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Show Paralysis of Accommodation With Preserved Pupillary Function as the Initial Manifestation of Guillain-Barré Syndrome Blake V. Fausett, MD, PhD, Jonathan D. Trobe, MD Abstract: A 7-year-old boy who complained of blurred reading vision was found on ophthalmologic examination to have subnormal distance visual acuity in both eyes and a hyperopic refractive error that was deemed too small to explain his symptoms. Within days, he developed speech and gait deficits that led to a diagnosis of Guillain-Barré syndrome (GBS). Inpatient examination confirmed a profound binocular loss of accommodation with preservation of iris sphincter function and eye movements. This is only the second detailed report of accommodative loss with sparing of the pupil as the initial clinical manifestation of GBS. Journal of Neuro-Ophthalmology 2012;32:148-149 doi: 10.1097/WNO.0b013e318241d4b5 © 2012 by North American Neuro-Ophthalmology Society Guillain-Barré syndrome (GBS) is an immune-mediated acute polyneuropathy principally affecting motor nerves and causing paralysis (1). Impairment of the autonomic ner-vous system is relatively common, manifesting usually as labile blood pressure and cardiac arrhythmia. Loss of accommoda-tion may occur but has been previously well documented as the initial clinical manifestation in only one case (2). That case involved a 7-year-old boy who presented for evaluation of unclear vision and was found to have paralysis of accommo-dation with pupils that constricted normally to light stimula-tion. Three days after being evaluated, he began having difficulty walking and was diagnosed with GBS. We describe a nearly identical case of a 9-year-old boy who reported newly blurred vision, especially for near viewing. Given an otherwise normal ophthalmologic examination, his bilateral subnormal distance acuity was attributed to a nonorganic cause. One day later, he developed slurred speech and impaired gait and was diagnosed as having GBS. CASE REPORT An otherwise healthy 9-year-old boy complained to his mother that he was having difficulty seeing clearly when reading and playing portable video games. Six days later, he was taken to an ophthalmologist, where distance visual acuity was recorded as 20/70 in both eyes. Manifest refraction of +1.25 diopters (D) in the right eye and +1.50 D in the left eye improved distant acuity to 20/30 in both eyes. In the absence of other ophthalmic findings and systemic symp-toms, the ophthalmologist attributed these visual deficits to lack of cooperation. The following day, his mother noted that the boy's speech was slurred and that he stumbled over a curb. She took him to a pediatrician who recommended that he be emergently evaluated in hospital. In the emergency department, the patient's mother con-firmed that her son denied constitutional symptoms and was taking no medications. Examination disclosed 2/4 strength in all muscle groups proximally and distally in all 4 extremities. Deep tendon reflexes were absent throughout. Brain and spine MRIs showed enhancement of cranial and spinal nerve roots. Lumbar puncture revealed a normal opening pressure, a cell count of 2 leukocytes per cubic millimeter, and a protein of 140 mg/dL (normal 15-45 mg/ dL), findings consistent with GBS. Electromyography and nerve conduction studies were omitted because of the ade-quate evidence for that diagnosis. GQ1B antibody was not tested because of the lack of ocular motor findings. One day after hospital admission, the patient's uncorrected distance (20 feet) visual acuities were 20/40 (right eye) and 20/60 (left eye) and near (13 inches) visual acuities were 20/400 (right eye) and 20/200 (left eye). Bedside cycloplegic retinoscopy disclosed a refractive error of +1.50 D in both eyes at distance. With a +1.50-D sphere lens placed before each eye, distance visual acuity improved to 20/30; with a +4.50-D Departments of Ophthalmology and Visual Sciences (BVF, JDT) and Neurology (JDT), University of Michigan Medical School, Ann Arbor, Michigan. The authors report no conflicts of interest. Address correspondence to Jonathan D. Trobe, MD, Kellogg, Eye Center, 1000 Wall Street, Ann Arbor, MI 48105; E-mail: jdtrobe@med. umich.edu 148 Fausett and Trobe: J Neuro-Ophthalmol 2012; 32: 148-149 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. lens placed before each eye, near visual acuity improved to 20/40, but the assessment of visual acuity was hampered by the patient's irritability and unsustained cooperation. The near point of accommodation could not be reliably tested, but dynamic retinoscopy showed no evidence of accommodation. The pupils measured 5 mm in dim illumination and con-stricted normally to light and near stimuli. Ocular ductions were full, and the eyes were aligned in all positions of gaze. There was no ptosis or orbicularis oculi weakness. Biomicro-scopy and ophthalmoscopy were