Elke’s Story: Myelin Oligodendrocyte Glycoprotein Antibody Disease

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At age 10, Elke was afflicted by MOGAD, a serious illness that started with acute and severe paralysis and an inability to speak. Thanks to support from multiple CHOP specialists, therapists and her parents, Elke has found her way back to the path of physical and mental wellness and recovery.

elke patient story After a normal school day, 10-year-old Elke and a friend planned to go shopping in Allentown, Pa., and then rehearse for a dance/lip sync show at the friend’s house. On the drive to and from the store, Elke had trouble getting into the car. She had to lift her right leg with her hands and physically move her foot into the car.

She didn’t think much of it. But then, after the shopping trip, as she was walking into her friend’s house, Elke felt a sudden and uncontrollable urge to go to the bathroom. She wasn’t able make it to the bathroom in time and wet herself.

Embarrassed, Elke asked her friend’s grandmother to take her home. When she got home, Elke broke down crying and told her parents, Terri and [father’s name?], she didn’t feel well.

Elke’s parents thought a warm bath would help her feel better. But after the bath, she threw up. She went to bed early and awoke a few hours later to find she’d wet herself again. Elke’s parents grew especially concerned when they saw her struggling to walk to the bathroom and sit upright on the toilet. They called 911 and by the time the paramedics arrived, Elke couldn’t move the right side of her body and couldn’t talk. Her condition was quickly deteriorating.

Diagnosis of MOGAD

Elke was taken to a nearby emergency room and, after an assessment there, she was flown by medical helicopter to Children’s Hospital of Philadelphia (CHOP). As soon as the helicopter landed and Elke was wheeled into CHOP’s pediatric intensive care unit (PICU), a team of more than 12 specialists got to work caring for her.

A four-hour sedated MRI revealed one very large “tumor-like” lesion that was causing Elke’s brain to swell. Phillip Storm, MD, Chief of CHOP’s Division of Neurosurgery and Co-Director of the Neuroscience Center, performed an emergency craniotomy, in which he surgically removed part of her skull to relieve pressure on her brain, and obtained cerebral spinal fluid and biopsies.

A serum blood test came back positive for MOG antibodies, and the brain biopsy showed a demyelinating process. Elke was diagnosed with myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD).

MOGAD is a demyelinating disorder of the central nervous system, meaning that damage is caused to the myelin, or protective covering, of pathways in the brain. In this disease, the immune system attacks the myelin oligodendrocyte glycoprotein (MOG), a protein found on the myelin covering in brain, spinal cord and optic nerves.

The exact cause of MOGAD is unknown. It can cause a wide range of symptoms, from vision loss and reduced color vision to weakness in the limbs, confusion, drowsiness, encephalopathy and seizures.

The Right Place for Expert Care

Elke’s care was placed in the knowing hands of Brenda Banwell, MD, Chief of CHOP’s Division of Neurology who co-directs the Neuroscience Center with Dr. Storm. Dr. Banwell, a world leader in MOGAD treatment and research, has studied MOGAD and related neuroimmune conditions for more than 20 years. She is Co-director of CHOP’s Pediatric Multiple Sclerosis and Neuroinflammatory Disorders Clinic and leads an international panel that recently created the first guidelines to help clinicians distinguish MOGAD from other neurological disorders.

Dr. Banwell initially prescribed intravenous corticosteroids for Elke and then added intravenous immunoglobulin (IVIG) and plasma exchange (PLEX). Elke did not immediately respond, necessitating use of Cytoxan, a powerful immunosuppressive medication.

To everyone’s relief, Elke’s condition gradually began to improve. After three weeks in the PICU and a brief stay in the neuroscience monitoring unit, Elke was transferred to CHOP’s Seashore House where she had physical therapy (PT), occupational therapy (OT) and speech therapy every day for a month.

When Elke was admitted to CHOP, she had been unable to talk or walk and needed a feeding tube because she couldn’t swallow. By the time she was discharged from the hospital after a total of two months, those abilities had been fully restored.

Moving From Illness to Wellness

elke patient story Elke continued outpatient PT and OT for another year and a half. Therapy focused on alleviating spasms, involuntary muscle contractions that tighten her ankle and cause her foot to turn in a bit. She also worked to increase use of her right hand. Fortunately, PT and OT appointments are offered at the CHOP Specialty Care Center in King of Prussia, which is closer to the family’s home than Philadelphia.

While Elke still has a small residual area of prior demyelination in her frontal lobe, she no longer needs medication and no longer tests positive for MOG antibodies. Dr. Banwell continues to monitor her with follow-up visits every three months and two MRIs a year.

“An MRI every six months reassures me,” says Elke, who is now 13 years old and in the seventh grade. “When I see my brain, my lesion is always getting smaller and smaller.”

One year to the day that Elke was admitted to the hospital with MOGAD symptoms, she experienced a functional neurological disorder (FND). Symptoms of FND can appear suddenly after a stressful event. In FND, the brain generally appears structurally normal on clinical MRI scans, but it functions incorrectly (like computer software crashing), resulting in patients experiencing symptoms including limb weakness, tremor, gait abnormalities and non-epileptic seizures.

“When I came out of the hospital, I was just happy to be home,” says Elke. “Two or three months later, I started saying to myself, ‘What if this happens again?’”

Elke’s FND symptoms lasted about nine months. At its worst, she suffered 20 to 30 non-epileptic seizures a day. Cognitive behavioral therapy has helped her overcome the condition.

“When I have a fever or I feel sick, I get scared. I think I’m going to get sick again,” says Elke. “That’s my brain telling me to go into lockdown mode. I had to learn coping skills to beat it.”

“The biggest challenge for her since being out of the hospital has been learning how to be well Elke again,” adds her mom, Terri.

Elke’s experience with both MOGAD and FND has sparked her interest in pursuing a career in the medical field, particularly child life therapy.

“It just makes me feel better to put smiles on kids’ faces,” says Elke. “And it would be really valuable for me to share my experience with them.”