Abstract
Neuromyelitis optica spectrum disorder (NMOSD) includes conditions with autoimmune genesis, which are manifested by attacks of optic neuritis (ON) and transverse myelitis (TM), and also express aquaporin 4 (NMO-IgG) or myelin oligo-endocytic glycoprotein (MOGAb) antibodies. In rare cases, the disease may also have a clinical presentation with only TM, without ON or with ON, without TM. These conditions are also included in the spectrum. We present a case of a 50-year-old patient with complaints of blurry vision in the left eye, neck pain, and numbness of the hands a few months prior to hospitalization. After a consultation with an ophthalmologist, magnetic resonance imaging (MRI) of the brain was performed, and findings showed left ON. Serological findings showed antibodies for aquaporin 4 with a ratio of 1:100, which is a pathognomic marker for NMOSD. Upon admission to the clinic, the complaints persisted. The neurological status revealed decreased vision of the left eye, pain in the Valleix points in the cervical region, and hyperesthesia including dermatomes C5-C8, more prominent for the right arm. Routine blood tests showed leukocytosis, neutrophilia, and lymphocytosis. Additional virology studies were performed to rule out neuroborreliosis, neurosyphilis, and human immunodeficiency virus (HIV). MRI of the cervical spine revealed dural sac compression from degenerative spinal disease. Electromyography (EMG) showed radiculopathy at the C5-C8 levels and neuropathy of n. ulnaris sinistra and n. medianus dextra. Consultation with an ophthalmologist revealed a decrease in the left eye visus. The treatment plan consisted of methylprednisolone 1000 mg for five days and gastroprotection with famotidine 40 mh p.o. Further consultations with the ophthalmologist were done in the following days to monitor the visual status of the patient. After completion of the diagnostic and treatment plan, slight recovery of the visus and reversal of the pain symptomatology was reported. Based on the history, clinical findings, ophthalmologic examination, brain and neck imaging (MRI), and laboratory results (NMO-IgG ratio 1:100 ), NMOSD with left retrobulbar neuritis (Devic's disease) was concluded as the final diagnosis. Multiple sclerosis (MS; retrobulbar neuritis) first attack, systemic connective tissue disease (systemic lupus erythematosus, Sjögren's syndrome, and antiphospholipid syndrome), and neuro-infection were discussed. Clinical suspicion of intracranial tumors was ruled out. Corticosteroids and gastroprotectors were prescribed for oral use under the supervision of a neurologist. Regular check-ups were performed. The reported case of NMOSD with unilateral optic nerve involvement is a rare condition in neurological practice. It requires an individualized approach to diagnose and apply treatment. Patient follow-up is a key starting point toward gathering more information about autoimmune processes in the central nervous system (CNS) and toward finding faster, more reliable, and cheaper clinical and instrumental methods of diagnosis, which in turn may lead to more timely initiation of treatment, proportionally leading to improvement in the patient's quality of life.