Letters to Editor
a
b
c
d
Figure 3: Immunohistochemical profile: Immunohistochemistry showing the tumor cells to be immunopositive for glial fibrillary acidic protein (GFAP) (a) and vimentin (b), with dot like positivity for epithelial membrane antigen (EMA) (c). MIB‑1 LI is very low (d) (×400)
have features similar to ependymomas, with intercellular junctions and microlumens filled with microvilli.[2] However, cortical location of AG is contrary to this hypothesis. An origin from neoplastic transformation of radial glial cells during neuronal migration has now been proposed, based on elongated bipolar morphology of tumor cells.[1]
caused by angiocentric glioma complicated with focal cortical dysplasia: A surgical case series. J Neurooncol 2012;110:375‑80. 4. Burger PC, Jouvet A, Preusser M, Hans VH, Rosenblum MK, Lellouch‑Tubiana A. Angiocentric glioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. World Health Organization Classification of Tumours of the Central Nervous System. 4th ed. Lyon: IARC; 2007. p. 92‑3. 5. Takada S, Iwasaki M, Suzuki H, Nakasato N, Kumabe T, Tominaga T. Angiocentric glioma and surrounding cortical dysplasia manifesting as intractable frontal lobe epilepsy‑‑Case report. Neurol Med Chir (Tokyo) 2011;51:522‑6. 6. Prayson RA. Tumours arising in the setting of paediatric chronic epilepsy. Pathology 2010;42:426‑31. 7. Marburger T, Prayson R. Angiocentric glioma: A clinicopathologic review of 5 tumors with identification of associated cortical dysplasia. Arch Pathol Lab Med 2011;135:1037‑41. 8. Raghunathan A, Olar A, Vogel H, Parker JR, Coventry SC, Debski R, et al. Isocitrate dehydrogenase 1 R132H mutation is not detected in angiocentric glioma. Ann Diagn Pathol 2012;16:255‑9. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.149405
Received: 04‑11‑2014 Review completed: 09‑11‑2014 Accepted: 20‑12‑2014
Outcome of AG patients is excellent, with most achieving freedom from seizures following gross total resection, but subtotal resection does not have such good results, with reported seizure‑free outcomes of 93% vs 36%.[3,5] Although benign in nature, one fatality has been attributed to AG, due to progressive disease.[2] The primary tumor showed typical histological features; however, the recurrent tumor displayed increased mitoses and elevated MIB‑1 LI (10%), indicating aggressive behavior.
Yolk sac tumor of the temporal bone: An unusual presentation as hydrocephalus
Aanchal Kakkar, Mehar Chand Sharma, Vaishali Suri, Seema Kaushal, Sarat P. Chandra1, Ajay Garg2, Chitra Sarkar
A 3‑year‑old‑male child presented with progressive increase in head size since birth and a right temporal swelling since 2 months. He had symptoms of raised intracranial pressure viz. irritability and decreased vision in both eyes. There was no history of fever, weight loss, or vomiting. On examination, the child had disproportionate head size (circumference 59 cm) with open and tense anterior fontenelle [Figure 1a]. A soft to firm, warm, non‑tender swelling was identified over right temporal region measuring 12 cm, extending from external auditory meatus to lateral canthus of right eye. There were no sensory, motor, or cranial nerve deficits. Computed tomography showed a right temporal soft tissue space‑occupying lesion with destruction of right temporal bone, extension into the cranial cavity, and evidence of hydrocephalus [Figure 1b]. Cerebrospinal fluid examination was normal. Patient underwent shunt
Departments of Pathology, Neurosurgery1 and Neuroradiology2, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India E‑mail: [email protected]
References 1. Lellouch‑Tubiana A, Boddaert N, Bourgeois M, Fohlen M, Jouvet A, Delalande O, et al. Angiocentric neuroepithelial tumor (ANET): A new epilepsy‑related clinicopathological entity with distinctive MRI. Brain Pathol 2005;15:281‑6. 2. Wang M, Tihan T, Rojiani AM, Bodhireddy SR, Prayson RA, Iacuone JJ, et al. Monomorphous angiocentric glioma: A distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. J Neuropathol Exp Neurol 2005;64:875‑81. 3. Liu CQ, Zhou J, Qi X, Luan GM. Refractory temporal lobe epilepsy Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
Sir, Yolk sac tumor (YST) occurring at extragonadal sites usually involves midline structures viz. brain, mediastinum, retroperitoneum. YST of head and neck is rare, and involvement of skull as a primary site is extremely so.[1]
679
Letters to Editor
Tumor cells were immunopositive for pancytokeratin and alpha fetoprotein (AFP), and were negative for epithelial membrane antigen, desmin, myogenin, CD99, S100, GFAP, synaptophysin, and chromogranin. A diagnosis of pure YST involving right temporal bone was made. Serum alpha‑fetoprotein (AFP) level was assessed and was 20,800 ng/mL. The patient was investigated to rule out a primary tumor in gonads as well as at other sites. He was started on chemotherapy with bleomycin, etoposide, and paclitaxel. After 4 months, there was marked reduction in serum AFP level as well as in tumor size. However, during the course of chemotherapy, he developed pneumothorax and died of respiratory failure.
