Correspondence
Chronic papilledema with vision loss as the presenting feature in racemose neurocysticercosis Neurocysticercosis is considered as the most common helminthic infection of the human central nervous system.1 The disease is common in the Indian subcontinent, Central and South America, Spain, and Eastern Europe, and the incidence is rising in nonendemic areas because of immigration.2 Neurocysticercosis can present with various manifestations, but papilledema with visual loss is described rarely as a presenting manifestation of neurocysticercosis. Racemose form is a type of neurocysticercosis consisting of a single large vesicle or mass of vesicles that often resembles a cluster of grapes and accounts for 10% of the cases of neurocysticercosis.3 It is most frequently found in the basal cisterns, Sylvian fissure, and ventricles, but can extend into the brain parenchyma and produce obstructive hydrocephalus.3 We report a case of racemose neurocysticercosis that presented to us as chronic papilledema with bilateral optic atrophy set in with total loss of vision in 1 eye and significant visual field loss in the other eye. A 22-year-old male presented with complaints of headache and blurring of vision for 4 months. There was no history of seizures, vomiting, and other focal neurologic signs. Ocular examination revealed a visual acuity of no perception of light in the right eye and 6/12 in the left eye. There was a total afferent pupillary defect in the right eye. Anterior segment examination was normal in both eyes. Fundus evaluation showed chronic papilledema with pallor
in both eyes (Fig. 1A). Visual field of the left eye showed severe constriction with sparing of less than 10 degrees of the temporal field. Neurologic evaluation was normal. In view of papilledema, he underwent magnetic resonance imaging (MRI) of brain with contrast. Neuroimaging confirmed fluid around the optic nerve (Fig. 2A) and showed multiloculated cysts in the basal cisterns, right sylvian, and anterior interhemispheric fissure without any scolex suggestive of racemose neurocysticercosis (Fig. 2) with moderate obstructive hydrocephalus (Fig. 2B). The patient was started on antihelminthic treatment. In view of loss of vision in the right eye and significant visual field loss in the left eye with chronic papilledema, he underwent emergency right craniotomy with decompression of the cysts. Histopathology of the cysticercous revealed large convoluted cysts with hyalinized degenerated wall showing outer cuticular layer and single-layered subcuticular cells; scolices were not seen (Fig. 3). Three weeks after neurosurgical intervention, because no resolution of the papilledema was noted, the patient underwent a ventriculoperitoneal shunt. Two weeks later, resolution of papilledema was noted (Fig. 1B), and although the vision in the right eye remained unchanged, the left eye vision improved to 6/9 from 6/12 with mild improvement in the visual field. Cysticercosis is caused by cysticercous larvae of Taenia solium, and neurocysticercosis is a common cause for intracranial space-occupying lesions in endemic areas. It can present as subarachnoid-cisternal, parenchymal, ventricular, or with spinal involvement.4 Parenchymal cysts commonly result in seizures, headache, or focal neurologic
Fig. 1 — Fundus photograph of right and left eyes. A, Chronic papilledema as presenting feature. B, Resolving disc edema with pallor in the right eye more than the left eye after treatment.
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CAN J OPHTHALMOL — VOL. 49, NO. 1, FEBRUARY 2014
Correspondence
Fig. 2 — Magnetic resonance imaging of brain with contrast showing multiple cysts of racemose neurocysticercosis. A, T2-weighted coronal scan showing fluid ring around the optic nerve in both eyes (thin arrows) suggestive of papilledema with racemose cysts in the anterior interhemispheric fissure (thick arrow). B, T2-weighted coronal scan showing cyst in right sylvian fissure (thick arrow) with dilated lateral ventricles suggestive of moderate obstructive hydrocephalus (thin arrow). C, T2-weighted axial FLAIR sequence showing cyst appearing like cluster of grapes in the anterior interhemispheric and right sylvian fissures (arrows). D, T2-weighted axial scan without FLAIR showing cyst appearing like cluster of grapes in the anterior interhemispheric and right sylvian fissures (arrows).
