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Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Originally published1 May 1996https://doi.org/10.1161/01.STR.27.5.996Stroke. 1996;27:996–1001

    Abstract

    Background Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a chorioretinal disease that causes acute visual symptoms with characteristic fundus findings. Although this entity has been associated with a variety of neurological complications, it has received little attention in the neurological literature. We wanted to emphasize the spectrum of neurological involvement, in particular the occurrence and management of strokes in patients with APMPPE.

    Case Descriptions We report three patients with APMPPE and neurological disease. All three presented with marked visual disturbances and headaches. One patient developed recurrent strokes involving different vascular territories of the brain and required immunosuppressive treatment for presumed cerebral vasculitis. The other two patients had cerebrospinal fluid pleocytosis and persistent headaches but recovered spontaneously. The review of the literature demonstrates a particular pattern of neurological complications in a subgroup of patients with APMPPE.

    Conclusions APMPPE should be considered among the causes of stroke and aseptic meningitis in young adults. The diagnosis is critically dependent on a thorough ophthalmologic examination. Severe neurological complications are difficult to predict at the onset of the ophthalmologic disease. The patients should be monitored closely. If investigations suggest cerebral vasculitis, immunosuppressive treatment may be helpful to prevent recurrences.

    Acute posterior multifocal placoid pigment epitheliopathy is a chorioretinal disease typically occurring in young adults who present with binocular visual blurring, metamorphopsia, or scotomas.12 Funduscopically, there are multiple circumscribed, gray-white, flat lesions at the level of the retinal pigment epithelium (Fig 1). The clinical course is self-limited with return of visual function over weeks to months. A third of these patients have a flulike illness at the onset. Systemic manifestations are rare; however, severe neurological involvement can cause strokes and sudden death (Table 1).

    We report three patients with APMPPE and neurological complications who were admitted to our neurology service since 1989. Although all three presented similarly, only one developed strokes. On the basis of the cumulative data from the reported cases, we suggest a strategy for the workup and management.

    Case Reports

    Patient 1

    A 23-year-old woman developed severe headaches and bilaterally decreased vision in May 1993. Initial ophthalmologic evaluation revealed visual acuity of 20/400 OD, 8/200 OS, and multiple creamy yellow patches involving the retinal pigment epithelium in both posterior poles (Figs 1 and 2). Her past medical history was significant for poor dentition with loss of all permanent teeth at an early age. She had Native American ancestry on her paternal side. There were no other systemic findings, and an initial MRI of the brain was normal. The patient started treatment of 40 mg prednisone daily for presumed Vogt Koyanagi Harada disease. Subsequently, there was some subjective visual improvement, but her headaches persisted intermittently. Three weeks after her initial symptoms, while still taking steroids, she abruptly developed a severe headache followed by unresponsiveness and right-sided weakness. On admission, she was stuporous with dense right hemiplegia. Ophthalmologic examination at this stage revealed early pigmentation of the subretinal lesions in both eyes. CSF analysis showed 60×106 WBC/L (90% lymphocytes), 20×106 RBC/L, protein of 0.45 g/L, and glucose of 3.2 mmol/L. Peripheral WBC count was 22×109/L, and erythrocyte sedimentation rate was 30 mm/h. There were a few cellular casts in the initial urinalysis. A repeat MRI revealed bilateral parieto-occipital infarcts with gyral enhancement, left-sided basal ganglia signal changes with an enhancing putaminal lesion, and a nonenhancing lesion in the splenium of the corpus callosum. The occipital lesions later underwent hemorrhagic conversion (Fig 3A through 3E). A cerebral angiogram was unremarkable, as was a chest radiograph and a transthoracic echocardiogram. APMPPE with associated cerebral vasculitis was diagnosed, and administration of intravenous methylprednisolone, 250 mg QID, was started. The patient improved gradually over 2 weeks. At discharge, she had residual right hemianopsia, difficulty in reading, and slight right hemiparesis. Visual acuity had improved to 20/40 OU, and mottled pigmentation was present in the posterior pole. Oral prednisone was continued. Extensive laboratory investigations (Table 2) did not reveal an etiology. Serum adenovirus antibodies at the fourth week of her illness were positive at a titer of 1:128, but a single measurement could not prove an acute infection. γ-Glutamyltransferase and alanine aminotransferase were initially slightly elevated at 176 U/L and 61 U/L, respectively. Human leukocyte antigen typing was A11, A26, B35, B44, Bw4, Bw6, Cw4, Cw5, DR14, DR15, DR52, and DQ1.

