|Year : 2015 | Volume
| Issue : 1 | Page : 75-76
Neuromyelitis optica with Hashimoto's thyroiditis: A new syndrome or just coincidence
Abhishek Singhai, Abhishek Agarwal, Ashwin Porwal, Rajesh Kumar Jha
Department of Medicine, Sri Aurobindo Medical College, Indore, Madhya Pradesh, India
|Date of Web Publication||14-Jan-2015|
Dr. Abhishek Singhai
Department of Medicine, Sri Aurobindo Medical College, Indore, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
Neuromyelitis optica (NMO) is an uncommon disease syndrome of the central nervous system that affects the optic nerves and spinal cord. NMO with endocrinopathies has been described as being unique to black Antillean and Afro-Brazilian women. We describe one case of NMO with hashimoto's thyroiditis in a young female, probably first case report in India.
Keywords: Endocrinopathies text, hypothyroidism, neuromyelitis optica
|How to cite this article:|
Singhai A, Agarwal A, Porwal A, Jha RK. Neuromyelitis optica with Hashimoto's thyroiditis: A new syndrome or just coincidence. CHRISMED J Health Res 2015;2:75-6
|How to cite this URL:|
Singhai A, Agarwal A, Porwal A, Jha RK. Neuromyelitis optica with Hashimoto's thyroiditis: A new syndrome or just coincidence. CHRISMED J Health Res [serial online] 2015 [cited 2019 Nov 14];2:75-6. Available from: http://www.cjhr.org/text.asp?2015/2/1/75/149354
| Introduction|| |
Neuromyelitis optica (NMO) is an uncommon disease syndrome of the central nervous system that affects the optic nerves and spinal cord. Individuals with NMO develop optic neuritis, which causes pain in the eye and vision loss, and transverse myelitis, which causes weakness, numbness, and sometimes paralysis of the arms and legs, along with sensory disturbances and loss of bladder and bowel control. In 1997, a distinct syndrome with clinical presentation of optic neuritis and acute myelitis, called recurrent optic neuromyelitis with endocrinopathies, was first described in eight patients from French Antilles, and then in six patients from Brazil and one from China. ,, We describe one case of NMO with hashimoto's thyroiditis in young females, probably first case report in India.
| Case Report|| |
A 25-year-old female had developed sudden diminution of vision from left eye. She consulted to ophthalmologist for that and diagnosed to have optic neuritis. She was started on oral prednisolone and continued for 6 months with little improvement in vision. Two years later, she developed acute spastic quadriparesis secondary to demyelination in cervico-dorsal spinal cord involving more than three spinal tracts. Same time she also developed optic neuritis of right eye. Investigations showed: Hb 11.1 g/dL, White blood cells count 5300 mm 3 , erythrocyte sedimentation rate 40 mm/h, liver function test normal, renal function test normal, fasting blood sugar 80 mg/dL, Antinuclear antibodies negative, anti NMO antibody positive. Patient was investigated for associated endocrinopathies for academic purpose. Thyroid function showed Free T 3 2.2 pmol/L (normal 3.5-7.5), Free T 4 8 pmol/L (10-25), thyroid-stimulating hormone (TSH) >100 mU/L (0.3-4.5). Anti-thyroid peroxidase antibody was positive in titre of >100 IU/mL. Fasting blood sugar, serum cortisol, serum follicle stimulating hormone, serum luteinizing hormone and serum prolactin levels were normal. Brain magnetic resonance imaging (MRI) was normal [Figure 1] while MRI spine showed diffuse Short tau inversion recovery (STIR) and T2 hyperintense signal changes suggesting cord edema and swelling of entire cervical and dorsal cord. The lesions were seen predominantly in central, posterior and right lateral parenchyma of the cord [Figure 2]. Based on these investigations, she was diagnosed to have NMO with Hashimoto's thyroiditis. She was started on oral prednisolone, azathioprine, L-thyroxine and active physiotherapy. She had little improvement with treatment. On discharge she had a vision in left eye 6/9, vision in right eye 6/60, power in upper limb 5/5 while power in lower limbs 3/5. After 2 months of discharge her neurological status remained same and TSH decreased to 4.4 mU/L.
|Figure 2: MRI spine showed diffuse STIR and T2 hyperintense signal changes suggesting cord edema.|
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| Discussion|| |
Our patient with two bouts of optic neuritis, acute transverse myelitis and absence of MRI lesions in the cerebral white matter, met the diagnostic criteria for NMO. 
Neuromyelitis optica with endocrinopathies has been described as being unique to black Antillean and Afro-Brazilian women. This syndrome with diabetes mellitus and amenorrhea has been reported in the literature among two Caucasians from Brazil.  The spectrum of endocrinopathies in NMO patients seems to be related to hypothalamus-pituitary dysfunction as gadolinium enhancement was observed in the pituitary of some patients from Martinique. Also, involvement of hypothalamic structures has been described in two patients with NMO, one of which has Haitian heritage.  Our patient had autoimmune hypothyroidism in association with NMO while workup for other endocrinopathies was negative. In the disease process of NMO, for reasons that aren't yet clear, immune system cells and antibodies attack and destroy myelin cells in the optic nerves and the spinal cord. After the initial attack, NMO follows an unpredictable course. Most individuals with the syndrome experience clusters of attacks months or years apart, followed by partial recovery during periods of remission. This relapsing form of NMO primarily affects women. The female to male ratio is >4:1. There is no cure for NMO, but there are therapies to treat an attack, to reduce symptoms, and to prevent relapses. Corticosteroid (methylprednisolone) is used to stop the attack, and an immunosuppressive drug (azathioprine) for prevention of subsequent attacks.
| Conclusion|| |
Although more studies are needed to define this disorder as a new syndrome, this case additionally supports the observation that endocrinopathies should be suspected in NMO.
| References|| |
Vernant JC, Cabre P, Smadja D, Merle H, Caubarrère I, Mikol J, et al
. Recurrent optic neuromyelitis with endocrinopathies: A new syndrome. Neurology 1997;48:58-64.
Papais-Alvarenga RM, Miranda-Santos CM, Puccioni-Sohler M, de Almeida AM, Oliveira S, Basilio De Oliveira CA, et al
. Optic neuromyelitis syndrome in Brazilian patients. J Neurol Neurosurg Psychiatry 2002;73:429-35.
Hui AC, Wong RS, Ma R, Kay R. Recurrent optic neuromyelitis with multiple endocrinopathies and autoimmune disorders. J Neurol 2002;249:784-5.
Wingerchuk DM, Hogancamp WF, O'Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology 1999;53:1107-14.
Poppe AY, Lapierre Y, Melançon D, Lowden D, Wardell L, Fullerton LM, et al
. Neuromyelitis optica with hypothalamic involvement. Mult Scler 2005;11:617-21.
[Figure 1], [Figure 2]