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Recurring meningoencephalitis in sinusitis-associated acute posterior multifocal placoid pigment epitheliopathy under
H Joswig1, C Flueckiger, A Infanger
1Department of Neurology, Cantonal Hospital St. Gallen, St. Gallen, Switzerland.
Abstract:
The authors describe a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with recurrent neurological complications. A 24-year-old man experienced subacute bilateral visual loss. Based on the characteristic findings in funduscopy and fluorescin angiography and after the exclusion of the differential diagnoses, APMPPE was diagnosed. During the course of the disease recurrent episodes of meningitis and encephalitis occurred when tapering of prednisone was attempted. Secondary to encephalitic lesions, the patient developed partial epileptic seizures, which made an anticonvulsive medication necessary. The authors considered a chronic sinusitis to be an aetiological factor of the underlying autoimmune process. Due to the complicated course of APMPPE, they decided to start long-term immunosuppressive therapy with azathioprine under which the patient remained stable and prednisone could be tapered successfully. Neurological complications of APMPPE are rare. Nevertheless, this case demonstrates that long-term immunosuppressive treatment might be necessary to prevent recurrent neurological complications in some cases.
Insights
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) can cause rare neurological issues. Long-term immunosuppression with azathioprine may be needed to manage recurrent complications effectively.
Area of Science:
- Ophthalmology
- Neurology
- Immunology
Background:
- Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an inflammatory eye condition.
- Neurological complications associated with APMPPE are uncommon but can be severe.
Observation:
- A 24-year-old male presented with subacute bilateral visual loss.
- The diagnosis of APMPPE was confirmed via funduscopy and fluorescein angiography.
- Recurrent meningitis and encephalitis occurred during prednisone tapering, leading to partial epileptic seizures.
Findings:
- Chronic sinusitis was suspected as a potential trigger for the autoimmune process.
- Long-term immunosuppressive therapy with azathioprine was initiated due to the complicated disease course.
- Azathioprine facilitated successful prednisone tapering and maintained patient stability.
Implications:
- This case highlights the potential need for prolonged immunosuppression in APMPPE management.
- Early recognition and aggressive treatment are crucial for preventing recurrent neurological sequelae.
- Understanding the autoimmune basis of APMPPE may guide future therapeutic strategies.
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