deficits, whereas ventricular or subarachnoid-cisternal cysts result in obstructive hydrocephalus caused by direct obstruction of the ventricular system by the cyst or secondary to meningeal inflammation. Subarachnoid and
intraventricular cysts are rare in India.5 These are larger, multilobulated, arranged in grapelike clusters, and called racemose neurocysticercosis. Racemose cysts do not show the temporal developmental stages like vesicular, colloid,
Fig. 3 — Histopathologic section of the cysts. A, Interconnecting cluster of cysticerci showing individual cysts (arrows). Hematoxylin and eosin staining; original magnification 5. B, Individual cyst with outer prominent investing tegument layer with hyalinized degenerated wall showing outer cuticular layer and single-layered subcuticular cells; scolices are not seen. Hematoxylin and eosin staining; original magnification 10. CAN J OPHTHALMOL — VOL. 49, NO. 1, FEBRUARY 2014
e27
Correspondence granular-nodular, and calcific that are seen with parenchymal cysts.6 This form of neurocysticercosis constitutes a hydropic change as degenerated cyst wall shows a rapid hyalinization and enlargement that leads to large or even giant vesicles usually devoid of a scolex. The cysts attain large size because the growth is not stopped by pressure effects exerted by brain parenchyma.7 MRI has been shown to be modality of choice for diagnosing neurocysticercosis,8 but histopathologic examination might be required in a few cases for confirmation.9 Differential diagnosis for multiple cysts includes cryptococcal cysts, with histopathology being confirmatory.10 Patients with neurocysticercosis mainly present with seizures/epilepsy (79%), severe headache (38%), focal deficits (16%), and signs of raised intracranial pressure (12%).11 Although visual loss is not a common cause of presentation in neurocysticercosis, it is a common feature occurring secondary to optic neuropathy resulting from papilledema. Other causes of visual loss include chiasmal involvement from inflammation or compression by large cysts and retrochiasmal damage from compression or vasculitic cerebral infarction.12 Sotelo et al.13 noted that 28% of their 753 cysticercal patients had papilledema, with 10% having decreased vision. Similarly, Takayanagui and Odashima14 reported obstructive hydrocephalus with progressive intracranial hypertension in 50% to 60% of cases with racemose neurocysticercosis. However, none of the reports mentions visual loss with papilledema as the presenting symptom. The treatment of neurocysticercosis is controversial, consisting of cysticidal drugs, steroids, ventriculoperitoneal shunt, and surgical excision of the cysts. The surgical intervention is reserved for those exhibiting compression of brain and cranial nerves, presence of raised intracranial hypertension with or without obstructive hydrocephalus. Prompt diagnosis is essential for treatment in cases of neurocysticercosis, mainly in the intraventricular or subarachnoid-cisternal racemose form because they present clinically in a more aggressive manner as compared with the parenchymal form.15 Racemose neurocysticercosis, although less common, is a serious variety of neurocysticercosis resulting in obstructive hydrocephalus. We present a case of a 22-year-old male with total visual loss in the right eye and visual field loss in the left eye after chronic papilledema secondary to racemose cysts in the subarachnoid-cisternal spaces causing hydrocephalus. Early recognition and surgical intervention can
help in preservation of vision. Ophthalmologists must be aware of this entity as a differential diagnosis of intracranial space-occupying lesions mainly in endemic areas.
Familial exudative vitreoretinopathy mimicking macular telangiectasia type 1
were unremarkable. Fundus examination of the right eye revealed several aneurysmal dilatations temporal to the macula, together with a small area of adjacent fibrosis (Fig. 1). Clinical examination of the left eye was unremarkable. Fundus fluorescein angiography revealed slow filling of the aneurysms with late faint leakage. Furthermore, there was evidence of avascularity of the temporal
A 21-year-old asymptomatic white male in good health was referred by his optometrist for evaluation of a right fundus lesion. His visual acuity was 20/20 OU, and his anterior segment examination and intraocular pressures
e28
