Acute multifocal placoid pigment epitheliopathy and central nervous system involvement

O'Halloran, Henry S.; Berger, Joseph R.; Lee, William B.; Robertson, Dennis M.; Giovannini, Joseph A; Krohel, Gregory B.; Meckler, Roy J; Selhorst, John B.; Lee, Andrew G.; Nicolle, David A.; O’Day, Justin

doi:10.1016/s0161-6420(01)00565-6

Cited by 82 publications

(79 citation statements)

References 34 publications

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“…2b). There are a few reports on associations of APMPPE with either probable sarcoidosis [1,2] or CNS abnormalities [3]. Histopathological studies have shown the latter to be caused by granulomatous arteritis of medium-sized arteries [4].…”

mentioning

confidence: 99%

Acute posterior multifocal placoid pigment epitheliopathy with concurrent cerebral vasculitis and sarcoidosis

Prokosch

Becker²,

Thanos

et al. 2009

Graefes Arch Clin Exp Ophthalmol

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Dear Editor, Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a distinct clinical entity of unknown origin, characterized by the acute onset of multiple lesions at the posterior retinal pole at the pigment epithelium level. The clinical course is usually self-limited. In this report, a case of APMPPE emerging simultanously with sarcoidosis and CNS vascular abnormalities is described, suggesting that APMPPE is associated with a generalized inflammatory disease.A 40-year-old woman presented with bilateral visual field defects. Ophthalmoscopy showed multiple yellowwhite placoid lesions at the posterior pole of both eyes (Fig. 1a). Fluorescein angiography demonstrated an early hypofluorescence and a late hyperfluorescence of the lesions characteristic of APMPPE (Fig. 1b). The medical history included episodes of fever, cough and painful nodular skin lesions. Biopsy of these skin lesions demonstrated granulomatous epitheloid cells consistent with erythema nodosum. X-ray disclosed bilateral enlargement of the hilar lymph nodes, suggestive of sarcoidosis. Bronchoalveolar lavage showed a high CD4/CD8 ratio of 3.6: transbronchial biopsy revealed non-caseating granulomas (Fig. 2a), and the serum level of angiotension-converting enzyme was elevated. Cranial MRI revealed stenosis of the left middle cerebral artery (Fig. 2b).There are a few reports on associations of APMPPE with either probable sarcoidosis [1,2] or CNS abnormalities [3]. Histopathological studies have shown the latter to be caused by granulomatous arteritis of medium-sized arteries [4]. Fig. 1 Multiple yellow-white placoid lesions at the posterior pole (a), showing late hyperfluorescence in fluorescence angiography (b) V. Prokosch (*) : S. Thanos :

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mentioning

confidence: 99%

Acute posterior multifocal placoid pigment epitheliopathy with concurrent cerebral vasculitis and sarcoidosis

Prokosch

Becker²,

Thanos

et al. 2009

Graefes Arch Clin Exp Ophthalmol

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“…The anterior circulation was involved in 12/31 (39%), the posterior circulation in 9/31 (29%) and in 10/31 (32%) patients cerebral infarcts occurred in multiple vascular territories. MRI was consistent with ischaemic strokes in the majority of patients but in one case series, one patient had an intracerebral haemorrhage complicating superior sagittal sinus thrombosis [17]. Seventy percent of patients with cerebrovascular complications were male.…”

Section: Neurological Complications Of Amppementioning

confidence: 77%

“…The features of headache complicating AMPPE are poorly characterised in published case histories but where described the headaches were severe [5,6,17,22,23], persistent [17,26,27], localised [5] or diffuse [6]. Meningitic symptoms were described in the minority with only 4/52 (8%) patients experiencing either neck stiffness [9,10], fever [18] or photophobia [22].…”

Section: Neurological Complications Of Amppementioning

confidence: 99%

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Neurological complications of acute multifocal placoid pigment epitheliopathy

Brownlee

Anderson

Sims

et al. 2016

Journal of Clinical Neuroscience

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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.

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“…Others 2,5,6 have reported substantial rates of persistent visual field defects, metamorphopsia, and incomplete visual recovery while remaining equivocal regarding the role of systemic therapy. Several 1,7,8 agree that despite the paucity of high-quality clinical evidence, rapid initiation of therapy is justified in patients with central nervous system involvement and that long-term immunomodulatory therapy may be necessary.…”

Section: Discussionmentioning

confidence: 99%