MRI findings in a patient with a familial form of motor neuron disease

The authors report the case of a 32-year-old woman who developed symptoms and signs possible manifestations of a familial motor neuron disease with left sided pyramidal signs and marked wasting and weakness in the ipsilateral upper girdle, progressively extended to the contralateral upper limb. The CT-scan showed only frontal cortical atrophy. MRI disclosed a restricted area of increased signal intensity in the centrum semiovale of the subcortical white matter of the right prefrontal cortex. This young woman did not disclose any risk factors for cerebrovascular diseases nor other disorders of the CNS; therefore the authors are of the opinion that the white matter changes observed on MRI are not occasional findings, but are related to the pathologic process occurring in consequence of her neurodegenerative disorder (possible ALS).