Artificial Intelligence to Detect Papilledema from Ocular Fundus Photographs.

BACKGROUND: Nonophthalmologist physicians do not confidently perform direct ophthalmoscopy. The use of artificial intelligence to detect papilledema and other optic-disk abnormalities from fundus photographs has not been well studied. METHODS: We trained, validated, and externally tested a deep-learning system to classify optic disks as being normal or having papilledema or other abnormalities from 15,846 retrospectively collected ocular fundus photographs that had been obtained with pharmacologic pupillary dilation and various digital cameras in persons from multiple ethnic populations. Of these photographs, 14,341 from 19 sites in 11 countries were used for training and validation, and 1505 photographs from 5 other sites were used for external testing. Performance at classifying the optic-disk appearance was evaluated by calculating the area under the receiver-operating-characteristic curve (AUC), sensitivity, and specificity, as compared with a reference standard of clinical diagnoses by neuro-ophthalmologists. RESULTS: The training and validation data sets from 6779 patients included 14,341 photographs: 9156 of normal disks, 2148 of disks with papilledema, and 3037 of disks with other abnormalities. The percentage classified as being normal ranged across sites from 9.8 to 100%; the percentage classified as having papilledema ranged across sites from zero to 59.5%. In the validation set, the system discriminated disks with papilledema from normal disks and disks with nonpapilledema abnormalities with an AUC of 0.99 (95% confidence interval [CI], 0.98 to 0.99) and normal from abnormal disks with an AUC of 0.99 (95% CI, 0.99 to 0.99). In the external-testing data set of 1505 photographs, the system had an AUC for the detection of papilledema of 0.96 (95% CI, 0.95 to 0.97), a sensitivity of 96.4% (95% CI, 93.9 to 98.3), and a specificity of 84.7% (95% CI, 82.3 to 87.1). CONCLUSIONS: A deep-learning system using fundus photographs with pharmacologically dilated pupils differentiated among optic disks with papilledema, normal disks, and disks with nonpapilledema abnormalities. (Funded by the Singapore National Medical Research Council and the SingHealth Duke-NUS Ophthalmology and Visual Sciences Academic Clinical Program.)

Infliximab-Associated Retrobulbar Optic Neuritis

Infliximab is a tumor necrosis factor blocker that has been approved for the treatment of Crohn's disease and rheumatoid arthritis. A single case of retrobulbar optic neuritis associated with Infliximab has been recently reported. In this case the patient developed a superior visual field defect

Early Bilateral External Ophthalmoplegia Caused by Metastatic Breast Carcinoma

Breast cancer is the most frequent type of metastasis to the orbit, which although rare is a well recognized occurrence. Involvement of extraocular muscles is usually discrete and unilateral. Furthermore, other systemic metastases are usually encountered at the time of orbital compromise and occur after the original diagnosis of breast cancer is made

Multiple Intracranial Lesions Following Treatment of Nelson's Symptoms (MRI)

Declining cognition; Alexia; AgraphiaA 75-year old female with no light perception OD and right third, fourth and sixth nerve pareses. Previous history significant for diabetes mellitus, hypertension, Cushing syndrome and Nelson syndrome. After CD Dx, a bilateral adrenalectomy was performed.VA: NLP OD, 20/40 OS; Color plates: Unable to identify OS, Colors of large objects OD; RADP ODMRIPituitary carcinomaSurgery; XRT1. Grua JR and Nelson DH: ACTH-Producing Pituitary Tumors. Endocrinol Metab Clin North Am 1991; 20: 319-62. 2. Pernicone PJ, Bernd WS, Sebo TJ, et al: Pituitary Carcinoma Cancer 1997; 79(4): 804-12. 3. Kemink SA, Wesseling P, Pieters GF, et al: Progression of a Nelson's Adenoma to Pituitary Carcinoma; a case report and review of the literature. J Endocrinol Invest 1999; 22(1): 70-5. 4. Kemink L, Picters G, Hermus A, Smals A, Kloppenborg P: Patient's Age is a Simple Predictive Factor for the development of Nelson's Syndrome after Total Adrenalectomy for Cushing's Disease. J Clin Endocrinol Metab 1994; 79(3): 887-9. 5. Frost AR, Tenner S, Tenner M, Rollhauser C, Tabbara SO: ACTH-Producing Pituitary Carcinoma Presenting as the Cauda Equina Syndrome. Arch Pathol Lab Med 1995; 199(1): 93-6. 6. Lormeau B, Miossec P, Sibony M, Valensi P, Attali JR: Adrenocorticotropin-Producing Pituitary Carcinoma with Liver Metastatis. J Endocrinol Invest 1997; 20(4): 230-6. 7. Buchfelder M, Fahlbusch R, Thierauf P, Muller OA: Observations on the Pathophysiology of Nelson's Syndrome: A Report of Three Cases. Neurosurgery 1990; 27(6): 961-8. 8. Saegler W, Geisler F, Ludecke DK: Pituitary Pathology in Cushing's Disease. Path Res Pract 1988; 183: 592-5. 9. Favia G, Boscaro M, Lumachi F, D'Amico DF: Role of Bilateral Adrenaletomy in Cushing's Disease. World J Surg 1994; 18:462-6

