WORKUP of Dementia in Motor Neuron Disease
Lab Studies
Obtain typical blood studies, thyroid function tests, vitamin B12 and folate levels, and Venereal Disease Research Laboratory test. Refer to Amyotrophic Lateral Sclerosis for a full listing of the MND workup.
Imaging Studies
Computed tomography (CT) scan may show mild, generalized cerebral atrophy or asymmetric frontotemporal atrophy.
Because of greater resolution than CT, MRI studies may reveal selective frontal and anterior temporal atrophy that cannot be appreciated on CT.
Single photon emission computed tomography imaging often demonstrates reduced blood flow in an asymmetric, frontotemporal pattern.
A recent study reported that glucose hypometabolism on PET imaging in patients with dementia associated with motor neuron disease affected only the frontal lobes, sparing the temporal lobes. This is in contrast to frontotemporal dementia where glucose hypometabolism is seen in both frontal lobes and temporal lobes. In Alzheimer disease, PET scans may reveal glucose hypometabolism in the parietal and temporal regions bilaterally.
Other Tests
Electroencephalogram can remain normal even in later stages of dementia, but often mild dysrhythmic slowing occurs that is sometimes asymmetric.
Electromyography may demonstrate widespread denervation in limb muscles. Early in the disease, especially in predominantly bulbar onset patients, findings may not fulfill the Lambert or El Escorial criteria for MND.
Histologic Findings
Early in the disease, FTD/MND preferentially affects frontal and temporal lobes, the hypoglossal nucleus, and spinal motor neurons. Later and terminal stages reveal histologic evidence of widespread cortical involvement. In the frontal and temporal lobes, microscopic changes include loss of pyramidal cells, spongiform neuropil change, and astrocytic gliosis. Ubiquinated, tau-negative inclusions are present within the frontal cortex and the dentate gyrus of the hippocampus. Pick cells (inflated neurons) and Pick bodies (ubiquitin and tau-positive intracellular inclusions) are absent. Betz cells in the precentral gyrus usually are affected.
In approximately 50% of patients, neuronal loss and pigmentary incontinence in the substantia nigra and other pigmented brainstem neurons occurs, even in patients without clinically overt parkinsonism. There can be marked hypoglossal and spinal motor neuron degeneration (although this is not essential for patients to progress to an anarthric state) and ubiquinated tau-negative inclusions in the spinal neurons.
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