present 2 more cases, both with right hemisphere infarction. (Since this manuscript was submitted, we have identified a third case, which is not discussed here.) The following criteria for lid opening apraxia, modified from Lepore and Duvoisin {4], were present in both of our patients: inability to initiate lid opening voluntarily, despite frontalis muscle contraction; normal spontaneous lid elevation; n o evidence of concurrent orbicularis oculi muscle contraction; n o ocular motor nerve dysfunction; n o ocular sympathetic dysfunction; and no ocular myopathy. Case Reports Patient 1 A 68-year-old right-handed woman previously in good health had acute onset of left-sided weakness. She was alert and oriented with fluent but dysarthric speech, left-sided neglect, and visual-spatial dysfunction. She had no evidence of From the Department of Neurology, University of Texas School of Medicine, Houston, TX. Received May 20, 1988, and in revised form Nov 7. Accepted for publication Dec 3, 1988. Address correspondence to Dr Johnston, Eastern Idaho Neurology Clinic, 2860 Channing Way, Suite 217, Idaho Falls, ID 83404. "Current address: Eastern Idaho Neurology Clinic, Idaho Falls, ID. tCurrent address: Stern Comprehensive Stroke Center, Montefiore Medical Center, Bronx, NY. orofacial or limb apraxia. She had central left facial weakness, a left hemiparesis, and left hemisensory deficit. The left plantar response was extensor, the right flexor. The pupils were 3 mm, symmetrical, and equally reactive. Extraocular movements were full, although she exhibited a right conjugate gaze preference. She rarely opened her eyes spontaneously, even when engaged in conversation; however, when her eyes were open, there was no evidence of ptosis. Closure of the eye was normal. Reflex lid opening was intact. The patient was unable to open her eyes on command, despite frontalis muscle contraction. She was aware of this deficit, replying that she did not know why she was unable to perform the task. Occasionally she used her nonparetic hand to elevate the lids. The patient improved over the next few days. By the fourth hospital day, she was able to open her lids on command without hesitation. Also during this time, neglect and visual-spatial dysfunction resolved. The motor and sensory deficits persisted. Serial computed tomography of the brain revealed an infarction in the right middle cerebral artery distribution (Fig 1). Patient 2 An 81-year-old right-handed man with a history of atrial fibrillation but no prior neurological events experienced the acute onset of left-sided paralysis. He was alert and oriented, with fluent but dysarthric speech, left-sided neglect, visualspatial dysfunction, and a left homonymous hemianopsia. He had no evidence of orofacial or limb apraxia. He had central left facial weakness, left hemiplegia, and left hemisensory deficit. The left plantar response was extensor, the right flexor. The pupils were 4 mm, symmetrical, and equally reactive. Extraocular movements were full, although he exhibited a right conjugate gaze preference. Although the patient kept his eyes closed most of the time, he at times opened them spontaneously. There was no evidence of ptosis. Closure of the eyes was normal. Reflex lid opening was intact. He was unable to open his eyes on command, despite frontalis muscle contraction. He was aware of this difficulty, replying that he did not know why he was unable to perform this task. The patient slowly improved, but it was not until the end of 1 month that he was able to open his eyes on command. During this time, parietal lobe dysfunction also resolved, but motor and sensory deficits persisted. Serial computed tomography of the brain revealed an infarction in the right middle cerebral artery distribution with hemorrhagic transformation (Fig 2). Discussion Both of our patients exhibited lid opening apraxia as defined by the above-mentioned criteria. They had difficulty initiating lid opening, even when alert and interacting with other people. This eventually resolved, as did other deficits of cortical function referable to the right parietal lobe. T h e Table summarizes the reported cases of lid opening apraxia. Extrapyramidal disease was responsible for cases reported by Schilder 151, Goldstein and Cogan {b],Dehaene {7], and Lepore and Duvoisin {4]. 