    Two months after the initial symptoms, the patient’s visual acuity was 20/25 OU with pigment stippling in both maculae, and her right hemianopsia had slightly improved. Shortly thereafter, she experienced brief attacks of left arm and face dysesthesia. An electroencephalogram was unremarkable. She received phenytoin for possible focal seizures. The attacks continued but were somewhat less pronounced. A month later, she had a right parietal stroke when her steroid tapering was at 40 mg/d (Fig 3F). CSF revealed 32×106 WBC/L (90% lymphocytes) and protein of 0.32 g/L. Cyclophosphamide (125 mg/d) was added to her treatment. She again showed marked clinical improvement. Prednisone was discontinued at 7 months because of side effects. Twelve months after initial presentation, cyclophosphamide was also discontinued. At 24-month follow-up, she was doing well without medication.

    Patient 2

    A previously healthy 20-year-old man presented in October 1989 complaining of 2 weeks of bilateral visual blurring, 1 week of black spots in the right eye, and 4 days of episodic right retro-orbital headaches. His visual acuity was 20/200 OD and 1/200 OS. There were mild cellular infiltrates in the vitreous bilaterally and multiple irregular-shaped placoid areas of pigment epithelial graying in different stages of evolution, with the older lesions displaying atrophic retinal pigment epithelium. Fluorescein angiogram findings were diagnostic for APMPPE. Two weeks later, his vision had decreased to 20/400 OU and his headaches had intensified. Neurological examination was unremarkable. CSF analysis showed 89×106 WBC/L (100% mononuclear cells), 3×106 RBC/L, protein of 0.54 g/L, and glucose of 3 mmol/L. An unenhanced CT scan and a cerebral angiogram were normal. Laboratory evaluation did not reveal an etiology (Table 2). Angiotensin-converting enzyme level was 143 U/L (normal, 44 to 125). The visual acuity improved spontaneously over the next 3 months to a final level of 20/25 OD and 20/20 OS. Fundus examination demonstrated areas of atrophy and hypertrophy of the retinal pigment epithelium at the location of the previously active lesions. His headaches also subsided without further intervention.

    Patient 3

    A previously healthy 23-year-old man presented in April 1989 with a 1-week history of severe headaches, difficulty in focusing, and perception of black patches and white dots. The patient also complained of slight fever, night sweats, chills, and nausea. Ophthalmologic evaluation revealed a visual acuity of 20/50 OU, multiple paracentral scotomas on Amsler grid visual-field testing, mild inflammation in both anterior chambers with 1+ cells and flare, and rare cells in the central vitreous of the left eye. Fundus examination demonstrated multiple grayish-green placoid lesions at the level of the retinal pigment epithelium, almost confluent in the macula, with smaller scattered nonconfluent areas in the periphery. Fluorescein angiogram findings further supported the diagnosis of APMPPE. Two weeks later, his visual acuity was 20/60 OD and 20/30 OS. Fundus examination showed pigment epithelial mottling in the evolving lesions. Despite the improvement of his visual symptoms, the headaches persisted. Neurological examination was unremarkable. A cerebral angiogram and unenhanced CT scan were normal. CSF revealed 61×106 WBC/L (97% mononuclear cells), 17×106 RBC/L, protein of 0.46 g/L, and glucose of 3 mmol/L. The patient improved spontaneously without further intervention. A brain MRI at the sixth week of the illness was normal.

