Accommodative spasm (AS) results from over-stimulation of the parasympathetic nervous system, and when it occurs with pupillary miosis and excessive convergence, it is termed spasm of the near reflex (SNR).1,2 The major symptoms of SNR include sudden onset of blurred vision as a result of apparent myopic refractive error (pseudomyopia), diplopia, limited ocular motility, ocular pain, and photophobia.3 The signs and symptoms are episodic and variable, and frequently disappear with cycloplegia. The most common etiology for SNR in otherwise healthy young individuals is thought to be mostly functional or psychogenic.3 Neurological investigations are recommended to rule out suspected organic causes. Closed head trauma, for example, has been reported as being causative for some cases of AS even several years after the occurrence.4 SNR is a differential diagnosis for acute comitant esotropia in children and adults.
Anatomical correlates of accommodation and the triad reflex in SNR are not well studied in the human visual system. In cats, the lateral suprasylvian cortex mediates bilateral innervation to the oculomotor nuclei, and stimulation of this area produces the triad reflex and selective activation of accommodation.4,5
Comitant esotropia in children is usually either infantile, accommodative, or non-accommodative. Late-onset comitant esotropia, otherwise known as acute acquired comitant esotropia (AACE), has drawn considerable attention in the literature because of its varied presentations and natural history. Burian and Miller (1958)6 first reported AACE and classified it into three types. Type I AACE (Swan) refers to AACE resulting from prolonged occlusion or loss of vision in one eye and may or may not be associated with hyperopic refractive error. Type II AACE (Franceschetti) is presumably a result of shock or psychogenic illness and may be associated with minimal hyperopic refractive error. Type III AACE (Bielschowsky) is associated with myopic refractive error of lesser than 5 D and also a larger distance deviation than near deviation. Increased tonicity of medial rectus associated with near work as a result of uncorrected myopia is considered to be a causative factor for this type.6 The role of accommodative spasm in causing the esotropia is not well defined in these classifications. AACE has been reported in patients with intracranial tumors5 and in children without any neurological abnormalities (type II AACE).7 In type III AACE associated with myopia, some authors8 have reported good ocular alignment through surgical management, but again the differential diagnosis with SNR is not mentioned. Goldstein (1996),3 however, has clearly pointed out the possible role of SNR in AACE in his review through case illustrations.
Management of SNR includes determining its underlying etiology and inhibiting the excessive accommodation and excessive convergence using strong cycloplegic agents and bifocal lenses.3 Atropine, homatropine, and cyclopentolate are the commonly prescribed cycloplegic agents, and each of them has a different course of action.9 These anti-cholinergic drugs paralyze the parasympathetic nervous system supplying the sphincter constrictor muscles and hence relaxes ocular accommodation and convergence. Cyclopentolate and homatropine take 15 to 60 minutes for the onset of maximum cycloplegia and their effect lasts for 24 to 48 hours for the former and 48 to 72 hours for the latter. Atropine has the slowest action and takes almost 2 weeks for the ocular accommodation to normalize. The residual accommodation following instillation of 1% cyclopentolate as assessed using objective measures has been reported to vary between 0.75 and 1.00 D for most individuals (Manny et al, 1993)10 whereas other individuals may exhibit up to 1.50 D,11–13 and darker irides take longer time to achieve mydriasis compared to lighter irides.10 Compared to atropine, cyclopentolate causes less adverse effects, but can cause central nervous system side effects including mental hallucination and restlessness.12–15 These adverse events could possibly be related to overdosage and hence care should be considered in darker irides whose cycloplegic effect is poorer compared to lighter irides.9 Systemic absorption of atropine causes side effects including skin rashes and tachycardia. Punctal pressure for a few seconds after the drug instillation reduces the systemic absorption and hence the side effects. Homatropine has similar side effects to cyclopentolate but lesser than that of atropine. Atropine is considered as the strongest cycloplegic agent and preferred for subjects with darker irides and in accommodative anomalies.9
Recurrence of SNR has been reported following discontinuation of treatment16 and hence gradual weaning of treatment and regular follow-ups are recommended. The role of cycloplegic agents in terms of response to treatment for recurrent SNR is poorly understood. That the differential diagnosis of AACE can be SNR has been reported by Goldstein (1996)3; recurrent episodes and their treatment, however, have not been reported. We report a case of a young healthy gentleman who presented with possible AACE that was diagnosed as SNR. The patient was successfully managed with optical and pharmacological intervention. The clinical findings observed initially, with treatment, and following treatment are reported.
