Presentation Time: 1030-1045

Alexander S. Easton 

Department of Pathology, Dalhousie University, Halifax, NS, Canada


Case 5: Acute presentation of an unusual frontal white matter disease

This 56 year old black woman, a retired music teacher, presented 13 May 2018 with acute onset of reduced hearing, enhanced verbal stutter, disturbed gait and confusion.  She was assessed for stroke, but brain MRI on 16 May showed only a small (5mm) acute infarct in the left globus pallidus, and the patient was discharged on low dose aspirin.  She presented again on 5 June, found wandering and confused outside the hospital, with stuttering speech and disorientation.  On examination, her language had impaired fluency with intact comprehension and repetition.  This was diagnosed as rapidly progressive delirium/dementia with transcortical motor aphasia.  Her past medical history included ulcerative colitis, proctocolectomy with end ileostomy for rectal adenocarcinoma in 2004 followed by right lung metastasis in 2007, Grave’s disease, depression, glaucoma and osteoarthritis.  Brain MRI on 6 June showed new bilateral symmetric white matter T2 hyperintensities without diffusion restriction in subcortical white matter with U-fiber sparing, most prominent in the frontal lobes.  This became more prominent on repeat MRI brain on 22 June with mild diffusion restriction.  Frontal lobe findings remained stable on repeat MRI brain scans through to September, with some interval volume loss and new bilateral T2 signals in the caudate nuclei, optic radiation and insular cortex.  Vessel wall imaging for vasculitis was negative.  Multiple additional tests including brain biopsy were negative.  The patient declined in function and needed admission to a long term care facility, where she died in October 2019.  A complete autopsy was performed, including brain examination.


Is this likely to be autoimmune?

Is this a case of primary or secondary demyelination?

What are the CNS complications of ulcerative colitis? 

Materials submitted: 

One H&E/LFB stained slide.

Reveal Diagnosis

To be added after the meeting.