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From a research, medical and clinical standpoint, little is known about Devic's disease. What is known is that the condition is characterized by inflammation of the optic nerve and spinal cord, which may result in temporary blindness, muscle weakness or paralysis.
Although current literature does not specify a relationship to any specific demographic, there is some evidence that non-Caucasian groups may be at higher risk for Devic's disease, or neuromyelitis optica.
"This indicator may be reflective of different methods of classification of symptoms/syndromes around the world and not necessarily a genetic or environmental predisposition," said L.A. Campbell, DPT, MBA, CPI. Dr. Campbell is supervisor of outpatient rehabilitation services at St. Joseph's Hospital and Medical Center/Barrow Neurological Institute in Phoenix, AZ.
St. Joseph's outpatient department has five PTs devoted to the rehabilitation of neurological patients. Many of these therapists work with patients who have Devic's disease. St. Joseph's also has a large acute-care therapy staff and an inpatient neurorehabilitation unit that provide services to these patients at different points throughout the continuum.
Mistaken Diagnosis
Strikingly similar to multiple sclerosis, Devic's disease is a rare and incurable neurologic disorder. It is so rare, in fact, that Shannon Perez, DPT, has evaluated and treated just three patients with Devic's disease in the acute-care setting during her four-year career at St. Joseph's.
According to Dr. Campbell, Devic's disease can be mistaken for multiple sclerosis based on general symptoms alone, but diagnostically, the patients have distinct differences based on radiographic findings of the brain and spinal cord. The cord lesions associated with neuromyelitis optica extend through three or more vertebral segments that are typically different from those found with multiple sclerosis. The physical impairments noted in these patients include severe motor deficits, vision and visual-spatial deficits, sensory impairments, neurological mediated pain and dysfunction of bowel and bladder.
The PT evaluation for this patient type should be neurologically focused but also assess other body systems, including postural alignment and control, cardiovascular function and stamina and general skin integrity. Additionally, the PT needs to consider the premorbid history-medical, social and physical-that could impact the patient's ability to regain motor skills.
Pregnant Predicament
Dr. Perez recently worked with a patient who was blind in one eye and had very low vision in the other eye. On top of her vision impairment, the 32-year-old woman was 24 weeks pregnant. When she was admitted to St. Joseph's, she had respiratory failure so her doctors put her on a ventilator and trach.
The patient knew she had Devic's disease before she became pregnant but because doctors had told her she was sterile, her impending motherhood was a bit of a surprise. She had been undergoing chemotherapy for Devic's disease when she discovered she was pregnant. "That was a scary situation for her, not knowing whether the chemotherapy was going to affect her baby or not," Dr. Perez said.
"During the time she was hospitalized and on a ventilator, we were working on the basics such as rolling in bed and sitting in bed," Dr. Perez explained.
The PTs in acute care helped her develop arm strength so she'd be able to carry her baby around once she was discharged from the hospital. "We worked on a lot of strength and endurance issues," Dr. Perez said. "Functioning as much as she could tolerate-it's always at a lower level when they are on the ventilator because we don't want to overexert them."
The doctors at St. Joseph's carried the patient until she was 34 weeks pregnant and then did a Cesarean section. She was treated at St. Joseph's for almost two months and was discharged to a facility for ventilator weaning. "On the hospital end of the care, we are looking at function and making sure that our patients are safe for discharge," Dr. Perez said. "The most important thing is making sure that they have the appropriate resources they need."
"Just the experience of her being here, getting to know her and her family was a great experience," Dr. Perez said. "She has stopped by since she has gotten better to show us her healthy baby. It's kind of an exciting thing for us."
The patient was originally diagnosed with MS at 11 years old. "I questioned it because that is a very early age to be diagnosed with MS," Dr. Perez said. "Later when she was 28, they changed the diagnosis to Devic's disease.
Devic's Disease Close-up
The hallmark symptom of Devic's disease is partial or full blindness. Optic neuritis is an inflammation of the optic nerve that connects the eye with the brain. The optic nerve fibers are coated with myelin, which is the fatty tissue that protects nerve cells and helps optic nerve fibers conduct electrical impulses to and from the brain. In most cases, inflammation of the optic nerve occurs as a result of demyelination or damage to the myelin. When the myelin is damaged, nerve impulses slow or even stop, causing neurological problems.
With Devic's disease, there are incidences of severe blindness but this is not necessarily a constant with every patient. "The patients get optic neuritis, which causes field cuts and distortions, and diminished sight, but this can change or resolve somewhat just like the other symptoms when remission occurs," Dr. Campbell said.
In addition to optic neuritis, Devic's disease causes an inflammation of the spinal cord, called transverse myelitis. This typically occurs at the same time as optic neuritis, but also can occur before or after. Transverse myelitis causes weakness, numbness and other sensory disturbances. Over time, the disease may alternate between periods of good health, or remission, and disabling flare-ups, or relapses, and the prognosis for patients with the disease varies. Though Devic's disease classically affects the optic nerve and spinal cord, other parts of the brain may be affected.
