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Show LETTERS TO THE EDITOR Isolated Bilateral Ptosis as the Only Ophthalmologic Sign in the Fisher Variant of Guillain- Barre ´ Syndrome Fisher syndrome ( FS) is a variant of the Guillain- Barre ´ syndrome ( GBS) that is typically characterized by the acute onset of the triad of ophthalmoplegia, ataxia, and areflexia ( 1). To our knowledge, bilateral ptosis as the only ophthalmic finding in FS has only been described only once before ( 2). A 48- year- old woman reported distal numbness in all four limbs and unsteadiness of 2 days'duration. Two weeks before presentation, she had experienced an upper respira-tory tract infection with a sore throat. Her medical history was otherwise unremarkable. Neurologic examination of the cranial nerves was normal. There were no pupillary defects, ptosis, abnormal eye movements, or nystagmus. Muscle strength was normal. Sensory examination showed a decrease in light touch and pinprick in both hands and feet, with normal vibration and joint sense in the limbs. She had mild gait ataxia. Limb dysmetria was absent. She had no dysdia-dochokinesia or rebound phenomenon. Gait was slightly unsteady but not wide- based. Stride and arm swing movements were normal. Deep tendon reflexes were absent. A complete blood count was normal, as were levels of serum electrolytes, glucose, C- reactive protein, and vitamins B6 and B12. Blood tests of coagulation and renal and thyroid function were normal. Lumbar puncture yielded a normal opening pressure and 3.9 mmol/ L glucose, 0.55 g/ L protein, and 6 leukocytes. Results of tests for human immunodefi-ciency virus, herpes simplex virus, varicella zoster virus, cytomegalovirus, Epstein- Barr virus, and Borrelia burgdorferi infection were negative in blood and cerebrospinal fluid ( CSF). A paraneoplastic antibody screen showed negative results. CSF sent for anti- GQ1b IgG antibody measurement was lost. Four days later she returned because of increasing dysesthesias and unsteadiness. Bilateral ptosis was now evident with pupils of normal size and reactivity ( Fig. 1). Eye movements were normal, and the eyes were aligned. She had no facial weakness, slurred speech, or swallowing difficulty. Muscle power was decreased in all 4 limbs ( Medical Research Council grade 4). There was marked extremity ataxia and such severe gait ataxia that the patient could not ambulate without holding on to the furniture. Results of brain MRI were normal. Electrodiagnostic studies, performed 6 days after onset of symptoms, showed normal motor and sensory conduction velocities in upper and lower limb nerves, normal ulnar sensory nerve action potential amplitudes, and only slightly diminished sural sensory nerve action potential amplitudes ( 2 and 7 mV). Compound muscle action potentials amplitudes of tested motor nerves were moderately diminished ( abductor digiti quinti 5.3 mVand extensor digitorum brevis 3.6 mV). In the left median motor nerve, partial conduction block was present ( 9.6 and 6.4 mVafter distal and proximal stimulation, respectively). F wave latencies were normal in the median nerve but could not be elicited in the ulnar and peroneal nerves. The H- reflex over S1 could not be elicited bilaterally. A 5- day course of 0.4 g/ kg/ day intravenous immu-noglobulin was given, during which neurologic symptoms started to resolve. Ataxia diminished and muscle power returned to normal in less than 2 weeks. The ptosis started to improve during the same period. Six weeks later mild bilateral ptosis was the only residual neurologic finding. At 3 months after disease onset, the ptosis had resolved and deep tendon reflexes had returned. Anti- GQ1b IgG antibody was negative at this time. FIG. 1. At presentation, the patient displayed bilateral ptosis with clear contraction of the frontalis muscle ( A) and isocoria ( B). 