An Elusive Seizure

Abstract

We present a case of a healthy 62-year-old woman who developed recurrent seizures preceded by subacute cognitive slowing, ataxia, night sweats, and weight loss. She was found to have cytopenias, multifocal T2/FLAIR hyperintensities on magnetic resonance imaging (MRI), and magnetic susceptibility artifact lesions on susceptibility weighted imaging (SWI). Her symptoms, imaging and laboratory abnormalities all improved with high-doses of steroids and intravenous immunoglobulin (IVIG). But recurred several weeks after completing treatment. Despite extensive work-up, she required multiple hospitalizations and repeat diagnostic studies to arrive at a diagnosis. With an expert discussant in hematology and oncology, we review the differential diagnosis and stepwise approach of unexplained neuro-inflammatory syndromes with cytopenias and systemic symptoms. Our case highlights how time, empiric treatment response, and repeated diagnostic studies refine differential diagnoses and subsequent evaluation. After revealing the diagnosis, we discuss the heterogenous clinical manifestations of this disease process.

Keywords: cerebral microhemorrhages; clinical reasoning; cytopenia; hematology; hemophagocytic lymphohistiocytosis; seizure.

Figure 1.

MRI Brain showing progression of disease from initial presentation. (A) Axial DWI, (B) corresponding axial ADC, and (C) axial T2 FLAIR images highlighting lesion within the right corona radiata taken from patient’s initial hospital admission. (D) Coronal T1 post-gadolinium contrast image demonstrating wispy enhancement within the inferior aspect of this lesion. (E) Axial DWI and (F) axial T2 FLAIR images from more inferior location demonstrating patchy lesions involving left corpus callosum and right juxtacortical white matter; of note, DWI changes were largely T2 shine-through for these lesions and so ADC is not presented here. (G) Coronal T1 post-gadolinium contrast image demonstrating faint enhancement within left corpus callosum lesion.

Figure 2.

MRI Brain showing progressive microhemorrhages over course of illness and PET imaging revealing systemic imaging findings. (A) Axial susceptibility-weighted image demonstrating multifocal susceptibility artifact especially involving right parietal cortex at initial presentation. (B) Axial susceptibility-weighted image at corresponding level and (C) more inferior level taken from patient’s fourth hospital admission demonstrating progressive microhemorrhages over the course of illness. (D) Whole-body PET scan during the fourth admission revealing diffuse R humeral FDG avidity and focal avidity in L humeral diaphysis.