Jyoti Matalia, Hemant Anaspure, Nirupama Kasturi, Bhujang K. Shetty Pediatric Ophthalmology and Strabismology Services, Anekal Taluk, Bangalore, India. Correspondence to: Jyoti Matalia, DNB: [email protected] REFERENCES 1. Kim SW, Kim MK, Oh SM, Park SH. Racemose cysticercosis in the cerebellar hemisphere. J Korean Neurosurg Soc. 2010;48:59-61. 2. Sawhney IM, Singh G, Lekhra OP, Mathuriya SN, Parihar PS, Prabhakar S. Uncommon presentations of neurocysticercosis. J Neurol Sci. 1998;154:94-100. 3. Pittella JE. Neurocysticercosis. Brain Pathol. 1997;7:681-93. 4. Kimura-Hayama ET, Higuera JA, Corona-Cedillo R, et al. Neurocysticercosis: radiologic-pathologic correlation. Radiographics. 2010;30: 1705-19. 5. Ghose D, Dubey TN, Prabhakar S. Brain parenchymal, subarachnoid racemose, and intraventricular cysticercosis in an Indian man. Postgrad Med J. 1999;75:164-7. 6. Mittal P, Mittal G. Intraventricular and subarachnoid racemose cysticercosis. Trop Parasitol. 2011;1:111-2. 7. Arora A, Puri SK, Upreti L. Intracranial Neurocysticercosis. In: Arora A, Puri SK, Upreti L (Eds). Brain Imaging: Case Review Series. New Delhi: Jaypee Brothers Medical Publishers; 2011;80. 8. Martinez HR, Rangel-Guerra R, Elizondo G, et al. MR imaging in neurocysticercosis: a study of 56 cases. AJNR Am J Neuroradiol. 1989;10:1011-9. 9. Bannur U, Rajshekhar V. Cisternal cysticercosis: a diagnostic problem—a short case report. Neurol India. 2001;49:206-8. 10. Mathews M, Pare L, Hasso A. Intraventricular crytococcal cysts masquerading as racemose neurocysticercosis. Surg Neurol. 2007;67:647-9. 11. Carabin H, Ndimubanzi PC, Budke CM, et al. Clinical manifestations associated with neurocysticercosis: a systematic review. PLoS Negl Trop Dis. 2011;5:e1152. 12. Chang GY, Keane JR. Visual loss in cysticercosis: analysis of 23 patients. Neurology. 2001;57:545-8. 13. Sotelo J, Guerrero V, Rubio F. Neurocysticercosis: a new classification based on active and inactive forms. A study of 753 cases. Arch Intern Med. 1985;145:442-5. 14. Takayanagui OM, Odashima NS. Clinical aspects of neurocysticercosis. Parasitol Int. 2006;55:S111-5. 15. Sinha S, Sharma BS. Intraventricular neurocysticercosis: a review of current status and management issues. Br J Neurosurg. 2012;26: 305-9. Can J Ophthalmol 2014;49:e26–e28 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.11.002
CAN J OPHTHALMOL — VOL. 49, NO. 1, FEBRUARY 2014
Chronic papilledema with vision loss as the presenting feature in racemose neurocysticercosis Neurocysticercosis is considered as the most common helminthic infection of the human central nervous system.1 The disease is common in the Indian subcontinent, Central and South America, Spain, and Eastern Europe, and the incidence is rising in nonendemic areas because of immigration.2 Neurocysticercosis can present with various manifestations, but papilledema with visual loss is described rarely as a presenting manifestation of neurocysticercosis. Racemose form is a type of neurocysticercosis consisting of a single large vesicle or mass of vesicles that often resembles a cluster of grapes and accounts for 10% of the cases of neurocysticercosis.3 It is most frequently found in the basal cisterns, Sylvian fissure, and ventricles, but can extend into the brain parenchyma and produce obstructive hydrocephalus.3 We report a case of racemose neurocysticercosis that presented to us as chronic papilledema with bilateral optic atrophy set in with total loss of vision in 1 eye and significant visual field loss in the other eye. A 22-year-old male presented with complaints of headache and blurring of vision for 4 months. There was no history of seizures, vomiting, and other focal neurologic signs. Ocular examination revealed a visual acuity of no perception of light in the right eye and 6/12 in the left eye. There was a total afferent pupillary defect in the right eye. Anterior segment examination was normal in both eyes. Fundus evaluation showed chronic papilledema with pallor
in both eyes (Fig. 1A). Visual field of the left eye showed severe constriction with sparing of less than 10 degrees of the temporal field. Neurologic evaluation was normal. In view of papilledema, he underwent magnetic resonance imaging (MRI) of brain with contrast. Neuroimaging confirmed fluid around the optic nerve (Fig. 2A) and showed multiloculated cysts in the basal cisterns, right sylvian, and anterior interhemispheric fissure without any scolex suggestive of racemose neurocysticercosis (Fig. 2) with moderate obstructive hydrocephalus (Fig. 2B). The patient was started on antihelminthic treatment. In view of loss of vision in the right eye and significant visual field loss in the left eye with chronic papilledema, he underwent emergency right craniotomy with decompression of the cysts. Histopathology of the cysticercous revealed large convoluted cysts with hyalinized degenerated wall showing outer cuticular layer and single-layered subcuticular cells; scolices were not seen (Fig. 3). Three weeks after neurosurgical intervention, because no resolution of the papilledema was noted, the patient underwent a ventriculoperitoneal shunt. Two weeks later, resolution of papilledema was noted (Fig. 1B), and although the vision in the right eye remained unchanged, the left eye vision improved to 6/9 from 6/12 with mild improvement in the visual field. Cysticercosis is caused by cysticercous larvae of Taenia solium, and neurocysticercosis is a common cause for intracranial space-occupying lesions in endemic areas. It can present as subarachnoid-cisternal, parenchymal, ventricular, or with spinal involvement.4 Parenchymal cysts commonly result in seizures, headache, or focal neurologic
Fig. 1 — Fundus photograph of right and left eyes. A, Chronic papilledema as presenting feature. B, Resolving disc edema with pallor in the right eye more than the left eye after treatment.
