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Patients with acute onset Vogt-Koyanagi-Harada disease (VKHD) were prospectively included in this study. They were systematically followed with clinical, posterior segment imaging exams and full-field electroretinogram during a minimum 24-month of follow-up. All patients were treated with 3-day methylprednisolone pulse therapy followed by 1mg/day oral prednisone with a slow tapper during a median of 13 months. Non-steroidal immunosuppressive therapy (IMT) was introduced in cases of refractory disease or in cases of prednisone intolerance. Outcome measured by full-field electroretinogram was analyzed and patient was grouped as electroretinogram stable or electroretinogram worsening. Clinical data was analyzed in these two electroretinogram-based groups.
Consecutive patients with acute onset VKHD were included and followed for a minimum 24-month as Part I of an ongoing prospective long-term study on VKHD. The main purpose was to understand the course of clinical and subclinical choroidal inflammation in patients receiving early and high-dose corticosteroid followed by high-dose oral prednisone and a very slow tapper. All patients were followed with clinical and posterior segment imaging (PSI) exams, i.e. fundus picture, fluorescein angiography, indocyanine green angiography and enhanced depth imaging optical coherence tomography, at inclusion, 1st month, and thereof every three months. Full-field electroretinogram was performed at inclusion, 1st month, and thereof every six months. Flare was defined as appearance or increase/worsening of inflammatory signs after the initial six-month from disease onset during the predefined treatment protocol. Inflammatory signs were cells in anterior chamber, macular edema; subclinical inflammatory signs were mainly those observed by PSI exams. Scotopic full-field electroretinogram results between 12 and 24 month were the main outcome. Clinical data was analyzed in the full-field electroretinogram-based groups.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Corticosteroid monotherapy | Other | All patients in the acute phase will be treated with corticosteroid monotherapy, unless presents a contraindication or persistence/ recurrence of inflammation with regressive corticosteroid monotherapy.This is the unique arm of the present study. Treatment protocol includes a three-day course of intravenous methylprednisolone (1000 mg per day) followed by a high dose of oral prednisone (1.0 mg/kg/day) with a slow tapering with dose equal or less than 0.1mg/kg/day after 10 months. The prednisone dose is scheduled to be reduced in a 0.1 mg/kg-step ladder until it reaches 0.3 mg/kg (i.e. for an individual with 60 kg, dose would be 20 mg): from 1 to 0.8 mg/kg every two to three weeks; at 0.7 mg/kg for 4 weeks; at 0.6 mg/kg for two to three weeks; from 0.5 mg/kg every 4 weeks; from 0.3 to 0.15 mg/kg every 6 weeks; and 0.1 mg/kg, 0.075 mg/kg and 0.05 mg/kg for 8 weeks each. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Corticosteroid monotherapy | Drug | Patients will receive corticosteroid monotherapy as a pulsetherapy (1000mg/ day for 3 days) followed by oral corticosteroid. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Median Values of ERG Scotopic Parameters at 12-month | Full-field electroretinogram (ERG) scotopic parameters were evaluated at 12 -month (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential). | assessed at 12-month |
| Median Values of ERG Scotopic Parameters at 24-month | Full-field electroretinogram (ERG) scotopic parameters were evaluated at 24 -month (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential). | assessed at 24-month |
| Variation (Worsening or Improvement) Between 24 and 12 Months of ERG Scotopic Parameters Comparing 24 and 12-months | Full-field electroretinogram (ERG) scotopic parameters at 12 and 24 months were compared (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential). The worsening of the ERG parameters was defined as a value reduction of ≥ 30 % in any these scotopic ERG parameters at month 12 and month 24. We report the change between the (value at month 24/ the value at month 12)*100. | 12 and 24-months |
| Measure | Description | Time Frame |
|---|---|---|