placement, followed by excision biopsy. Microscopic examination [Figure 2] showed a cellular tumor with mixed glandular and microcystic pattern, which was infiltrating the bone. Tumor cells were cuboidal to polygonal, with eosinophillc to clear vacuolated cytoplasm and vesicular nuclei. At places, Schiller‑Duval bodies were noted. An occasional mitotic figure was seen. Intracytoplasmic and extracellular eosinophilic hyaline globules were identified, which were periodic acid‑Schiff (PAS)‑positive and diastase‑resistant. No high‑grade glandular structures or solid areas suggestive of embryonal carcinoma or seminomatous differentiation, respectively, were noted.
a
YST is the commonest non-seminomatous germ cell tumor in infants and children, and most frequent site of occurrence is the gonads. Extragonadal tumors account for 20%, and location in head and neck region is rare, with brain, parapharyngeal space, nose, and maxillary sinuses being involved.[1‑3] In intracranial location, pineal gland, and suprasellar regions are most frequently involved. There are only two case reports describing temporal bone involvement by primary YST [Table 1].[4,5] In addition, another case of YST of external ear was reported by
b Figure 1: (a) Photograph of child showing right temporal swelling and disproportionate head size; (b) computed tomography showing infiltrative tumor involving temporal bone with intracranial extension
a
b
c
d
e
f
g
h
Figure 2: Photomicrographs showing tumor arranged in glandular and microcystic pattern with bone destruction (a, b, c; H and E x200). At places intracytoplasmic eosinophilic globules are seen which are periodic acid-Schiff (PAS) positive and diastase resistant (d, H&E x200; e, PAS stain x200). The tumor cells show diffuse immunopositivity for α-fetoprotein and pancytokeratin but are negative for epithelial membrane antigen (EMA) (f-h x200 each)
Table 1: Clinicopatholological features of published cases of temporal bone involvement by primary yolk sac tumor
Author and year of publication
Age/sex
Size (cm) Site
Frank TC et al. (2000)
18 months/female
N/A
Kebudi R et al. (1993)
20 months/female
5 cm
36 months/male
10 cm
Present case
Left temporal bone Left temporal region Right temporal region
Pure/ mixed
Serum AFP
Treatment
Follow up
Pure
Raised
Chemotherapy
Pure
Raised
Chemotherapy
Pure
Raised
Chemotherapy
Complete response (36 months) Complete response (5 months) Partial response Died after 4 months
AFP ‑ Alpha fetoprotein
680
Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
Letters to Editor
Stanley et al., and this patient had abnormal temporal bone development.[3] Temporal bone involvement with a large extra‑ and intracranial soft tissue lesion in the pediatric age‑group raises the possibilities of rhabdomyosarcoma, congenital cholesteatoma, high‑grade lymphoma, meningocele, hemangioma, cystic hygroma, and germ cell tumors, including teratoma. Other close differential diagnoses for YST in temporal bone are glomus tympanicum and endolymphatic sac tumor, but these have characteristic histomorphology. Immunohistochemisty is helpful as both of these tumors are immunonegative for AFP unlike YST. Pathogenesis of extragonadal YST is not known. A commonly accepted hypothesis is origin from primordial germ cells misplaced during migration in embryogenesis.[6] An alternative school of thought is that pluripotential cells which escape the influence of primary development are later on the source of germ cell tumors at ectopic sites.[7] Extra‑gonadal YSTs are less aggressive than gonadal tumors. Surgery followed by chemotherapy is treatment of choice regardless of disease stage. Surgical exenteration is usually not possible due to anatomic constraints. Upfront triple chemotherapy yields favorable outcome with significant remission.[6] However, complications of chemotherapy may lead to significant morbidity and mortality, as in the present case.
