Pdf longitudinally extensive transverse myelitis with. Parainfectious longitudinally extensive transverse. Patients diagnosed with multiple sclerosis during the first myelitis episode were excluded. Case a 50 year old caucasian man presented with left arm paraesthesia, sensory and gait disturbance.
Brain mri revealed leptomeningeal enhancement with no basal ganglia or thalami involvement. Hemorrhagic longitudinally extensive transverse myelitis. Pdf hemorrhagic longitudinally extensive transverse myelitis. Longitudinal extensive transverse myelitis letm is defined as a spinal cord lesion that extends over three or more vertebrae, as seen on mri of the spine. Transverse myelitis associate diseases the johns hopkins. Refractory longitudinally extensive transverse myelitis responsive to cyclophosphamide volume 44 issue 6 laura j. Longitudinally extensive transverse myelitis following. The differential diagnosis of longitudinally extensive. Spinal mri showed a swollen longitudinally extensive cord lesion 6 spinal segments with peripheral enhancement. Modifications of longitudinally extensive transverse. Longitudinally extensive transverse myelitis letm may be associated with viral triggers, including both infections and vaccinations.
T2 signal abnormality extending craniocaudally over at least three. It has been mainly reported with lung cancers and lymphoproliferative malignancy. Magnetic resonance imaging is the diagnostic modality of choice for longitudinally extensive transverse myelitis,6, 7 allowing compressive lesions such as hematomas or intramedullary tumours to be ruled out. Longitudinally extensive transverse myelitis letm is a neurological condition characterised by a contiguous inflammatory lesion of the spinal cord.
Neoplastic and autoimmune causes were ruled out, including negative neuromyelitis optica igg nmoigg. Longitudinally extensive transverse myelitis letm is defined as spinal cord lesions affecting at least 3 spinal cord segments and resulting in hyperintensities on sagittal t2weighted magnetic resonance imaging mri sequences. Pdf diagnosis of longitudinally extensive transverse. Oxford covers the southern half of the nationally funded uk nmo service and herein we describe the characteristics and diagnoses of patients referred to the. Letm, a propagate spinal cord sc inflammation causing hyperintensity on t2weighted magnetic resonance imaging mri over 3 or more vertebral segments, is widely regarded a spectrum of neuromyelitis optica nmosd. Chikungunya viral chikv fever is often a selflimiting febrile illness associated with severe debilitating arthralgia. Letm in systemic malignancy can be caused by either intramedullary metastases or paraneoplastic syndrome.
The differential diagnosis of longitudinally extensive transverse. Refractory longitudinally extensive transverse myelitis. Differential diagnosis and prognosis for longitudinally. A case of longitudinally extensive transverse myelitis. Initially, letm and neuromyelitis optica nmo were thought to be synonymous due to their frequent association. It is typically characterized by longitudinally extensive transverse myeli. Neuromyelitis optica spectrum disorder nmosd is a rare relapsing autoimmune disorder that preferentially causes inflammation in the optic nerve and spinal cord. In some cases, however, transverse myelitis can present in the form of a longitudinally extensive transverse myelitis letm, which is defined as myelitis affecting three or more contiguous segments of the spinal the cord 5.
Neuromyelitis optica igg predicts relapse after longitudinally extensive transverse myelitis. An approach to the diagnosis of acute transverse myelitis. Some patients are unresponsive to conventional immune therapy. Systemic lupus erythematosus presented as extensive longitudinal myelitis. Longitudinally extensive transverse myelitis letm is a frequently devastating clinical syndrome which has come into focus for its association with neuromyelitis optica nmo. Objective to describe a case of longitudinally extensive transverse myelitis associated with vaccination with a nasal attenuated novel influenza ah1n1 vaccine. Importance aquaporin 4 antibody aqp4abnegative patients with longitudinally extensive transverse myelitis letm behave differently from. Longitudinally extensive transverse myelitis june 14, 2014 regional ms summit pavle repovic, md, phd ms center at swedish. What is the link between transverse myelitis and multiple.