e26
CAN J OPHTHALMOL — VOL. 49, NO. 1, FEBRUARY 2014
Correspondence
Fig. 2 — Magnetic resonance imaging of brain with contrast showing multiple cysts of racemose neurocysticercosis. A, T2-weighted coronal scan showing fluid ring around the optic nerve in both eyes (thin arrows) suggestive of papilledema with racemose cysts in the anterior interhemispheric fissure (thick arrow). B, T2-weighted coronal scan showing cyst in right sylvian fissure (thick arrow) with dilated lateral ventricles suggestive of moderate obstructive hydrocephalus (thin arrow). C, T2-weighted axial FLAIR sequence showing cyst appearing like cluster of grapes in the anterior interhemispheric and right sylvian fissures (arrows). D, T2-weighted axial scan without FLAIR showing cyst appearing like cluster of grapes in the anterior interhemispheric and right sylvian fissures (arrows).
deficits, whereas ventricular or subarachnoid-cisternal cysts result in obstructive hydrocephalus caused by direct obstruction of the ventricular system by the cyst or secondary to meningeal inflammation. Subarachnoid and
intraventricular cysts are rare in India.5 These are larger, multilobulated, arranged in grapelike clusters, and called racemose neurocysticercosis. Racemose cysts do not show the temporal developmental stages like vesicular, colloid,
Fig. 3 — Histopathologic section of the cysts. A, Interconnecting cluster of cysticerci showing individual cysts (arrows). Hematoxylin and eosin staining; original magnification 5. B, Individual cyst with outer prominent investing tegument layer with hyalinized degenerated wall showing outer cuticular layer and single-layered subcuticular cells; scolices are not seen. Hematoxylin and eosin staining; original magnification 10. CAN J OPHTHALMOL — VOL. 49, NO. 1, FEBRUARY 2014
e27
Correspondence granular-nodular, and calcific that are seen with parenchymal cysts.6 This form of neurocysticercosis constitutes a hydropic change as degenerated cyst wall shows a rapid hyalinization and enlargement that leads to large or even giant vesicles usually devoid of a scolex. The cysts attain large size because the growth is not stopped by pressure effects exerted by brain parenchyma.7 MRI has been shown to be modality of choice for diagnosing neurocysticercosis,8 but histopathologic examination might be required in a few cases for confirmation.9 Differential diagnosis for multiple cysts includes cryptococcal cysts, with histopathology being confirmatory.10 Patients with neurocysticercosis mainly present with seizures/epilepsy (79%), severe headache (38%), focal deficits (16%), and signs of raised intracranial pressure (12%).11 Although visual loss is not a common cause of presentation in neurocysticercosis, it is a common feature occurring secondary to optic neuropathy resulting from papilledema. Other causes of visual loss include chiasmal involvement from inflammation or compression by large cysts and retrochiasmal damage from compression or vasculitic cerebral infarction.12 Sotelo et al.13 noted that 28% of their 753 cysticercal patients had papilledema, with 10% having decreased vision. Similarly, Takayanagui and Odashima14 reported obstructive hydrocephalus with progressive intracranial hypertension in 50% to 60% of cases with racemose neurocysticercosis. However, none of the reports mentions visual loss with papilledema as the presenting symptom. The treatment of neurocysticercosis is controversial, consisting of cysticidal drugs, steroids, ventriculoperitoneal shunt, and surgical excision of the cysts. The surgical intervention is reserved for those exhibiting compression of brain and cranial nerves, presence of raised intracranial hypertension with or without obstructive hydrocephalus. Prompt diagnosis is essential for treatment in cases of neurocysticercosis, mainly in the intraventricular or subarachnoid-cisternal racemose form because they present clinically in a more aggressive manner as compared with the parenchymal form.15 Racemose neurocysticercosis, although less common, is a serious variety of neurocysticercosis resulting in obstructive hydrocephalus. We present a case of a 22-year-old male with total visual loss in the right eye and visual field loss in the left eye after chronic papilledema secondary to racemose cysts in the subarachnoid-cisternal spaces causing hydrocephalus. Early recognition and surgical intervention can
help in preservation of vision. Ophthalmologists must be aware of this entity as a differential diagnosis of intracranial space-occupying lesions mainly in endemic areas.
