Six months after delivering a healthy baby girl, a physician experiences intermittent blurred vision and tingling in her left leg. Her diagnosis might surprise you.
A 32-year-old physician had a baby six months prior to the onset of new neurological symptoms. She reports that she has been experiencing intermittent blurred vision and tingling of her left leg for about two weeks. The episodes occur several times per week, and each tend to last for about 5 to 10 hours. She has not had any recent trauma, injuries, or infections, and she does not have any muscle weakness.
Past medical history
The patient is healthy and had a normal pregnancy with a normal labor and delivery. She gave birth to a healthy baby girl six months ago.
The patient has worn corrective lenses for many years and has had problems with her prescription for the past three years, frequently getting her prescription renewed without any improvement. She is in her last year of a medical training fellowship, and she has been doing well at work. She initially thought that her visual symptoms could be related to her work with diagnostic images, looking at them for hours at a time, but she now thinks that the problem is related to her eyes or to a neurological dysfunction. Family history: Her father and mother are in their 50s, and they are good health.
Her blood pressure is 120/65, pulse is 82, and respiratory rate is 16. The patient is alert, oriented x3, and cooperative. She appears in good health and does not have any tremors, jerking, or involuntary movements. Her skin is normal without any lesions or discoloration. Her pulse is normal, and her heart sounds are regular without any murmurs. Her breathing is normal. Her abdomen is non-tender and non-distended.
Her face is symmetric without any weakness or ptosis. Her extraocular movements are normal with no nystagmus. Her pupils are normal, equal, and reactive to light. Her vision is blurred, and she has trouble reading a pamphlet and the sign on the door with her left eye. She can read close-up and far away with her right eye, but her visual acuity was not checked during this visit. On her funduscopic examination, she has normal vessels bilaterally. She has the appearance of inflammation of her left optic nerve, consistent with optic neuritis, and a normal appearance of the right optic nerve.
Her motor strength is 5/5 in bilateral upper and lower extremities. The left patellar reflex is diminished; her other reflexes are normal. Her sensory examination is normal to light touch, pinprick, vibration, and position in all extremities except for diminished sensation to pinprick in her left leg. Her upper extremity coordination is normal bilaterally on finger to nose, and her rapid alternating movements are normal. Her heel to shin testing is normal on the right and impaired on the left. Gait is normal, but she has difficulty with tandem walking.
The patient had a brain MRI, which was normal. She had a lumbar spine MRI, which showed two small areas that were consistent with demyelination in the lumbar spine. She had visual evoked potentials, which were consistent with optic neuritis of the left eye.
DIAGNOSIS: DEVIC DISEASE
Also known as neuromyelitis optica, Devic disease, a demyelinating condition, is considered a variant of multiple sclerosis (MS); it involves the optic nerves and the spinal cord, sparing the brain. The acute treatment includes high dose steroids or plasmapheresis. The long-term management of Devic disease is the same as that of MS-disease-modifying therapies (DMTs) can be used chronically to prevent progression and relapses.
Pregnancy and the postpartum period are common times of disease exacerbation, and the first signs of disease can occur during pregnancy or the first year after delivery1. It is unclear whether this patient’s vision complaints over the past few years are related to the optic neuritis.
At the time of her diagnosis, this patient was breastfeeding her baby and regularly pumping milk. It was decided that she would be treated with a course of high dose steroids for her exacerbation, and she discontinued breastfeeding. Her paresthesias improved within a few days, but her vision did not improve right away. About a week after treatment, the vision in her left eye began to improve, but not completely.
After the course of steroids was complete, she was given the option of initiating a DMT. She wanted to have another baby within the next few years, and she was concerned about the possible effects of a DMT on a future pregnancy. Thus, she decided to postpone initiation of DMT treatment.
The patient returned after four months, at which time she was eight weeks pregnant. She had decided at the time of her diagnosis that she would not try to postpone a future pregnancy. There is a risk of Devic’s exacerbation during pregnancy, and experts suggest that immunosuppressive therapy is safe during pregnancy.2 Additionally, Devic disease can increase the risk of adverse pregnancy outcomes, such as miscarriages and and pre-term births.3
Nevertheless, the patient opted not to use a DMT during her pregnancy despite these risks. Near the end of her pregnancy, she began to experience a recurrence of paresthesias and visual changes. She opted not to use DMT or immunosuppressive therapy during the remainder of her pregnancy, and her symptoms did not worsen or resolve. Her pregnancy and delivery were otherwise uncomplicated. About three weeks after she delivered her healthy full-term baby boy, she was treated with a course of high dose steroids and experienced substantial improvement of her symptoms. She then opted to start using a DMT for treatment of Devic disease.
At a one-year follow up visit, she had not experienced any further symptoms or exacerbations and remained on her DMT.
• Pregnancy influences symptoms of MS and variants such as Devic disease.
• Treatment for MS is considered safe during pregnancy and can help prevent exacerbations.
• Women who are of childbearing age often base their decisions about DMTs and immunosuppressive therapies on their plans to become pregnant, and thus they may not follow medical advice.
1. Tong Y, Liu J, Yang T, et al. Influences of pregnancy on neuromyelitis optica spectrum disorders and multiple sclerosis. Mult Scler Relat Disord. 2018;25:61-65.
2. Wuebbolt D, Nguyen V, D'Souza R, Wyne A. Devic syndrome and pregnancy: A case series. Obstet Med. 2018;11:171-177.
3. Shi B, Zhao M, Geng T et al. Effectiveness and safety of immunosuppressive therapy in neuromyelitis optica spectrum disorder during pregnancy. J Neurol Sci. 2017;377:72-76.