Clinical Message Neuromyelitis optica is certainly a rare inflammatory demyelinating disease of the central nervous system that predominantly targets the optic nerves and spinal cord. myelitis. The syndrome was eponymously named Devic’s disease 2. The discovery of a specific NMO immunoglobulin (NMO‐IgG) opened a new era in the classification and understanding of the pathogenesis of NMO 3. NMO‐IgG binds to aquaporin‐4 which is the main channel that regulates water homeostasis in the CNS. Diagnostic criteria for NMO with aquaporin‐4 antibodies (AQP4‐Ab) requires at least one core clinical characteristic a positive test for AQP4‐Ab using best available detection method (cell‐based assay recommended) and exclusion of alternative diagnoses 1. The core clinical characteristics are for example optic neuritis severe myelitis severe brainstem symptoms symptomatic narcolepsy or symptomatic cerebral symptoms with regular NMO human brain lesions 1. Neuromyelitis optica should be MK-2206 2HCl recognized from various other demyelinating diseases for instance multiple sclerosis. The current presence of AQP4‐Ab differentiates NMO from multiple sclerosis (MS) with high specificity 4. As opposed to regular MS the scientific occasions in NMO are often more serious 5 6 Cerebrospinal liquid (CSF) results in NMO may also be recognized to differ considerably from those in traditional MS. CSF‐limited oligoclonal IgG rings are absent generally in most NMO sufferers. Cerebrospinal liquid pleocytosis is certainly a common acquiring (50%) in NMO. Nevertheless pleocytosis is normally mild and sometimes contains neutrophils eosinophils turned on lymphocytes and/or plasma cells 6 7 MK-2206 2HCl AQP4‐Ab may also be detectable in the CSF of all AQP4‐Ab‐seropositive sufferers with NMO 8. The prevalence and incidence of NMO aren’t well known. Studies completed in European countries South East and Southern Asia the Caribbean and Cuba claim that the occurrence and prevalence of NMO runs from 0.05-0.4 to 0.52-4.4 per 100 0 9 respectively. The condition is sporadic MK-2206 2HCl although several familial cases have already been reported 10 mainly. We describe an instance of a unique and severe span of NMO impacting almost the complete spinal-cord and human brain. Clinical Information A 45‐12 months‐old woman was referred to hospital with acute‐onset chest pain. Examination on the day of admission revealed normal results as regards ECG troponin I and computed tomography (CT) of the chest and abdomen. The next day the patient reported headache and neurological examination showed right‐sided hemiparesis and afferent pupillary defect of the left eye suggesting an afferent optic nerve defect. Within a few hours the patient showed a rapid neurological deterioration NT5E with progressive tetraplegia and global decline. Brain CT precerebral and intracranial vessel CT angiography showed no abnormalities. Magnetic resonance imaging (MRI) of the brain was MK-2206 2HCl MK-2206 2HCl also normal. MRI of the spinal cord showed myelitis in the spinal cord segments C2 to Th5 (Fig. ?(Fig.1A).1A). CSF examination revealed polymorphonuclear pleocytosis (leukocytes 1210 × 106/L neutrophils 95%) and an increased total protein concentration (2273 mg/L). Oligoclonal banding was unfavorable. Due to spinal cord MRI and CSF findings infectious transverse myelitis could not be excluded and the patient was treated with dexamethasone acyclovir ceftriaxone ampicillin and levofloxacin. Dexamethasone was given for 5 days according to the treatment protocol of bacterial meningitis. Possible infectious etiology was treated with wide‐range antibiotic therapy. Physique 1 (A) Sagittal T2‐FSE MRI of the spinal cord showing high signal changes. (B) Axial MK-2206 2HCl T2‐FLAIR brain MRI showing high signal changes in thalami internal capsule and corpus callosum. (C) Axial T2‐FLAIR brain MRI showing high signal … The next day the patient’s condition worsened; she became comatose and experienced a respiratory failure that required assisted ventilation; this might be caused by bilateral phrenic nerve involvement as its roots originate from C3 to C5 where the lesion was also seen (Fig. ?(Fig.1A).1A). Brain MRI showed high signal changes in thalami internal capsule and corpus callosum (Fig. ?(Fig.1B)1B) and also similar changes in pons medulla oblongata cerebellum and middle cerebellar peduncle (Fig. ?(Fig.1C).1C). These changes were also seen in the.