354 J Neuro- Ophthalmol, Vol. 29, No. 4, 2009 Although bilateral ptosis was the only ophthalmo-logic finding in this case, the numbness, areflexia, ataxia, electromyography/ nerve conduction velocity results, and clinical course supported the diagnosis of the Fisher variant of GBS. Since the original description of this syndrome in 1956 ( 1), there has been a continuing debate about the site of the lesion. Fisher ( 1) considered the syndrome to be a variant of GBS because of weakness and sensory changes in the limbs, absent deep tendon reflexes, and raised protein in CSF. However, he admitted that the signs were difficult to explain on a purely peripheral basis, because of the remarkable symmetry of the ophthalmologic manifestations during progression and recovery. Others believe that this syndrome involves both the central and the peripheral nervous system ( 3,4). The presence of bilateral ptosis as the only ophthalmologic finding does not resolve the issue of lesion location, the cause being either a central dorsal nuclear lesion of the oculomotor complex or lesions of both nerves innervating the levator palpebrae muscles. Najim al- Din et al. ( 3) have shown that the ophthalmologic manifestations of the Fisher variant of GBS can vary greatly. Our patient confirms that they can also be minimal. Xenia L Stalpers, MD, MSc Wim I. M. Verhagen MD, PhD Jan Meulstee MD, PhD Department of Neurology, Canisius- Wilhelmina Hospital, Nijmegen, The Netherlands x. stalpers@ cwz. nl REFERENCES 1. Fisher M. An unusual variant of acute idiopathic polyneuritis ( syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 1956; 255: 57- 65. 2. Yip PK. Bilateral ptosis, ataxia and areflexia- a variant of Fisher's syndrome. J Neurol Neurosurg Psychiatry 1991; 54: 1121. 3. Najim al- Din AS, Anderson M, Eeg- Olofsson O, et al. Neuro-ophthalmic manifestations of the syndrome of ophthalmoplegia, ataxia and areflexia. Observations on 20 patients. Acta Neurol Scand 1994; 89: 87- 94. 4. Meienberg O, Ryffel E. Supranuclear eye movement disorders in Fisher's syndrome of ophthalmoplegia, ataxia and areflexia. Report of a case and literature review. Arch Neurol 1983; 40: 402- 405. Papilledema and Visual Loss as the Presenting Signs of a Primary Spinal Primitive Neuroectodermal Tumor We report a patient with a primary spinal primitive neuroectodermal tumor ( PNET) who presented with papilledema and visual loss before the appearance of myelopathic symptoms. A 28- year- old woman had a 5- month history of intense headaches and progressive visual loss that affected her left eye more than her right eye. Best- corrected visual acuities were 20/ 50 in the right eye and 20/ 400 in the left eye. She was able to identify 11/ 11 Ishihara color plates with the right eye and 0/ 11 with the left eye. A relative afferent pupillary defect was noted in the left eye. Ophthalmoscopy revealed severe bilateral optic disc swelling associated with macular edema, retinal hemor-rhages, and hard exudates ( Fig. 1). Automated visual fields demonstrated severe constriction. Results of the neurolog-ical examination were normal. Results of CTof the head and orbits and of brain MRI and magnetic resonance venography were normal. Lumbar puncture opening pressure was 630 mm H2O. There were 2 white blood cells ( all mononuclear), 694 red blood cells, 500 mg/ dL protein, and 16 mg/ dL glucose. The elevated protein and decreased glucose levels raised concern for fungal or tuberculosis meningitis, although the patient did not have symptoms to suggest this diagnosis. Results of serum tests for syphilis, toxoplasmosis, HIV, hepatitis A, B, and C, tuberculosis, and Bartonella henselae were negative, as were results of cerebrospinal ( CSF) fungal culture, India ink and KOH preparations, Gram stain, and acid fast bacilli ( AFB) smear and culture. The patient was treated with 250 mg methylprednis-olone intravenously every 6 hours for 5 days and 500 mg acetazolamide intravenously every 6 hours for 5 days with no improvement in visual function. A left optic nerve sheath fenestration was performed with stabilization of vision. Approximately 1 week after the optic nerve sheath fenestration was performed, the patient developed back pain and difficulty walking. Complete spine MRI showed 2 cervical intradural extramedullary masses at C3 and C7, 3 thoracic intramedullary enhancing masses at T4, T8, and FIG. 1. Fundus photographs obtained at presentation show bilateral optic disc edema, hard exudates, and macular edema. 355 Letters to the Editor J Neuro- Ophthalmol, Vol. 29, No. 4, 2009 T12- L1, and 2 cauda equina masses, one at the left S1 root and the other in the posterior lumbar vertebrae at L2 ( Fig. 2). Chest and abdominopelvic MRI showed no abnormalities. The patient refused further medical treatment. Two months later, she returned to the hospital complaining of decreased movement and sensation of both lower extrem-ities and worsening of vision. She subsequently underwent optic nerve sheath fenestration on the right side. A T5- T7 laminectomy with intramedullary biopsy showed a primitive neuroectodermal tumor ( PNET). She refused further intervention and died 3 months later of respiratory failure. Papilledema associated with spinal