Neurological symptoms associated with acute multifocal placoid pigment epitheliopathy: treatment dilemma and diagnostic issues
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Published on: September 20, 2018
Justyna D Oleszczuk1, Muhammad Usman Saeed
1Sutton Hospital, The Roy Harfitt Eye Unit , Cotswold Road, London , UK.
Abstract:
Acute multifocal placoid pigment epitheliopathy (AMPPE) is thought to be caused by vasculitis of the choroid. Central nervous system involvement is rare. We report a case of a 28-year-old male who developed blurred vision (6/12 OD, 6/24 OS). Past medical history involved non-specific viral meningitis treated with intravenous antibiotics and antivirals. Subsequently, the patient complained of recurrent headaches. Based on typical clinical findings and fluorescein angiogram, he was diagnosed with AMPPE. Visual disturbance resolved without any systemic treatment. Six months later, visual symptoms recurred and resolved spontaneously, but his headache continued. A few months later, the patient developed acute progressive neurological signs and symptoms necessitating inpatient admission. Lacunar infarcts in the CNS were found, which had not been noted in previous neuro-imaging studies. Cerebral vasculitis associated with AMPPE was diagnosed and treated with systemic steroids and immune suppression. Currently, the patient remains asymptomatic under joined care of an ophthalmologist and a neurologist.
Insights
Acute multifocal placoid pigment epitheliopathy (AMPPE) can involve the central nervous system. This case highlights rare neurological complications of AMPPE, emphasizing the need for combined ophthalmology and neurology care.
Area of Science:
- Ophthalmology
- Neurology
- Immunology
Background:
- Acute multifocal placoid pigment epitheliopathy (AMPPE) is an inflammatory eye condition typically affecting the choroid.
- Central nervous system (CNS) involvement in AMPPE is considered rare, with limited documented cases.
Observation:
- A 28-year-old male presented with blurred vision and was diagnosed with AMPPE.
- He experienced recurrent visual disturbances and persistent headaches following the initial diagnosis.
- The patient later developed acute progressive neurological deficits, including lacunar infarcts on neuro-imaging.
Findings:
- The patient was diagnosed with cerebral vasculitis associated with AMPPE.
- Neuroimaging revealed CNS lacunar infarcts not previously identified.
- Treatment involved systemic steroids and immunosuppressive therapy.
Implications:
- This case underscores the potential for rare but serious CNS manifestations in AMPPE.
- Highlights the importance of a multidisciplinary approach involving ophthalmologists and neurologists for managing complex AMPPE cases.
- Suggests that vasculitis associated with AMPPE may require systemic immunosuppressive treatment for neurological complications.