Longitudinally extensive transverse myelitis with aqp4 antibodies revealing ovarian teratoma. Evidence of increased signal on t 2weighted images of the spinal cord extending beyond four vertebrae is considered diagnostic of this condition. Although commonly associated with neuromyelitis optica, there are many other causes that should be considered. Longitudinally extensive transverse myelitis as presenting. Recent studies have indicated that the brainstem is an important site of attack in nmo. Longitudinally extensive transverse myelitis refers to inflammation of the spinal cord causing t2 hyperintensity on spinal mri that extends over three or more vertebral segments.
We present a case of a middleaged man with a 1week history of. We describe a patient presenting acutely with a corticosteroidresponsive longitudinally extensive transverse myelitis and granulomatous lung lesions with a previous history of. Longitudinal extensive transverse myelitis letm is defined as an intramedullary spinal cord. Treatment of longitudinally extensive transverse myelitis hinges on distinguishing inflammatory and noninflammatory aetiologies and identifying patients who are at high risk of a recurrent course. In a prospective study, the risk of developing recurrent myelitis or new onset optic neuritis in patients with an isolated longitudinally extensive transverse myelitis was more than 50% among those. These include other inflammatory monophasic myelitises, infection, and. Tm is a clinical syndrome in which an immunemediated process causes neural injury to the spinal cord, resulting in varying degrees of weakness, sensory alterations, and autonomic dysfunction. We report a patient who developed longitudinally extensive transverse myelitis after c. Pdf longitudinally extensive transverse myelitis letm is a neurological condition characterised by a contiguous inflammatory lesion of the. The frequency of brainstem and letm lesions, changes over time of letm. Aciclovir was suspended after serology test results were obtained. Differentiating neuromyelitis optica from other causes of. Pdf download for the differential diagnosis of longitudinally extensive transverse. Longitudinally extensive transverse myelitis letm is defined as a hyperintense spinal cord lesion extending over three or more vertebral levels on sagittal t2weighted t2w spinal magnetic resonance imaging mri.
Longitudinally extensive transverse myelitis in four children. Mri lesions are often centrally located with high t2 signal intensity involving gray and neighboring white matter. Letm refers to spinal cord lesions extending at least three vertebral segments. Neuromyelitis optica nmo, characterised by longitudinally extensive transverse myelitis letm, was previously thought to be a variant of multiple sclerosis. We describe a patient presenting acutely with a corticosteroidresponsive longitudinally extensive transverse myelitis and granulomatous lung lesions with a previous history of recurrent generalised lymphadenopathy, pyogenic. Retrospective cohort study of 192 patients initially presenting with tm of unknown etiology. Recent advances in the diagnosis of nmo have led to very sensitive and specific tests and advances in therapy for this disorder. Spinal cord mri showed a longitudinally extensive transverse myelitis figures 1 and 2. Longitudinally extensive transverse myelitis letm partial transverse myelitis means that the inflammation across the spinal cord is patchy or not fully complete, so symptoms below the spinal segment tend to be milder and asymmetric. Longitudinally extensive transverse myelitis in systemic lupus erythematosus. The differential diagnosis of longitudinally the authors.
Longitudinally extensive transverse myelitis associated. Transverse myelitis transverse myelitis tm is a rare inflammatory disease causing injury to the spinal cord with varying degrees of weakness, sensory alterations, and autonomic dysfunction the part of the nervous system that controls involuntary activity, such as the heart, breathing, the digestive system, and reflexes. Transverse myelitis tm is the prototype inflammatory myelopathy. Longitudinal extensive transverse myelitisits not all. However, differential diagnosis reveals a wide range of aetiologies. To report on differential diagnosis and prognosis for letm observed in a group of patients in buenos aires. It is mostly imaged with mri, which generally shows a long segment 34 segments or more of t2 increased signal occupying greater than twothirds of the crosssectional area of the cord, with. However, longitudinally extensive transverse myelitis commonly has a parainfectious cause, triggered by a wide range of microorganisms including viruses and bacteria. Longitudinally extensive transverse myelitis letm is an important component of the clinical diagnosis of nmo. Longitudinally extensive transverse myelitis letm has classically been grouped with the full or limited neuromyelitis optica spectrum disorders nmosd. Longitudinally extensive transverse myelitis letm is a neurological condition characterised by a contiguous inflammatory lesion of the spinal. Abstract longitudinal extensive transverse myelitis letm is defined as a spinal cord lesion that extends over three or more vertebrae. We present a case of a healthy immunocompetent 33yearold.