    Discussion

    In 1968, Gass1 reported three young women with bilateral rapid loss of central visual acuity secondary to multifocal yellow-white placoid lesions at the level of the pigment epithelium and choroid of the retina. Further reports revealed that this illness affects males and females equally, with striking preponderance for young adults.2 Simultaneous onset in both eyes is the rule; however, involvement of the second eye can be delayed for a few days or even weeks. The lesions are mainly at the posterior pole and never anterior to the equator. The placoid lesions start to fade within a few days and by two weeks are replaced by areas of partly depigmented pigment epithelium with irregular pigment clumping. Fluorescein angiography demonstrates characteristic hypofluorescent lesions in the early phase of the angiogram followed by late hyperfluorescence of the same areas (Fig 2, top and bottom). If the primary lesions do not involve the fovea, the prognosis is good, with nearly complete visual function returning over several weeks. Recurrences are infrequent and usually occur in the first 6 months.3 Occasionally, progressive disease can lead to chorioretinal atrophy and significant visual loss.4

    Ocular histopathology of APMPPE is not available. The primary lesion was originally considered to be at the pigment epithelium; however, more recent evidence suggests that the lamina choriocapillaris is the site mainly affected.5 Indocyanine green angiography studies support choroidal hypoperfusion as a likely mechanism of injury rather than primary pigment epitheliopathy.67 Choroidal vasculitis has been proposed as the underlying pathological process.8

    The etiology of APMPPE is unknown. The frequent history of a preceding flulike illness suggests a viral or parainfectious process. Isolated cases were associated with adenovirus,9 Lyme disease,1011 systemic lupus erythematosus,12 juvenile rheumatoid arthritis,13 sarcoidosis,14 schistosomiasis,15 hepatitis B vaccination,16 thyroiditis,17 Crohn’s disease,18 erythema nodosum,19 and nephritis.20 HLA-B7 or DR2 antigens were reported in a group of patients21 and in two familial occurrences.2223 Despite lacking definite associations, APMPPE appears to be a manifestation of various infectious or autoimmune processes, analogous to aseptic meningitis.

    The differential diagnosis of the retinal findings include serpiginous choroiditis, multiple evanescent white-dot syndrome, birdshot chorioretinitis, acute retinal pigment epitheliitis, multifocal choroiditis, and multifocal retinal metastasis.224 Certain systemic illnesses can involve both the brain and the eyes, leading to the so-called uveoretinal-meningoencephalitis syndromes. Specific entities include Vogt Koyanagi Harada disease, sarcoidosis, Behçet’s disease, systemic lupus erythematosus, Crohn’s disease, metastatic malignancies, primary intraocular lymphoma, histoplasmosis, toxoplasmosis, cytomegalovirus, and syphilis. Of these entities, Vogt Koyanagi Harada disease is a primarily ophthalmologic disease that can mimic APMPPE with initial funduscopic and angiographic findings,25 and it has been suggested that these may represent a continuum of a single disease.25

    The common neurological complications of APMPPE are CSF pleocytosis2627 and headaches, which usually improve spontaneously, as in two of our patients. However, some patients develop transient hearing loss,28 optic neuritis,3 meningoencephalitis,29 transient focal deficits,26 and strokes30313233 due to vasculitic changes.3435 There are two reported cases of death from large strokes.3637 Of nine previously described cases of strokes with APMPPE (Table 1), five had angiographic findings consistent with vasculitis. In one case, brain histopathology revealed focal granulomatous inflammation of medium-sized arteries.36 In another case, a muscle biopsy was suggestive of vasculitis.33 In two previous cases, angiography was normal, as in our first patient.

    The pattern apparent from the reported patients with strokes is that these are mostly young adults with evidence of inflammation in their CSF. Cerebral vasculitis is the presumed mechanism of injury, although angiographic evidence may be lacking. The onset of stroke is typically concurrent with the ocular disease (in one case preceding it) or within 4 to 5 months. In one instance, meningoencephalitis29 developed 5 years after the onset of ocular disease. Infarcts involve predominantly the posterior circulation and the basal ganglia. There may be a biphasic course of the neurological illness,31 and the strokes can recur during the tapering of immunosuppressives. This can be heralded by transient ischemic attacks, which may have been the case in our first patient rather than the suspected focal seizures.

