Neurology Resident and Fellow Section

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Presentation transcript:

Neurology Resident and Fellow Section A 17 year old girl with progressive weakness Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology

Vignette 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. Sushma et al.

MRI Cervical spine Sushma et al. Legends to figures Fig 1A- MRI cervical spine Axial T1W sequence showing symmetrical hypointense signals in the anterior horn. Fig 1B- MRI cervical spine Axial T2W sequence showing symmetrical hyperintense signals in the anterior horn. Sushma et al.

Snake eye appearance in MRI in patient with ALS MRI of cervical spine revealed symmetrical T1 hypo, T2 hyper intense signal in the anterior horns ("snake eyes" appearance) 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 was made. Snake eye appearance has been described in disorders like ALS, Cervical Spondylotic Amyotrophy, Hirayama disease and Ossification of Posterior Longitudinal Ligament.(1, 2) References: 1.Friedman DP, Tartaglino LM, Fisher AR, Flanders AE. MR imaging in the diagnosis of intramedullary spinal cord diseases that involve specific neural pathways or vascular territories. AJR. 1995;165:515–23. 2. Mizuno J, Nakagawa H, Inoue T, Hashizume Y. Clinicopathological study of “snake-eye appearance” in compressive myelopathy of the cervical spinal cord. J. Neurosurg. 2003 ;99(2 Suppl):162–8. Sushma et al.