Having diagnosed longitudinally extensive transverse myelitis 3 associated with vzv, we started treatment with intravenous iv aciclovir and corticosteroids, which achieved good outcomes. Longitudinally extensive transverse myelitis springerlink. Clinical characteristics and mri of the spinal cord classify transverse myelitis tm into acute partial or longitudinally extensive variants. Longitudinally extensive transverse myelitis refers to florid and widespread. Longitudinally extensive transverse myelitis with and without. Longitudinally extensive transverse myelitis letm is characterized by contiguous immunemediated inflammatory lesion of spinal cord extending to three or more vertebral segments. Keywords demyelinative myelitis, devics neuromyelitis optica, infectious myelitis, inflammatory myopathy, letm, necrotizing mye. Severe longitudinally extensive transverse myelitis letm can cause quadriplegia, marked sensory dysfunction, and respiratory failure. It is characterized by longitudinally extensive myelitis myelitis which is 3 vertebral segments in length or greater, which can leave patients quite debilitated at presentation, and unilateral or bilateral optic neuritis. Longitudinally extensive transverse myelitis letm is an unusual manifestation of systemic malignancy. Nmo is a rare relapsing autoimmune disorder that preferentially causes inflammation in the optic nerve and spinal cord.
Recent advances in the diagnosis of nmo have led to very sensitive and specific tests and advances in. Evaluation and management of longitudinally extensive transverse. Distinctive clinical and neuroimaging characteristics of longitudinally extensive transverse myelitis associated with aquaporin4 autoantibodies. This study sought to identify factors that increased the risk of recurrence after an initial transverse myelitis tm presentation. Longitudinally extensive transverse myelitis after. Acute transverse myelitis atm is an inflammatory condition affecting both halves of the spinal cord and associated with rapidly progressive motor, sensory, and autonomic dysfunction. Pdf diagnosis of longitudinally extensive transverse myelitis. Longitudinally extensive spinal cord lesions lescl, also known as longitudinally extensive transverse myelitis letm, represent extensive involvement of the spinal cord, with abnormal t2 signal traversing at least three vertebral body segments in length. A retrospective analysis of sixtyfour patients at tertiary care center of northwest india, clinical neurology and neurosurgery, 148.
Transverse myelitis symptoms, diagnosis and treatment. Differential diagnosis of longitudinally extensive. Longitudinally extensive transverse myelitis refers to florid and widespread inflammation of the spinal cord causing t2 hyperintensity on spinal magnetic resonance imaging that is seen to extend over three or more vertebral segments. Hereby, we present a case of letm that occurred in a patient with sle. Differential diagnosis and prognosis for longitudinally extensive. Longitudinally extensive transverse myelitis with and. We present a case of a healthy immunocompetent 33yearold woman who developed a hemorrhagic letm 2 weeks after seasonal influenza vaccination. Longitudinally extensive transverse myelitis refers to florid and widespread inflammation of the spinal cord causing t2 hyperintensity on spinal magnetic. Neurological complications associated with chikv, although rare, have been reported in literature. Transverse myelitis may be a manifestation of autoimmune connective tissue diseases and nmo is now recognised to be a humorally mediated autoimmune disease. Longitudinally extensive transverse myelitis mayo clinic. Neuromyelitis optica nmo includes transverse myelitis, optic neuritis and brain lesions. To report the differential diagnosis of longitudinally extensive transverse myelitis letm patients.
Background transverse myelitis has been reported in association with vaccination, including influenza vaccination. We report a case of longitudinally extensive transverse myelitis mimicking nmo that was pathologically confirmed to be sarcoidosis. Letm is often associated with the autoimmune central nervous system disease neuromyelitis optica nmo and rarely with multiple sclerosis. Confirmed by lp demonstrating increased wbc count and absence of infection, with or without spinal cord mri revealing a cord lesion that enhances after gadolinium administration. Longitudinally extensive spinal cord lesion radiology. Longitudinally extensive transverse myelitis is a hallmark of nmonmosd. Transverse myelitis requires careful investigation, as although many causes respond acutely to immunomodulation, the longer term management depends upon its precise cause. Hemorrhagic letm has not to our knowledge been reported after influenza.
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