| Recurrence or Worsening of Cells in Anterior Chamber | Evaluation of anterior chamber (AC) cells was performed at visits 6 months, 12 months, 18 months and 24 months from disease onset, according to the Standardization of Uveitis Nomenclature´s classification of anterior chamber cells. (Am J Ophthalmol, 2005) Any step increase (fluctuation), when comparing sequential dates of follow up (e.g 6 and 12 months) were considered as one episode of clinical worsening in AC cells |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Joyce H Yamamoto, PhD | Universidade São Paulo | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hospital das Clinicas HCFMUSP, Faculdade de Medicina Universidade de Sao Paulo | São Paulo | São Paulo | 05403-000 | Brazil |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 26875727 | Background | Du L, Kijlstra A, Yang P. Vogt-Koyanagi-Harada disease: Novel insights into pathophysiology, diagnosis and treatment. Prog Retin Eye Res. 2016 May;52:84-111. doi: 10.1016/j.preteyeres.2016.02.002. Epub 2016 Feb 11. | |
| 27008848 | Background | Lavezzo MM, Sakata VM, Morita C, Rodriguez EE, Abdallah SF, da Silva FT, Hirata CE, Yamamoto JH. Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes. Orphanet J Rare Dis. 2016 Mar 24;11:29. doi: 10.1186/s13023-016-0412-4. |
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Date of recruitment: June, 2011 through January, 2017 Location: Ophthalmology service -Hospital das Clínicas da Universidade São Paulo
| ID | Title | Description |
|---|---|---|
| FG000 | Corticosteroid Monotherapy | All patients in the acute phase will be treated with corticosteroid monotherapy, unless presents a contraindication or persistence/ recurrence of inflammation with regressive corticosteroid monotherapy. Treatment protocol includes a three-day course of intravenous methylprednisolone (1000 mg per day) followed by a high dose of oral prednisone (1.0 mg/kg/day) with a slow tapering with dose equal or less than 0.1mg/kg/day after 10 months. The prednisone dose is scheduled to be reduced in a 0.1 mg/kg-step ladder until it reaches 0.3 mg/kg (i.e. for an individual with 60 kg, dose would be 20 mg): from 1 to 0.8 mg/kg every two to three weeks; at 0.7 mg/kg for 4 weeks; at 0.6 mg/kg for two to three weeks; from 0.5 mg/kg every 4 weeks; from 0.3 to 0.15 mg/kg every 6 weeks; and 0.1 mg/kg, 0.075 mg/kg and 0.05 mg/kg for 8 weeks each. |
| Title | Milestones | Reasons Not Completed | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Overall Study |
|
Study of clinical, subclinical and electroretinographic outcomes of patients with acute Vogt-Koyanagi-Harada disease treated with corticosteroid monotherapy
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| ID | Title | Description |
|---|---|---|
| BG000 | Corticosteroid Monotherapy | All patients in the acute phase will be treated with corticosteroid monotherapy, unless presents a contraindication or persistence/ recurrence of inflammation with regressive corticosteroid monotherapy. Treatment protocol includes a three-day course of intravenous methylprednisolone (1000 mg per day) followed by a high dose of oral prednisone (1.0 mg/kg/day) with a slow tapering with dose equal or less than 0.1mg/kg/day after 10 months. The prednisone dose is scheduled to be reduced in a 0.1 mg/kg-step ladder until it reaches 0.3 mg/kg (i.e. for an individual with 60 kg, dose would be 20 mg): from 1 to 0.8 mg/kg every two to three weeks; at 0.7 mg/kg for 4 weeks; at 0.6 mg/kg for two to three weeks; from 0.5 mg/kg every 4 weeks; from 0.3 to 0.15 mg/kg every 6 weeks; and 0.1 mg/kg, 0.075 mg/kg and 0.05 mg/kg for 8 weeks each. prednisolone and prednisone |
| Units | Counts |
|---|---|
| Participants |
|
| Title | Description | Population Description | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Denominator Units Selected | Denominators | Classes |
|---|---|---|---|---|---|---|---|---|---|
| Age, Categorical | Count of Participants |
| Type | Title | Description | Population Description | Reporting Status | Anticipated Posting Date | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Time Frame | Units Analyzed | Denominator Units Selected | Arm/Group Information | Denominators | Classes | Analyses | |||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Primary | Median Values of ERG Scotopic Parameters at 12-month | Full-field electroretinogram (ERG) scotopic parameters were evaluated at 12 -month (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential). | Posted | Median | Full Range | microvolts | assessed at 12-month | eyes | eyes |
|
12 and 24 months
