|Year : 2021 | Volume
| Issue : 1 | Page : 80-82
Devic's disease: A rare case presentation
Sahebaan S Sethi, Aditya Sethi
Consultant Ophthalmologist, Arunodaya Deseret Eye Hospital, Gurugram, Haryana, India
|Date of Submission||02-Sep-2020|
|Date of Acceptance||13-Jan-2020|
|Date of Web Publication||27-Mar-2021|
Dr. Sahebaan S Sethi
Arunodaya Deseret Eye Hospital, NH-4, Sector 55, Gurugram - 122 011, Haryana
Source of Support: None, Conflict of Interest: None
We report a case of a young patient with sudden drop in vision and lower limb weakness. On examination was found to have left eye disc edema and abnormal CNS function. On further investigation, the patient was diagnosed to have optic neuritis with associated transverse myelitis-a syndrome referred to neuromyelitis optica or Devic's Disease. This case emphasizes the need for timely intervention to prevent permanent vision loss.
Keywords: Devic's disease, neuromyelitis optica, optic neuritis, transverse myelitis
|How to cite this article:|
Sethi SS, Sethi A. Devic's disease: A rare case presentation. TNOA J Ophthalmic Sci Res 2021;59:80-2
| Introduction|| |
Neuromyelitis optica (NMO), characterized by concurrent inflammation and demyelination of the optic nerve (optic neuritis [ON] and the spinal cord (myelitis), is an immune-mediated inflammatory disorder of the central nervous system. It occurs as an autoimmune disorder of the central nervous system, mainly involving the optic nerves and spinal cord, and sometimes in the brain.
NMO may occur after an infection but the cause remains largely unknown, although it may be associated with another autoimmune condition. Thus, it is often misdiagnosed as multiple sclerosis (MS) or as a type of MS, even though it is a distinct condition.
NMO though extremely rare, affecting between 0.05 and 4.4 in every 100,000 people worldwide, affects the age group above 40 years more often. NMO episodes may be reversible, but can be severe enough to cause unilateral or bilateral blindness, weakness, or paralysis in the legs or arms.
| Presentation|| |
A 26-year-old male presented to us with complaints of sudden diminution of vision and pain on movement in the left eye since a day. He gave a history of suffering from fever with chills with rigors and loss of bladder control 2 days back which was associated with pain and distention of abdomen. He also gave a history of weakness in right lower limb, followed by left lower limb since the past 3 days.
The patient was conscious, alert, and oriented. On systemic examination, he was found to be essentially normal. Examination of the central nervous system revealed GCS of 15/15,
- Right lower limb-3+/5
- Left lower limb-4+/5
- Both upper limbs-4+/5
- Sensation: Bilaterally reduced below the sternum. Altered sensation D10-D11
- Plantar reflex: Right–exaggerated; left-reduced
- Deep tendon reflexes: brisk more in lower limb
- Tone: Both upper and lower limb-normal.
Ocular examination findings showed right eye normal findings, left eye showed a drop in vision to counting finger at 50 m, drop in color vision to 10/21 (Ishihara Chart). Pupil examination of the left eye showed a relative afferent pupillary defect. Fundus examination revealed left eye disc edema and right eye normal fundus, which was captured on fundus photography [Figure 1]. Magnetic resonance imaging (MRI) brain was done [Figure 2] and was suggestive of demyelination at T7-T9 level with left ON. Cerebrospinal fluid oligoclonal bands were positive. NMO antibodies were positive.
|Figure 2: Magnetic resonance imaging brain suggestive of left optic neuritis|
Click here to view
On the basis of clinical examination and investigations, a diagnosis of acute transverse myelitis with left on also known as Devic's disease was made.
The patient was immediately treated with high-dose intravenous steroids iv methylprednisolone 1 gm/day for 3 days followed by tapering dose of oral steroids 1 mg/kg body weight over 3 weeks. The patient's visual function recovered fully at 1 month follow-up.
| Discussion|| |
Devic's disease is an autoimmune inflammatory disorder wherein patient's own immune system-antibodies called NMO-immunoglobulin G (IgG) attack protein of nervous system cells called aquaporins in the cell membrane of astrocytes. This produces ON and myelitis. Symptoms include loss of vision or decreased visual acuity, visual field defect, loss of color vision, loss of spinal cord function leading to muscle weakness, spastic paraparesis or tetraparesis, reduced sensation, and loss of bladder/bowel control. Optic nerve involvement can be unilateral or bilateral and is a painful condition worsened by movement with decreased visual acuity in the affected eye and is the most common initial symptom. Half of the patients lose functional vision within 5 years of onset. Myelitis involvement classically involves more than three spinal cord segments.
Currently, three supposed causes of the disease are:
- A first subset of patients is anti-AQP4+. In them, the cause of the NMO (understood as the syndrome) is an autoimmune aquaporin-4 channelopathy, due to these specific autoantibodies. In these cases, astrocytes are the victims of the autoimmune attack
- A second subset of patients is anti-MOG+. In them, the cause is an anti-MOG associated encephalomyelitis
- In a third subset of patients, NMO is due to the presence of anti-neurofascin auto-antibodies which damage the Ranvier nodes of the neurons. These antibodies are more related to the peripheral nervous demyelination, but they were also found in NMO.
The etiology is actually unknown. It is said to have been associated with other autoimmune diseases such as collagen vascular disease, autoantibody syndrome, infections such as varicella zoster, Epstein–Barr virus, human immunodeficiency virus, and exposure to clioquinol and antituberculosis drugs.
The Mayo Clinic proposed a revised set of criteria for the diagnosis of Devic's disease in 2006. Those new guidelines require two absolute criteria plus at least two of three supportive criteria.
Absolute criteria: (1) optic neuritis, (2) acute myelitis.
Supportive criteria: (1) Brain MRI not meeting criteria for MS at disease onset. (2) Spinal cord MRI with continuous T2-weighted signal abnormality extending over three or more vertebral segments, indicating a relatively large lesion in the spinal cord. (3) NMO-IgG seropositive status (The NMO-IgG test checks the existence of antibodies against the aquaporin 4 antigen.)
Currently, there is no cure for Devic's disease, but symptoms can be treated. Some patients recover, but many are left with impairment of vision and limbs, which can be severe.
| Conclusion|| |
Devic's disease is a rare encounter which when diagnosed correctly and intervened timely can have a good prognosis. This case also emphasizes the need for good history taking and thorough examination including general and systemic.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]