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Botulinum Therapy for Apraxia of Eyelid Opening
718
AMERICAN JOURNAL OF OPHTHALMOLOGY
We hope that calling attention to the histopathologic features of subconjunctival corticosteroid depots will lessen errors and delays in diagnosis.
May, 1987
Inquiries to Barrett Katz, M.D., Director, NeuroOphtlmlmology Unit, Department of Ophthalmology, UCSD Eye Center M-018, La jolla, CA 92093. Apraxia of eyelid opening is a nonparalytic motor abnormality of eyelid elevation. It is characterized by a transitory inability to initiate eyelid opening, vigorous frontalis muscle con traction without apparent orbicularis muscle contraction, and the absence of oculomotor or ocular sympathetic dysfunction, or ocular myopathy. 1 Whether it is a true apraxia (incapacity of deliberate movement under some circum stances that can be made under others) is de batable. 2 It has also been regarded as an akinesia of eyelid function, and an involuntary inhibition of the levator muscles. Apraxia of eyelid opening can be differentiated from blepharospasm by the absence of Charcot's sign (forced lowering of the eyebrows beneath the superior orbital margins). Botulinum A toxin has been successfully used as therapy for blepharospasm 3 and associ-
ated syndromes. It inhibits conduction in pe ripheral nerves by presynaptic blockade, pre venting the release of acetylcholine by disrupting calcium ion metabolism in nerve terminals. 4 Its success has been based upon ensuing local weakening of the orbicularis oculi muscle after local use of the agent around the eye. We have successfully treated a patient with apraxia of eyelid opening, with botulinum injections. A 68-year-old man had been followed up for four years. He had sporadic difficulty initiating eyelid opening, which was characterized by varying degrees of levator and orbicularis mus cle relaxation accompanied by vigorous front alis contraction (Fig. 1). Oculomotor innervation was normal, as was sympathetic functioning. No evidence of a myopathic proc ess was present. The patient had signs of extrapyramidal dysfunction, though no involuntary orofacial-cervical movements or supranuclear disorder of gaze. He denied exposure to neuroleptic medication. There was no prior central nervous system disease or family history of movement disorder. He had increased disabili ty because of cosmetic deformity and function al blindness. We injected purified botulinum A toxin3 into the immediate supraorbital frontalis muscu lature, after a trial of normal saline injec tions demonstrated no improvement. The botulinum-induced paresis of the frontalis muscle was accompanied by an increased abili ty to open both eyelids voluntarily (Fig. 2). This effect lasted as long as the paresis of the front alis muscle, generally lasting three months. Repeated therapy with titrating dosages dem onstrated clinical success with five injection
Fig. 1 (Katz and Rosenberg). Pretreatment appear ance of patient, with frontalis muscle contraction and levator muscle relaxation.
Fig. 2 (Katz and Rosenberg). Posttreatment ap pearance of patient, with paretic frontalis muscle, more normal eyebrow position, and open eyelids.
Botulinum Therapy for Apraxia of Eyelid Opening Barrett Katz, M . D . , and Jay H. Rosenberg, M . D . Departments of Ophthalmology (B.K) and Neurosciences (B.K. and J.H.R.), University of California, San Diego.
Vol. 103, No. 5
Letters to the Journal
sites of 5.0 units each, placed above each eye brow. The patient improved both subjectively and objectively, and showed no signs of de creased responsiveness to or complication of therapy (14 months of follow-up). Type A botulinum toxin injection proved a safe and efficacious treatment for the tempo rary relief of visually incapacitating apraxia of eyelid opening, when injected into the frontalis muscle of our affected patient. The neuroanatomic basis of apraxia of eyelid opening and the pathophysiologic explanation of this patient's success are still unknown. Previous reports of treatment failure in this condition with levodopa and 5-hydroxytrytophan 5 lead us to con sider further clinical trials.
References 1. Lepore, F. E., and Duvoisin, R. C : "Apraxia" of eyelid opening. An involuntary levator inhibition. Neurology 35:423, 1985. 2. Liepmann, H.: Das krankheitsbild der apraxie ("Motorischen Asymbolie"). Berlin, S. Karger, 1900. 3. Scott, A. B., Kennedy, R. A., and Stubbs, H. A.: Botulinum A toxin injection as a treatment for blepharospasm. Arch. Ophthalmol. 103:347, 1985. 4. Kuo, I., Drachman, D. B., and Price, D. L.: Botulinum toxin. Mechanism of presynaptic block ade. Science 193:1256, 1976. 5. Tokigucki, S., Natsukawa, M., Kawase, Y., Hayashi, H., and Tsukada, Y.: Pure akinesia with apraxia of lid-opening. Rinsho Shinkeigaku 18:192, 1978.
