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Measuring Progressive Neurological Disability in a Mouse Model of Multiple Sclerosis
Published on: November 14, 2016
Neuropsychological Disability in the Case of Natalizumab-Related Progressive Multifocal Leukoencephalopathy
Viviana Lo Buono1, Giangaetano D'Aleo1, Simona Cammaroto1
1IRCCS Centro Neurolesi Bonino Pulejo, 98100 Messina, Italy.
Background:
Progressive multifocal leukoencephalopathy (PML) is a viral disease characterized by progressive damage or inflammation of the cerebral white matter that can be encountered in patients with multiple sclerosis (MS). There are cases of PML caused by pharmacological agents including natalizumab. Therefore, in patients treated with this drug, early identification of PML allows changes in the treatment plan, reducing the risks of morbidity and mortality.
Case Presentation:
We reported the case of a 57-year-old female diagnosed with relapsing-remitting MS, who presented with PML related to natalizumab. The patient presented with change in behavioral, radiological abnormalities in the left parieto-temporal lobes. We described the longitudinal course of PML, from the diagnosis until the patient's death, documenting the progressive deterioration of her cognitive functioning, supported by changes on sequential brain scans and neurophysiological data.
Conclusion:
The neuropsychological impairment documented in this case study expands the range of treatment-related complications associated with natalizumab, and provides evidence that occurrence of "atypical" cognitive deficits in MS may support the early diagnosis of PML.
Insights
Progressive multifocal leukoencephalopathy (PML), a rare brain infection, can occur in multiple sclerosis (MS) patients treated with natalizumab. Early detection of atypical cognitive deficits in MS patients is crucial for diagnosing PML and improving outcomes.
Area of Science:
- Neurology
- Neuroimmunology
- Infectious Diseases
Background:
- Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease affecting the brain's white matter.
- PML can be a complication in patients with multiple sclerosis (MS).
- Natalizumab, a treatment for MS, is associated with PML development.
Observation:
- A 57-year-old female with relapsing-remitting MS developed PML while on natalizumab therapy.
- The patient exhibited behavioral changes and parieto-temporal lobe abnormalities on imaging.
- The case details the progression of PML, cognitive decline, and neurophysiological changes.
Findings:
- The case illustrates PML as a treatment-related complication of natalizumab in MS.
- Neuropsychological impairment was a key feature, progressing over time.
- Sequential brain scans and neurophysiological data correlated with cognitive deterioration.
Implications:
- Atypical cognitive deficits in MS patients may indicate early PML.
- Timely diagnosis of PML allows for treatment modification, potentially reducing morbidity and mortality.
- This case highlights the importance of vigilant monitoring for PML in MS patients receiving natalizumab.