A 23-year-old man presented to the clinic with complaints of blurred distance vision for the past month. He also complained of sudden onset inward deviation of his right eye and was sure that he had never noticed this eye turn earlier. Cover testing showed an esotropia that was constant and associated with varying amounts of diplopia at all distances. General health history was normal and there was no recent history of fever or febrile illness. There was also no history of head trauma or psychogenic ailments. The patient was an aspiring law student and was accompanied by his uncle who was a general physician who confirmed the history revealed by the patient.
The patient had never used any prescription eyeglasses until 5 months before, when he was first diagnosed to be myopic and was prescribed glasses of −0.75 DS/−0.50 DC × 90 and −0.50 DS/−0.50 DC × 90 in the right eye (OD) and left eye (OS), respectively. The patient was comfortable with the glasses for almost a month after which he had noticed fluctuating vision with the glasses. His glasses were changed to −4.50 DS in both the eyes at that time. With the increased myopic prescription, there was no esotropia or diplopia. The last prescription advised was −5.50 D and −5.00 D in OD and OS, respectively, which was not obtained. Since then, he has noticed inward deviation of his right eye, diplopia, and fluctuating visual acuity. The patient subsequently sought an opinion from us. The patient had copies of his prescriptions and visual acuity was documented to be 20/20 with all three prescriptions at the time of dispensing. No other medical records were available. The patient denied having undergone previous cycloplegic refractions.
Visit 1 (Baseline)
The patient’s visual acuity and binocular vision findings are summarized in Table 1. Only the key findings are reported and the entire examination was limited because of symptoms of diplopia and blurred vision. No signs of worsening of visual acuity on occlusion or pupillary miosis during convergence were noted. Color vision was normal with Ishihara pseudo-isochromatic color plates in both eyes. Indirect ophthalmoscopy revealed normal fundus findings. Foveal reflex was normal and retinal abnormalities were ruled out. Neurological investigation was reported to be normal with normal imaging findings of the brain by computed tomography (based on reports brought by the patient). Ocular motility was full with normal saccadic reflexes and oculomotor, trochlear, and abducens cranial nerve paresis were ruled out. Pupillary reflexes were brisk and equally reacting to light in both eyes. Measurement of the ocular deviation revealed 30 PD of constant right esotropia for distance and near. Visual acuity with the myopic prescription (−4.50 D in both the eyes) was 20/25 in either eye and visual acuity without any correction was 20/200 in both the eyes with normal near visual acuity at a closer distance of 20 cm from the spectacle plane.
Cycloplegic refraction using 1% cyclopentolate revealed refractions of OD: +0.25 DS/−1.25 DC × 90 and OS: −0.75 DS/−1.00 DC × 90. The patient’s subjective acceptance and binocular vision was checked under cycloplegia to assess the effect of accommodative relaxation on visual acuity and binocular vision. Visual acuity was 20/25 in both eyes with the cycloplegic refraction values. The esotropia and diplopia persisted even after the cycloplegia without an apparent change in the amount of eso-deviation (findings same as baseline). A diagnosis of SNR was made because of the presence of acute acquired comitant esotropia associated with accommodative spasm. The differential diagnoses considered in this case included decompensated esophoria, divergence insufficiency, lateral rectus palsy, ocular motor apraxia, and ocular myasthenia.
The patient was prescribed 1% atropine (atropine sulfate) eye ointment, twice a day for three consecutive days, and a re-evaluation was advised on the fourth day. The patient was instructed to discontinue the use of prescription glasses until the next visit. The patient was also informed about the potential side effects of atropine, including fever, skin rashes, and facial flushing, and to discontinue the drug if any side effects were observed.
