Defining the course of neurosarcoidosis according to presentation at onset and disease modifying treatment: a cohort study of 84 patients

Therapeutic Advances in Neurological Disorders, 2023 · DOI: 10.1177/17562864231205954 · Published: September 15, 2023

Simple Explanation

Neurosarcoidosis (NS) is a rare form of sarcoidosis that affects the nervous system, presenting in various ways and posing management challenges due to limited knowledge about its long-term progression. This study aimed to identify different patterns of how neurosarcoidosis develops over time based on the initial symptoms and test results at the beginning of the illness. The study found that neurosarcoidosis typically follows a relapsing course, with most patients experiencing a single episode or recurring episodes of the same symptoms, and only a small percentage developing new symptoms over time.

Study Duration

6.6 Years

Participants

84 patients diagnosed with definite, probable, or possible neurosarcoidosis

Evidence Level

Not specified

Key Findings

1
Neurosarcoidosis is primarily a relapsing disease, with no patients exhibiting a progressive course in this cohort.
2
Most patients experienced a monophasic disease course, meaning a single episode, while others had polyphasic courses with either the same or different neurological symptoms recurring.
3
Patients with isolated cranial nerve injury or aseptic meningitis had better short-term prognoses and fewer long-term sequelae compared to those with other forms of neurosarcoidosis.

Research Summary

This retrospective cohort study of 84 patients with neurosarcoidosis (NS) aimed to define disease courses based on initial presentation and treatment. The study identified three main disease courses: monophasic, polyphasic with recurrence of the same symptoms, and polyphasic with different symptoms. Most patients had a monophasic course. The researchers concluded that the initial presentation of NS can help guide therapeutic strategies and patient management, with isolated cranial nerve injury and aseptic meningitis showing better prognoses.

Practical Implications

Personalized Treatment Strategies

Understanding the different disease course profiles of neurosarcoidosis can help clinicians tailor treatment strategies based on the initial clinical and paraclinical presentation of the patient.

Prognostic Assessment

Identifying specific disease courses can aid in predicting the likely progression of neurosarcoidosis, allowing for more informed patient counseling and follow-up planning.

Targeted Therapies

Recognizing that certain presentations, such as spinal cord involvement, may require more aggressive first-line treatments, like TNF-alpha blockers, to achieve remission and limit sequelae.

Study Limitations

1
The retrospective design could introduce measurement bias and missing data.
2
The non-standardized nature of data entry, patient follow-up, and treatment initiation in a real-life study influences the evaluation of clinical and paraclinical courses.
3
The study only included patients from neurology departments, potentially leading to selection bias by excluding patients treated in other specialties.

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