622 Copyright 0 1989 by the American Neurological Association Fig I. Computed tomograph of Patient 1 one week after presentation. Patient’s right is viewer’s left. F i g 2. Computed tornograph of Patient 2 one week aferpresentation. Patient’s right is viewer’s left. Nashold and Gillis [8} reported 1 patient who developed lid opening apraxia after placement of a thermal lesion in Forel’s field H and the red nucleus for involuntary movements. Our patients had no evidence of extrapyramidal dysfunction. The hypothesis that the cortex influences lid movements is not new. Early animal studies by Ferrier 191, Leyton and Sherrington 1101, and others established this concept, and Penfield and Rasmussen 1111 evoked lid opening by stimulating awake humans in the prefrontal as well as more posterior cortical regions. Of all lid movements, ptosis has most frequently been associated with cortical lesions. While many early cases of ptosis were simply attributed to a mass effect, Marquez 1121 suggested that ptosis may be due to supranuclear disruption of ocular motor nerve pathways produced by lesions of the cerebral cortex or internal capsule. Years later, Caplan fl31 described 13 cases of unilateral or bilateral ptosis after hemispheric vascular events. None of his patients had apraxia. Other lid abnormalities including reflex blepharospasm [ 141 and motor impersistence 115, 161 have been associated with cortical lesions. Additionally, Walsh and Hoyt El71 reported a case of unilateral ptosis, contralateral to a temporal lobe epileptic focus. Cortical lesions causing apraxia of lid movements, in particular of lid opening, have been much less frequently reported. Riese 1181 described a patient who was unable to initiate lid elevation after a missile wound affecting both hemispheres. Nutt 1191 described 2 stroke patients with difficulty initiating lid Reported Cases of Apraxia of Eyelid Opening Author Schilder (1927) E51 Riese (1930) 1181 Goldstein and Logan (1965) E61 Nashold and Gillis (1967) C81 Nutt (1977) El91 Colombo et al. (1982) No. of Patients Pathological Findings 2 1 4 Extrapyramidal Cortical (bilateral) Extrapyramidal 1 Forel’s H, red nucleus 2 Cortical (bilateral) Cortical (bilateral) 1 rzoi Dehaene (1984) r71 Lepore and Duvoisin (1985) C41 De Renzie et al (1986) 1211 3 6 1 Extrapyramidal Extrapyramidal Cortical (right hemisphere) opening, but both also had bilateral ptosis. These patients had bilateral cortical disease, which the author stressed was necessary for the phenomenon of lid opening apraxia to occur. Colombo and colleagues 1201 described 1 patient with lid opening apraxia who had bilateral cortical infarctions. Our patients exhibited lid opening apraxia without other ocular or lid abnormalities, and the clinical and radiographic examinations indicated unilateral right hemispheric cortical involvement. De Renzi and colleagues [21} described several lid abnormalities in patients with hemispheric vascular lesions, and noted l Brief Communication: Johnston et al: Apraxia of Eyelid Opening 623 patient with right brain disease who was unable to initiate lid elevation. However, little other clinical information was provided. Our cases do not identify the exact region responsible for lid opening apraxia, since the area involved by each infarct was quite extensive. However, as the apraxia resolved coincident with other signs of right parietal lobe dysfunction, we speculate that the same regions involved with nondominant cortical function are responsible for lid opening apraxia. These cases suggest that eyelid opening apraxia may be due to right parietal lobe cortical dysfunction. The authors wish to thank Betty Cobb for her assistance in the preparation of this article. References 1. Lewandowsky M. Ueber apraxie dyes lidschlusses. Klin Wochenschr 1907;44:921-923 2. Rosati G, DeBastiani P, Granieri E, Agnetti V. Voluntary lid closure inability-release of a compulsive reaction to the exploration of the environment. Arch Psychiatr Nervenkr 1978; 226(1):11-17 3. Russel RWR. Supranuclear palsy of eyelid closure. Brain 1980;108:71-82 4. Lepore