Case Report
Sarcoidosis associated with neuromyelitis optica

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Abstract

Neuromyelitis optica (NMO) is an autoimmune disorder diagnosed by an elongated spinal cord lesion associated with unilateral or bilateral optic neuritis and anti-aquaporin 4 (AQP4) antibodies in the serum. It is triggered by or associated with several autoimmune diseases, but not with sarcoidosis. It responds to immunomodulators better than to steroid treatment. Sarcoidosis is an autoimmune disorder which manifests as non-caseating granulomas, usually in the lung parenchyma, but also in other tissues, including the brain. The involvement of the central nervous system in neurosarcoidosis differs considerably from that in neuromyelitis optica and the association of these two diseases concurrently in the same patient is unusual.

Introduction

Neuromyelitis optica (NMO) is an autoimmune disorder diagnosed by an elongated spinal cord lesion, unilateral or bilateral optic neuritis, and is sometimes associated with anti-aquaporin 4 (AQP4) antibodies in the serum. It has been reported to occur in patients with other autoimmune diseases that affect the immune system, but not with sarcoidosis. It responds to immunomodulators better than to steroid treatment. Sarcoidosis is a disorder, possibly autoimmune, which manifests as non-caseating granulomas, usually in the lung parenchyma, but also in other tissues, including the brain. The involvement of the central nervous system (CNS) in neurosarcoidosis differs considerably from that in neuromyelitis optica and the association of these two diseases concurrently in the same patient is unusual.

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Case report

We describe a 50-year-old woman who developed type 1 diabetes mellitus at the age of 19 years. She also suffered from secondary amenorrhea and infertility, and was found to have Hashimoto’s thyroiditis. At the age of 45 years she presented with the clinical picture of an acute cervical spinal cord lesion with acute, progressive weakness in the upper extremities, hyperreflexia in the lower extremities, bilateral Babinski signs and L’hermites sign. Cranial nerves and fundi were normal. Blood and

Discussion

NMO is an autoimmune inflammatory demyelinating disease of the CNS which predominantly affects the spinal cord and optic nerves. It differs from multiple sclerosis in several ways: patients with NMO experience more severe attacks; the optic neuritis may result in visual loss; patients have centrally located spinal cord lesions across more than three vertebral bodies; oligoclonal bands are not usually present in the CSF; and cerebral lesions, when present, are more extensive, usually contiguous

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    Although the lesion of anti-AQP4 antibody-positive NMOSD was previously considered restricted to the CNS, recent studies have suggested that tissues beyond the CNS, including the skeletal muscles [9–12], intestines [13–15], and ears [16], may be involved. Regarding the pulmonary involvement in patients with NMOSD, there are only a few case reports showing the development of lung disorders, including organizing pneumonia (OP), pulmonary sarcoidosis, and unclassifiable interstitial pneumonia [17–21]. However, the pathological relevance of complicated pulmonary disorders in anti-AQP4 antibody-positive NMOSD remains unclear.

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    In the field of radiological features, NMOSD may present with simultaneous bilateral optic nerve involvement and a long optic nerve lesion, posterior optic nerve involvement also including the chiasm, periaqueductal lesions, periependymal lesions surrounding the lateral ventricles (long lesions that parallel and involve the long axis of the corpus callosum), lesions on area postrema, LETM, spinal cord lesions involving only the gray matter, medullary lesions contiguous to spinal cord lesions, and/or cloud-like gadolinium enhancement. There are several reports proving the coexistence between NMOSD and other autoimmune diseases, mainly rheumatoid arthritis [1], sarcoidosis [2,3], myasthenia gravis, Sjogren's syndrome and SLE [4]. However, it is not possible yet to estimate its prevalence and incidence in this population [5].

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