    APMPPE should be considered in young patients with unexplained strokes or aseptic meningitis, particularly when associated with symptoms such as scotomas or visual blurring. Although the diagnosis can be firmly established with typical fundus and fluorescein angiography findings, the lesions are difficult to identify with direct funduscopy. Therefore, a thorough ophthalmologic evaluation is necessary in suspected cases. Etiologic investigation for previously reported associations may identify medically treatable cases and help with prognostication. An initial brain MRI for patients without neurological signs is not necessary. CSF analysis, MRI, and cerebral angiogram should be undertaken if persistent headache, neck stiffness, or transient ischemic attack is present. Meningeal biopsy may be indicated if the workup is negative but the patient continues to have ischemic attacks. Once cerebral vasculitis is diagnosed, immunosuppressive treatment should be started. Although some patients with cerebral vasculitis improved spontaneously, given the risk of mortality and recurrent strokes, we advocate aggressive treatment. Combination of steroids with cyclophosphamide or azathioprine is reported to improve the outcome in primary cerebral vasculitis.38 The immunosuppressive agents can be stopped after 6 to 12 months, since there are no reported strokes after 5 months from the disease onset. It remains difficult to predict which patients with CSF pleocytosis and headaches will evolve to have strokes. We suggest that these patients be followed closely for neurological deterioration rather than treated with low-dose steroids.

    Selected Abbreviations and Acronyms

    APMPPE=acute posterior multifocal placoid pigment epitheliopathy
    CSF=cerebrospinal fluid
    OD=oculus dexter
    OS=oculus sinister
    OU=oculus unitas
    RBC=red blood cell
    WBC=white blood cell

    Figure 1.

    Figure 1. Color photograph of the left fundus at the time of presentation. Note normal optic disk and yellow multifocal patchy lesions.

    Figure 2.

    Figure 2. Top, Early frame of fluorescein angiogram in the left eye. Note dark patchy lesions that block background fluorescence. Bottom, Late frame of fluorescein angiogram. Note intense staining of the lesions described.

    Figure 3.
    Figure 3.
    Figure 3.
    Figure 3.
    Figure 3.
    Figure 3.

    Figure 3. A and B, T2-weighted images 1 day after the first stroke. Note bioccipital, splenium, and left putamen lesions. C, T1-weighted contrast image at the same time. Note the enhancement of the left occipital meninges and the left putamen. D, Proton-density image 1 week after the first stroke. Note the bioccipital as well as left basal ganglia lesions. E, T1-weighted image 1 week after the first stroke. Note the hemorrhagic conversion. F, T1-weighted contrast images showing the new enhancing right parietal stroke.

