While MRI is usually performed to exclude alternate causes of symptoms, several features have been reported in patients with ALS.  Examples may include cerebral cortex atrophy, motor band sign, and corticospinal tract hyperintensity; however, these changes are variable and often subtle. 

46 year-old right-handed woman was admitted to the neurological intensive care unit for respiratory distress in the setting of progressive decline over the preceding 5 months. 

Her disease course initially began as dysphagia; however, gastroenterology evaluation including modified barium swallow study (MBSS) and esophagogastroduodenoscopy (EGD) were unremarkable.   She then developed dysarthria and eventually distal muscle weakness months later.  Just prior to her hospitalization, she had magnetic resonance imaging (MRI) of the brain, cervical spine, and 3 limb nerve conduction study (NCS) with electromyography (EMG) to further investigate her symptoms.  These results were highly concerning for motor neuron disease, specifically amyotrophic lateral sclerosis (ALS).  Particularly, MRI brain demonstrated hyperintense changes from the precentral cortex through the pons along the corticospinal tracts.  Her neurologic exam while admitted was additionally notable for both upper and lower motor neuron signs including tongue fasciculations. 

She was diagnosed with bulbar onset ALS, started on Riluzole, and referred to our regional ALS medical home at time of discharge.