Clinical Reasoning: An 82-Year-Old Woman With Subacute Ophthalmoparesis and Ataxia

Abstract

We present the case of an 82-year-old woman with subacute altered mental status, oculomotor disturbances, and ataxia. On examination, she exhibited bilateral ptosis, complete horizontal ophthalmoplegia, and limited vertical eye movements during upgaze associated with prominent truncal ataxia. Cerebral MRI showed a mild hyperintensity on T2 and fluid-attenuated inversion recovery sequences in the posterior brainstem extending to the upper cervical cord, without gadolinium enhancement. Clinical and radiologic features suggested an encephalomyelitis with prominent brainstem involvement. We summarize the comprehensive differential diagnosis in patients with subacute brainstem encephalitis, which includes infectious paraneoplastic syndromes and inflammatory disorders. This case highlights the relevance of performing a wide methodical screening for malignancy in case of negative initial workup.

Figure 1. Algorithm for the Diagnosis of Subacute Ophthalomoparesis and Ataxia

Categories of differential diagnosis show in white boxes with specific examples to the right and recommended confirmatory testing shown in italics. ADEM = acute disseminated encephalomyelitis; CLIPPERS = chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; CMV = cytomegalovirus; CO = carbon monoxide; EBV = Epstein-Barr virus; GFAP = glial fibrillary acidic protein; HSV = herpes simplex virus; MOGAD = myelin oligodendrocyte glycoprotein antibody–associated disease; NMOSD = neuromyelitis optica spectrum disorders; OCB = oligoclonal bands; SCLC = small cell lung cancer; SREAT = steroid-responsive encephalopathy and associated autoimmune thyroiditis; TPO = thyroid peroxidase; TSH = thyroid-stimulating hormone.

Figure 2. Brain MRI

Fluid-attenuated inversion recovery MRI brain in the axial (A) and coronal (B) planes. Arrow indicates the presence of a periependymal hyperintensity and swelling ventral to the fourth ventricle of the medulla. Sagittal plane of a T2-weighted sequence (C) showing the extension of the hyperintensity from the brainstem to the cervical spine (C1-2 levels, arrow).