FE, Duvoisin RC. Apraxia of eyelid opening: an involuntary levator inhibition. Neurology 1985;35:423-427 5. Schilder P. Neuer beitrag zur pathologie der lidbewegungen. Dtsch Z Nervenheilk 1927;98:161-168 6. Goldstein JE, Cogan DG. Apraxia of lid opening. Arch Ophthalmol 1965;73: 15 5- 159 7. Dehaene I. Apraxia of eyelid opening in progressive supranuclear palsy. Ann Neurol 1984;15(1):115-116 8. Nashold BS Jr, Gillis JP Jr. Ocular signs from brain stimulation and lesions. Arch Ophthalmol 1967;77:609-618 9 . Ferrier D. Experiments on the brains of monkeys. Proc R SOC Lond 1875;23:409-432 10. Leyton ASF, Sherrington CS. Observations on the excitable cortex of the chimpanzee, orang-utan and gorilla. Q J Exp Physiol 1917;11:135-22 7 11. Penfield W, Rasmussen T. The cerebral cortex of man, vol 75. New York Macmillan, 1950:5 1-52 12. Marquez M. La lendidura palpebral normal y patologica. Medicinya Ibera, Madrid 1936;1:209-222 13. Caplan LR.Ptosis. J Neurol Neurosurg Psychiatry 1974;37:1-7 14. Fisher CM. Reflex blepharospasm. Neurology 1963;13:77-78 15. Fisher CM. Left hemiplegia and motor impersistence. J Nerv Ment Dis 1956;1233(3):201-2 18 16. Joynt RJ, Benton AL, Fogel ML. Behavioral and pathological correlates of motor impersistence. Neurology 1962;12:87688 1 17. Walsh FB, Hoyt WF. Clinical neurophthalmology. 3rd ed. Baltimore: Williams & Wilkins, 1969 18. Riese W. Apraxie der lidoffnung. J Psych Neurol Lpz 1930; 40~347-355 19. Nutt JG. Lid abnormalities secondary to cerebral hemisphere lesions. Ann Neurol 1977;1:149-151 20. Colombo A, De Renzi E, Gibertani M. Eyelid movement disorders following unilateral hemispheric stroke. Ital J Neurol Sci 1982;3(1):25-30 21. De Renzi E, Gentilini M, Bazolli C. Eyelid movement disorders and motor impersistence in acute hemispheric disease. NeurolO ~ Y1986;36:414-418 Induction of Aromatic-L-Amino Acid Decarboxylase by Decarbox&se Inhibitors in Idiopathic-Parkinsonism F. Boomsma, PhD,"J. D. Meerwaldt, MD,t A. J. Man in 't Veld, MD," A. Hovestadt, MD,t and M. A. D. H. Schalekamp, MD" We evaluated the effect of administration of L-dopa, alone or in combination with a peripheral decarboxylase inhibitor, on plasma levels of aromatic-L-amino acid decarboxylase (ALAAD). After single-dose administration of L-dopa plus benserazide (Madopar) in healthy subjects and in chronically treated patients with parkinsonism, plasma ALAAD followed for 2 to 3 hours fell, but returned to predosing levels within 90 minutes. Four groups of patients with idiopathic parkinsonism were studied during chronic treatment: Group I, no L-dopa treatment (n = 31); Group 11, L-dopa alone (n = 15); Group 111,L-dopa plus benserazide (n = 28); and Group IV, L-dopa plus carbidopa (Sinemet, n = 30). Plasma ALAAD 2 hours after dosing was normal in Groups I and 11. ALAAD was increased threefold in Groups 111 and IV, suggesting induction of ALAAD by the coadministration of a peripheral decarboxylase inhibitor. In a study of 3 patients in whom L-dopdbenserazide was started, plasma ALAAD rose gradually over 3 to 4 weeks. Further detailed pharmacokinetic studies of Ldopa, dopamine, and ALAAD in plasma and cerebrospinal fluid are required to determine if the apparent ALAAD induction by a peripheral decarboxylase inhibitor may be related to the loss of clinical efficacy of combination therapy in some patients and how it is related to end-of-dose deterioration and on-off phenomena. Boomsma F, Meerwaldt JD, Man in 't Veld AJ, Hovestadt A, Schalekamp MADH. Induction of aromatic-L-amino acid decarboxylase by decarboxylase inhibitors in idiopathic parkinsonism. Ann Neurol 1989;25:624-628 In patients suffering from Parkinson's disease L-dopa is often administered combined with so-called peripheral decarboxylase inhibitors (PDIs). PDIs inhibit the conFrom the Departments of *Internal Medicine I and ?Neurology, University Hospital Dijkzigt, Erasrnus University, Rotterdam, The Netherlands. Received Sep 16, 1988, and in revised form Nov 14. Accepted for publication Dec 3, 1988. Address correpondence to Dr Boomsma, Dept. of Internal Medicine I, Erasmus University, Dr Molewaterplein 40, 3015 G D Rotterdam, The Netherlands. 624 Copyright 0 1989 by the American Neurological Association