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Susac syndrome: the first case report in Peru.
Medwave 2017; 17(7):e7033M

Abstract

Susac Syndrome is a rare entity, characterized by a triad of subacute encephalopathy, retinal artery occlusion and sensorineural hearing loss. It is more common in women and the age of onset fluctuates between 9-58 years of age. The pathogenesis is presented as microangiopathic changes at the cerebral, retinal and cochlear levels associated with an autoimmune mechanism. We present the case of a 31-year-old woman who started with a diffuse headache, puerile behavior, bradylalia and somnolence. As the disease progressed, she had auditory deficit and arterial obstruction of the right temporal retinal branch in retinal fluorescein angiography. Brain magnetic resonance showed rounded hyperintense lesions in the corpus callosum, periventricular region and cerebellum. This is the first reported case of Susac Syndrome in Peru, presented with the classic triad, which is an infrequent presentation. However, cases that show incomplete forms should be evaluated in a timely manner to initiate timely treatment and avoid irreversible consequences.

Authors+Show Affiliations

Centro de investigación básica en demencias y enfermedades desmielinizantes del sistema nervioso central, Instituto Nacional de Ciencias Neurológicas, Lima, Perú. Address: Jirón Ancash 1271, Barrios Altos, Distrito de Lima, Lima, Perú. Email: sheics2@gmail.com.Centro de investigación básica en demencias y enfermedades desmielinizantes del sistema nervioso central, Instituto Nacional de Ciencias Neurológicas, Lima, Perú; Facultad de medicina de la Universidad Nacional Mayor de San Marcos, Lima, Perú.Centro de investigación básica en demencias y enfermedades desmielinizantes del sistema nervioso central, Instituto Nacional de Ciencias Neurológicas, Lima, Perú.Centro de investigación básica en demencias y enfermedades desmielinizantes del sistema nervioso central, Instituto Nacional de Ciencias Neurológicas, Lima, Perú; Instituto Peruano de Neurociencias, Lima, Perú.

Pub Type(s)

Case Reports

Language

spa eng

PubMed ID

28885997

Citation

Rivadeneira-Sotelo, Carolina, et al. "Susac Syndrome: the First Case Report in Peru." Medwave, vol. 17, no. 7, 2017, pp. e7033.
Rivadeneira-Sotelo C, Meza Vega M, Segura-Chávez D, et al. Susac syndrome: the first case report in Peru. Medwave. 2017;17(7):e7033.
Rivadeneira-Sotelo, C., Meza Vega, M., Segura-Chávez, D., & Castro-Suarez, S. (2017). Susac syndrome: the first case report in Peru. Medwave, 17(7), pp. e7033. doi:10.5867/medwave.2017.07.7033.
Rivadeneira-Sotelo C, et al. Susac Syndrome: the First Case Report in Peru. Medwave. 2017 Aug 30;17(7):e7033. PubMed PMID: 28885997.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Susac syndrome: the first case report in Peru. AU - Rivadeneira-Sotelo,Carolina, AU - Meza Vega,María, AU - Segura-Chávez,Darwin, AU - Castro-Suarez,Sheila, Y1 - 2017/08/30/ PY - 2017/06/05/received PY - 2017/08/08/accepted PY - 2017/9/9/entrez PY - 2017/9/9/pubmed PY - 2018/5/29/medline KW - Susac syndrome KW - autoimmune endotheliopathy KW - hearing loss KW - microangiopathy KW - retinocochleocerebral vasculopathy KW - encephalopathy SP - e7033 EP - e7033 JF - Medwave JO - Medwave VL - 17 IS - 7 N2 - Susac Syndrome is a rare entity, characterized by a triad of subacute encephalopathy, retinal artery occlusion and sensorineural hearing loss. It is more common in women and the age of onset fluctuates between 9-58 years of age. The pathogenesis is presented as microangiopathic changes at the cerebral, retinal and cochlear levels associated with an autoimmune mechanism. We present the case of a 31-year-old woman who started with a diffuse headache, puerile behavior, bradylalia and somnolence. As the disease progressed, she had auditory deficit and arterial obstruction of the right temporal retinal branch in retinal fluorescein angiography. Brain magnetic resonance showed rounded hyperintense lesions in the corpus callosum, periventricular region and cerebellum. This is the first reported case of Susac Syndrome in Peru, presented with the classic triad, which is an infrequent presentation. However, cases that show incomplete forms should be evaluated in a timely manner to initiate timely treatment and avoid irreversible consequences. SN - 0717-6384 UR - https://www.unboundmedicine.com/medline/citation/28885997/Susac_syndrome:_the_first_case_report_in_Peru_ L2 - http://www.diseaseinfosearch.org/result/6937 DB - PRIME DP - Unbound Medicine ER -