Demyelinating disease

Transverse myelitis and spinal demyelinating diseases • MS is by far the most common demyelinating disease Transverse myelitis • inflammatory condition affecting both halves of the spinal cord and associated with rapidly progressive motor, sensory, and autonomic dysfunction • It affects individuals of all ages with peaks at ages 10-19 years and 30-39 years • There is no sex or familial predisposition and usually no prior history of neurologic abnormality Etiology • • Transverse myelitis may occur in isolation or in the setting of another illness When it occurs without apparent underlying cause, it is referred to as idiopathic • Idiopathic TM is assumed to be the result of abnormal activation of the immune system against the spinal cord  Patients with an acute short segment TM are at risk of developing MS if there is one of the following: • partial TM, i.e short segment TM • Family history of MS Brain lesions on MR • Oligoclonoal bands in CSF Radiographic features • Lesions may occur anywhere within the cord, with the thoracic cord being the most frequently involved site • Up to 40% of cases have no findings on MRI • most commonly extend for 3-4 spinal segments • More than 2/3 of the cross sectional area is involved • Focal enlargement • Enhancement + / - 18 years Male Acute paraparesis These images are of a 31 year old male with headache, voiding disturbances, urinary retention, sensory level C3 The CSF analysis revealed 400/3 cells (meaning no infection) and a slightly higher protein level Longitudinal case series of TM reveal that approximately 1/3 of patients recover with little to no sequelae, 1/3 are left with moderate degree of permanent disability, and 1/3 have severe disabilities Multiple Sclerosis • adolescence and the sixth decade, with a peak at approximately 35 yr • female predilection  • The most common demyelinating • A acquired chronic relapsing demyelinating disease • An immune-mediated inflammatory of the brain and the spinal cord • Multiple lesions disseminated over time and space (multiple lesions at different times) • CSF monoclonal bands Radiographic features • In MS there is typically a short segment involved, i.e less than segments • Partial involvement is typically seen in MS • Location: posterior, lateral • pathologic studies have shown that 95% of MS patients have spinal cord lesions, whether they have spinal symptoms or not On transverse images MS lesions typically have a round or triangular shape and are located posteriorly or laterally This 24-year old patient had visual disturbances on one eye followed by weakness and sensory disturbances of the lower and upper extremities a couple of years later Now she presents with sensory disturbances of both lower extremities So we already think MS • • Location: infratentorial, in the deep white matter, periventricular, juxtacortical a very early sign is called ependymal dot-dash sign (tiny (~1 mm) dots of high signal along the ependymal surface that may coalesce into short dashes  1-2 It presence has been reported as being very sensitive (>95%) and moderately specific (>70%) in younger individuals (