Familial exudative vitreoretinopathy mimicking macular telangiectasia type 1
were unremarkable. Fundus examination of the right eye revealed several aneurysmal dilatations temporal to the macula, together with a small area of adjacent fibrosis (Fig. 1). Clinical examination of the left eye was unremarkable. Fundus fluorescein angiography revealed slow filling of the aneurysms with late faint leakage. Furthermore, there was evidence of avascularity of the temporal
A 21-year-old asymptomatic white male in good health was referred by his optometrist for evaluation of a right fundus lesion. His visual acuity was 20/20 OU, and his anterior segment examination and intraocular pressures
e28
Jyoti Matalia, Hemant Anaspure, Nirupama Kasturi, Bhujang K. Shetty Pediatric Ophthalmology and Strabismology Services, Anekal Taluk, Bangalore, India. Correspondence to: Jyoti Matalia, DNB: [email protected] REFERENCES 1. Kim SW, Kim MK, Oh SM, Park SH. Racemose cysticercosis in the cerebellar hemisphere. J Korean Neurosurg Soc. 2010;48:59-61. 2. Sawhney IM, Singh G, Lekhra OP, Mathuriya SN, Parihar PS, Prabhakar S. Uncommon presentations of neurocysticercosis. J Neurol Sci. 1998;154:94-100. 3. Pittella JE. Neurocysticercosis. Brain Pathol. 1997;7:681-93. 4. Kimura-Hayama ET, Higuera JA, Corona-Cedillo R, et al. Neurocysticercosis: radiologic-pathologic correlation. Radiographics. 2010;30: 1705-19. 5. Ghose D, Dubey TN, Prabhakar S. Brain parenchymal, subarachnoid racemose, and intraventricular cysticercosis in an Indian man. Postgrad Med J. 1999;75:164-7. 6. Mittal P, Mittal G. Intraventricular and subarachnoid racemose cysticercosis. Trop Parasitol. 2011;1:111-2. 7. Arora A, Puri SK, Upreti L. Intracranial Neurocysticercosis. In: Arora A, Puri SK, Upreti L (Eds). Brain Imaging: Case Review Series. New Delhi: Jaypee Brothers Medical Publishers; 2011;80. 8. Martinez HR, Rangel-Guerra R, Elizondo G, et al. MR imaging in neurocysticercosis: a study of 56 cases. AJNR Am J Neuroradiol. 1989;10:1011-9. 9. Bannur U, Rajshekhar V. Cisternal cysticercosis: a diagnostic problem—a short case report. Neurol India. 2001;49:206-8. 10. Mathews M, Pare L, Hasso A. Intraventricular crytococcal cysts masquerading as racemose neurocysticercosis. Surg Neurol. 2007;67:647-9. 11. Carabin H, Ndimubanzi PC, Budke CM, et al. Clinical manifestations associated with neurocysticercosis: a systematic review. PLoS Negl Trop Dis. 2011;5:e1152. 12. Chang GY, Keane JR. Visual loss in cysticercosis: analysis of 23 patients. Neurology. 2001;57:545-8. 13. Sotelo J, Guerrero V, Rubio F. Neurocysticercosis: a new classification based on active and inactive forms. A study of 753 cases. Arch Intern Med. 1985;145:442-5. 14. Takayanagui OM, Odashima NS. Clinical aspects of neurocysticercosis. Parasitol Int. 2006;55:S111-5. 15. Sinha S, Sharma BS. Intraventricular neurocysticercosis: a review of current status and management issues. Br J Neurosurg. 2012;26: 305-9. Can J Ophthalmol 2014;49:e26–e28 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.11.002
CAN J OPHTHALMOL — VOL. 49, NO. 1, FEBRUARY 2014
