General paresis of the insane: a diagnosis not to forget, 2025, Masud Husain

[Mij]

Just over 200 years ago something remarkable happened in post-Revolutionary Paris. Alienists, the forerunners of modern day psychiatrists and so-called because they cared for people alienated from society, had observed an alarming trend. A growing number of relatively young people—mostly men—had developed a startling, progressive illness. It was characterized by abnormal behaviour (sometimes referred to as mania and often associated with delusions of grandeur), cognitive decline and eventual dementia, gait disturbance leading to paralysis, and ultimately to death.

The mental institutions in Paris and elsewhere in France soon began to register that this hitherto unrecognized constellation of symptoms was a new condition. Moreover, it seemed utterly unresponsive to conventional psychological or physical treatments and always led to a terrible demise, usually within months, at the very most a couple of years.
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[Mij]

Although Alzheimer’s disease (AD) would perhaps not be the first consideration in the differential diagnosis of a patient presenting with GPI, it is interesting to note that many individuals in the Wang et al. study had hippocampal atrophy on MRI. Some also had evidence of amyloid positivity (on either CSF analysis or PET imaging) or elevated tau levels (in the CSF).

More likely than a clinical diagnosis of AD being made erroneously is the consideration of behavioural variant frontotemporal dementia and its overlap with movement disorders and amyotrophic lateral sclerosis (ALS). Further, if a neurological examination is not performed, some clinicians may diagnose a primary psychiatric diagnosis as the likely cause.

Late neurosyphilis remains an elusive entity and a challenging diagnosis to make unless we entertain it as a possibility. It remains a diagnosis not to forget.