Multiple Intracranial Lesions Following Treatment of Nelson's Symptoms (Pathology)

Declining cognition; Alexia; AgraphiaA 75-year old female with no light perception OD and right third, fourth and sixth nerve pareses. Previous history significant for diabetes mellitus, hypertension, cushing syndrome and Nelson syndrome. After CD Dx, a bilateral adrenalectomy was performed.VA: NLP OD, 20/40 OS; Color plates: Unable to identify OS, Color of large objects OD; RAPD ODMRIPituitary carcinomaSurgery; XRT1. Grua JR and Nelson DH: ACTH-Producing Pituitary Tumors. Endocrinol Metab Clin North Am 1991; 20: 319-62. 2. Pernicone PJ, Bernd WS, Sebo TJ, et al: Pituitary Carcinoma Cancer 1997; 79(4): 804-12. 3. Kemink SA, Wesseling P, Pieters GF, et al: Progression of a Nelson's Adenoma to Pituitary Carcinoma; a case report and review of the literature. J Endocrinol Invest 1999; 22(1): 70-5. 4. Kemink L, Picters G, Hermus A, Smals A, Kloppenborg P: Patient's Age is a Simple Predictive Factor for the development of Nelson's Syndrome after Total Adrenalectomy for Cushing's Disease. J Clin Endocrinol Metab 1994; 79(3): 887-9. 5. Frost AR, Tenner S, Tenner M, Rollhauser C, Tabbara SO: ACTH-Producing Pituitary Carcinoma Presenting as the Cauda Equina Syndrome. Arch Pathol Lab Med 1995; 199(1): 93-6. 6. Lormeau B, Miossec P, Sibony M, Valensi P, Attali JR: Adrenocorticotropin-Producing Pituitary Carcinoma with Liver Metastatis. J Endocrinol Invest 1997; 20(4): 230-6. 7. Buchfelder M, Fahlbusch R, Thierauf P, Muller OA: Observations on the Pathophysiology of Nelson's Syndrome: A Report of Three Cases. Neurosurgery 1990; 27(6): 961-8. 8. Saegler W, Geisler F, Ludecke DK: Pituitary Pathology in Cushing's Disease. Path Res Pract 1988; 183: 592-5. 9. Favia G, Boscaro M, Lumachi F, D'Amico DF: Role of Bilateral Adrenaletomy in Cushing's Disease. World J Surg 1994; 18:462-6

