Neurological complications of acute multifocal placoid pigment epitheliopathy

Abstract

Acute multifocal placoid pigment epitheliopathy (AMPPE) is an autoimmune chorioretinal disease that can be complicated by neurological involvement. There is limited information on this potentially treatable condition in the neurological literature. The objective of this patient series is to describe the neurological complications of AMPPE. We retrospectively identified patients with neurological complications of AMPPE seen at Auckland Hospital between 2008 and 2013 and summarised cases in the literature between 1976 and 2013. We identified five patients with neurological complications of AMPPE at Auckland Hospital and 47 reported patients. These patients demonstrated a spectrum of neurological involvement including isolated headache, stroke or transient ischaemic attack, seizures, venous sinus thrombosis, optic neuritis, sensorineural hearing loss and peripheral vestibular disorder. We propose criteria to define AMPPE with neurological complications. A cerebrospinal fluid (CSF) lymphocytosis in a patient with isolated headache may predict the development of cerebrovascular complications of AMPPE. Patients with cerebrovascular complications of AMPPE have a poor prognosis with high rates of death and neurological disability among survivors. Predictors of poor outcome in those who develop neurological complications of AMPPE are a relapsing course, generalised seizures and multifocal infarction on MRI. All patients with neurological complications of AMPPE, including headache alone, should be investigated with an MRI brain and CSF examination. Patients with focal neurological symptoms should receive intravenous (IV) methylprednisolone followed by a tapering course of oral steroids for at least 3months. Patients with AMPPE and an isolated headache with a CSF pleocytosis should be treated with oral steroids.

Acute posterior multifocal placoid pigment epitheliopathy associated with CN III palsy

“… 4 , 5 Rarely, cerebral vasculitis, and even death may occur. 5 , 6 , 7 Here, we present a novel association of APMPPE and isolated CN III palsy in a 20-year-old woman.…”

“… 6 Brownlee et al identified five patients with neurological complications of AMPPE seen at Auckland Hospital between 2008 and 2013, and summarised cases in the literature between 1976 and 2013. 7 Published complications most commonly include cerebral vasculitis and stroke, but have also been known to include headaches, meningoencephalitis, viral meningitis, seizures, peripheral neuropathy, CN VI palsy, transient hearing loss, cavernous sinus thrombosis, hypoesthesia & paresthesia. 6 , 7 CNS complications were observed to occur twice as often in men, and may occur simultaneously with ocular symptoms or months later, 6 , 7 , 13 with two thirds of ischemic events occurring within 4 weeks of visual symptom onset.…”

“… 7 Published complications most commonly include cerebral vasculitis and stroke, but have also been known to include headaches, meningoencephalitis, viral meningitis, seizures, peripheral neuropathy, CN VI palsy, transient hearing loss, cavernous sinus thrombosis, hypoesthesia & paresthesia. 6 , 7 CNS complications were observed to occur twice as often in men, and may occur simultaneously with ocular symptoms or months later, 6 , 7 , 13 with two thirds of ischemic events occurring within 4 weeks of visual symptom onset. 7 Most of the neurological complications resolve completely, but prognosis may vary, as 18–25% of patients sustain long-term disability, and 11–13% of CNS complications result in death.…”

“… 6 , 7 CNS complications were observed to occur twice as often in men, and may occur simultaneously with ocular symptoms or months later, 6 , 7 , 13 with two thirds of ischemic events occurring within 4 weeks of visual symptom onset. 7 Most of the neurological complications resolve completely, but prognosis may vary, as 18–25% of patients sustain long-term disability, and 11–13% of CNS complications result in death. 6 , 7 …”

“… 7 Most of the neurological complications resolve completely, but prognosis may vary, as 18–25% of patients sustain long-term disability, and 11–13% of CNS complications result in death. 6 , 7 …”