Neuromyelitis Optica (Devic`s Disease)

Karuna Thapar; Gaurav Dhawan

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Karuna Thapar, Gaurav Dhawan.

Department of Pediatrics, Government Medical College, Amritsar.

ADDRESS FOR CORRESPONDENCE
Dr Karuna Thapar, Department of Pediatrics, Government Medical College, Amritsar, India.
Email: kthapar2000@yahoo.com

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Introduction

Neuromyelitis optica (NMO) (Devic's disease) is an uncommon clinical syndrome in pediatric age group comprising of unilateral/ bilateral optic neuritis and transverse myelitis within 8 weeks (1-3). Although once believed to be a variant of multiple sclerosis, diagnostic criteria have recently been proposed for neuromyelitis optica (4), making it a clinically distinct syndrome. Here we report a case because of its rarity.

Case Report

An 8-year old female child presented with cough, abdominal pain, backache and dysuria for 5-6 days, pain in legs, inability to stand and walk, urinary retention and dribbling of urine for 3 days. There is history of fever of short duration 1-month back. Development of child was normal and child was immunized. There was no history of any trauma, recent vaccination, diarrhea and back pain. On examination, vital parameters were stable, general physical examination was normal, with no organomegaly. Neurological findings included grade 1 power and hypotonia of lower limbs. Superficial and deep reflexes of lower limbs were absent. Sensory loss was present up to the level of umbilicus. There was no sensory and motor loss in upper limbs; cranial nerves and spine examination were normal. There were no signs of meningeal irritation, and raised intracranial tension so child was then diagnosed as a case of transverse myelitis. Child had a sudden loss of vision on 7th day. This led to the possibility of Devic's disease. Fundus examination was normal. Routine investigations including complete blood counts, ESR and chest X-ray were normal and Mantoux test was negative. CSF examination showed 4 cells/cu mm, predominant lymphocytes, proteins 34 mg%, globulin negative, sugar 45.5 mg%, and chloride 125.2 mg% with no micro organism. MRI spine showed hyper intense regional areas at dorsal cord D3-D5 and D7-D9 level.

Child was put on intravenous dexamethasone instead of methylprednisolone (due to cost factor), within 4 hours of loss of vision. Vision showed improvement in 24 hours and full vision was restored within 72 hours. Sensory loss of lower limbs progressively decreased and child was able to stand. On 15th day of treatment with steroids, child was able to walk without support. Urinary retention improved slowly. There was no residual neurological deficit at three weeks follow-up.

Discussion

Eugene Devic and Fernand Gault initially described Neuromyelitis optica in the late 19th century (5). Neuromyelitis optica is a clinical syndrome characterized by: (a) an acute or subacute onset, (b) involvement of both optic nerves and spinal cord, and (c) an interval between spinal cord involvement and optic nerve not exceeding one month (6).

Diagnostic criteria for NMO have been proposed by Wingerchuck et al (4). Diagnosis requires all absolute criteria and one major supportive criterion OR two minor supportive criteria

Absolute Criteria:
1. Optic neuritis
2. Acute myelitis and
3. No evidence of clinical disease outside the optic nerve or spinal cord

Major Supportive Criteria:
1. Negative brain MRI at onset
2. Spinal cord MRI with signal abnormality extending over 3 vertebral segments
3. CSF pleocytosis of >50 WBC/mm3 or >5neutrophils/ mm3

Minor Supportive Criteria:
1. Bilateral optic neuritis
2. Severe optic neuritis
3. Severe, fixed, attack-related weakness in one or more limbs (MRC grade 2 or less)

Our patient presented with acute myelitis, optic neuritis, with no evidence of clinical disease outside the spinal cord or optic nerve, had spinal cord MRI with signal abnormality extending from D5-7 and D9-11, with attack related weakness in both the limbs. Three absolute criteria, one major supportive criteria and three minor supportive criteria were present. Thus our patient's signs and symptoms are consistent with the diagnosis of NMO according to Wingerchuck et al (4).

Specific etiological causes of neuromyelitis optica have not been identified, although connective tissue disorders, tuberculosis, and acute disseminated encephalomyelitis have been associated with it (7). Neuromyelitis optica may follow either a monophasic or relapsing course (4). In monophasic NMO, patients experience either unilateral or bilateral optic neuritis and a single episode of myelitis, typically but not always, within a very short time of one another, but do not have further attacks. In contrast, patients with a relapsing course continue to have discrete exacerbations of optic neuritis and/or myelitis after they meet NMO diagnostic criteria. Our patient had experienced a single episode (follow up for 9 months) of myelitis followed by bilateral optic neuritis thus showing a monophasic course. Near complete visual loss, color blindness, bilateral hemianopia, generalized field constriction with central or paracentral scotomas, uveitis, anterior disc edema and Horner's syndrome are some of the visual defects reported (1,8). Our patient had complete loss of vision in both the eyes. Pathological changes reported in clinically typical Devic's disease include white matter plaques, demyelination with vascular infiltrates, necrosis or cystic degeneration(1,9).

Mainstay of therapy is treatment of acute attacks, prevention of medical complications, and rehabilitation. Glucocorticoids are typically used to treat cases acutely and may be beneficial (4,10). Treatment with intravenous methyl prednisolone (IVMP) followed by oral prednisone has been tried in cases of isolated optic neuritis (11) and for acute transverse myelitis. Isolated cases have shown excellent recovery with steroids (12). Prevention of medical complications is critically important. Acute cervical cord attacks may cause respiratory failure. Medical measures to prevent thromboembolic complications, aspiration pneumonia and urinary tract infections are also required.

Compliance with Ethical Standards

Funding None

Conflict of Interest None

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