Follow-up #1 (4 Days from Baseline Visit)
On the fourth day of medication, the patient was reviewed and was found to be comfortable using the medication without any side effects. He also felt that the distance vision was clear though the near vision remained blurred (likely a result of the cycloplegia) and diplopia was present for distance and near. The visual examination details are as given in Table 1. Visual acuity was 20/20 in either eye and the angle of esotropia remained 30 PD of constant esotropia for distance and near. The patient was informed of the need for constant use of cycloplegic eye drops with the addition of near-vision glasses. He was advised to use Homide (Homatropine 2%) eye drops (considering the possible side effects of atropine because of systemic absorption) twice a day for 1 month and a prescription based on full cycloplegic refraction with near addition of +2.50 DS was given. Photochromic lenses were advised to tolerate photophobia and outdoor activities. He was counseled about the condition and need for stress management during the course of treatment. The patient was advised to return for follow-up in 2 months. The patient was also instructed to discontinue Homide 2 weeks before the follow-up visit.
Follow-up #2 (2 Months From Baseline)
On follow-up after 2 months, the patient had discontinued Homide eye drops 2 weeks before the scheduled appointment and reported recurrence of symptoms after discontinuing the cycloplegics. The esotropia and diplopia had reduced during the application of cycloplegia and bifocals. The patient had complaints of diplopia only for distance with reduction in esotropia angles (approximately 20 PD intermittent esotropia for distance and near during this visit) and reduction in the myopic shift. It was not a typical response to have persistent diplopia only for distance in the presence of comitant angles for distance and near, and the possible reason could be suppression only at near. Visual acuity with the prescribed glasses was 20/80 and 20/60 in the right and left eyes, respectively. The clinical findings are summarized in Table 1 (follow-up #2). The patient was assured that the condition is resolving and was advised to restart Homide (2% Homatropine) twice a day for 2 months.
Follow-up #3 (4 Months From Baseline)
At the third follow-up appointment, 2 months after the previous visit, the patient had tolerated the cycloplegic eye drops well. He had also discontinued the medications 2 weeks before this follow-up as the visual symptoms of blur and diplopia had resolved. Following the cessation of cycloplegia, the angles of esotropia increased from 20 PD to 60 PD and the patient experienced symptoms of fluctuating vision and diplopia. Visual acuity with the glasses remained same as previous visit. The findings are summarized in Table 1. A neurology evaluation was done again at this point and no organic abnormalities were detected in the repeat CT scan. The pupillary examination and ocular motility findings were normal. In view of the recurrence of SNR on discontinuation of cycloplegic eye drops, the patient was switched to atropine eye drops (1%) once a day for the first week followed by once a week with bifocals. The patient was advised to stop atropine 2 weeks before the scheduled review after 2 months. The patient was also given accommodative facility exercises once the atropine was weaned. Accommodative flippers of power +2.00/−2.00 D were prescribed for near accommodative training along with word rock card of N10 and N8 font sizes. The patient was advised to start the accommodative facility exercises once the atropine was stopped. With the bifocal lenses, the patient was advised to focus with and without the minus lenses through the bifocal segment when the near blur persisted. Once the patient felt that the near vision was clear without the bifocals (considering that the effect of cycloplegia resolved), the patient was advised to use the flippers by focusing through both plus and minus lenses, without the bifocal glasses. The bifocal glasses were advised to be continued for regular day-to-day activities.
The accommodative facility exercises are prescribed to improve the flexibility of the accommodative system considering the risk of recurrence of accommodative spasm after the cycloplegic is weaned. As the action of atropine lasts for 10 to 14 days, patients may get only a couple of days to a maximum of 1 week to perform the facility exercises after the last instillation of atropine that could still be considered beneficial. As there are individual variations in the cycloplegic effect, it is generally left to the patient to start the exercises and to begin with larger fonts such as N10.
Follow-up #4 (6 Months From Baseline)
At the 2 months’ follow-up visit, refraction was stable with uncorrected visual acuity of 20/20 in both eyes. There was minimal esophoria (8 PD BO) for distance and near with a stereo acuity of 20 arc seconds.
The patient was able to perform the accommodative flipper exercises as advised since the past 1 week and felt that the symptoms related to blurring of vision were much reduced. Considering persistent SNR, he was advised to continue atropine eye drops once every 2 weeks to stabilize the accommodation along with accommodative facility exercises. He was called for a review after 4 months with weaning of atropine 1 month before the follow-up.