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| ID | Title | Description | Deaths (Affected) | Deaths (At Risk) | Serious Events (Affected) | Serious Events (At Risk) | Other Events (Affected) | Other Events (At Risk) |
|---|---|---|---|---|---|---|---|---|
| EG000 | Corticosteroid Monotherapy | All patients in the acute phase will be treated with corticosteroid monotherapy, unless presents a contraindication or persistence/ recurrence of inflammation with regressive corticosteroid monotherapy. Treatment protocol includes a three-day course of intravenous methylprednisolone (1000 mg per day) followed by a high dose of oral prednisone (1.0 mg/kg/day) with a slow tapering with dose equal or less than 0.1mg/kg/day after 10 months. The prednisone dose is scheduled to be reduced in a 0.1 mg/kg-step ladder until it reaches 0.3 mg/kg (i.e. for an individual with 60 kg, dose would be 20 mg): from 1 to 0.8 mg/kg every two to three weeks; at 0.7 mg/kg for 4 weeks; at 0.6 mg/kg for two to three weeks; from 0.5 mg/kg every 4 weeks; from 0.3 to 0.15 mg/kg every 6 weeks; and 0.1 mg/kg, 0.075 mg/kg and 0.05 mg/kg for 8 weeks each. |
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| Term | Organ System | Source Vocabulary | Assessment Type | Notes | Statistical Information |
|---|---|---|---|---|---|
| Psychosis/ depression | Psychiatric disorders | Non-systematic Assessment | Psychosis/ depression |
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| Title | Organization | Phone | Extension | |
|---|---|---|---|---|
| Dra Joyce Hisae Yamamoto | Hospital das clínicas- Faculdade de Medicina da Universidade São Paulo | 41988263757 | vmsakata@yahoo.com.br |
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| Type | Includes Protocol | Includes SAP | Includes ICF | Document Label | Document Date | Document Uploaded Date | Document File Name |
|---|---|---|---|---|---|---|---|
| Prot_SAP | Yes | Yes | No | Study Protocol and Statistical Analysis Plan | Mar 26, 2025 | Mar 26, 2025 | Prot_SAP_002.pdf |
| ICF | No | No | Yes | Informed Consent Form | Mar 26, 2025 | Mar 26, 2025 | ICF_003.pdf |
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| ID | Term |
|---|---|
| D014607 | Uveomeningoencephalitic Syndrome |
| D007249 | Inflammation |
| D015862 | Choroid Diseases |
| D014786 | Vision Disorders |
| ID | Term |
|---|---|
| D020274 | Autoimmune Diseases of the Nervous System |
| D009422 | Nervous System Diseases |
| D014605 | Uveitis |
| D014603 | Uveal Diseases |
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All patients were treated with a standard high-dose corticosteroid
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|
| 6 to 24 months from disease onset. |
| Increase in the Score of Dark Dots on Indocyanine Green Angiography | Dark dots scores had a maximum value of 8, any increase of 0.5 after 6 months from disease onset will be considered (Int Ophthalmo 2010) Dark dots Score based on pattern of distribution (Sparse/ Numerous) Minimum: 0 (better outcome) Maximum: 8 (worse outcome) | 6 to 24 months from disease onset |
| Change in Subfoveal Choroidal Thickness on Enhanced Depth Optical Coherence Tomography | Increase of 30% or more in choroidal thickness EDI in consecutive exams on horizontal scan | 6 to 24 months from disease onset |
| Change in Perivascular Leakage on Fluorescein Angiography | Perivascular leakage was observed as an increase in hyperfluorescence around retinal vasculature over time on FA exam at midperiphery. | 6 to 24 months after disease onset |
| Choroidal Neovascularization | Choroidal neovascularization was diagnosed when increasingly localized hyperfluorescence at the posterior pole is detected on FA or a hyperreflective subretinal lesion associated with sub or intraretinal fluid on OCT. | 6 to 24 months after disease onset |
| Cataract | Cataract was defined as any lens opacification greater than nuclear or cortical 2+/4 or subcapsular 1+/4 | 6 to 24 months after disease onset |
| Ocular Hypertension | Ocular hypertension was defined as an intraocular pressure (IOP) above 21mmHg | 6 to 24 months after disease onset |
| 17435969 | Background | Rao NA. Pathology of Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2007 Apr-Jun;27(2-3):81-5. doi: 10.1007/s10792-006-9029-2. Epub 2007 Apr 14. |
| 2025171 | Background | Rubsamen PE, Gass JD. Vogt-Koyanagi-Harada syndrome. Clinical course, therapy, and long-term visual outcome. Arch Ophthalmol. 1991 May;109(5):682-7. doi: 10.1001/archopht.1991.01080050096037. |
