Auteurs : K. Bigaut a,*, L. Kremer a,b, A. Hacquard d, B. Wolff e, N. Collongues a,b,c, J. De Seze a,b,c
A 24-year-old male with no medical history presented with a bilateral blurred vision that progressively worsened for three days without influenza-like illness. Fundoscopy showed multiple cream-colored placoid lesions. Optical coherence tomography revealed multiple hyperreflective lesions located in the outer nuclear layer and subfoveal photoreceptor layer disruption in both eyes. Fluores- cein angiogram showed early hypofluorescent lesions with a staining in the late sequence. Indocyanine green chorioangiography angiograms showed bilateral multiple hypofluorescent lesions in the early phase which persisted throughout the late phase. Based on clinical features, the patient has been diagnosed with bilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE).
The next day, the patient was referred to the neurology department for a thunderbolt headache. He did not show any meningeal symptoms nor signs of focal lesion. Magnetic resonance imaging (MRI) showed no abnormalities. The analysis of the cerebrospinal fluid (CSF) revealed a lymphocyte count of 34.106/L and a protein level of 0.46 g/L without any evidence of oligoclonal bands, a glucose level of 0.56 g/L. PCR for HSV, VZV and enterovirus in the CSF was reported negative. A lupus anticoagulant was detected in blood samples without any other immunological abnormalities. Borrelia burgdorferi serology, syphilis serology and tuberculin skin test were negative. Minor salivary gland biopsy and lung computed tomography were normal.
The diagnosis of APMPPE with aseptic meningitis was made. Intravenous methylprednisolone therapy was induced (3 g in 3 days) and followed by an oral steroid therapy of 1 mg/ kg/day.
After two months, while decreasing steroid therapy, the patient presented with sudden-onset visual impairment, Weber’s syndrome and impaired consciousness (NIHSS 20) requiring intensive care. Brain MRI showed mesencephalic and cerebral infarction in the territories of the posterior
cerebral artery and its paramedian branches, of the middle cerebral artery and of the anterior cerebral artery. Magnetic resonance angiography and contrast-enhanced MRI showed signs of vasculitis and meningitis (Fig. 1C). Cardiovascular investigation was normal. Intravenous methylprednisolone therapy and monthly cyclophosphamide infusion were induced, resulting in a partial improvement (NIHSS 9).
a Service de neurologie, hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France
b Inserm U1434, centre d’investigation clinique, 67000 Strasbourg, France
c Inserm U1119, biopathologie de la myéline, neuroprotection et stratégies thérapeutiques, fédération de médecine translationnelle de Strasbourg, 67000 Strasbourg, France
d Service de neurologie, groupe hospitalier de la région de Mulhouse et Sud Alsace, 68100 Mulhouse, France
e Centre d’ophtalmologie de la maison rouge, 67000 Strasbourg, France
* Corresponding author.