Hackensack Meridian JFK Johnson Rehabilitation Institute’s Alexander Shustorovich, DO, co-authors the study
A new visual vignette published in the Journal of Physical Medicine & Rehabilitation reports on the case of a 21-year-old woman* who was admitted to a community hospital with rapidly progressive paralysis on her left side and 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 magnetic resonance imaging (MRI) which revealed demyelinating lesions suspicious for neuromyelitis optica (NO) (a subtype of multiple sclerosis (MS)). NO is a central nervous system disorder that causes inflammation in the nerves of the eye and spinal cord. MS is a demyelinating disease that causes damage to the myelin sheath or protective covering that surrounds nerve fibers in the brain. The neuromyelitis optica testing was negative, and cerebrospinal fluid (CSF) 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 computed tomography (CT) was obtained, showing a large brain lesion involving the right frontal and parietal lobes, with gyral effacement, or 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. Multiple sclerosis is caused by inflammation in the nerves and myelin, and steroids can help relieve MS symptoms because they help to reduce nerve inflammation. 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.
”This case increases awareness of the clinical presentation of tumefactive multiple sclerosis with the goal of helping clinicians to identify and treat it appropriately in the future,” said Alexander Shustorovich, DO, Pain Medicine Physician, JFK Johnson Rehabilitation Institute, a co-author of the case study. “The patient was presumed to have had neuromyelitis optica given her age and family history. The CT scan after her functional decline in rehab looked almost identical to a brain bleed versus an enhancing mass. Treatment is very different for brain bleed versus diffuse demyelination seen in MS. It was imperative to transfer her for advanced imaging to identify the lesion. In fact, the problem was not a bleed, but a tumefactive multiple sclerosis presentation, and she subsequently was treated for that condition with symptomatic improvement.”
This case reports that the diagnosis of tumefactive multiple sclerosis can be made on MRI, PET scan, or cerebrospinal fluid analysis, whereas 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.
Multiple sclerosis is an autoimmune disorder that causes the destruction of myelin and oligodendrocytes (myelinating cells) within the central nervous system. It classically presents with lesions that appear at different times and in different areas of the body. One example is a transient case of optic neuritis followed by sudden limb paralysis that subsequently resolves. Tumefactive multiple sclerosis (TMS) is considered a subcategory of multiple sclerosis that presents as large demyelinating lesions usually greater than 1.5 cm, however, the definition continuously evolves. MRI will show large demyelinating ring-enhancing lesions >2 cm with mass effect and edema.
*The patient was fully informed regarding the intention to publish this case report in a medical journal and provided full consent to publish details of her medical condition.
About Hackensack Meridian JFK Johnson Rehabilitation Institute:
Named one of the Top Rehabilitation Hospitals in the Country by U.S. News and World Report, JFK Johnson Rehabilitation Institute is a 94-bed facility in Edison, New Jersey, that offers the state’s most comprehensive rehabilitation services to restore function and quality of life to those with physical impairments or disabilities. For nearly 50 years, JFK Johnson has served children and adults in the tri-state area — and has developed programs in specialties such as brain injury, stroke rehabilitation, orthopedics/musculoskeletal and sports injuries, cardiac rehabilitation, pediatrics, and prosthetics and orthotics. JFK Johnson Rehabilitation Institute is part of the Johnson Rehabilitation Institute, which also includes Johnson Rehabilitation Institute at Ocean University Medical Center and Johnson Rehabilitation Institute at Riverview Medical Center. Each rehabilitation hospital brings together highly specialized physicians and professionals with the goal of “Advancing What’s Possible” for every patient. The JFK Johnson Rehabilitation Institute also partners with the St. Joseph’s Health Acute Rehabilitation Unit at St. Joseph’s Wayne Medical Center in Wayne, New Jersey. JFK Johnson Rehabilitation Institute serves as the Physical Medicine and Rehabilitation Department of the Hackensack Meridian Medical School and Rutgers Robert Wood Johnson Medical School. To learn more, visit JFKJohnson.org.