Recurrent Vertebrobasilar Strokes Associated With Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an ophthalmologic condition of likely immune origin. Typically, it presents as a chorioretinitis with bilateral visual disturbance and characteristic funduscopic lesions of the retinal pigment epithelium. APMPPE has been associated with several systemic and neurological complications, including cerebrovascular diseases.

A 58-year-old woman presented with sudden right hemiparesis and dysarthria, with magnetic resonance imaging evidence of an acute ischemic lesion in the left pons. Five days later, she developed contralateral hemiparesis and evolved into a locked-in syndrome. A new lesion located at the right pontomedullary junction was detected by magnetic resonance imaging. The patient developed a visual deterioration that had started 1 week before admission. An ophthalmologic evaluation showed visual acuity loss (20/200 in both eyes) and characteristic yellow-white lesions in the posterior pole of both eyes. Laboratory analyses were remarkable for positive antinuclear antibodies, an elevated erythrocyte sedimentation rate, and C-reactive protein. The cerebrospinal fluid showed elevated protein levels, lymphocytic pleocytosis, and normal glucose levels. The fundoscopy findings together with recurrent strokes led to the diagnosis of APMPPE and appropriate immunomodulatory treatment with corticosteroids and azathioprine was started.

This case illustrates the importance of careful evaluation and high clinical suspicion for this entity when dealing with patients with new-onset headache or stroke associated with visual impairment. Proper ophthalmologic evaluation is important so that adequate therapy is established.