Transverse myelitis tm may present with either an lmn or an upper motor neuron umn injury, depending on areas affected on the cord. Clinical signs are due to the involvement of the ascending and descending tracts in the transverse plane of the spinal cord. Recent advances in the diagnosis of nmo have led to very sensitive and specific tests and. Cerebrospinal fluid csf analysis showed pleocytosis of 96 cells, 73% of. Transverse myelitis 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. Causes motor weakness, a sensory impairment below the lesions, and bowel and bladder dysfunction. Acute transverse myelitis is acute inflammation of gray and white matter in one or more adjacent spinal cord segments, usually thoracic. If no other disease is found responsible for the myelitis attack, the risk of recurrence or other cns attack is best predicted by brain mri. This study aimed to describe the conversion rate to multiple sclerosis ms and variables associated with conversion, and to analyze functional outcome and prognostic factors associated with. Longitudinally extensive transverse myelitis is a hallmark of nmonmosd. A fatty tissue called myelin protects these nerve fibers. The widespread use of mri revealed letm more frequency than ever. Transverse myelitis fact sheet national institute of.
Several cases of longitudinal myelitis have been described in the literature, all in the context of sle except for one case with sjogrens syndrome. Lupus related longitudinal myelitis the journal of. 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 clinical inflammatory team with an letm. Longitudinally extensive transverse myelitis letm is a frequently devastating clinical syndrome which has come into focus for its association with neuromyelitis optica nmo. Pdf acute longitudinal myelitis following cryptococcus. Longitudinally extensive transverse myelitis letm is characterized by spinal cord inflammation extending vertically through three or more vertebral segments. Review of the etiological causes and diagnosis of myelitis and its. Acute longitudinal myelitis following cryptococcus laurentii pneumonia in a patient with systemic lupus erythematosus. Perivascular monocytic and lymphocytic infiltration, demyelination, and axonal injury are prominent histopathogic features of tm. Letm is often associated with the autoimmune central nervous system disease neuromyelitis optica nmo and rarely with multiple sclerosis. Request pdf longitudinal extensive transverse myelitisits not all neuromyelitis optica longitudinal extensive transverse myelitis letm is defined as a. Transverse implies that the inflammation extends horizontally across the spinal cord. Longitudinal extensive transverse myelitis letm refers to spinal cord lesions extending at least three vertebral segments.
Clinical and neuroimaging features of sarcoid associated. 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. Anaesthetic considerations in patients with transverse. Acute transverse myelitis atm is an inflammatory demyelinating disorder that affects. Transverse myelitis is an inflammation of your spinal cord. Letm is observed in neuromyelitis optica nmo1,2 and several other inflammatory disorders of the central nervous system cns including paraneoplastic diseases. Pdf diagnosis of longitudinally extensive transverse myelitis. The inflammation impairs messages nerve impulses travelling along the spinal cord. A characteristic analysis of longitudinally extensive. Acute transverse myelitis neurologic disorders msd. Clinical and neuroimaging features of sarcoid associated myelopathy jorge a.
Longitudinal myelitis is a rare form of lupusrelated myelopathy with poor prognosis. 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 variable pattern. Longitudinally extensive spinal cord lesion radiology. The child had leucocytosis with elevated c reactive protein. Transverse myelitis 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. The word myelitis is derived from greek words myelos spinal cord and itis inflammation. Longitudinally extensive atm occurs in the majority. 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.
Lm is a rare variant of acute transverse myelitis and it frequently presented poor responses to. Review article transverse myelitis spectrum disorders. Longitudinal extensive transverse myelitis letm in children. Given its rarity and the diversity of possible manifestations, establishing the diagnosis of spinal cord sarcoidosis is often very difficult. The clinical manifestations of tm are consequent to dysfunction of motor, sensory, and autonomic pathways. About europe pmc funders joining europe pmc governance roadmap outreach tools. 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. Although commonly associated with neuromyelitis optica, there are many other causes that should be considered. Tm can occur as an independent entity, usually as a postinfectious complication, but tm also exists on a continuum of neuroinflammatory disorders that. Acute transverse myelitis is an intramedullary spinal disorder yamada et al. Transverse refers to the inflammation being across the width of the spinal cord though often it may not affect the whole width. Neck flexion triggers an electrical sensation that ra. These findings were consistent with acute transverse myelitis involving the entire spinal cord, also referred to as longitudinal myelitis. Transverse myelitis is an acute or subacute inflammatory disorder involving the spinal cord.
