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Updated: Jan 27, 2026

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
Published on: July 29, 2007
Frontotemporal Dementia with Motor Neuron Disease in a Patient with Antiphospholipid Syndrome: A Case Report
Yoon-Cheol Jeong1, Jin-Seok Park1, Seung-Hyun Kim1
1Department of Neurology, Hanyang University College of Medicine, Seoul, Korea.
Background:
Frontotemporal dementia (FTD) with motor neuron disease (MND) is a syndrome of progressive changes in behavior, language, muscle weakness and atrophy due to loss of function of neurons in the frontal and temporal lobes and in motor neurons. Etiology and pathogenesis of FTD with MND are still uncertain.
Case Report:
A 71-year-old man presented with a 2-year history of progressive muscle weakness and cognitive deficits. We diagnosed this patient as FTD with MND by neurological examination, electromyography, brain imaging and neuro-psychological evaluation. We also confirmed antiphospholipid syndrome (APS) in this patient as a way to rule out secondary causes of MND.
Conclusions:
This was a very rare case of FTD with MND in APS. We should focus study on the possible role of autoimmune pathogenesis in FTD with MND.
Insights
This rare case highlights frontotemporal dementia with motor neuron disease (FTD with MND) in a patient with antiphospholipid syndrome (APS). Further research into autoimmune factors in FTD with MND is warranted.
Area of Science:
- Neurology
- Immunology
Background:
- Frontotemporal dementia with motor neuron disease (FTD with MND) is a progressive neurodegenerative syndrome with uncertain etiology.
- FTD with MND involves neuronal loss in the frontal/temporal lobes and motor neurons, leading to behavioral, language, and muscle deficits.
Observation:
- A 71-year-old male presented with a two-year history of progressive muscle weakness and cognitive decline.
- Diagnostic workup included neurological examination, electromyography, brain imaging, and neuropsychological evaluation, confirming FTD with MND.
- Antiphospholipid syndrome (APS) was identified, serving to exclude secondary causes of MND.
Findings:
- The case presents a rare co-occurrence of FTD with MND and APS.
- This association suggests a potential link between autoimmune processes and FTD with MND.
Implications:
- Investigating the role of autoimmune pathogenesis in FTD with MND is crucial.
- This case underscores the importance of considering autoimmune etiologies in complex neurodegenerative presentations.
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