Follow-up #5 and #6 (8 Months and 1 Year of Follow-up)
Two months later (unscheduled follow-up was done as the patient wanted an assessment), visual acuity and refraction were stable (emmetropic) with atropine and minimal esophoria (1 PD BO for distance and 5 PD BO for near) was found. The main reason for the review was that the patient wanted to know if atropine could be discontinued as the symptoms had resolved and remained stable. Atropine had been discontinued only a week before and hence a review with complete weaning of atropine was advised. He was continued with the weekly atropine dosage for two more months. The patient was adequately counseled about the condition, the possibilities of recurrence with abrupt cessation of cycloplegia, and the need for long-term follow-up and repeat neurological assessment in case of recurrence. The patient was convinced and gave consent to use atropine for two more months. As the patient had an anxious personality and was at high risk for recurrence, the duration of atropine was prolonged before cessation. During the 1-year follow-up visit, visual acuity was 20/15 in either eye and ocular alignment was orthophoric for distance and near. Atropine had been discontinued 2 months before this visit and accommodative parameters were found to be stable. Regular follow-up, once in 6 months, was advised and the patient was advised to continue accommodative facility exercises and yoga for mental relaxation because of his anxious nature. Yoga has gained anecdotal evidence in stress management and hence was considered as part of the treatment protocol.
Summary of Findings
A 23-year-old young healthy male patient reported with sudden onset esotropia and accommodative spasm with variable refractive error for a period of past 6 months. A diagnosis of SNR was made and cycloplegic eye drops were prescribed along with bifocal glasses. In the first follow-up, the patient showed considerable improvement in symptoms, reduced myopic refraction, and reduction in esotropia angle. However, the patient was bothered by recurrence of symptoms on weaning of cycloplegia and an increase in the angle of esotropia was noticed during the third follow-up visit. At that point, the patient was changed to 1% atropine eye drops from 2% homatropine eye drops for a stronger effect of cycloplegia, and the condition responded favorably with reduction of the magnitude of the esotropia until orthotropia was achieved. The refraction also returned to the emmetropic state with 6 months of atropine use along with accommodative facility exercises. There was no recurrence of the SNR noticed in the past 1 year even after the discontinuation of atropine.
Accommodative spasm (AS), also termed accommodative excess, hyperaccommodation, pseudomyopia, and ciliary spasm, has been attributed to either excessive action of the ciliary muscle or excessive flexibility of the crystalline lens, and difficulty with tasks that require relaxation of accommodation has been reported.2 Clinically, AS is defined as a condition in which the accommodative response exceeds the accommodative stimulus and a lead of accommodation is noticed. Presentations of AS vary from simple fluctuating visual acuity with pseudomyopic refractive shift to more complex presentations of SNR. Acute presentations are the most common involving one or both eyes and respond to shorter course of cycloplegic eye drops.
Accommodative spasm is rare. In a retrospective review of clinical records of 114 pre-presbyopic patients with accommodative dysfunctions, Daum (1983)17 has reported that almost 96 of 114 (84%) patients had accommodative insufficiency, whereas only 4 out of 114 patients (2.5%) had accommodative spasm. The overall prevalence of accommodative dysfunctions varies between 22.3% (Lara et al, 2001)18 in a clinical population and 32.3% in the community (Porcar, 1997)19 among which the prevalence of accommodative excess varies between 6.4% and 10.8%, respectively. Accommodative excess can thought to be a mild form of AS with less severe clinical presentation20 and thus shown to be more prevalent than accommodative spasm.
SNR with AS manifests with a wide variety of symptoms, clinical signs, and response to treatment, and it does not follow a common pattern of presentation. Individuals with SNR usually present with complaints of intermittent diplopia, eyestrain, headache, blurred distance vision, and sometimes blurred near vision.18 A number of neurological associations have been reported in literature such as trauma, Wernicke-Korsakoff syndrome, Arnold-Chiari malformation, pituitary disease, pineal gland tumor, and sixth nerve palsy.21 It is therefore important to rule out a central nervous system etiology by thorough ophthalmic and neurological evaluation in combination with neuroimaging. Our patient is a typical case of AS with SNR associated with marked esotropia at presentation and highly fluctuating refraction that responded to 1% atropine but not to 2% homatropine. Rutstein et al (1988),2 in their case series on accommodative spasm, reported only 1 of 17 cases that had esotropia equal to 30 prism diopters and also history of multiple surgeries for strabismus. Only 4 of the 17 patients had complete resolution of spasm during a follow-up that ranged between 4 and 30 months. They have also reported a strong association between psychogenic ailments and AS. Similar to our patient, persistent SNR with cycloplegia has been reported by Faucher and De Guise (2004).22 Rutstein and Marsh-Tootle (2004)23 have reported AS without strabismus precipitated by occlusion of right eye in a 27-year-old female patient whose symptoms were relieved with cycloplegic drug.
