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Show 70 LETTERS TO THE EDITOR Hemifacial Spasm and Idiopathic Intracranial Hypertension To the Editor: We read with great interest the recent article by Selky and Purvin entitled Hemifacial spasm: an unusual manifestation of idiopathic intracranial hypertension ( 1). We would like to validate this unusual association by describing a similar patient. A 54- year- old woman was referred for evaluation of disc edema. Three weeks prior, she developed involuntary facial spasms that were elicted by chewing. The left side of her face developed uncontrollable spasms, eyelid closure and mouth distortion. After each spasm, she heard a whooshing sound. The spasms occurred on several occasions and were observed by us. She described frequent bifrental headaches. She had a vague sense of visual blurring, suggestive of transient visual obscuration. Her past history was significant for hypertension and smoking. On examination her best corrected visual acuity was 20/ 25 in each eye. Pupils were briskly reactive without an afferent pupillary defect. Bilateral disc edema greater in the right eye was evident. Humphrey visual fields were constricted bilaterally. Brain CT and MRI were normal. There was no dolichoectasia of the vertebrobasilar system noted. A lumbar puncture revealed an opening pressure of 480 mm H20. Her episodes of hemifacial spasm stopped after the spinal tap and did not recur while on acetazolamide 500 mg b. i. d. When the patient discontinued the acetazolamide against physician's advice, the facial twitching returned but to a much lesser degree. The spasms ceased when she restarted her acetazolamide. At four month follow- up, she had no recurrence of hemifacial spasm, headache, or audible intracranial bruit. Her visual acuity was now 20/ 20 OU, and there was less disc edema. Her visual fields were stable. Her acetazolamide was decreased to 500 mg/ day and she has remained asymptomatic for six months. Our patient had hemifacial spasm as a manifestation of idiopathic intracranial hypertension. Like Selky and Purvin, we interpret this as causally related based on the simultaneous development of the spasm, headache, and audible intracranial noise. Furthermore, the resolution of all symptoms with treatment and their recurrence with cessation of acetazolamide supports the relationship between the hemifacial spasm and elevated intracranial pressure. We agree that intermittent compression of the seventh nerve from " pressure induced intracranial structural shifts which pull and stretch the extra-axial segment of the involved nerve" is a plausible explanation ( 1). It is also noteworthy that both of these patients were older than most patients with idiopathic intracranial hypertension; most patients with hyper-excitable facial nerve syndromes are also older. We believe that this second patient establishes hemifacial spasm as an unusual manifestation of intracranial hypertension. Nancy Mayer Benegas, M. D. Nicholas J. Volpe, M. D. Grant T. Liu, M. D. Steven L. Galetta, M. D. Scheie Eye Institute University of Pennsylvania Medical Center Philadelphia, Pennsylvania REFERENCE 1. Selky AK, Purvin VA. Hemifacial spasm: An unusual manifestation of idiopathic intracranial hypertension. / Neuro- Ophthalmol 1994; 14: 196- 8. Porcelinizing Discolorization of the Periocular Skin Following Botulinum A Toxin Injections To the Editor: Botulinum A toxin has gained popularity in the medical treatment of strabismus and a variety of neuromuscular disorders, such as essential blepharospasm, hemifacial spasm, and Meige syndrome. In addition, this treatment has been applied to lower lid entropion, ocular myokymia, abberant regeneration of the seventh nerve, nystagmus, lid retraction, and corneal exposure. The most common side effects of botulinum A toxin injections are ptosis and ocular malalignment. We would like to call attention to three patients who were treated with repeated injections of botulinum A toxin ( BOTOX) and developed por-celin discoloration of the periocular skin accompanied by long- standing edema of the lower lid. A 62- year- old man was treated with botulinum A toxin ( BOTOX) injections for essential blepharospasm. He received a series of BOTOX injections to the upper and lower lids bilaterally. Each series consisted of 80 units of BOTOX ( 40 units to each eye). The patient tolerated the procedure well and no side effects were noted until 10 days after the / Neuro- Ophthalmol, Vol. 16, No. 1, 1996 LETTERS TO THE EDITOR 71 second series of injections, when a bilateral whitening of his periocular skin and bilateral lower lid edema were noted. Follow- up of 18 months did not reveal significant changes in his appearance. The eye opening was significantly improved by the BOTOX. A 60- year- old man was treated by BOTOX injections for right hemifacial spasm. He had five series of BOTOX injections to his right periocular, malar and buccal area. The total dosage administered was 210 units. He tolerated the injections well and no complications were observed until after the fifth course when a periocular whitening and lower lid edema were observed for the first time. These were unchanged on a follow- up visit at 16 months. The right facial contractions were significantly improved following each series of injections. A 62- year- old man was treated with BOTOX injections for essential blepharospasm which progressed to become Meige syndrome. He had six series of BOTOX injections to the periocular, malar, and buccal areas bilaterally. The total dosage administered was 350 units. He tolerated the injections well with no side effects. His twitchings and lid closure were markedly improved. After the last treatment, periocular whitening and bilateral lower lid edema were noted ( Fig. 1). These were unchanged on a 6- month follow- up. Botulinum A toxin injections are considered to be a popular, effective and safe procedure in the treatment of paralytic and comitant strabismus, various neuromuscular disorders, facial spasm associated with dystonia, and other ophthalmologi-cal and neurological disorders ( 1,2). The most common local side effects are ptosis and