Teaching Case

A 53 year old male patient with sudden onset of myelopathy; amaurosis and motor worsening 5 months later.

After an upper respiratory tract infection, he had an acute paraplegia, urinary retention and constipation. Neurological examination: hypotonic paraplegia, absent deep tendon reflexes in the lower limbs, bilateral Babinski and bilateral sensory loss below D4. Five months later occured left amaurosis and bilateral two weeks after. Admitted four weeks later, he had bilateral amaurosis, bilateral mydriasis, non-reacting pupils, right internuclear ophthalmoplegia, hypotonic tetraplegia (severe paresis in lower limbs, subsequent mild improvement in upper limbs) with absent deep tendon reflexes in the lower limbs, bilateral Babinski and bilateral sensory loss below C5.
All laboratoy blood studies were normal. First Lumbar puncture revealed an elevated CSF proteins; no oligoclonal bands in CSF or other anomalies were found. Subsequent CSF results were normal. Spine MR showed cord enlargment and intramedullary high signal on T2WI extending from C5 to D3 (more at C6 and C7). Initial Brain MR did not show intracranial lesions. Brain and Spine MR were repeated 7 months later. Then, Brain MR showed an high signal on periventricular white matter (mainly posteriorly) and a new small and central pons lesion with high signal on long TR sequences and low signal on T1, without enhancement. Spine MR showed extensive intramedullary high signal on T2WI extending from medullary-cord junction to level of C5 ; without enhancement after gadolinium. The other levels of intra-medullary high signal weren't evident.
In this case, clinical history and imaging studies support the diagnosis of Devic's Syndrome (acute transverse myelitis, then optic neuritis and ascending myelitis).

We present a typical case of NMO clinical defined as severe CNS demyelinating syndrome traditionally consisting of an acute-subacute onset of loss of vision in one or often in both eyes, preceded or followed within several days or weeks by a transverse or ascending myelitis. The age of patients is variable but usually affect young adults (rarely older than 50 years) and affects females almost exclusively, while the case presented is an older male. Until recently, the presence of CNS symptoms outside the optic nerves and spinal cord excluded the diagnosis. Earlier and accurate diagnosis is important to an appropriate treatment aproach and prognosis. Wingerchuk et al. recently (2006) have proposed for definitive diagnostic criteria of NMO: optic neuritis, acute myelitis and at least two of three supportive criteria (contiguous spinal cord MRI lesion extending over ≥ 3 vertebral bodies; brain MRI not meeting diagnostic criteria for MS; NMO-IgG seropositive status). A serum autoantibody marker for NMO (NMO-IgG) has been recently developed. This autoantibody is reported to have a sensitivity of 73% and a specificity of 91% for NMO. It may be helpful for distinguishing this form of IIDD from MS and may predict relapse and conversion to NMO in patients presenting with a single attack of longitudinally extensive myelitis. In this case we couldn´t assess the presence of NMO-Ig.
Transverse myelitis (TM) has usually a thoracic localization. Inflammatory and demyielinating lesions of the spine are usually considered under the clinical syndrome acute TM, characterized by an acutely developing ascending or static spinal cord lesion affecting both halves of the cord in the absence of any known neurologic disease or cord compression. Spine MR may directly show an abnormal intramedullary signal, i.e, low intensity on T1 and high intensity on T2, the cord may be of normal caliber or slighly expanded, occasionally suggesting a neoplasm. The abnormal signal may extend above the level of clinical deficit. After gadolinium administration, the abnormal areas may show enhancement. TM may be associated with infections, vaccination, disorders of the immune system such as systemic lupus and MS or paraneoplastic syndromes. Patients Follow-up MRI may demonstrate resolution of the abnormal signal and return of the cord to a normal caliber or cord athrophy. Although all the inflammatory and demyielinating diseases can produce a clinical picture of TM, they are by no means the exclusive causes of TM; in particular venous ischemia secondary to radicullomedullary fistulas, spinal cord infarction due to occlusion of the anterior spinal artery, infectious myelitides should be excluded before the diagnosis of idiopathic TM is made. While the pathology of NMO appears distinct from classical MS, individuals surviving the acute episode may subsequently develop a disease course typical of MS, suggesting the underlying processes are similar or identical. Recent advances in the clinical, neuroimaging, laboratory and pathological hallmarks ( such as a low frequency of CSF oligoclonal bands, unusually extensive spinal cord lesions and a high frequency of organ-specific autoantibodies) have defined NMO as a distinct demyelinating disease of the CNS.