Botulinum toxin treatment of acute sixth and third nerve palsy

Thirty-four patients with acute sixth nerve palsy and nine patients with acute third nerve palsy were treated with botulinum toxin injection to the antagonist, nonparalytic horizontal rectus muscle. In a control group of 52 patients with acute sixth nerve palsy not treated with botulinum in the acute stage, only 16 (31 %) recovered spontaneously and did not require surgery. Twenty-two of the 31 surviving patients who could be followed with acute sixth nerve palsy had lateral rectus recovery and surgery was avoided. Four required prisms in their glasses to obtain fusion. Nine patients developed chronic sixth nerve palsy and required surgery. In this group of acute sixth nerve palsy patients, eleven were bilateral. Seven of these eleven developed chronic sixth nerve paralysis, and required strabismus surgery. This suggests the prognosis for recovery following botulinum treatment in cases of acute bilateral sixth nerve palsy is not as good as in the unilateral cases. Botulinum toxin treatment does not appear to be effective in chronic sixth nerve palsy, as judged by results of treatment in one patient known to have a chronic palsy. Nine of nine patients with acute third nerve palsy had medial rectus recovery with fusion horizontally in primary gaze. None have required surgery. Only four of nine showed improvement in vertical rotations. The remaining five patients avoid vertical diplopia by a compensatory chin position. Botulinum toxin treatment of patients with acute sixth and third nerve palsy appears beneficial. However, since some in this group of patients may recover spontaneously, a randomized, double-blind study may be necessary to more definitively determine the effectiveness of this therapy.Dedicated to Dr. G.K. von Noorden on the occasion of his 60th birthday     

The diagnostic yield of neuroimaging in sixth nerve palsy - Sankara Nethralaya Abducens Palsy Study (SNAPS): Report 1

Aims:The aim was to assess the etiology of sixth nerve palsy and on the basis of our data, to formulate a diagnostic algorithm for the management in sixth nerve palsy.Design:Retrospective chart review.Results:Of the 104 neurologically isolated cases, 9 cases were attributable to trauma, and 95 (86.36%) cases were classified as nontraumatic, neurologically isolated cases. Of the 95 nontraumatic, isolated cases of sixth nerve palsy, 52 cases were associated with vasculopathic risk factors, namely diabetes and hypertension and were classified as vasculopathic sixth nerve palsy (54.7%), and those with a history of sixth nerve palsy from birth (6 cases) were classified as congenital sixth nerve palsy (6.3%). Of the rest, neuroimaging alone yielded a cause in 18 of the 37 cases (48.64%). Of the other 19 cases where neuroimaging did not yield a cause, 6 cases were attributed to preceding history of infection (3 upper respiratory tract infection and 3 viral illnesses), 2 cases of sixth nerve palsy were found to be a false localizing sign in idiopathic intracranial hypertension and in 11 cases, the cause was undetermined. In these idiopathic cases of isolated sixth nerve palsy, neuroimaging yielded no positive findings.Conclusions:In the absence of risk factors, a suggestive history, or positive laboratory and clinical findings, neuroimaging can serve as a useful diagnostic tool in identifying the exact cause of sixth nerve palsy. Furthermore, we recommend an algorithm to assess the need for neuroimaging in sixth nerve palsy.     

Differential diagnosis and management of acquired sixth cranial nerve palsy.

BACKGROUND: Cranial nerve VI innervates the lateral rectus muscle. A lesion will result in esotropia greater at distance and an ipsilateral abduction deficiency. After the age of 50 years, vascular diseases are the most commonly known causes. CASE REPORT: A 55-year-old white man reporting a 2-week history of horizontal diplopia that was worse at distance was found to have a left sixth cranial nerve paresis. The patient was diagnosed with hypertension and placed on medications. At the 4-week follow-up visit, the abduction deficiency had resolved. DISCUSSION: The incidence of sixth nerve palsy is 11.3 in 100,000. A lesion anywhere along the course of the nerve, from the pons to the orbit, can cause a paresis or palsy. After ruling out trauma and non-neurological problems, cases should be classified into neurologically isolated or non-neurologically isolated cases. Neurologically isolated sixth nerve palsies are associated most commonly with vascular disease. Non-neurologically isolated sixth nerve palsies typically are associated with more grave conditions. CONCLUSION: A sixth nerve palsy of vascular or undetermined causes typically resolves within 6 to 8 weeks. If resolution does not occur within 2 to 3 months, the condition progresses, or if additional neurologic signs or symptoms develop, imaging studies are indicated.     