Transverse myelitis spectrum disorders bioline international. Longitudinal myelitis in systemic lupus erythematosus. It results from damage to nerve cells in a certain area. Transverse myelitis complications bmj best practice. A previously healthy 9yearold girl was admitted to the emergency department of a district hospital due to persistent headache for 3 days. Longitudinal extensive transverse myelitisits not all neuromyelitis. We highlight two cases of spinal cord sarcoidosis, each presenting with longitudinally extensive transverse myelitis, that demonstrate the dilemmas that physicians face with regard to diagnosis and treatment. Longitudinal transverse extensive myelitis is the most common form of itm in oman. Tm is uncommon, with an approximate incidence of between one to five cases per million population annually 5. These patients may present with various clinical findings with which it is important for. Early identification and establishment of the underlying aetiology is vital in. Systemic lupus erythematosus presented as extensive. 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. It classically can cause weakness, loss of sensation, pain and bowel or.
Mri lesions are often centrally located with high t2 signal intensity involving gray and neighboring white matter. A little later, a brain biopsy showed findings of cerebral neutrophilic vasculitis. Neuromyelitis optica spectrum disorder as an initial. An approach to the diagnosis of acute transverse myelitis. Both entities are inflammatory and autoimmune in nature. Longitudinal myelitis may be the presenting manifestation of systemic lupus erythematosus and may be associated with the presence of antiphospholipid antibodies apl 2. Nmo was diagnosed because of characteristic longitudinal myelitis and positive nmoigg. Acute transverse myelitis tm is a rare acquired neuroimmune spinal cord disorder that can present with the rapid onset of weakness, sensory alterations, and bowel or bladder dysfunction. Whilst rare, longitudinally extensive transverse myelitis is clinically important as it can lead to catastrophic morbidity, and a group of these patients are at risk of further attacks. In general, complete transverse myelitis is associated with a long spinal cord lesion exceeding three vertebral segments in length, often central within the cord, and an incomplete trans verse myelitis is associated with a short spinal cord lesion, typically one to two segments in length and peripheral. Neurological rarities longitudinally extensive transverse. An uncommon cause of longitudinally extensive transverse myelitis. The differential diagnosis of longitudinally extensive.
An otherwise healthy 28yearold woman presents with progressive weakness for the past 3 days, numbness below her breasts, and urinary urgency. Differentiating neuromyelitis optica from other causes of. Diagnosis of longitudinally extensive transverse myelitis. Longitudinal extensive transverse myelitisits not all. These include other inflammatory monophasic myelitises, infection, and mimics such as malignancy. Acute longitudinal myelitis as the initial manifestation. Longitudinally extensive transverse myelitis with and. We report the case of a patient with pathologically confirmed smallcell lung carcinoma metastasis into the spinal cord presenting as letm. Most patients experience partial response to treatment or relapse within the first year of diagnosis 1. Two cases of sarcoidosis presenting as longitudinally. 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.
Transverse myelitis is an inflammation of the spinal cord, a major part of the central nervous system. As for her brain lesion, an extensive infectious disease workup was undertaken with multiple viral, bacterial, and fungal cultures and polymerase chain reactions ordered. The patient was initially treated with methylprednisolone, mgd, for 5 days and 7 cycles of plasmapheresis. A case of systemic lupus erythematosus associated with. Transverse myelitis tm is a focal inflammatory disorder of the spinal cord.
Pdf longitudinally extensive transverse myelitis letm is a neurological condition characterised by a contiguous inflammatory lesion of the. The weakness may become severe at times, leading to complete paralysis. In 2002, the transverse myelitis consortium working group tmcwg proposed the diagnostic criteria for idiopathic acute transverse myelitis iatm to delimit and unify this group of patients. The heterogeneous signal extended from the cervicomedullary junction to the conus medullaris. Longitudinally extensive transverse myelitis letm is a neurological condition characterised by a contiguous inflammatory lesion of the spinal cord. Transverse myelitis tm is a rare neurological condition in which the spinal cord is inflamed.
Differentiating neuromyelitis optica from other causes of longitudinally extensive transverse myelitis on spinal magnetic resonance imaging yeliz pekcevik, charles h mitchell, maureen a mealy, gunes orman, in h lee, scott d newsome, carol b thompson, carlos a pardo, peter a calabresi. Transverse myelitis tm is the prototype inflammatory myelopathy. Here, we present a patient with longitudinal myelitis who was found to have both primary ss and a positive antibody to aquaporin4 nmoigg. The spinal cord carries nerve signals to and from the brain through nerves that extend from each side of the spinal cord and connect to nerves elsewhere in the body. The longitudinal extent of mri lesions is possibly useful in determining the cause of tm multiple class iii studies, specifically in distinguishing between nmo spectrum disorders and ms. Letm is often associated with the autoimmune central nervous system disease neuromyelitis. Longitudinally extensive transverse myelitis mayo clinic.
At peak deficit, 50% of patients with tm are completely paraplegic with no. Causes include multiple sclerosis, neuromyelitis optica, infections, autoimmune or postinfectious inflammation, vasculitis, and certain drugs. Heterogeneous focal inflammatory disorder of the spinal cord characterized by acute or subacute development of motor weakness, sensory impairment, and autonomic dysfunction. No previous infections or recent immunisations were reported.