Objectives Rapidly progressive dementias manifest with cognitive decline that progresses over weeks to months. It is rare for dementia to present early in the course of multiple sclerosis (MS). However, several case series on rapidly progressive dementia include a small number of patients with MS, and one small case series specifically examined dementia presenting within five years of MS diagnosis.
Methods Case study.
Results A 66 year old woman presented to hospital with progressive left upper limb weakness and subacute cognitive decline over six to nine months. MRI brain revealed a right frontoparietal tumefactive lesion. Oligoclonal bands were detected in the CSF and a brain biopsy confirmed demyelination. The patient’s serum was tested in our research lab and revealed a novel antibody.
The patient experienced a fluctuating clinical course but responded well to treatment with intravenous (IV) methylprednisolone, IV immunoglobulin (IVIG) and Cyclophosphamide. Unfortunately, further deterioration occurred five months after treatment with Cyclophosphamide and Rituximab was initiated. Clinical improvement in cognition was again observed, albeit briefly.
Six months later, the patient died due to bronchopneumonia. The period from initial presentation to death spanned 30 months. A non-coronial autopsy was performed and revealed widespread incomplete cortical myelin loss and atrophy.
Conclusions Although a few cases have previously been reported in the published medical literature, dementia in the early stages of MS is extremely rare and may in fact be due to a distinct immunopathologic process.
Zhang W, et al, Analysis of clinical features for 8 patients with autoimmune dementia, Zhonghua Yi Xue Za Zhi. 2014 Feb 11;94(5):359–63.
Mendes MF, et al. Early and severe cognitive impairment in multiple sclerosis. Dement Neuropsychol 2012 Jan-Mar;6(1);48–52.
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