| 29948499 | Background | Herbort CP Jr, Abu El Asrar AM, Takeuchi M, Pavesio CE, Couto C, Hedayatfar A, Maruyama K, Rao X, Silpa-Archa S, Somkijrungroj T. Catching the therapeutic window of opportunity in early initial-onset Vogt-Koyanagi-Harada uveitis can cure the disease. Int Ophthalmol. 2019 Jun;39(6):1419-1425. doi: 10.1007/s10792-018-0949-4. Epub 2018 Jun 11. |
| 18672226 | Background | Yang P, Fang W, Wang L, Wen F, Wu W, Kijlstra A. Study of macular function by multifocal electroretinography in patients with Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol. 2008 Nov;146(5):767-71. doi: 10.1016/j.ajo.2008.05.044. Epub 2008 Jul 30. |
| 17103022 | Background | Chee SP, Jap A, Bacsal K. Spectrum of Vogt-Koyanagi-Harada disease in Singapore. Int Ophthalmol. 2007 Apr-Jun;27(2-3):137-42. doi: 10.1007/s10792-006-9009-6. Epub 2006 Nov 11. |
| 29702075 | Background | Yuan W, Zhou C, Cao Q, Du Z, Hu R, Wang Y, Kijlstra A, Yang P. Longitudinal Study of Visual Function in Vogt-Koyanagi-Harada Disease Using Full-Field Electroretinography. Am J Ophthalmol. 2018 Jul;191:92-99. doi: 10.1016/j.ajo.2018.04.013. Epub 2018 Apr 25. |
| 15761760 | Background | Chee SP, Luu CD, Cheng CL, Lim WK, Jap A. Visual function in Vogt-Koyanagi-Harada patients. Graefes Arch Clin Exp Ophthalmol. 2005 Aug;243(8):785-90. doi: 10.1007/s00417-005-1156-3. Epub 2005 Mar 11. |
| 19781687 | Background | da Silva FT, Hirata CE, Olivalves E, Oyamada MK, Yamamoto JH. Fundus-based and electroretinographic strategies for stratification of late-stage Vogt-Koyanagi-Harada disease patients. Am J Ophthalmol. 2009 Dec;148(6):939-45.e3. doi: 10.1016/j.ajo.2009.06.029. Epub 2009 Sep 24. |
| 18795232 | Background | Tugal-Tutkun I, Herbort CP, Khairallah M; Angiography Scoring for Uveitis Working Group (ASUWOG). Scoring of dual fluorescein and ICG inflammatory angiographic signs for the grading of posterior segment inflammation (dual fluorescein and ICG angiographic scoring system for uveitis). Int Ophthalmol. 2010 Oct;30(5):539-52. doi: 10.1007/s10792-008-9263-x. Epub 2008 Sep 16. |
| 29666121 | Background | Nakayama M, Keino H, Watanabe T, Okada AA. Clinical features and visual outcomes of 111 patients with new-onset acute Vogt-Koyanagi-Harada disease treated with pulse intravenous corticosteroids. Br J Ophthalmol. 2019 Feb;103(2):274-278. doi: 10.1136/bjophthalmol-2017-311691. Epub 2018 Apr 17. |
| 19151926 | Background | Kawaguchi T, Horie S, Bouchenaki N, Ohno-Matsui K, Mochizuki M, Herbort CP. Suboptimal therapy controls clinically apparent disease but not subclinical progression of Vogt-Koyanagi-Harada disease. Int Ophthalmol. 2010 Feb;30(1):41-50. doi: 10.1007/s10792-008-9288-1. Epub 2009 Jan 17. |
| 27844182 | Background | Herbort CP Jr, Abu El Asrar AM, Yamamoto JH, Pavesio CE, Gupta V, Khairallah M, Tugal-Tutkun I, Soheilian M, Takeuchi M, Papadia M. Reappraisal of the management of Vogt-Koyanagi-Harada disease: sunset glow fundus is no more a fatality. Int Ophthalmol. 2017 Dec;37(6):1383-1395. doi: 10.1007/s10792-016-0395-0. Epub 2016 Nov 14. |
| 27535102 | Background | Abu El-Asrar AM, Dosari M, Hemachandran S, Gikandi PW, Al-Muammar A. Mycophenolate mofetil combined with systemic corticosteroids prevents progression to chronic recurrent inflammation and development of 'sunset glow fundus' in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease. Acta Ophthalmol. 2017 Feb;95(1):85-90. doi: 10.1111/aos.13189. Epub 2016 Aug 18. |
| Participants |
|
| Age, Continuous | Median | Full Range | years |
|
| Sex: Female, Male | Count of Participants | Participants |
|
| Race (NIH/OMB) | Count of Participants | Participants |
|
| Region of Enrollment | Number | participants |
|
|
|
|
| Primary | Median Values of ERG Scotopic Parameters at 24-month | Full-field electroretinogram (ERG) scotopic parameters were evaluated at 24 -month (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential). | Posted | Median | Full Range | microvolts | assessed at 24-month | eyes | eyes |
|
|
|
| Primary | Variation (Worsening or Improvement) Between 24 and 12 Months of ERG Scotopic Parameters Comparing 24 and 12-months | Full-field electroretinogram (ERG) scotopic parameters at 12 and 24 months were compared (scotopic parameters: amplitude of scotopic a and b wave, amplitude of maximum scotopic a and b wave, oscillatory potential). The worsening of the ERG parameters was defined as a value reduction of ≥ 30 % in any these scotopic ERG parameters at month 12 and month 24. We report the change between the (value at month 24/ the value at month 12)*100. | One patient had an eye classified as ERG stable group and the other eye as ERG worsening group thus the sum of both groups exceed the total 12 participants | Posted | Number | percentage of change on ERG parameters | 12 and 24-months | eyes | eyes |