According to Dr. Campbell, physical therapy for patients with Devic's disease focuses on an assortment of deficits depending on the individual's needs. Impaired motor function and general mobility are a main focus within the treatment sessions, but addressing proximal control and postural stability impacts the gait cycle and standing balance significantly.
In addition to treatment of the impairments, particular attention is made to equipment and long-term planning as many of these individuals become severely impaired very quickly after onset, and while some may recover over time, a portion of them will continue to have relapses with progressive disability resulting.
"Therapeutically, the physical therapy treatments of MS and Devic's disease are very similar," Dr. Campbell said. "However, the diagnoses of Devic's disease and MS are slightly different."
Devic's disease demonstrates longitudinal spinal cord lesions of three or more segments, while MS does not. There are also specific radiologic diagnostic criteria in a brain magnetic resonance image for MS that Devic's disease does not meet. Additionally, whereas optic neuritis occurs in many patients with MS, Devic's disease patients typically, but not always, present with bilateral ON and acute myelitis simultaneously.
Dr. Campbell's clinical focus in recent years has been on MS patients, which helps her with Devic's disease patients because the symptoms and corresponding PT treatment approaches are very similar. She works closely with the physicians in the Neuroimmunology Department at Barrow Neurological Institute in program planning and treatment, including the development of their monthly Interdisciplinary Multiple Sclerosis Rehabilitation Clinic.
PT Focus
Dr. Campbell recognizes that the PTs at St. Joseph's are fortunate to be able to treat their outpatient neurological clients in hourly, one-on-one sessions because this attention is not available in all hospitals and clinics. The average treatments are one to two times per week as the patient tolerates treatment, with an average length of stay of about 12 weeks.
Each PT session varies according to the patient's needs and the perspective of her therapist. Dr. Campbell typically focuses on proximal stability and postural awareness as an aspect of neuromuscular re-education and spends time educating the patient in movement awareness and self-correction.
Each session also is spent addressing specific mobility needs that may include gait training if appropriate. Dr. Campbell has completed the NDTA three-week course and is a Certified Pilates Instructor. She uses many principles of normalized movement analysis and proximal stabilization as the foundation for her neuromuscular re-education activities with each patient depending on need.
Modified Pilates-based, core-muscle exercises are safe for patients with Devic's disease because many of the exercises are done lying down and are non-impact and non-weight bearing, although they can work best in closed-chain positions such as hook-lying or side-lying.
The most important thing in acute care is to clearly communicate with the patients, especially if they have visual impairments already. "We need to be very clear and concise with instructions," Dr. Perez stated. "It's important that we tell patients exactly what we want to do and how we are going to do it before we begin any course of action."
The PTs at St. Joseph's work in tandem with OTs. The physical therapists in acute care work on compensation strategies from a gross-motor functional level, such as getting out of bed, walking and getting to the bathroom. The OTs also work on compensation strategies but focus on self care, feeding, grooming and hygiene.
Compensation strategies include counting steps to the bathroom, making sure that the patients know their environment and using tactile cues in the environment to help with low vision. The PTs also use assistive devices to help with depth perception and balance issues that go hand-in-hand with vision loss.
"Once a patient's vision progressively declines, so does the balance and depth perception," Dr. Perez explained. The typical patient is ambulatory, so the PTs need to teach him to get around safely and comfortably as his vision declines. "We give them tools to manage their condition at home as well as resources in the community," she said. "The Association for the Blind and Visually Impaired is a great resource for the patients."
Rebecca Mayer is regional editor of ADVANCE and can be reached at rmayer@merion.com
Case Study of a Patient with Devic's Disease
Provided by L.A. Campbell, DPT, MBA, CPI, supervisor of outpatient rehabilitation services at St. Joseph's Hospital and Medical Center/Barrow Neurological Institute in Phoenix, AZ.
Patient: 57 year-old patient diagnosed with Devic's disease six years ago.
History of Devic's disease: Has had more than four relapsing episodes of acute myelitis with progressively worsening lower-extremity weakness and history of falls. Has had physical therapy for each episode both at the inpatient and outpatient levels of care. Most recent episode was July 2007.
Clinical Presentation: Urinary tract infection, thrombocytopenia, enhanced thoracic lesions in two places, across three segments each, pain in the low back and extremities scored at 4 to 6/10.
Medications: Baclofen, Neurontin, Retuxan.
Medical Procedures: Plasmaphoesis and dialysis.
Co-morbidities: Recurrent diplopia, edema in bilateral ankles, clonus present in the lower extremities, muscle weakness RLE greater than LLE averaging 2+/5.
Prior level of function: Power wheelchair used for most mobility, adapted to LUE for assistance with all ADLs, stands with FWW and assistance. Has used an AFO in the past.
Estimated Length of Treatment: 12 weeks.
Frequency of Treatment: one to two times per week per patient tolerance.
Treatment Plan: Therapeutic exercise, neuromuscular re-education, balance training, patient/family education, mobility training and home program development to revise current one.
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