From the Guidelines
Multisystem tauopathy, a neurodegenerative disorder characterized by abnormal tau protein accumulation, requires a comprehensive management approach focusing on symptom relief and quality of life improvement, as outlined in the most recent guidelines, such as those from the Journal of the American College of Radiology 1.
Clinical Presentation and Diagnosis
Multisystem tauopathy presents with a range of symptoms including parkinsonism, cognitive decline, and behavioral changes, making diagnosis challenging without imaging and thorough clinical evaluation. The condition is part of a broader group of tauopathies, which include corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), distinguished by the accumulation of tau protein in different brain regions 1.
Management and Treatment
Given the complexity and variability of multisystem tauopathy, management strategies are tailored to the individual's symptoms. For parkinsonism symptoms, levodopa is commonly initiated at a dose of 100mg three times daily, with gradual increases as needed and tolerated 1. Additionally, medications like SSRIs (e.g., sertraline 50-200mg daily) may be used for mood disturbances, and cholinesterase inhibitors (e.g., donepezil 5-10mg daily or rivastigmine 1.5-6mg twice daily) for cognitive impairment.
Multidisciplinary Care
- Physical therapy is essential for maintaining mobility and preventing complications such as falls.
- Occupational therapy helps patients adapt to their condition, preserving independence.
- Speech therapy addresses communication and swallowing issues. Regular monitoring, ideally every 3-6 months, is crucial to assess disease progression and adjust treatment plans accordingly, ensuring the best possible quality of life for patients with multisystem tauopathy, as supported by recent clinical guidelines 1.
From the Research
Definition and Classification of Multi-System Tauopathy
- Multi-system tauopathy refers to a group of neurodegenerative disorders characterized by the accumulation of tau protein in the brain, leading to a range of symptoms including cognitive decline, behavioral changes, dementia, and motor deficits 2, 3.
- These disorders can be classified into primary and secondary tauopathies, with primary tauopathies including Pick disease, corticobasal degeneration, progressive supranuclear palsy, and argyrophilic grain disease 3.
Clinical Presentation and Diagnosis
- The clinical presentation of tauopathies varies based on the brain areas affected, generally presenting with a combination of cognitive and motor symptoms either earlier or later in the disease course 3.
- Accurate clinical diagnosis is challenging when biomarkers are unavailable, as symptoms overlap and tauopathies such as Alzheimer's disease and argyrophilic grain disease often coexist 3.
Treatment Options
- Currently, there are no approved and established pharmacologic treatment options for tauopathies, and available treatment strategies are based mainly on small clinical trials, miscellaneous case reports, or small case-controlled studies 2.
- Approved therapeutic agents for Alzheimer's dementia, such as acetylcholinesterase inhibitors and memantine, have been used off-label to treat cognitive and behavioral symptoms in tauopathies, but the outcome has not been consistent 2.
- Therapeutic agents for the symptomatic treatment of Parkinson's disease, such as levodopa or dopamine agonists, are used for motor symptoms in tauopathies, and antidepressants, especially selective serotonin reuptake inhibitors, could be helpful for behavioral or psychopathological symptoms 2, 4.
Current Research and Future Prospects
- Current research focuses on targeting tau protein pathology, including pathologic aggregation or phosphorylation, and several therapeutic approaches may slow the progression of tauopathies or improve symptoms 2, 5.
- There are four clinical candidates in phase III trials and 16 in phase II trials, and various therapeutic approaches are being explored, including disease-modifying therapies that target Aß and tau pathology in Alzheimer's disease 5.