vertical deviations which are due to the diffusion of the toxin from the lid to the extraocular muscles ( 1,3). Other local side effects which have been reported include: corneal exposure, lagophthalmos, superficial punctate keratopathy, scleral perforation, subconjunctival hemorrhage, entropion, ectropion, skin rash, ecchymosis and pupillary dilatation ( 3,4). No clinically significant systemic side effects of botulinum A toxin have been reported. However, abnormal neuromuscular transmission in arm muscles was observed in single fiber EMG, after periocular injection of 12.5 units of toxin ( 5). To our knowledge, these are the first reported cases of long- standing periocular discoloration and lower lid edema associated with BOTOX injections. The underlying mechanism is unclear. Our hypothesis would be that there is a connection, in some way, between botulinum toxin, skin melanocytes, and tyrosinase activity, but we cannot find any explanation for this peculiar phenomenon associated with botulinum A toxin injections. Has anyone noted a similar reaction? This work was supported in part by an unrestricted Departmental grant from RPB, Inc., New York. Shmuel Friedland, M. D. Ronald M. Burde, M. D. Department of Ophthalmology and Visual Sciences Albert Einstein College of Medicine/ Montefiore Medical Center FIG. 1. Case 3. Periocular whitening and bilateral lower lid edema after BOTOX injection. / Neuro- Ophthalmol, Vol. 16, No. 1, 1996 72 LETTERS TO THE EDITOR References 1. Osako M, Keltner JL. Botulinum A toxin ( oculinum) in ophthalmology. Surv Ophthalmol 1991; 36: 28- 46. 2. Perman KI, Baylis HI, Rosenbaum AL, Kirschner DG. The use of botulinum toxin in the medical management of benign essential blepharospasm. Ophthalmology 1986; 93: 1- 3. 3. Klara HK, Magoom EH. Side effects of the use of botulinum toxin for treatment of benign essential blepharospasm and hemifacial spasm. Ophthalmol Surg 1990; 21: 5; 335- 8. 4. Dutton JJ, Buckley EG. Long- term results and complications of botulinum A toxin in the treatment of blepharospasm. Ophthalmology 1988; 95: 1529- 34. 5. Sanders DB, Massey EW, Buckley EG. Botulinum toxin for blepharospasm: single fiber EMG fibers. Neurology 1986; 36: 545- 7. Chronic Headaches Due to Vitamin A Abuse To the Editor: Vitamin A or retinol is a fat- soluble vitamin that plays an essential role in various biochemical and physiological processes ( retina, growth and development of epithelial tissue, bone, reproduction, etc.) ( 1). Excess intake of vitamin A results in hy-pervitaminosis A, which can cause chronic headaches, vomiting, nausea, loss of appetite, fatigue, dermatitis, myalgia, gingivitis, nystagmus, and the entity called pseudotumor cerebri ( 2,3). Hyper-vitaminosis A is frequently an overlooked cause of chronic headaches. We would like to call attention to a patient who was evaluated in the headache clinic and a neuro- ophthalmology service for 2 years with a history of chronic headache and nausea. A 62- year- old man was evaluated by the headache unit and the neuro- ophthalmology service for chronic headaches and nausea. He had an extensive workup, including neuroimaging studies of the head, which were all normal. The headache was described as an ever- present pressure and tightness sensation. These headaches wake the patient from sleep, accompanied by nausea and sometimes vomiting. The patient was asked several times about his medications, and he reported the use of Ibuprofen, aspirin, and Advil. No focal triggers were found. Neurological, neuro- ophthal-mological and ENT examinations were all essentially normal. After 2 years of follow- up, the patient reported to us that he was (?) taking 20,000 IU of vitamin A daily, and, although he was asked about his medication consumption, he never realized that vitamin intake is considered to be a medication. The vitamin A intake was discontinued and the patient reported significant relief of his symptoms within 2- 3 weeks. Although in most instances, the average American diet does not require vitamin supplementation, many people are convinced that they need additional and better nutrients than their diet provides. Advertising, the need and desire to be fit, and the natural food popularity are examples of factors that have resulted in a significant increase in the consumption of vitamins. Vitamin A is obtained from organ meats, fish, butter, eggs, milk, carrots, squash, and pumpkin. Since the vitamin is sufficiently stored in the liver, high doses can result in excessive amount and toxicity ( 4,5). The U. S. recommended daily allowance values for vitamin A is 5,000 IU for adult males and 4,000 IU for adult females. The dose of vitamin A necessary to induce pseudotumor cerebri varies from 20,000 to 6,000 IU daily ( 3). The increased popularity of vitamin A consumption even in the oph-thalmological community ( possible role in retinitis pigmentosa patients?) should alert us to the side effects of its abuse, especially since patients do not recognize the fact that vitamins are considered as medication and thus do not report its intake. Special attention should be paid to vitamin A consumption whenever the clinical setting is adequate. This work was supported in part by an unrestricted departmental grant from Research to Prevent Blindness, Inc., New York. Shmuel Friedland, M. D. Ronald M. Burde, M. D. Department of Ophthalmology and Visual Sciences Albert Einstein College of Medicine/ Montefiore Medical Center 111 East 210th Street Bronx, NY 10467 References 1. Mandel HG, Cohn VH. Fat- soluble vitamins: vitamins A, K and E. In: Goodman and Gilmans' the pharmacological basis of therapeutics, 6th Ed. New York: Macmillan; 1980: 67 and 1583. 2. Howroth JC, Ranee CP, Roy C, et al. The use and abuse of vitamin A. Can Med Assoc J 1971; 104: 521- 2. 3. Lombart A, Carton H. Benign Intracranial hypertension due to A hypervitaminosis in adults and adolescents. Eur Neurol 1976; 14: 340- 50. 4. Fenny L, Berman ER. Oxygen toxicity: membrane damage by free radicals. Invest Ophthalmol Vis Sci 15: 789- 92, 1976. 5. Pfander H. Carotenoids: an overview. Methods Enzymol 1973; 123: 3- 13. / Neuro- Ophthalmol, Vol. 26, No. 1, 19% |