Mukund Sable, Aanchal Kakkar, Richa Ranjan, Ajay Garg1, Sameer Bakhshi2, Mehar Chand Sharma Departments of Pathology, 1Neuroradiology, and 2Medical Oncology, All India Institute of Medical Sciences, New Delhi, India E‑mail: [email protected]
References 1.
Devaney K, Ferlito A. Yolk sac tumors (endodermal sinus tumors) of the extracranial head and neck regions. Ann Otol Rhinol Laryngol 1997;106:254‑60. 2. Filho BC, McHugh JB, Carrau RL, Kassam AB. Yolk sac tumor in the nasal cavity. Am J Otolaryngol 2008;29:250‑4. 3. Stanley RJ, Scheithauer BW, Thompson EI, Kispert DB, Weiland LH, Pearson BW. Endodermal sinus tumor (yolk sac tumor) of the ear. Arch Otolaryngol Head Neck Surg 1987;113:200‑3. 4. Kebudi R, Ayan I, Darendeliler E, Ağaoğlu L, Kinay M, Olgaç V, et al. Non‑midline endodermal sinus tumor in the head and neck region: A case report. Med Pediatr Oncol 1993;21:685‑9. 5. Frank TC, Anand VK, Subramony C. Yolk sac tumor of the temporal bone: Report of a case. Ear Nose Throat J 2000;79:183, 187‑8, 191‑2. 6. Dede M, Pabuccu R, Yagci G, Yenen MC, Goktolga U, Gunhan O. Extragonadal yolk sac tumor in pelvic localization. A case report and literature review. Gynecol Oncol 2004;92:989‑91. 7. Kusumakumari P, Geetha N, Chellam VG, Nair MK. Endodermal sinus tumors in the head and neck region. Med Pediatr Oncol 1997;29:303‑7. Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.149406
Received: 06-11-2014 Review completed: 23-11-2014 Accepted: 05-12-2014
Cluster‑like headache as presenting feature of Vogt– Koyanagi–Harada disease Sir, A 26‑year‑old lady presented with acute, severe, right periorbital/frontal headache for 1 week. She had three to four headache episodes/day, each lasted an hour and usually clustered around night, sometimes waking her from sleep. Headache was piercing‑type associated with intense lacrimation/redness of right eye. Three days later, she complained of transient blurring of vision from right eye which improved after subsidence of headache. There was no double vision, painful eye movements, or past/family history of migraine. General/neurological examination was normal. Fundus examination revealed normal optic disc. A trial of oxygen inhalation (8l/min) promptly relieved her headache. Indomethacin failed to reduce headache frequency. During the hospital course, she developed mild continuous headache overlying cluster‑like attacks and blurred vision both eyes. Ophthalmic evaluation revealed vision 6/18 both eyes and multiple areas of exudative retinal detachments that were confirmed on fluoresce in angiography (FA) and optical coherence tomography (OCT) [Figure 1]. Cerebrospinal fluid (CSF) examination, cranial magnetic resonance imaging (MRI) and immunological work‑up were normal. With diagnosis of Vogt–Koyanagi–Harada disease (VKH), she was prescribed oral prednisolone (1 mg/kg). Within 1 week of initiation of steroids, her headache disappeared. At 1 month, her vision improved to 6/6 andretinal exudative detachments showed near‑complete recovery. VKH disease is a multisystem disease of presumed autoimmune etiology that commonly involves eye in the form of anterior uveitis, vitritis, optic nerve edema/hyperemia, and subretinal exudates with multiple retinal detachments. VKH also produce recurrent aseptic meningitis, encephalitis, deafness, blindness, and cutaneous manifestations. VKH evolves 681
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
a
b
c
d
Figure 3: Immunohistochemical profile: Immunohistochemistry showing the tumor cells to be immunopositive for glial fibrillary acidic protein (GFAP) (a) and vimentin (b), with dot like positivity for epithelial membrane antigen (EMA) (c). MIB‑1 LI is very low (d) (×400)
have features similar to ependymomas, with intercellular junctions and microlumens filled with microvilli.[2] However, cortical location of AG is contrary to this hypothesis. An origin from neoplastic transformation of radial glial cells during neuronal migration has now been proposed, based on elongated bipolar morphology of tumor cells.[1]