A thorough review of literature except for the cases illustrated by Goldstein and Schneekloth (1996)3 points out the infrequent mention of considering SNR as one of the causes in the presentation of AACE. Even in the classification of AACE originally proposed by Burian (1958)6 and subsequently used by many authors, the role of SNR and the role of cycloplegia in the diagnostic protocol warrants more attention. As many authors emphasize neurological investigations in cases of acute esotropia, Cogan and Freese (1955)24 in their paper state that patients with SNR are subjected to unnecessary neurological investigations because of the dilemma in diagnosis between AACE and SNR. The authors conclude that the “spasm of near reflex is functional and nonorganic disorder characteristically found in psychogenic individuals” and may be rarely associated with neurological abnormalities. However, ruling out central nervous system disorder should be part of the ophthalmic workup as stressed by subsequent papers. Through our case, we emphasize the need for a thorough investigation of accommodative parameters and the role of cycloplegic medications in the successful management of SNR. Another area of emphasis is the varied presentation of SNR. Our patient did not manifest any abduction limitation or pupillary miosis as frequently reported in the literature. This could be possible when there are episodic presentations of SNR with and without pupillary miosis and ocular motility restriction. Also in our patient, the dark complexion of the subject could have made it difficult to pick up subtle changes in pupillary reaction.
The role of over-minus prescription in precipitating accommodative spasm or accommodative excess related dysfunctions has not been well documented. Accommodative spasm can itself be the most possible cause for pseudomyopic presentations. However, it could also be hypothesized that an existing ill-sustained accommodative dysfunction could be disturbed or aggravated by increased minus prescriptions though there is no clear evidence available in the literature. A combination of both the hypotheses possible provides a better understanding of the clinical presentation in our subject.
Cycloplegic agents, typically atropine sulfate, in combination with reading glasses for near work, are recommended in the management of SNR. Atropine breaks the accommodative spasm by paralyzing the ciliary muscle rather than by altering accommodative effort as in the case of added plus lenses for near. On the other hand, atropine may stimulate an excessive accommodative effort and could induce convergence spasm as the patient unsuccessfully attempts to overcome the blurred near vision induced by cycloplegia, and hence near addition during cycloplegia and constant follow-ups are strongly recommended. Goldstein and Schneekloth (1996)3 report varied presentations of SNR and emphasize the need for relaxing accommodation in the management of these patients before adopting surgical management. Surgical management in these cases cannot only result in consecutive exodeviations but can possibly cause the recurrence of esodeviation if the accommodative dysfunction is not addressed. Through our case, we recommend the same and along with documentation of variations in binocular vision parameters that may be seen through the course of treatment. Although as there is no gold standard protocol for the duration of cycloplegic agents in the management of SNR, we suggest regular and long follow-ups (even up to a year) and modification of the cycloplegic agent when there is poor response to one cycloplegic agent before calling it a treatment failure. We also recommend the utilization of vision therapy such as accommodative facility exercises using plus or minus lenses and Hart chart that should be continued even after the discontinuation of cycloplegic agent. Near plus should be considered for near work that can reduce the accommodative effort and induced convergence resulting from the triad reflex. Stress management in the form of psychological counseling or yoga and meditation should also be considered as a conservative management in accommodative spasm because of the role of psychogenic illness in these anomalies.
It is necessary for an eye care professional to diagnose the condition accurately; discuss the diagnosis, the risks, and potential benefits of existing treatment options with the patient; and initiate and prolong the treatment when appropriate for sustained improvement in visual acuity and binocular vision.
Unique and varied presentation of SNR is emphasized through this case. Accommodative spasm can be associated with convergence spasm without pupillary involvement in SNR. The importance of long-term follow-up and prolonged use of cycloplegic medications and accommodative facility training in the successful management of SNR is emphasized. Also, the varied presentations of SNR in the literature along with the gap in knowledge in managing this unique entity is discussed.
Our sincere thanks to Dr. Robert Rutstein, Professor, School of Optometry, University of Alabama, Birmingham, for his valuable suggestions with language and technical content of the manuscript.
Received June 10, 2013; accepted December 3, 2013.
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