An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes
Purpose: To report an atypical case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with central nervous system (CNS) vasculitis and recurrent strokes. Observations: A 57 year-old female presented with APMPPE after a febrile illness and rash. She developed an acute infarct on m...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2019-12-01
|
| Series: | American Journal of Ophthalmology Case Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2451993619300313 |
| _version_ | 1828160713159344128 |
|---|---|
| author | Evan Berger Ghassan Ghorayeb Jeffery Hogg |
| author_facet | Evan Berger Ghassan Ghorayeb Jeffery Hogg |
| author_sort | Evan Berger |
| collection | DOAJ |
| description | Purpose: To report an atypical case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with central nervous system (CNS) vasculitis and recurrent strokes. Observations: A 57 year-old female presented with APMPPE after a febrile illness and rash. She developed an acute infarct on magnetic resonance imaging. Computed tomography angiography of the cerebral vasculature was normal. Cerebrospinal fluid (CSF) analysis and an extensive serum lab workup were also unremarkable. She was treated with high-dose corticosteroids and eventually transitioned to methotrexate. A month after being on treatment she developed a second stroke. A cerebral angiogram was obtained and did not show evidence of CNS vasculitis. The methotrexate was eventually stopped and the prednisone was tapered. Approximately 3 months later she developed a third stroke and worsening APMPPE-associated maculopathy in both eyes. She was eventually started on oral cyclophosphamide. Conclusions & importance: Although rare, CNS vasculitis is a known complication of APMPPE. This case is atypical given the development of multiple recurrent strokes, lack of inflammatory evidence on CSF analysis, and normal imaging of the cerebral vasculature. This report highlights the need for a high level of clinical suspicion for CNS vasculitis with APMPPE despite noncontributory cerebral angiographic imaging and normal CSF analysis. Keywords: Acute posterior multifocal placoid pigment epitheliopathy, APMPPE, Cerebral vasculitis, Stroke |
| first_indexed | 2024-04-12T00:25:23Z |
| format | Article |
| id | doaj.art-628c7d30780144a58b19d4cdc92f5acd |
| institution | Directory Open Access Journal |
| issn | 2451-9936 |
| language | English |
| last_indexed | 2024-04-12T00:25:23Z |
| publishDate | 2019-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | American Journal of Ophthalmology Case Reports |
| spelling | doaj.art-628c7d30780144a58b19d4cdc92f5acd2022-12-22T03:55:31ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362019-12-0116An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokesEvan Berger0Ghassan Ghorayeb1Jeffery Hogg2West Virginia University, Department of Ophthalmology & Visual Sciences, 1 Medical Center Drive, Morgantown, WV, United States; Corresponding author. West Virginia University Eye Institute, 1 Medical Center Drive, Morgantown, WV, 26506, United States.West Virginia University, Department of Ophthalmology & Visual Sciences, 1 Medical Center Drive, Morgantown, WV, United StatesWest Virginia University, Department of Radiology, 1 Medical Center Drive, Morgantown, WV, United StatesPurpose: To report an atypical case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with central nervous system (CNS) vasculitis and recurrent strokes. Observations: A 57 year-old female presented with APMPPE after a febrile illness and rash. She developed an acute infarct on magnetic resonance imaging. Computed tomography angiography of the cerebral vasculature was normal. Cerebrospinal fluid (CSF) analysis and an extensive serum lab workup were also unremarkable. She was treated with high-dose corticosteroids and eventually transitioned to methotrexate. A month after being on treatment she developed a second stroke. A cerebral angiogram was obtained and did not show evidence of CNS vasculitis. The methotrexate was eventually stopped and the prednisone was tapered. Approximately 3 months later she developed a third stroke and worsening APMPPE-associated maculopathy in both eyes. She was eventually started on oral cyclophosphamide. Conclusions & importance: Although rare, CNS vasculitis is a known complication of APMPPE. This case is atypical given the development of multiple recurrent strokes, lack of inflammatory evidence on CSF analysis, and normal imaging of the cerebral vasculature. This report highlights the need for a high level of clinical suspicion for CNS vasculitis with APMPPE despite noncontributory cerebral angiographic imaging and normal CSF analysis. Keywords: Acute posterior multifocal placoid pigment epitheliopathy, APMPPE, Cerebral vasculitis, Strokehttp://www.sciencedirect.com/science/article/pii/S2451993619300313 |
| spellingShingle | Evan Berger Ghassan Ghorayeb Jeffery Hogg An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes American Journal of Ophthalmology Case Reports |
| title | An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes |
| title_full | An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes |
| title_fullStr | An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes |
| title_full_unstemmed | An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes |
| title_short | An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes |
| title_sort | atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes |
| url | http://www.sciencedirect.com/science/article/pii/S2451993619300313 |
| work_keys_str_mv | AT evanberger anatypicalcaseofacuteposteriormultifocalplacoidpigmentepitheliopathywithrecurrentstrokes AT ghassanghorayeb anatypicalcaseofacuteposteriormultifocalplacoidpigmentepitheliopathywithrecurrentstrokes AT jefferyhogg anatypicalcaseofacuteposteriormultifocalplacoidpigmentepitheliopathywithrecurrentstrokes AT evanberger atypicalcaseofacuteposteriormultifocalplacoidpigmentepitheliopathywithrecurrentstrokes AT ghassanghorayeb atypicalcaseofacuteposteriormultifocalplacoidpigmentepitheliopathywithrecurrentstrokes AT jefferyhogg atypicalcaseofacuteposteriormultifocalplacoidpigmentepitheliopathywithrecurrentstrokes |