caused by angiocentric glioma complicated with focal cortical dysplasia: A surgical case series. J Neurooncol 2012;110:375‑80. 4. Burger PC, Jouvet A, Preusser M, Hans VH, Rosenblum MK, Lellouch‑Tubiana A. Angiocentric glioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. World Health Organization Classification of Tumours of the Central Nervous System. 4th ed. Lyon: IARC; 2007. p. 92‑3. 5. Takada S, Iwasaki M, Suzuki H, Nakasato N, Kumabe T, Tominaga T. Angiocentric glioma and surrounding cortical dysplasia manifesting as intractable frontal lobe epilepsy‑‑Case report. Neurol Med Chir (Tokyo) 2011;51:522‑6. 6. Prayson RA. Tumours arising in the setting of paediatric chronic epilepsy. Pathology 2010;42:426‑31. 7. Marburger T, Prayson R. Angiocentric glioma: A clinicopathologic review of 5 tumors with identification of associated cortical dysplasia. Arch Pathol Lab Med 2011;135:1037‑41. 8. Raghunathan A, Olar A, Vogel H, Parker JR, Coventry SC, Debski R, et al. Isocitrate dehydrogenase 1 R132H mutation is not detected in angiocentric glioma. Ann Diagn Pathol 2012;16:255‑9. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.149405
Received: 04‑11‑2014 Review completed: 09‑11‑2014 Accepted: 20‑12‑2014
Outcome of AG patients is excellent, with most achieving freedom from seizures following gross total resection, but subtotal resection does not have such good results, with reported seizure‑free outcomes of 93% vs 36%.[3,5] Although benign in nature, one fatality has been attributed to AG, due to progressive disease.[2] The primary tumor showed typical histological features; however, the recurrent tumor displayed increased mitoses and elevated MIB‑1 LI (10%), indicating aggressive behavior.
Yolk sac tumor of the temporal bone: An unusual presentation as hydrocephalus
Aanchal Kakkar, Mehar Chand Sharma, Vaishali Suri, Seema Kaushal, Sarat P. Chandra1, Ajay Garg2, Chitra Sarkar
A 3‑year‑old‑male child presented with progressive increase in head size since birth and a right temporal swelling since 2 months. He had symptoms of raised intracranial pressure viz. irritability and decreased vision in both eyes. There was no history of fever, weight loss, or vomiting. On examination, the child had disproportionate head size (circumference 59 cm) with open and tense anterior fontenelle [Figure 1a]. A soft to firm, warm, non‑tender swelling was identified over right temporal region measuring 12 cm, extending from external auditory meatus to lateral canthus of right eye. There were no sensory, motor, or cranial nerve deficits. Computed tomography showed a right temporal soft tissue space‑occupying lesion with destruction of right temporal bone, extension into the cranial cavity, and evidence of hydrocephalus [Figure 1b]. Cerebrospinal fluid examination was normal. Patient underwent shunt
Departments of Pathology, Neurosurgery1 and Neuroradiology2, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India E‑mail: [email protected]
References 1. Lellouch‑Tubiana A, Boddaert N, Bourgeois M, Fohlen M, Jouvet A, Delalande O, et al. Angiocentric neuroepithelial tumor (ANET): A new epilepsy‑related clinicopathological entity with distinctive MRI. Brain Pathol 2005;15:281‑6. 2. Wang M, Tihan T, Rojiani AM, Bodhireddy SR, Prayson RA, Iacuone JJ, et al. Monomorphous angiocentric glioma: A distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. J Neuropathol Exp Neurol 2005;64:875‑81. 3. Liu CQ, Zhou J, Qi X, Luan GM. Refractory temporal lobe epilepsy Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
Sir, Yolk sac tumor (YST) occurring at extragonadal sites usually involves midline structures viz. brain, mediastinum, retroperitoneum. YST of head and neck is rare, and involvement of skull as a primary site is extremely so.[1]
679
Letters to Editor
Tumor cells were immunopositive for pancytokeratin and alpha fetoprotein (AFP), and were negative for epithelial membrane antigen, desmin, myogenin, CD99, S100, GFAP, synaptophysin, and chromogranin. A diagnosis of pure YST involving right temporal bone was made. Serum alpha‑fetoprotein (AFP) level was assessed and was 20,800 ng/mL. The patient was investigated to rule out a primary tumor in gonads as well as at other sites. He was started on chemotherapy with bleomycin, etoposide, and paclitaxel. After 4 months, there was marked reduction in serum AFP level as well as in tumor size. However, during the course of chemotherapy, he developed pneumothorax and died of respiratory failure.