Multiple Intracranial Lesions Following Treatment of Nelson's Symptoms

Cognitive decline; Alexia with agraphiaA 75-year old female with no light perception OD and right third, fourth and sixth nerve pareses. Previous history significant for diabetes mellitus, hypertension, Cushing syndrome and Nelson syndrome. After CD Dx, a bilateral adrenalectomy was performed.VA: NLP OD, 20/40 OS; Color plates: Unable to identify OS, Colors of large objects OD; RAPD ODMRIPituitary carcinomaSurgery; XRT1. Grua JR and Nelson DH: ACTH-Producing Pituitary Tumors. Endocrinol Metab Clin North Am 1991; 20: 319-62. 2. Pernicone PJ, Bernd WS, Sebo TJ, et al: Pituitary Carcinoma Cancer 1997; 79(4): 804-12. 3. Kemink SA, Wesseling P, Pieters GF, et al: Progression of a Nelson's Adenoma to Pituitary Carcinoma; a case report and review of the literature. J Endocrinol Invest 1999; 22(1): 70-5. 4. Kemink L, Picters G, Hermus A, Smals A, Kloppenborg P: Patient's Age is a Simple Predictive Factor for the development of Nelson's Syndrome after Total Adrenalectomy for Cushing's Disease. J Clin Endocrinol Metab 1994; 79(3): 887-9. 5. Frost AR, Tenner S, Tenner M, Rollhauser C, Tabbara SO: ACTH-Producing Pituitary Carcinoma Presenting as the Cauda Equina Syndrome. Arch Pathol Lab Med 1995; 199(1): 93-6. 6. Lormeau B, Miossec P, Sibony M, Valensi P, Attali JR: Adrenocorticotropin-Producing Pituitary Carcinoma with Liver Metastatis. J Endocrinol Invest 1997; 20(4): 230-6. 7. Buchfelder M, Fahlbusch R, Thierauf P, Muller OA: Observations on the Pathophysiology of Nelson's Syndrome: A Report of Three Cases. Neurosurgery 1990; 27(6): 961-8. 8. Saegler W, Geisler F, Ludecke DK: Pituitary Pathology in Cushing's Disease. Path Res Pract 1988; 183: 592-5. 9. Favia G, Boscaro M, Lumachi F, D'Amico DF: Role of Bilateral Adrenaletomy in Cushing's Disease. World J Surg 1994; 18:462-6

Multiple Intracranial Lesions Following Treatment of Nelson's Symptoms: (MRI)

Declining cognition; Alexia; AgraphiaA 75-year old female with no light perception OD and right third, fourth and sixth nerve pareses. Previous history significant for diabetes mellitus, hypertension, cushing syndrome and Nelson syndrome. After CD Dx, a bilateral adrenalectomy was performed.VA: NLP OD, 20/40 OS; Color plates: Unable to identify OS, Colors of large objects OD; RAPD ODMRIPituitary carcinomaSurgery; XRT1. Grua JR and Nelson DH: ACTH-Producing Pituitary Tumors. Endocrinol Metab Clin North Am 1991; 20: 319-62. 2. Pernicone PJ, Bernd WS, Sebo TJ, et al: Pituitary Carcinoma Cancer 1997; 79(4): 804-12. 3. Kemink SA, Wesseling P, Pieters GF, et al: Progression of a Nelson's Adenoma to Pituitary Carcinoma; a case report and review of the literature. J Endocrinol Invest 1999; 22(1): 70-5. 4. Kemink L, Picters G, Hermus A, Smals A, Kloppenborg P: Patient's Age is a Simple Predictive Factor for the development of Nelson's Syndrome after Total Adrenalectomy for Cushing's Disease. J Clin Endocrinol Metab 1994; 79(3): 887-9. 5. Frost AR, Tenner S, Tenner M, Rollhauser C, Tabbara SO: ACTH-Producing Pituitary Carcinoma Presenting as the Cauda Equina Syndrome. Arch Pathol Lab Med 1995; 199(1): 93-6. 6. Lormeau B, Miossec P, Sibony M, Valensi P, Attali JR: Adrenocorticotropin-Producing Pituitary Carcinoma with Liver Metastatis. J Endocrinol Invest 1997; 20(4): 230-6. 7. Buchfelder M, Fahlbusch R, Thierauf P, Muller OA: Observations on the Pathophysiology of Nelson's Syndrome: A Report of Three Cases. Neurosurgery 1990; 27(6): 961-8. 8. Saegler W, Geisler F, Ludecke DK: Pituitary Pathology in Cushing's Disease. Path Res Pract 1988; 183: 592-5. 9. Favia G, Boscaro M, Lumachi F, D'Amico DF: Role of Bilateral Adrenaletomy in Cushing's Disease. World J Surg 1994; 18:462-6

CSF Characteristics in Patients with High Frisén Papilledema Grades from the IIHTT

"Papilledema is optic disc edema due to increased intracranial pressure. The severity of papilledema grade may be related to the density of trabeculations in the subarachnoid space, magnitude of CSF pressure, or another mechanism. We investigated the relationship of CSF pressure to high Frisén papilledema grades (4 and 5), and the response to randomized treatment with acetazolamide plus diet versus placebo plus diet in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT).

IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil

Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4 –related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. In both patients, there was treatment response to mycophenolate mofetil