Ocular Neuromyotonia Noted after Recent Botulinum Toxin Injection for Sixth Nerve Palsy Following Resection of a Posterior Fossa Skull Base Meningioma

A 56-year-old female complained of diplopia immediately after surgical excision of a recurrent left skull base tuberculum meningioma. She was found to have a left sixth nerve palsy, which was subsequently treated with botulinum toxin injection to the medial rectus muscle. Three months post injection, the patient had partial recovery of the sixth nerve palsy and new-onset ocular neuromyotonia.     

Vertical rectus muscle transposition with intraoperative botulinum injection for treatment of chronic sixth nerve palsy

Six adult patients with acquired, chronic, complete sixth nerve palsy had vertical rectus muscle transposition to the lateral rectus muscle insertion with intraoperative injection of botulinum toxin into the ipsilateral medial rectus muscle. Five of six patients were orthophoric in primary gaze, and the remaining patient achieved single binocular vision with a small head turn. This method compares favorably with previously described transposition procedures for sixth nerve palsy in terms of amount of correction and size of the window of single binocular vision with a lower risk of anterior segment ischemia.     

Paralytic Strabismus in Children: Etiologic Incidence and Management of the Third,Fourth, and Sixth Nerve Palsies

The results of a retrospective study of the etiologic incidence of all cases of third, fourth, and sixth nerve palsies in patients at Wills Eye Hospital and St Christopher's Hospital are reported. A total of 121 patients ranging in age from birth to sixteen were studied. Thirty-two cases of third nerve palsy, eighteen cases of fourth nerve palsy, and sixty-two cases of sixth nerve palsy were identified in this series. Nine additional cases illustrated combinations of multiple nerve involvement. With few exceptions, acquired third nerve palsy in children is an ominous sign whereas the congenital form is generally indicative of developmental anomaly or birth trauma. Acquired sixth nerve palsy in a child is always a matter of concern since it may represent the first sign of a brain-stem glioma. Active force studies may be carried out periodically to assess the speed of recovery and indicate when it is time to initiate a more aggressive role in the surgical management. The surgical results in children with paralytic strabismus that have been carefully evaluated offer a favorable prognosis.     

Incidence, associations, and evaluation of sixth nerve palsy using a population-based method

PURPOSE: To determine the incidence of sixth nerve palsy in a population-based study, with particular emphasis on associated coexisting medical conditions and to use these data to develop a management algorithm. DESIGN: Retrospective, population-based case series. PARTICIPANTS: All residents of Olmsted County, Minnesota, USA, diagnosed with sixth nerve palsy between January 1, 1978 and December 31, 1992. METHODS: All cases were identified by using the Rochester Epidemiology Project medical records linkage system, which captures all patient-physician encounters in Olmsted County. The entire medical record of each patient was reviewed to confirm the diagnosis, document county residency, and to determine associated medical conditions. We used stringent predetermined criteria to define diabetes mellitus and hypertension as associations. Incidence rates were adjusted to the age and gender distribution of the 1990 white population in the United States. MAIN OUTCOME MEASURES: Etiology or systemic associations of the palsy. RESULTS: We identified 137 new cases of sixth nerve palsy over the 15-year period. The age- and gender-adjusted annual incidence of sixth nerve palsy was 11.3/100 000 (95% confidence interval, 9.3-13.2/100 000). Causes and associations were: undetermined (26%), hypertension alone (19%), coexistent hypertension and diabetes (12%), trauma (12%), multiple sclerosis (7%), neoplasm (5%), diabetes alone (4%), cerebrovascular accident (4%), postneurosurgery (3%), aneurysm (2%), and other (8%). When sixth nerve palsy was the presenting sign in cases of neoplasm (n = 1) and aneurysm (n = 3), history and examination revealed the presence of other neurologic symptoms or signs. CONCLUSIONS: We provide population-based data on the incidence of sixth nerve palsy with a notably lower incidence of neoplasm and higher incidence of diabetes and hypertension than previous institution-based series. We suggest that patients with nontraumatic neurologically isolated sixth nerve palsy may undergo a focused medical evaluation followed by close observation, whereas non-neurologically isolated cases warrant a full neurologic evaluation, including prompt neuroimaging.     

