BackgroundHuntington's disease (HD) is a fatal inherited neurodegenerative disease, caused by a

The pupil dilation response to tropicamide has been proposed as a simple test for demonstrating the central cholinergic deficit present in Alzheimer's disease (AD). We examined this response in 20 patients with clinically diagnosed mild probable AD and in 20 unrelated, cognitively intact, age-matched controls. A marked increase in pupil diameter was observed within 30 minutes in both groups. Differences between the groups did not attain statistical significance. The ability of the proposed test to discriminate between AD patients and healthy controls may be influenced by experimental procedures and by the ethnic background of the subjects under study. At present the pupil dilation response to tropicamide cannot be considered a useful diagnostic aid.

Seven typical cases of dementia with motor neuron disease (D-MND) are reported. Among 1,000 dementia cases, D-MND was more frequent than Pick’s disease, Lewy body disease or Creutzfeldt-Jakob disease. D-MND accounted for 30.4% of all forms of frontal lobe dementia (FLD) including FLD and Pick’s disease. These data support that this combined syndrome may be more frequent than previously reported. As the subcortical neuropathology of D-MND is identical with MND, D-MND is rather the cortical manifestation of MND than a new disease entity.

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