placement, followed by excision biopsy. Microscopic examination [Figure 2] showed a cellular tumor with mixed glandular and microcystic pattern, which was infiltrating the bone. Tumor cells were cuboidal to polygonal, with eosinophillc to clear vacuolated cytoplasm and vesicular nuclei. At places, Schiller‑Duval bodies were noted. An occasional mitotic figure was seen. Intracytoplasmic and extracellular eosinophilic hyaline globules were identified, which were periodic acid‑Schiff (PAS)‑positive and diastase‑resistant. No high‑grade glandular structures or solid areas suggestive of embryonal carcinoma or seminomatous differentiation, respectively, were noted.
a
YST is the commonest non-seminomatous germ cell tumor in infants and children, and most frequent site of occurrence is the gonads. Extragonadal tumors account for 20%, and location in head and neck region is rare, with brain, parapharyngeal space, nose, and maxillary sinuses being involved.[1‑3] In intracranial location, pineal gland, and suprasellar regions are most frequently involved. There are only two case reports describing temporal bone involvement by primary YST [Table 1].[4,5] In addition, another case of YST of external ear was reported by
b Figure 1: (a) Photograph of child showing right temporal swelling and disproportionate head size; (b) computed tomography showing infiltrative tumor involving temporal bone with intracranial extension
a
b
c
d
e
f
g
h
Figure 2: Photomicrographs showing tumor arranged in glandular and microcystic pattern with bone destruction (a, b, c; H and E x200). At places intracytoplasmic eosinophilic globules are seen which are periodic acid-Schiff (PAS) positive and diastase resistant (d, H&E x200; e, PAS stain x200). The tumor cells show diffuse immunopositivity for α-fetoprotein and pancytokeratin but are negative for epithelial membrane antigen (EMA) (f-h x200 each)
Table 1: Clinicopatholological features of published cases of temporal bone involvement by primary yolk sac tumor
Author and year of publication
Age/sex
Size (cm) Site
Frank TC et al. (2000)
18 months/female
N/A
Kebudi R et al. (1993)
20 months/female
5 cm
36 months/male
10 cm
Present case
Left temporal bone Left temporal region Right temporal region
Pure/ mixed
Serum AFP
Treatment
Follow up
Pure
Raised
Chemotherapy
Pure
Raised
Chemotherapy
Pure
Raised
Chemotherapy
Complete response (36 months) Complete response (5 months) Partial response Died after 4 months
AFP ‑ Alpha fetoprotein
680
Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
Letters to Editor
Stanley et al., and this patient had abnormal temporal bone development.[3] Temporal bone involvement with a large extra‑ and intracranial soft tissue lesion in the pediatric age‑group raises the possibilities of rhabdomyosarcoma, congenital cholesteatoma, high‑grade lymphoma, meningocele, hemangioma, cystic hygroma, and germ cell tumors, including teratoma. Other close differential diagnoses for YST in temporal bone are glomus tympanicum and endolymphatic sac tumor, but these have characteristic histomorphology. Immunohistochemisty is helpful as both of these tumors are immunonegative for AFP unlike YST. Pathogenesis of extragonadal YST is not known. A commonly accepted hypothesis is origin from primordial germ cells misplaced during migration in embryogenesis.[6] An alternative school of thought is that pluripotential cells which escape the influence of primary development are later on the source of germ cell tumors at ectopic sites.[7] Extra‑gonadal YSTs are less aggressive than gonadal tumors. Surgery followed by chemotherapy is treatment of choice regardless of disease stage. Surgical exenteration is usually not possible due to anatomic constraints. Upfront triple chemotherapy yields favorable outcome with significant remission.[6] However, complications of chemotherapy may lead to significant morbidity and mortality, as in the present case.
