Amyotrophic lateral sclerosis with predominant pyramidal signs -- early diagnosis by magnetic resonance imaging

To evaluate the role of magnetic resonance imaging (MRI) in the early diagnosis of amyotrophic lateral sclerosis (ALS) with predominant upper motor neuron (UMN) signs in the initial stage of the disease.

Two patients with ALS were found to have spastic quadriparesis with no wasting or fasciculation in the limbs in the early stage of the disease. Both were subjected to MRI of the head and cervical spine to look for any specific diagnostic feature. Both of them were followed with clinical evaluation and electroneuromyography (ENMG) for the definitive diagnosis of ALS.

Magnetic resonance imaging showed selective degeneration of the pyramidal tracts in the contiguous axial cuts from subcortical white matter to cerebral peduncles. The finding was more visible in the coronal section. In addition, there was T1 hyperintensity visible along the anterior aspect of the spinal cord in the cervical region. These findings were suggestive of ALS, the diagnosis that was subsequently confirmed by serial clinical follow-up and ENMG.

It is difficult to diagnose ALS in the early stage of the disease especially if the pyramidal signs predominate over the lower motor neuron (LMN) signs; MRI might be useful in such cases.