neuromyelitis optica ( nmo ) or devic 's disease is a rare inflammatory , demyelinating disease of the central nervous system ( cns ) that predominantly targets the optic nerves and spinal cord 1 . the disease was first described in 1870 by albutt and 24 years later , devic described the clinical characteristics of nmo the discovery of a specific nmo immunoglobulin ( nmoigg ) opened a new era in the classification and understanding of the pathogenesis of nmo 3 . nmoigg binds to aquaporin4 , which is the main channel that regulates water homeostasis in the cns . diagnostic criteria for nmo with aquaporin4 antibodies ( aqp4ab ) requires at least one core clinical characteristic , a positive test for aqp4ab using best available detection method ( cellbased assay recommended ) and exclusion of alternative diagnoses 1 . the core clinical characteristics are , for example , optic neuritis , acute myelitis , acute brainstem syndrome , symptomatic narcolepsy , or symptomatic cerebral syndrome with typical nmo brain lesions 1 . neuromyelitis optica must be distinguished from other demyelinating diseases , for example , multiple sclerosis . the presence of aqp4ab differentiates nmo from multiple sclerosis ( ms ) with high specificity 4 . in contrast to typical ms , the clinical events in nmo are usually more severe 5 , 6 . cerebrospinal fluid ( csf ) findings in nmo are also known to differ significantly from those in classical ms . csfrestricted oligoclonal igg bands are absent in most nmo patients . however , pleocytosis is usually mild , and frequently includes neutrophils , eosinophils , activated lymphocytes , and/or plasma cells 6 , 7 . studies carried out in europe , south east and southern asia , the caribbean and cuba suggest that the incidence and prevalence of nmo ranges from 0.050.4 to 0.524.4 per 100,000 , respectively 9 . the disease is mainly sporadic , although a few familial cases have been reported 10 . we describe a case of an unusual and severe course of nmo affecting almost the entire spinal cord and brain . 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 rightsided 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 with progressive tetraplegia and global decline . mri of the spinal cord showed myelitis in the spinal cord segments c2 to th5 ( fig . csf examination revealed polymorphonuclear pleocytosis ( leukocytes 1210  10/l , neutrophils 95% ) and an increased total protein concentration ( 2273 mg / l ) . 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 . ( a ) sagittal t2fse mri of the spinal cord showing high signal changes . ( b ) axial t2flair brain mri showing high signal changes in thalami , internal capsule , and corpus callosum . ( c ) axial t2flair brain mri showing high signal changes in pons , medulla oblongata , cerebellum , and middle cerebellar peduncle . the next day , the patient 's condition worsened ; she became comatose and had 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 . brain mri showed high signal changes in thalami , internal capsule , and corpus callosum ( fig . 1b ) and also similar changes in pons , medulla oblongata , cerebellum , and middle cerebellar peduncle ( fig . treatment with methylprednisolone and immunoglobulin was attempted and as serum aqp4ab was confirmed as positive ( indirect immunofluorescence assay was used , the titer was 19.85 , normal < 10 ) and the patient did not respond to the treatment , the patient was also treated by means of plasmapheresis . histological examination of the cns revealed extensive , sharply limited demyelination and axon defect ( fig . 2a and b ) . the spinal cord was almost entirely affected . only the lumbar area was partly spared . smaller demyelination foci were found in the periventricular area of the hippocampus and in the corpus callosum . demyelination was verified by showing both cd68positive macrophage infiltration and betaapp positivity as signs of axonal damage . ( b ) the axon defect is shown by immunostaining ( brown staining ) of betaamyloid precursor protein ( app ) . this is the first case report of nmo described from finland from the aqp4ab era . there is only one older publication of a finnish nmo patient from the preaqp4antibody era 11 . the patient was referred to hospital with an acuteonset chest pain , which is an unusual first symptom of nmo . demyelination affected almost the entire spinal cord , sparing only partly the lumbar cord , which is unusual at first myelitis . lesions involving the lumbar or sacral spinal cord in addition to the cervical and thoracic portions have been reported only in 11% of patients at first myelitis . previous reports have revealed that 92% of the patients have at least one spinal cord lesion extending over three or more vertebral segments at their first myelitis . it has been reported that seropositive women have more severe clinical attacks than males , as evidenced by high lesion load in the spinal cord and other types of coexisting autoimmunity 6 . brain mri abnormalities are relatively common and may be relatively unique by virtue of localization and configuration 12 , as seen also in our patient . the histopathological findings in the cns , csf , and aqp4ab seropositivity are consistent with neuromyelitis opticatype demyelination , although the disease course of our case was not typical of nmo due to its rapid and severe course . according to hospitalbased observational studies , mortality of nmo ranges from 2.9% to 25% and reported that disease duration at the time of death ranged from 6 months to 23.6 years 6 . in addition , our patient 's histological examination revealed extensive , sharply limited demyelination of the spinal cord and brain in the acute phase of the disease . to the best of our knowledge , the treatment in this particular case was targeted to multiple causes of the symptoms due to the unknown etiology in the beginning of the disease . acute attacks and relapses of nmo are generally treated with intravenous glucocorticoids followed by plasmapheresis for refractory or progressive symptoms 13 , 14 . however , there are no controlled trials evaluating the treatment of nmo , and recommendations are primarily supported by data from observational studies and by the clinical experience of experts . despite several forms of treatment , the patient did not survive .
key clinical messageneuromyelitis optica is a rare inflammatory , demyelinating disease of the central nervous system that predominantly targets the optic nerves and spinal cord . 
 our case represents an unusual and severe course of neuromyelitis optica . despite several forms of treatment 
 , our patient died after a severe and shortterm attack .