The patient was presumed to have had neuromyelitis optica, a subtype of multiple sclerosis, given her age and family history. A CT scan appeared to show a brain bleed. However, the patient actually had a tumefactive multiple sclerosis presentation.
This case reports that the diagnosis of tumefactive multiple sclerosis can be made on MRI, PET scan, or cerebrospinal fluid analysis. Biopsies are not recommended, given associated risks with the procedure, especially in cases where TMS is apparent on imaging.
Patient Admitted with Rapidly Progressive Paralysis
The visual vignette focuses on a 21-year-old woman who was admitted to a community hospital with rapidly progressive paralysis on her left side and a worsening headache. She had a recent diagnosis of pseudotumor cerebri, also known as idiopathic intracranial hypertension, a rare medical condition that occurs when pressure inside the skull increases for no clear reason. Work-up included MRI, which revealed demyelinating lesions suspicious for neuromyelitis optica, a subtype of multiple sclerosis. The neuromyelitis optica testing was negative and a cerebrospinal fluid analysis was pending at time of the patient’s transfer to acute comprehensive inpatient rehabilitation. Shortly after entering rehab, the patient started having functional decline. A non-contrast CT showed a large brain lesion involving the right frontal and parietal lobes, with damage to space or cavity in these brain regions. She was rushed to the tertiary medical center where an MRI showed large right hemispheric lesions crossing the midline with obvious mass effect, meaning local pressure on adjacent parts of the brain. The patient received five days of intravenous solumedrol, a steroid, and plasma exchange. In certain forms of multiple sclerosis, plasma exchange is used to manage sudden, severe attacks. It is believed that the plasma may have proteins that in essence attack the body. By replacing the plasma, these proteins are eliminated and symptoms may improve. Ten days after treatment, the patient returned to the acute inpatient rehab unit. No further functional regression occurred, and the patient was discharged at a modified independent wheelchair level with transition to outpatient rehabilitation.
Biopsies Not Recommended to Diagnose Tumefactive MS
This case reports that the diagnosis of tumefactive multiple sclerosis can be made on MRI, PET scan or cerebrospinal fluid analysis. Biopsies are not recommended, given associated risks with the procedure, especially in cases where TMS is apparent on imaging. After the diagnosis has been made the treatment plan is altered based on the needs of the patient. During the acute occurrence or recurrence of a lesion, IV steroids can be used with good outcomes, which can be followed by disease modifying drugs to monitor the underlying multiple sclerosis. “It was imperative to transfer her for advanced imaging to identify the lesion, since treatment is very different for brain bleed versus diffuse demyelination seen in MS,” said Dr. Shustorovich, DO, Pain Medicine Physician, JFK Johnson Rehabilitation Institute, and a co-author of the case study.