719
healthy, full-term infant with typical persistent hyperplastic primary vitreous in one eye and retinopathy of prematurity-like changes in the other eye, born to a mother whose pregnancy was complicated by regular cocaine use during the entire nine months of her pregnancy. A 3,360-g infant boy was born at 40 weeks of gestation by normal vaginal delivery to a 39year-old woman. The apgar score was 8, and the infant required no supplemental oxygen therapy. At 10 weeks of age, the child was examined for apparent microphthalmos of the right eye. A presumptive diagnosis of persistent hyper plastic primary vitreous was made. At 12 weeks of age, the child underwent an examination under anesthesia that disclosed a microphthalmic right globe with a corneal diameter of 9.0 mm. Ophthalmoscopic examination disclosed a fibrovascular stalk extending from the optic nerve head to the posterior surface of the lens. A computed tomographic scan (Fig. 1) con firmed the diagnosis of persistent hyperplastic primary vitreous and failed to show any calcifi cation suggestive of a retinoblastoma. Exami nation of the patient's left eye showed a normal corneal diameter of 10.5 mm, and a normal anterior segment. Ophthalmoscopic examina tion, however, showed changes suggestive of retinopathy of prematurity (Fig. 2). The periph eral retina was avascular for 360 degrees and was associated with a shallow temporal ridge. There were a few new vessels growing over the ridge at the 5:00 o'clock position. Additionally, there was a circumferential vitreous condensa tion more posterior to this in an area of vascu-
Retinopathy of Prematurity-Like Fundus and Persistent Hyperplastic Primary Vitreous Associated With Maternal Cocaine Use Michael P. Teske, M.D., and Michael T. Trese, M.D. Kresge Eye Institute. Inquires to Michael T. Trese, M.D., Kresge Ei/e Institute, 3994 John R, Detroit, MI 48201. Recent reports of cocaine use by women dur ing pregnancy have disclosed an increased inci dence of spontaneous abortions, abruptio pla centae, impairment of neonatal interactive and state organization abilities, 1 and perinatal cere bral infarction. 2 We examined an otherwise
Fig. 1 (Teske and Trese). Orbital computed tomog raphy demonstrating microphthalmia and fibrovas cular stalk connecting the optic nerve to the posterior lens surface typical of persistent hyperplastic pri mary vitreous. Left globe appears normal.
AMERICAN JOURNAL OF OPHTHALMOLOGY
We hope that calling attention to the histopathologic features of subconjunctival corticosteroid depots will lessen errors and delays in diagnosis.
May, 1987
Inquiries to Barrett Katz, M.D., Director, NeuroOphtlmlmology Unit, Department of Ophthalmology, UCSD Eye Center M-018, La jolla, CA 92093. Apraxia of eyelid opening is a nonparalytic motor abnormality of eyelid elevation. It is characterized by a transitory inability to initiate eyelid opening, vigorous frontalis muscle con traction without apparent orbicularis muscle contraction, and the absence of oculomotor or ocular sympathetic dysfunction, or ocular myopathy. 1 Whether it is a true apraxia (incapacity of deliberate movement under some circum stances that can be made under others) is de batable. 2 It has also been regarded as an akinesia of eyelid function, and an involuntary inhibition of the levator muscles. Apraxia of eyelid opening can be differentiated from blepharospasm by the absence of Charcot's sign (forced lowering of the eyebrows beneath the superior orbital margins). Botulinum A toxin has been successfully used as therapy for blepharospasm 3 and associ-
ated syndromes. It inhibits conduction in pe ripheral nerves by presynaptic blockade, pre venting the release of acetylcholine by disrupting calcium ion metabolism in nerve terminals. 4 Its success has been based upon ensuing local weakening of the orbicularis oculi muscle after local use of the agent around the eye. We have successfully treated a patient with apraxia of eyelid opening, with botulinum injections. A 68-year-old man had been followed up for four years. He had sporadic difficulty initiating eyelid opening, which was characterized by varying degrees of levator and orbicularis mus cle relaxation accompanied by vigorous front alis contraction (Fig. 1). Oculomotor innervation was normal, as was sympathetic functioning. No evidence of a myopathic proc ess was present. The patient had signs of extrapyramidal dysfunction, though no involuntary orofacial-cervical movements or supranuclear disorder of gaze. He denied exposure to neuroleptic medication. There was no prior central nervous system disease or family history of movement disorder. He had increased disabili ty because of cosmetic deformity and function al blindness. We injected purified botulinum A toxin3 into the immediate supraorbital frontalis muscu lature, after a trial of normal saline injec tions demonstrated no improvement. The botulinum-induced paresis of the frontalis muscle was accompanied by an increased abili ty to open both eyelids voluntarily (Fig. 2). This effect lasted as long as the paresis of the front alis muscle, generally lasting three months. Repeated therapy with titrating dosages dem onstrated clinical success with five injection
Fig. 1 (Katz and Rosenberg). Pretreatment appear ance of patient, with frontalis muscle contraction and levator muscle relaxation.