cord tumors was first described by Taylor and Collier in 1901 ( 1). Since then the association of papilledema and spinal cord tumors has been well documented ( 2- 12). However, papilledema and visual loss preceding myelopathic symptoms occurs much less frequently. There are 11 reported cases, including 1 in French and 1 in Spanish ( 13- 19). In those patients, myelopathic signs developed between 1 and 7 months after the onset of visual symptoms. Five patients had hydro-cephalus at presentation. All had elevated CSF protein with normal CSF glucose except for 1 patient with a low glucose level ( 19). The patient with the low CSF glucose level had a PNET but was treated for tuberculous meningitis before the correct diagnosis was made. We emphasize not only that visual symptoms from papilledema may be a presenting feature of a spinal cord tumor before myelopathic manifestations have appeared but also that spinal fluid analysis may show elevated protein and decreased glucose levels and thus be mistaken for signs of infectious meningitis unless proper spinal imaging is done. Cristobal Cruz Colon, MD Lilliam Dı ´ az, MD Julio A. Rodrı ´ guez- Padilla, MD Luis Serrano, MD Department of Ophthalmology University of Puerto Rico Rı ´ o Piedras, Puerto Rico lserrano@ coqui. net REFERENCES 1. Taylor J, Collier J. The occurrence of optic neuritis in lesions of the spinal cord: injury, tumor, myelitis: an account of twelve cases and one autopsy. Brain 1901; 24: 532- 53. 2. Teng P, Wagner JH, Buxbaum MV. Giant ependymoma of the spinal cord associated with papilledema: review of literature and report of a case. Arch Neurol 1960; 2: 657- 62. 3. Raynor RB. Papilledema associated with tumors of the spinal cord. Neurology 1969; 19: 700- 4. 4. Luzecky M, Siegel BA, Coxe WS, et al. Papilledema and communicating hydrocephalus: association with a lumbar neurofi-broma. Arch Neurol 1974; 30: 487- 89. 5. Farmilo RW, McAuley DL, Osborne DR. Papilloedema and spinal cord tumours. N Z Med J 1974; 80: 100- 4. 6. Ammerman BJ, Smith DR. Papilledema and spinal cord tumors. Surg Neurol 1975; 3: 55- 7. 7. Luxon LM, Harrison MJ. Subarachnoid hemorrhage and papilledema due to a cervical neurilemmoma.. J Neurosurg 1978; 48: 1015- 18. 8. Tanaka K, Waga S, Shimosaka S. Papilledema and spinal cord tumors. Surg Neurol 1988; 29: 462- 66. 9. Hardten DR, Wen DY, Wirtschafter JD, et al. Papilledema and intraspinal lumbar paraganglioma. J Clin Neuroophthalmol 1992; 12: 158- 62. 10. Amlashi SF, Riffaud L. Images in clinical medicine: papilledema and spinal cord tumor. N Engl J Med 2004; 350: e18. 11. Kounin GK, Romansky KV, Traykov LD, et al. Primary spinal melanoma with bilateral papilledema. Clin Neurol Neurosurg 2005; 107: 525- 27. FIG. 2. Three weeks after presentation, when the patient first reported back pain and difficulty walking, postcontrast T1 sagittal MRI shows intramedullary masses at C3 ( A), C7 ( A, B), T4 ( B), T12 ( C), and caudal equina ( C), together with thickened, enhancing leptomeninges ( C). Biopsy of the thoracic lesion showed findings consistent with primitive neuroectodermal tumor. ( Courtesy of Liana Lopez, MD). 356 q 2009 North American Neuro- Ophthalmology Society J Neuro- Ophthalmol, Vol. 29, No. 4, 2009 Letters to the Editor 12. Wu XJ, Chen HR, Chen JX, et al. Papilledema caused by a thoracic schwannoma. J Neuroophthalmol 2009; 29: 156- 67. 13. Milla ´ n- Rodrı ´ guez AC, La ´ zaro- Gonza ´ lez V, Dios- Castro E, et al. Visual acuity loss, the initial sympton of a spinal cord neoplasm. Arch Soc Esp Oftalmol 2008; 83: 437- 40. 14. Costello F, Kardon RH, Wall M, et al. Papilledema as the presenting manifestation of spinal schwannoma. J Neuroophthalmol 2002; 22: 199- 203. 15. Oikawa S, Kyoshima K, Takemae T, et al. Multiple spinal neurinomas presenting visual disturbance as the initial symptom: case report. Surg Neurol 1992; 38: 309- 14. 16. Guigou S, Mercie ´ M, Blanc JL, et al. Bilateral papilledema as the manifestation of Schwannoma of the cauda equina. J Fr Ophtalmol 2006; 29: 312- 18. 17. Kesler A, Manor RS. Papilloedema and hydrocephalus in spinal cord ependymoma. Br J Ophthalmol 1994; 78: 313- 15. 18. Porter A, Lyons MK, Wingerchuk DM, et al. Spinal cord astrocytoma presenting as " idiopathic" intracranial hypertension. Clin Neurol Neurosurg 2006; 108: 787- 89. 19. Chen YC, Tang LM, Chen CJ, et al. Intracranial hypertension as an initial manifestation of spinal neuroectodermal tumor. Clin Neurol Neurosurg 2005; 107: 408- 11. 357 Letters to the Editor J Neuro- Ophthalmol, Vol. 29, No. 4, 2009 |