无需肌电仪引导的肉毒素联合或不联合透明质酸钠内直肌注射治疗外伤性外展神经麻痹

目的探讨无需肌电仪引导的A型肉毒素（BTA）联合或不联合透明质酸钠注射治疗外伤性外展神经麻痹的有效性及安全性。方法 前瞻性随机对照临床研究。采用抽签法将入选的27例年龄≥16岁病程＜6个月的外伤性外展神经麻痹患者随机分成2组,A组无需肌电仪引导直视下行患眼内直肌注射0.05 ml BTA 2.5～7.5 U联合透明质酸钠;B组无需肌电仪引导直视下行患眼内直肌注射0.03 ml BTA溶液2.5～7.5 U。分别于注射前、注射后2周、6个月测定患者斜视度、眼球运动功能及睑裂大小。数据采用配对t检验、独立样本t检验和卡方检验。结果 2组患者在注射后2周均出现斜视度明显下降（t=8.75、8.79,P均＜0.01﹚;A、B组的治愈率分别为62%及71%,2组比较差异没有统计学意义（χ2=0.297,P>0.05）;A、B组均未出现并发性上睑下垂及垂直斜视。结论 无需肌电仪引导的肉毒素联合或不联合透明质酸钠注射均能有效治疗外伤性外展神经麻痹,而且有较好的安全性。     

Adjustable-suture strabismus surgery: a review of 255 consecutive cases

Adjustable-suture strabismus surgery was performed on 255 patients under general anesthesia. Prophylactic topical and oral antibiotic therapy was used in all cases; only one postoperative infection occurred. Adjustments were needed for 123 patients and were done under topical tetracaine anesthesia within a few hours after recovery from general anesthesia. Overall the adjustments appeared to be reliable. The use of droperidol, however, made reliable adjustment impossible in two cases and is therefore strongly contraindicated if suture adjustment is to be done within a few hours of general anesthesia. There were two cases of temporary palsy of the muscle with the adjustable suture, presumed to be due to the topical anesthetic. Unexpectedly large effects of the surgery occurred in three cases of acquired sixth cranial nerve palsy in which full abduction had been recovered but a concomitant esotropia remained and in a few cases in which the muscles had previously been resected and there were normal active ductions preoperatively. Contraindications to the use of adjustable sutures in strabismus surgery may include multiple orbital fractures, previous orbital floor decompression for dysthyroid ophthalmopathy, and a variable angle.     

Adaptive neural mechanism for Listing's law revealed in patients with sixth nerve palsy.

PURPOSE: During fixation and saccades, human eye movements obey Listing's law, which specifies the torsional eye position for each combination of horizontal and vertical eye positions. To study the mechanisms that implement Listing's law, the authors measured whether the law was violated in peripheral and central unilateral sixth nerve palsy. METHODS: Twenty patients with peripheral (13 chronic, 7 acute) sixth nerve palsy, 7 patients with central sixth nerve palsy caused by brainstem lesions, and 10 normal subjects were studied with scleral search coils. With the head immobile, subjects made saccades to a target that moved between straight ahead and eight eccentric positions. At each target position, fixation was maintained for 3 seconds before the next saccade. To quantify violations of Listing's law, we measured ocular torsion during fixation and during saccades, and compared it with the torsion predicted by the law. The SD of the differences between the predicted and measured torsion was called Listing deviation. RESULTS: Patients with central sixth nerve palsy had abnormal ocular torsion in both the paretic and nonparetic eyes, which violated Listing's law. During fixation, Listing deviation averaged 2.4 degrees in the paretic eye and 1.7 degrees in the nonparetic eye, compared with 0.8 degrees in normal control subjects (P < 0.05). During saccades, the Listing deviation averaged 2.7 degrees in the paretic eye, and 1.6 degrees in the nonparetic eye, compared with 0.8 degrees in normal control eyes (P < 0.05). Donders' law was also violated in both eyes of patients with central sixth nerve palsy. They showed an abnormally wide range of ocular torsion in any given gaze direction. In contrast, patients with acute peripheral palsy had abnormal ocular torsion only in the paretic eye. Listing deviation of the paretic eye averaged 2.3 degrees during fixation and 3.2 degrees during saccades (P < 0.05). Donders' law was obeyed in acute peripheral palsy. Patients with chronic peripheral sixth nerve palsy obeyed Listing's and Donders' laws during both fixation and saccades. CONCLUSIONS: Patients with central unilateral sixth nerve palsy have abnormal ocular torsion in both eyes, demonstrating that brainstem circuits normally participate in the maintenance of Listing's law. Eye movements in patients with acute peripheral sixth nerve palsy violate Listing's law, whereas those in patients with chronic peripheral palsy obey it, indicating that neural adaptation can restore Listing's law, even when the eye muscle remains abnormal.     