    Table 1. Strokes Associated With APMPPE

    Author, YearAge (y) and SexOnset and Presentation (After Visual Symptoms)Diagnostic StudiesTherapy/Outcome
    Holt, 197622 MAttacks of R-sided weakness and aphasia followed by L-sided numbness (TIAs). Nine days later, first lesions of APMPPE.CT: not obtained. Angiogram: focal narrowing. CSF: 125 WBC, protein 1.22 g/L (high for 2 mo).No treatment for neurological symptoms. For eye findings, prednisone 100 mg/d for 10 days, then tapered over a month.
    Sigelman, 197918 M4 d; L hemianopsia.CT: R occipital stroke. Angiogram: disseminated focal narrowing. CSF: protein 0.55 g/L.No treatment; L hemianopsia persisted.
    Smith, 198325 M4 wk; headache and stiff neck. 7 wk; L visual blurring, L hemidysesthesia.CT (7 wk): R occipital stroke. Angiogram: focal narrowing. CSF (7 wk): 45 WBC; protein 0.3 g/L.Initial symptoms resolved on prednisone 60 mg/d. Stroke occurred 3 days after steroids were stopped. Prednisone 60 mg/d resumed and patient was stable at 6-mo follow-up.
    Wilson, 198824 M3 wk; comatose.CT: normal. Angiogram was not performed. CSF: 0 WBC; protein 0.66 g/L. Autopsy: granulomatous cerebral arteritis and strokes.Patient was taking prednisone for eye findings and became comatose 2 d after the dose decreased to 20 mg/d. Brain death occurred in 2 d.
    Weinstein, 198823 M8 wk; R leg numbness, R hand clumsiness, and mildly slurred speech. 12 wk; severe headache and L hemianopsia.CT (12 wk): R basal ganglia, L occipital strokes. MRI: hemorrhagic conversion. Angiogram: variations in arterial caliber. CSF: 19 WBC; protein 0.33 g/L.Patient did not seek medical attention at the first event. After the second event, methylprednisolone 1 g/d IV for 3 d; then prednisone 80 mg/d. Tapered after 4 mo. Well at 10-mo follow-up with stable deficit.
    Hammer, 198925 F3 wk; sudden hemiplegia and coma.CT: diffuse cerebral infarct. Angiogram or CSF was not performed.Eye findings and headaches resolved on prednisone 40 mg/d. Large stroke and death occurred at taper to 20 mg/d.
    Stoll, 1991154 M4 mo; sudden dysarthria, unsteadiness, and L hemiparesis.CT: normal. MRI: white matter, pons, and L thalamic strokes. Angiogram: stringlike narrowing. CSF: 18 WBC; protein 0.67 g/L.Dexamethasone 24 mg/d for 6 d; then methylprednisolone 80 mg/d for 8 d, followed by slow taper over 2 mo. Azathioprine 150 mg/d for long-term immunosuppression. Stable at 10-mo follow-up.
    Bodiguel, 199235 M5 wk; dysarthria, R brachiofacial paresis. Eye findings included anterior uveitis and retinal periphlebitis.CT and MRI: L internal capsule, genu of corpus callosum, and two white-matter lesions. Angiogram: normal. CSF: 75 WBC; protein 0.65 g/L.Prednisolone 60 mg/d, tapered over 7 mo without recurrences. Eye findings and mediastinal lymph nodes on chest CT suggested sarcoidosis; however, salivary gland biopsy was unremarkable.
    Bewermeyer, 1993127 M5 mo; severe stupor and dysarthria; few days later, nystagmus and L hemiparesis.CT, MRI, and PET: R pontine stroke. Angiogram: normal. Muscle biopsy: arteriolar vasculitis. CSF: 55 WBC; protein 0.36 g/L.Oral methylprednisolone 100 mg/d; after 6 wk switched to azathioprine 150 mg/d for long-term immunosuppression. Remained in good health at 18-mo follow-up.
    Present case23 F3 wk; sudden stupor, R hemiplegia, and global aphasia. 2 mo; brief L hemidysesthesia attacks. 3 mo; sudden L hemiparesis.MRI: Bilateral parieto-occipital, L basal ganglia, and splenium strokes. Angiogram: normal. MRI (3 mo): new R parietal stroke. CSF: 60 WBC; protein 0.45 g/L.Methylprednisolone 1 g/d IV for 5 d, then prednisone 80 mg/d. At the tapering level of 40 mg/d, second stroke occurred. Cyclophosphamide 125 mg/d added. Steroids and cyclophosphamide discontinued at 7 and 12 mo, respectively. Good functional state at 24-mo follow-up.

    1These two cases are also reported by Althaus et al (Ger J Ophthalmol. 1993;2:150-154).

    Table 2. Laboratory Investigations in Three Patients

    Patient 1Patient 2Patient 3
    CSFBacterial/fungal/viral cultures, cryptococcal antigen, herpes IgG/M, VDRL, Lyme antibodies, lactate/pyruvateBacterial cultures, immunoelectrophoresisBacterial cultures, cryptococcal antigen, immunoelectrophoresis, cytology
    SerologyToxoplasma IgG/M, mycoplasma IgG/M, EBV, LymeLymeToxoplasma IgG/M, HIV
    AutoimmuneANA, DNA crithidia, antiribosomal, SS-A/B, single-stranded DNA, rheumatoid factor, anticardiolipinANA, DNA crithidia, SS-A/B, ribonucleoprotein, rheumatoid factorANA, SS-A/B, rheumatoid factor
    MiscellaneousACE, CH100, PT/PTT, CBC, LFT, amino acid screen, urine CMV cultureACE, PT/PTT, CBC, LFT, thyroid function tests, RPRCH100, PT/PTT, CBC, LFT, PPD

    EBV indicates Epstein-Barr virus; ANA, antinuclear antibodies; ACE, angiotensin-converting enzyme; CH100, total hemolytic complement activity; PT/PTT, prothrombin time/partial thromboplastin time; CBC, complete blood count; LFT, liver function tests; RPR, rapid plasma reagin; PPD, purified protein derivative; and CMV, cytomegalovirus. The results were negative unless indicated in the text.

    Footnotes

    Correspondence to Sinan Çomu, MD, Children’s Hospital of Pittsburgh, Division of Child Neurology, 3705 Fifth Ave, Pittsburgh, PA 15213-2583.

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