Fig. 2 (Katz and Rosenberg). Posttreatment ap pearance of patient, with paretic frontalis muscle, more normal eyebrow position, and open eyelids.
Botulinum Therapy for Apraxia of Eyelid Opening Barrett Katz, M . D . , and Jay H. Rosenberg, M . D . Departments of Ophthalmology (B.K) and Neurosciences (B.K. and J.H.R.), University of California, San Diego.
Vol. 103, No. 5
Letters to the Journal
sites of 5.0 units each, placed above each eye brow. The patient improved both subjectively and objectively, and showed no signs of de creased responsiveness to or complication of therapy (14 months of follow-up). Type A botulinum toxin injection proved a safe and efficacious treatment for the tempo rary relief of visually incapacitating apraxia of eyelid opening, when injected into the frontalis muscle of our affected patient. The neuroanatomic basis of apraxia of eyelid opening and the pathophysiologic explanation of this patient's success are still unknown. Previous reports of treatment failure in this condition with levodopa and 5-hydroxytrytophan 5 lead us to con sider further clinical trials.
References 1. Lepore, F. E., and Duvoisin, R. C : "Apraxia" of eyelid opening. An involuntary levator inhibition. Neurology 35:423, 1985. 2. Liepmann, H.: Das krankheitsbild der apraxie ("Motorischen Asymbolie"). Berlin, S. Karger, 1900. 3. Scott, A. B., Kennedy, R. A., and Stubbs, H. A.: Botulinum A toxin injection as a treatment for blepharospasm. Arch. Ophthalmol. 103:347, 1985. 4. Kuo, I., Drachman, D. B., and Price, D. L.: Botulinum toxin. Mechanism of presynaptic block ade. Science 193:1256, 1976. 5. Tokigucki, S., Natsukawa, M., Kawase, Y., Hayashi, H., and Tsukada, Y.: Pure akinesia with apraxia of lid-opening. Rinsho Shinkeigaku 18:192, 1978.
719
healthy, full-term infant with typical persistent hyperplastic primary vitreous in one eye and retinopathy of prematurity-like changes in the other eye, born to a mother whose pregnancy was complicated by regular cocaine use during the entire nine months of her pregnancy. A 3,360-g infant boy was born at 40 weeks of gestation by normal vaginal delivery to a 39year-old woman. The apgar score was 8, and the infant required no supplemental oxygen therapy. At 10 weeks of age, the child was examined for apparent microphthalmos of the right eye. A presumptive diagnosis of persistent hyper plastic primary vitreous was made. At 12 weeks of age, the child underwent an examination under anesthesia that disclosed a microphthalmic right globe with a corneal diameter of 9.0 mm. Ophthalmoscopic examination disclosed a fibrovascular stalk extending from the optic nerve head to the posterior surface of the lens. A computed tomographic scan (Fig. 1) con firmed the diagnosis of persistent hyperplastic primary vitreous and failed to show any calcifi cation suggestive of a retinoblastoma. Exami nation of the patient's left eye showed a normal corneal diameter of 10.5 mm, and a normal anterior segment. Ophthalmoscopic examina tion, however, showed changes suggestive of retinopathy of prematurity (Fig. 2). The periph eral retina was avascular for 360 degrees and was associated with a shallow temporal ridge. There were a few new vessels growing over the ridge at the 5:00 o'clock position. Additionally, there was a circumferential vitreous condensa tion more posterior to this in an area of vascu-
Retinopathy of Prematurity-Like Fundus and Persistent Hyperplastic Primary Vitreous Associated With Maternal Cocaine Use Michael P. Teske, M.D., and Michael T. Trese, M.D. Kresge Eye Institute. Inquires to Michael T. Trese, M.D., Kresge Ei/e Institute, 3994 John R, Detroit, MI 48201. Recent reports of cocaine use by women dur ing pregnancy have disclosed an increased inci dence of spontaneous abortions, abruptio pla centae, impairment of neonatal interactive and state organization abilities, 1 and perinatal cere bral infarction. 2 We examined an otherwise
Fig. 1 (Teske and Trese). Orbital computed tomog raphy demonstrating microphthalmia and fibrovas cular stalk connecting the optic nerve to the posterior lens surface typical of persistent hyperplastic pri mary vitreous. Left globe appears normal.