Mukund Sable, Aanchal Kakkar, Richa Ranjan, Ajay Garg1, Sameer Bakhshi2, Mehar Chand Sharma Departments of Pathology, 1Neuroradiology, and 2Medical Oncology, All India Institute of Medical Sciences, New Delhi, India E‑mail: [email protected]
References 1.
Devaney K, Ferlito A. Yolk sac tumors (endodermal sinus tumors) of the extracranial head and neck regions. Ann Otol Rhinol Laryngol 1997;106:254‑60. 2. Filho BC, McHugh JB, Carrau RL, Kassam AB. Yolk sac tumor in the nasal cavity. Am J Otolaryngol 2008;29:250‑4. 3. Stanley RJ, Scheithauer BW, Thompson EI, Kispert DB, Weiland LH, Pearson BW. Endodermal sinus tumor (yolk sac tumor) of the ear. Arch Otolaryngol Head Neck Surg 1987;113:200‑3. 4. Kebudi R, Ayan I, Darendeliler E, Ağaoğlu L, Kinay M, Olgaç V, et al. Non‑midline endodermal sinus tumor in the head and neck region: A case report. Med Pediatr Oncol 1993;21:685‑9. 5. Frank TC, Anand VK, Subramony C. Yolk sac tumor of the temporal bone: Report of a case. Ear Nose Throat J 2000;79:183, 187‑8, 191‑2. 6. Dede M, Pabuccu R, Yagci G, Yenen MC, Goktolga U, Gunhan O. Extragonadal yolk sac tumor in pelvic localization. A case report and literature review. Gynecol Oncol 2004;92:989‑91. 7. Kusumakumari P, Geetha N, Chellam VG, Nair MK. Endodermal sinus tumors in the head and neck region. Med Pediatr Oncol 1997;29:303‑7. Neurology India | Nov-Dec 2014 | Vol 62 | Issue 6
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.149406
Received: 06-11-2014 Review completed: 23-11-2014 Accepted: 05-12-2014
Cluster‑like headache as presenting feature of Vogt– Koyanagi–Harada disease Sir, A 26‑year‑old lady presented with acute, severe, right periorbital/frontal headache for 1 week. She had three to four headache episodes/day, each lasted an hour and usually clustered around night, sometimes waking her from sleep. Headache was piercing‑type associated with intense lacrimation/redness of right eye. Three days later, she complained of transient blurring of vision from right eye which improved after subsidence of headache. There was no double vision, painful eye movements, or past/family history of migraine. General/neurological examination was normal. Fundus examination revealed normal optic disc. A trial of oxygen inhalation (8l/min) promptly relieved her headache. Indomethacin failed to reduce headache frequency. During the hospital course, she developed mild continuous headache overlying cluster‑like attacks and blurred vision both eyes. Ophthalmic evaluation revealed vision 6/18 both eyes and multiple areas of exudative retinal detachments that were confirmed on fluoresce in angiography (FA) and optical coherence tomography (OCT) [Figure 1]. Cerebrospinal fluid (CSF) examination, cranial magnetic resonance imaging (MRI) and immunological work‑up were normal. With diagnosis of Vogt–Koyanagi–Harada disease (VKH), she was prescribed oral prednisolone (1 mg/kg). Within 1 week of initiation of steroids, her headache disappeared. At 1 month, her vision improved to 6/6 andretinal exudative detachments showed near‑complete recovery. VKH disease is a multisystem disease of presumed autoimmune etiology that commonly involves eye in the form of anterior uveitis, vitritis, optic nerve edema/hyperemia, and subretinal exudates with multiple retinal detachments. VKH also produce recurrent aseptic meningitis, encephalitis, deafness, blindness, and cutaneous manifestations. VKH evolves 681
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