|
|
|
| Secondary | Recurrence or Worsening of Cells in Anterior Chamber | Evaluation of anterior chamber (AC) cells was performed at visits 6 months, 12 months, 18 months and 24 months from disease onset, according to the Standardization of Uveitis Nomenclature´s classification of anterior chamber cells. (Am J Ophthalmol, 2005) Any step increase (fluctuation), when comparing sequential dates of follow up (e.g 6 and 12 months) were considered as one episode of clinical worsening in AC cells | One patient had an eye classified as ERG stable group and the other eye as ERG worsening group thus the sum of both groups exceed the total 12 participants | Posted | Count of Units | eyes | 6 to 24 months from disease onset. | eyes | eyes |
|
|
|
|
| Secondary | Increase in the Score of Dark Dots on Indocyanine Green Angiography | Dark dots scores had a maximum value of 8, any increase of 0.5 after 6 months from disease onset will be considered (Int Ophthalmo 2010) Dark dots Score based on pattern of distribution (Sparse/ Numerous) Minimum: 0 (better outcome) Maximum: 8 (worse outcome) | One patient had an eye classified as ERG stable group and the other eye as ERG worsening group thus the sum of both groups exceed the total 12 participants | Posted | Number | eyes | 6 to 24 months from disease onset | eyes | eyes |
|
|
|
|
| Secondary | Change in Subfoveal Choroidal Thickness on Enhanced Depth Optical Coherence Tomography | Increase of 30% or more in choroidal thickness EDI in consecutive exams on horizontal scan | One patient had an eye classified as ERG stable group and the other eye as ERG worsening group thus the sum of both groups exceed the total 12 participants | Posted | Count of Units | eyes | 6 to 24 months from disease onset | eyes | eyes |
|
|
|
| Secondary | Change in Perivascular Leakage on Fluorescein Angiography | Perivascular leakage was observed as an increase in hyperfluorescence around retinal vasculature over time on FA exam at midperiphery. | One patient had an eye classified as ERG stable group and the other eye as ERG worsening group thus the sum of both groups exceed the total 12 participants | Posted | Count of Units | eyes | 6 to 24 months after disease onset | eyes | eyes |
|
|
|
|
| Secondary | Choroidal Neovascularization | Choroidal neovascularization was diagnosed when increasingly localized hyperfluorescence at the posterior pole is detected on FA or a hyperreflective subretinal lesion associated with sub or intraretinal fluid on OCT. | One patient had an eye classified as ERG stable group and the other eye as ERG worsening group thus the sum of both groups exceed the total 12 participants | Posted | Number | eyes | 6 to 24 months after disease onset | eyes | eyes |
|
|
|
|
| Secondary | Cataract | Cataract was defined as any lens opacification greater than nuclear or cortical 2+/4 or subcapsular 1+/4 | One patient had an eye classified as ERG stable group and the other eye as ERG worsening group thus the sum of both groups exceed the total 12 participants | Posted | Number | eyes | 6 to 24 months after disease onset | eyes | eyes |
|
|
|
|
| Secondary | Ocular Hypertension | Ocular hypertension was defined as an intraocular pressure (IOP) above 21mmHg | One patient had an eye classified as ERG stable group and the other eye as ERG worsening group thus the sum of both groups exceed the total 12 participants | Posted | Number | eyes | 6 to 24 months after disease onset | eyes | eyes |
|
|
|
|
| 0 |
| 12 |
| 0 |
| 12 |
| 6 |
| 12 |
|
| Weight gain | General disorders | Non-systematic Assessment |
|
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| D005128 | Eye Diseases |
| D001327 | Autoimmune Diseases |
| D007154 | Immune System Diseases |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D012678 | Sensation Disorders |
| D009461 | Neurologic Manifestations |
| D012816 | Signs and Symptoms |
|
| Oscillatory potential |
|
| Photopic a amplitude |
|
| Photopic B amplitude |
|
| Flicker |
|
| Maximum scotopic b amplitude |
|
| Oscillatory potential |
|
| Photopic a amplitude |
|
| Photopic b amplitude |
|
| Flicker |
|