Neuro-ophthalmic manifestations of intracranial cavernous hemangiomas

PURPOSE: To describe the neuro-ophthalmic manifestations of patients with intracranial cavernous hemangiomas (cavernomas). METHODS: A retrospective review of all patients with intracranial cavernomas with neuroophthalmic manifestations who were treated at the Royal Adelaide Hospital in Australia between 1994 and 2004. RESULTS: There were nine patients (three men and six women), with a mean age of 39 years (range 22-61). There was one cerebellar lesion, two thalamic, four pontine, one midbrain/pontine, and one midbrain. Ophthalmic presentations included internuclear ophthalmoplegia (one patient), third cranial nerve (CN) palsy (one patient), fourth CN palsy (one patient), and sixth CN palsy (six patients). Three patients underwent extraocular muscle surgery, and six were treated medically or observed. In five patients the diplopia resolved, in three it was only mild, and in one patient no significant change was noted during the follow-up period. CONCLUSIONS: Diplopia is the main neuro-ophthalmic manifestation of intracranial cavernomas. Sixth CN palsy is the most common cause. Neurosurgical or conservative treatment leads to improvement in most cases, and later use of spectacles or extraocular muscle surgery may lead to further improvement.     

Pediatric third, fourth, and sixth nerve palsies: a population-based study

PURPOSE: To determine the population-based incidence and cause of cranial nerve palsies affecting ocular motility in children in the circumscribed population of Olmsted County, Minnesota. METHODS: The Rochester Epidemiology Project medical records linkage system captures virtually all medical care provided to Olmsted County residents. By means of this database, all cases of third, fourth, and sixth cranial nerve palsy were identified among county residents less than 18 years of age from 1978 through 1992. Medical records were reviewed to confirm the diagnosis, determine the cause, and document county residency. Incidence rates were adjusted to the age and sex distribution of the 1990 white population in the United States. RESULTS: Over this 15-year period, 36 incidence cases of cranial nerve palsy were identified in 35 children in this defined population. The age-adjusted and sex-adjusted annual incidence of third, fourth, and sixth nerve palsies combined was 7.6 per 100,000 (95% confidence interval, 5.1 to 10.1). The most commonly affected nerve was the fourth (36%), followed by the sixth (33%), the third (22%), and multiple nerve palsies (9%). The most common cause was congenital for third and fourth nerve palsy, undetermined for sixth, and trauma for multiple nerve palsies. Although three cases were associated with neoplasia, a cranial nerve palsy was not present at the time of diagnosis in any case. CONCLUSIONS: Unlike many institutionally based referral series, our population-based study provides data on the incidence and cause of third, fourth, and sixth nerve palsies in a geographically defined population. In contrast to previous institutionally based series, nearly half the cases were congenital in origin, and in no case did intracranial neoplasia present as an isolated nerve palsy.     