unremarkable. During his hospitalization, he received 5 daily doses of 10 g of intravenous immunoglobulin and underwent physical therapy for approximately 10 days. On follow-up examination 7 weeks after hospital admission, visual acuity was 20/15 bilaterally at distance and near without correc-tion, and the ophthalmologic and neurologic examinations were normal apart from reduced deep tendon reflexes. DISCUSSION This patient had accommodative loss with preserved pupillary function as the first clinical manifestation of GBS; only the second time this phenomenon has been reported in detail (2). Binocular accommodative loss is well known after expo-sure to systemic anticholinergic agents and in the setting of botulism and diphtheria. In chronic exposure to systemic anticholinergic agents, the pupils need not be affected and the patient may have no other systemic manifestations (3). The history of anticholinergic use provides explanation for the loss of accommodation. In botulism, loss of accommodation may be the earliest or most prominent neurologic finding, but there should be a preceding exposure to contaminated food and a bout of severe abdominal pain and diarrhea (1). Abduction deficits and bulbar and extremity weakness usually follow within days. Because GBS can also follow an abdominal flu-like illness, distinguishing botulism fromGBSmay be difficult. In diphtheria, paralysis of accommodation may occur without pupillary dysfunction, but patients usually report a preceding sore throat. Nasal speech, hoarseness, and difficulty swallowing are often present. Duke-Elder (4) points out that "it is not uncommon for the diagnosis of diphtheria to be made by the development of paralysis of accommodation occurring some weeks after a mild sore throat which had excited no attention." Accommodation may take years to recover (5). Isolated paralysis of accommodation may also be idiopathic. Almog (6) reported a case series of 5 patients aged 8-21 years who were referred for neuro-ophthalmologic examination due to difficulty reading. They had loss of accommodation with normally reactive pupils and normal convergence amplitudes. No one developed other neurologic symptoms. Four had nor-mal brain MRIs, and 1 patient was examined by a pediatric neurologist who found no other neurologic abnormalities. The presence or absence of deep tendon reflexes was not reported, and none of the patients underwent lumbar puncture or nerve conduction studies, so a mild form of GBS cannot be excluded in these patients. Accommodation returned to normal after several months. Chrousos et al (7) described 10 patients with asthenopia whose accommodative amplitudes were 6 D lower than expected for age. Three had convergence insufficiency. The patients were otherwise asymptomatic and were managed with spectacles. None underwent evaluation to exclude GBS. Tornqvist (8) described 2 young patients with apparently iso-lated lack of accommodation that persisted for at least 8 years in 1 patient and 2 years in another. There is no mention of the status of deep tendon reflexes, and none of the patients under-went brain imaging or nerve conduction studies. Loss of accommodation also occurs in Adie syndrome, which consists of tonic pupils and often absent deep tendon reflexes. But in Adie syndrome, pupil sphincter disturbances are much more prominent than accommodative dysfunc-tion. Bell and Thompson (9) did report 2 patients with Adie syndrome involving a tonic pupil in one eye who later developed paralysis of accommodation with normal pupil-lary function in the fellow eye. Adie (10) himself postulated that there might be an incomplete form of this syndrome manifesting as internal ophthalmoplegia alone. How might the lesions of GBS, a peripheral nervous system demyelinating disorder, cause paralysis of the ciliary body without affecting the iris sphincter, given that far more axons emanating from the ciliary ganglion are believed to innervate the ciliary body than the iris sphincter (11)? Perhaps the fibers that innervate the ciliary body express specific glycoproteins on their surface that make them partic-ularly susceptible to the immune response generated in GBS. Alternatively, the topographic organization of the postgangli-onic fibers within the short ciliary nerves might render fibers that innervate the iris sphincter less susceptible to damage. 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Acta Ophthalmol (Copenh). 1971;49:702-706. 9. Bell RA, Thompson HS. Ciliary muscle dysfunction in Adie's syndrome. Arch Ophthalmol. 1978;96:638-642. 10. Adie WJ. Complete and incomplete forms of the benign disorder characterized by tonic pupils and absent tendon reflexes. Br J Ophthalmol. 1932;16:449-461. 11. Warwick R. The ocular parasympathetic nerve supply and its mesencephalic sources. J Anat. 1954;88:71-93. Fausett and Trobe: J Neuro-Ophthalmol 2012; 32: 148-149 149 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
