Teaching Neuro Images : Snake eyes appearance in MRI in patient with ALS

A 17-year-old girl presented with 6 months of progressive asymmetrical distal weakness of upper limbs with fasciculation. There were no bulbar symptoms, weakness in lower limbs, or sensory symptoms. There was no history of neck injury or pain. Deep tendon reflexes were all brisk. MRI of cervical spine revealed symmetrical T1 hypointense, T2 hyperintense signal in the anterior horns ("snake eyes" appearance) (figure, A and B) without evidence of extradural compression. EMG showed diffuse denervation changes in both upper and lower limb muscles. Sensory nerve action potentials were normal. A diagnosis of amyotrophic lateral sclerosis (ALS) was made. Snake eyes appearance has been described in disorders like ALS, cervical spondylotic amyotrophy, Hirayama disease, and ossification of posterior longitudinal ligament.1,2