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Late-onset multiple sclerosis presenting with cognitive dysfunction and severe cortical/infratentorial atrophy
Massimiliano Calabrese1, Alberto Gajofatto2, Francesca Gobbin2
1Neurology Section, Department of Neurological and Movement Sciences calabresem@hotmail.it.
Multiple Sclerosis (Houndmills, Basingstoke, England)
|November 30, 2014
Summary
Cognitive dysfunction can be the first sign of multiple sclerosis (MS), especially in older adults. Brain atrophy, including the "Hummingbird sign," may be present even without other neurological symptoms.
Area of Science:
- Neurology
- Neuroimmunology
- Cognitive Neuroscience
Background:
- Cognitive dysfunction is a common but often overlooked early symptom of multiple sclerosis (MS).
- Cognitive onset as the primary manifestation of MS is rare, potentially delaying diagnosis.
Purpose of the Study:
- To describe patients with multiple sclerosis presenting with cognitive dysfunction as their initial symptom.
- To investigate the clinical and radiological characteristics of these patients.
Main Methods:
- Retrospective study of 12 patients with cognitive onset MS (CI-MS).
- Comparison with cognitively normal MS patients (CN-MS), healthy controls, and progressive supranuclear palsy (PSP) patients.
- Analysis of cognitive function, clinical course, and neuroimaging findings (cortical, infratentorial, and midbrain atrophy).
Main Results:
- CI-MS patients typically had a late disease onset (median 49 years) and a progressive course.
- Frontal cognitive domains were most affected.
- CI-MS patients showed significant cortical and infratentorial atrophy, with 8/12 exhibiting the midbrain "Hummingbird sign."
Conclusions:
- Cognitive dysfunction, particularly with a slowly progressive course and significant brain atrophy, warrants consideration for MS diagnosis.
- The
- Hummingbird sign
- may be observed in MS with cognitive onset.
- Early diagnosis of MS is crucial, even in the absence of overt non-cognitive neurological deficits.
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