APPENDIX 4
Employing Neuroimaging Studies in the Diagnosis of ALS

In patients with Clinically Definite ALS with bulbar or pseudobulbar involvement, neuroimaging studies are not essential.

In all other patients, appropriate neuroimaging studies should be performed to rule out structural lesions that may explain the observed signs and symptoms.

Neuroimaging features that cast doubt on the diagnosis of ALS or may be confounding factors in interpretation of diagnostic or clinical trial results include the following:

1. significant bony abnormalities on plain X-rays of skull or spinal canal that might explain clinical findings,
2. significant abnormalities of head or spinal cord MRI suggesting intra- or extra-parenchymal processes. However, abnormalities confined to the corticospinal tract are consistent with ALS.
3. significant abnormalities of spinal cord myelography with/without CT or CT alone suggesting lesions as noted above,
4. significant abnormalities on spinal cord angiography suggesting vascular malformations.

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