Fourth and sixth cranial nerve injury after halo traction in children: A report of two cases

BACKGROUND: Spinal traction is the application of a longitudinal force to the spinal column as a means of stabilizing a damaged or abnormal spine. Although not well documented in the ophthalmic literature, complications include cranial nerve palsies, with the sixth nerve being most commonly affected. Fourth nerve palsies have not previously been reported to our knowledge. We present 2 cases of combined fourth and sixth palsies after cervical traction. METHODS: Retrospectively, we reviewed the ophthalmic findings in 2 children with diplopia after spinal traction. RESULTS: Case 1 suffered a traumatic rotatory atlantoaxial subluxation and underwent halo traction. Case 2 required traction to correct a scoliosis secondary to osteogenesis imperfecta. In both cases, sixth nerve palsies were apparent soon after traction. Careful orthoptic examination revealed additional fourth nerve involvement. After 3 months, both cases showed partial resolution of the cranial nerve injuries. CONCLUSIONS: Cranial nerve injury may occur with spinal traction. Fourth nerve palsy may be underreported because of masking by a coinciding sixth nerve palsy.     

Augmented superior rectus muscle transposition for the treatment of strabismus in Möbius syndrome

M?bius syndrome is a rare congenital disorder characterized by bilateral facial nerve palsy and abducens nerve palsy. It is associated with a wide spectrum of systemic and ocular manifestations; esotropia is commonly the reason for ophthalmic referral. There are few published studies in the literature on strabismus surgery in M?bius syndrome. An augmented vertical rectus muscle transposition in which lateral fixation sutures are used on the transposed vertical rectus muscles (Foster augmentation) has been used to correct esotropia caused by severe sixth nerve palsy and type 1 Duane syndrome. We describe the surgical outcome after treating moderate- to large-angle esotropia in 3 patients with M?bius syndrome with the augmented vertical rectus muscle transposition.     

Vertical misalignment in unilateral sixth nerve palsy

OBJECTIVE: To detect and determine the magnitude of vertical deviation in patients with unilateral sixth nerve palsy. DESIGN: Prospective consecutive comparative case series. PARTICIPANTS: Twenty patients with unilateral peripheral sixth nerve palsy, 7 patients with central palsy caused by brainstem lesions, and 10 normal subjects. METHODS: Subjects were tested by the prism and cover test, Maddox rod and prism test, and magnetic search coil recordings in nine diagnostic eye positions. They were also tested during static lateral head tilt by the prism and cover, and Maddox rod and prism tests. MAIN OUTCOME MEASURES: The magnitudes of horizontal and vertical deviations. RESULTS: All patients had an abduction deficit and incomitant esodeviation that increased in the field of action of the paretic muscle, indicating sixth nerve palsy. Mean vertical deviations, for all positions of gaze in peripheral palsy were 0.3 +/- 0.8 prism diopters (PD) by prism and cover test, 1.3 +/- 1.6 PD by Maddox rod and prism test, and 2.0 +/- 1.4 PD by coil recordings. Mean vertical deviations in normal subjects were 0.0 +/- 0.0 PD by prism and cover test, 1.0 +/- 0.9 PD by Maddox rod and prism test, and 1.9 +/- 2.1 PD by coil recordings. Therefore, peripheral palsy did not cause abnormal vertical deviation. In central palsy, for all positions together mean vertical deviations were 0.9 +/- 1.3 PD by prism and cover test, 1.4 +/- 1.6 PD by Maddox rod and prism test, and 2.5 +/- 1.6 PD by coil recordings; they were not different from normal values. During static head roll, patients with peripheral palsy had a right hyperdeviation on right head tilt and a left hyperdeviation on left head tilt, regardless of the side of the palsy. In contrast, in central palsy, head tilt caused vertical strabismus that remained on the same side on head tilt to either side. CONCLUSIONS: Small vertical deviations in sixth nerve palsy are consistent with normal hyperphorias that become manifest in the presence of esotropia. In peripheral sixth nerve palsy, static head roll to either side induces hyperdeviation in the eye on the side of the head tilt. Hyperdeviation of the same eye induced by head tilt to either direction implicates a brainstem lesion as the cause of paretic abduction. Quantitative study of sixth nerve palsy demonstrates that if a vertical deviation falls within the normal range of hyperphoria, multiple cranial nerve palsy or skew deviation may not be responsible. Conversely, vertical deviation > 5 PD indicates skew deviation or peripheral nerve palsy in addition to abduction palsy.     

