Abstract Details

Title Creutzfeldt-Jakob Disease Associated Longitudinally Extensive Transverse Myelitis; a Rare Presentation Accompanied By Rapid Disease Progression

Topic General Neurology

Presentation(s) P5 - Poster Session 5 (5:30 PM-6:30 PM)

Poster/Presentation
Number 7-037

Objective NA

Background Creutzfeldt-Jakob Disease (CJD) usually affects the grey-matter structures in the brain like the cortex and the basal ganglia, although cranial white-matter involvement is not unheard of. Spinal cord involvement is not clearly described in literature. Our case describes an extremely rare association between a probable CJD and longitudinally-extensive-transverse-myelitis (LETM).

Design/Methods Reporting a 57-year-old-male with past history of indolent non-hodjkin’s lymphoma who was admitted for progressive clumsiness, gait instability and bowel/bladder incontinence over the past two months. Neurological examination revealed an alert and oriented patient but with poor attention, dysarthria, right side weakness, ataxia and up-going toe for which he was admitted.

Results The patient had MRI-spine that showed an extensive longitudinal lesion in the cervicothoracic region. His initial CSF-analysis was non-specific with only increased proteins and negative cytology. He received plasmapheresis and high dose steroids empirically without improvement. Meanwhile, the patient became increasingly mute that further progressed to becoming lethargic requiring further cranial work-up and transfer to the ICU. Brain-MRI showed an increased diffusion sequence in the caudate and putamen nuclei bilaterally as well as patchy bilateral frontal cortical ribbon increased signal. Shortly after, the patient started developing myoclonic jerks in his extremities not reflected on the EEG by any epileptic activity. Repeat CSF analysis revealed positive protein 14.3.3 and real-time quaking-induced conversion (RT-QuIC) assay. Thus, a diagnosis of sporadic CJD was probable based on the history, symptomatology and investigations. The patient was sent to hospice care.

Conclusions Our patient meets criteria of probable CJD. This is evident in the rapidly progressive dementia, extrapyramidal symptoms, mutism and clinical myoclonus. This is confirmed by MRI findings and laboratory testing. Our case also reports very rapid progression of the disease after the development of the LETM. Whether this can be considered an indicator of rapid disease progression and worse prognosis remains to be investigated.

Authors/Disclosures
Zachary B. Jordan, MD (Ohio State University Wexner Medical Center) PRESENTER	Dr. Jordan has nothing to disclose.
Ahmed Abd Elazim, MD (Sanford USD Medical Center)	Dr. Abd Elazim has nothing to disclose.
Stevon H. Boyett, DO (Ohio State University)	No disclosure on file
	No disclosure on file
Omar M. Hussein, MD (University of New Mexico)	No disclosure on file
Amro Stino, MD (Michigan Medicine - University of Michigan)	Dr. Stino has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for CSL Behring. Dr. Stino has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Dr. Stino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda. Dr. Stino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Dr. Stino has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Annexon. The institution of Dr. Stino has received research support from GBS-CIDP Foundation. The institution of Dr. Stino has received research support from Bristol Myers Squibb.

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