糖尿病性眼肌麻痹临床分析

目的 探讨糖尿病性眼肌麻痹的临床特点.方法 对25例(25只眼)经常规眼科检查,眼肌检查及实验室生化检查后,确诊为糖尿病性眼肌麻痹进行分析.结果 25只眼均单眼发病,受累神经以动眼神经麻痹多见,为15例,其次外展神经麻痹6例,滑车神经麻痹3例,复合神经(Ⅲ+Ⅳ)麻痹1例.结论 中老年糖尿病患者易并发眼肌麻痹,糖尿病性微循环病变是导致神经缺血缺氧以致变性的病理基础.     

Abducens nerve palsy and Horner's syndrome revisited

Sympathetic fibers destined for the eye join the abducens nerve for a short distance within the cavernous sinus; thus, a unilateral sixth nerve palsy with an ipsilateral Horner's syndrome is of significant localizing value. We report two cases of cavernous sinus lesions producing an ipsilateral abducens palsy and Horner's syndrome: one case due to an intrinsic and the other to an extrinsic lesion of the sinus region.     

Supramaximal horizontal rectus surgery in the management of third and sixth nerve palsy

Supramaximal medial and lateral rectus surgery was performed to correct horizontal deviations in seven cases of sixth nerve palsy and three cases of third nerve palsy, with satisfactory results in most cases. The advantages and disadvantages of such procedures are discussed.     

Cause and prognosis of nontraumatic sixth nerve palsies in young adults

OBJECTIVE: To review the causes and prognosis of sixth nerve palsies in patients who are 20 to 50 years of age. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: All patients aged 20 to 50 years with a nontraumatic sixth nerve palsy seen in a neuro-ophthalmic practice from 1994 to 2000. INTERVENTION: Diagnostic testing to determine the cause of the palsy and surgical intervention to correct persistent stable strabismus. MAIN OUTCOME MEASURES: Cause of the palsy. The patients' clinical courses were reviewed. RESULTS: The most common cause for a sixth nerve palsy in this age group was a central nervous system (CNS) mass lesion, although the most common cause for an isolated sixth nerve palsy in this age group was multiple sclerosis. Patients with a CNS mass lesion responsible for their palsy had the highest rate of nonresolution requiring strabismus surgery. CONCLUSIONS: Sixth nerve palsies are unusual in young adults, but in the practice of author (GBK) most are secondary to CNS mass lesions and, when isolated, multiple sclerosis. Deferring neuroimaging or other appropriate investigations presuming a microvascular cause for the palsy in this age group is not recommended.     

Cause and prognosis of neurologically isolated third, fourth, or sixth cranial nerve dysfunction in cases of oculomotor palsy

Purpose To determine the cause and prognosis of neurologically isolated third, fourth, or sixth cranial nerve dysfunction in cases of oculomotor palsy, and to determine the best imaging methods to make a correct diagnosis. Methods The medical records of 221 consecutive patients with oculomotor palsy caused by neurologically isolated cranial nerve dysfunction were reviewed. There were 63 cases of third, 41 of fourth, and 117 of sixth cranial nerve dysfunction. The patients were examined at the Neuro-ophthalmology Clinic of Kyoto University Hospital between 1993 and 2001. Results Vascular disorders accounted for 34.9% of the third nerve dysfunction, and 90% of these recovered completely in 6 months. Ninety percent of the patients with an isolated third nerve dysfunction that was caused by an aneurysm also had anisocoria, and 68% of the patients with a third nerve dysfunction caused by a vascular disorder had anisocoria. In all of the vascular cases with anisocoria, the difference in the pupillary diameter was <1.0 mm. The presence of ptosis did not play an important role in making a diagnosis of third nerve dysfunction. Ninety percent of the patients with fourth nerve dysfunction and 60% of the patients with sixth nerve dysfunction recovered within 9 months. Conclusions The age of the patient, signs of an improvement, and associated alterations are important diagnostic markers to determine the best type of imaging methods for evaluating neurologically isolated third, fourth, and sixth cranial nerve dysfunction.