Neurological aspects in Sjögren's - Alessia Alunno ACSP2023

Summary

The video discusses the neurological manifestations of Sjögren's syndrome, a chronic, disabling systemic disease that significantly impacts quality of life. It explains how different parts of the nervous system can be affected, including the central, peripheral, and autonomic nervous systems. While neurological symptoms are common, they are often nonspecific, making diagnosis and treatment challenging. The video emphasizes the importance of awareness among rheumatologists and other specialists, highlighting the lack of strong pharmacological treatments. Non-pharmacological approaches, including physiotherapy and cognitive behavioral therapy, are crucial in managing symptoms. The speaker also introduces a research survey aimed at gathering patient perspectives.

Takeaways

• 😀 Sjögren's syndrome is a chronic autoimmune disease that can severely impact the nervous system, causing both central and peripheral neurological symptoms.
• 😀 The central nervous system (CNS) and peripheral nervous system (PNS) are both affected by Sjögren, with symptoms ranging from sensory disturbances to motor dysfunction.
• 😀 Neurological manifestations of Sjögren can include stroke, trigeminal neuralgia, cognitive dysfunction, and peripheral neuropathy, with symptoms often being nonspecific.
• 😀 Peripheral nervous system involvement is most common, with symptoms like pain, numbness, and weakness, affecting 18-45% of individuals with Sjögren.
• 😀 Damage to small fibers in the nervous system can cause autonomic dysfunction, including problems with temperature regulation, digestion, and cardiovascular issues.
• 😀 Diagnosing neurological issues in Sjögren is challenging due to the nonspecific nature of symptoms and their overlap with other diseases like diabetes or fibromyalgia.
• 😀 Diagnostic tests for neurological manifestations include physical exams, imaging, and nerve biopsies, though some tests may yield false negatives.
• 😀 While immunosuppressive treatments are used, there is limited evidence on their effectiveness specifically for neurological manifestations of Sjögren.
• 😀 Non-pharmacological treatments such as physiotherapy, cognitive behavioral therapy, and nutritional supplementation are crucial in managing symptoms and improving quality of life.
• 😀 Raising awareness of neurological manifestations of Sjögren is essential, not only within rheumatology but across other medical specialties, including general practice and neurology.
• 😀 Current treatment recommendations are based on evidence from other autoimmune diseases, but more research is needed to establish effective, targeted therapies for Sjögren-related neurological symptoms.

Q & A

What is Sjögren's syndrome, and how does it affect the nervous system?

Sjögren's syndrome is a chronic autoimmune disease that causes systemic effects, including damage to the nervous system. Neurological manifestations are common and can severely impact a person's quality of life. These manifestations can affect both the central and peripheral nervous systems, leading to symptoms like pain, sensory loss, cognitive dysfunction, and autonomic dysfunction.

How is the nervous system structured, and what roles do different types of nerves play?

The nervous system is divided into the central nervous system (CNS) and the peripheral nervous system (PNS). The CNS, consisting of the brain and spinal cord, controls bodily functions. The PNS is made up of cranial nerves, spinal nerves, and autonomic nerves, which transmit sensory information and control movements, including involuntary functions like heart rate and digestion.

What are the main types of nerves in the peripheral nervous system, and what functions do they serve?

The peripheral nervous system contains three main types of nerves: cranial nerves, spinal nerves, and autonomic nerves. Cranial nerves transmit sensory information (e.g., smell, taste) and control motor functions like eye and facial movements. Spinal nerves relay sensory and motor signals between the body and the brain. Autonomic nerves regulate involuntary functions such as stress responses and digestive processes.

What is the impact of neurological manifestations in Sjögren's syndrome, and how common are they?

Neurological manifestations affect 18-45% of people with Sjögren's syndrome, with peripheral nervous system involvement being the most common. These symptoms can include pain, sensory abnormalities, muscle weakness, and cognitive dysfunction. Central nervous system manifestations like stroke and headache can also occur, though these are nonspecific and difficult to attribute directly to the disease.

Why is diagnosing neurological manifestations in Sjögren's syndrome difficult?

Diagnosis is challenging because neurological symptoms in Sjögren's syndrome overlap with those of other conditions, such as diabetes, vitamin deficiencies, and fibromyalgia. Tests like nerve conduction studies may yield false negatives, and small fiber neuropathy may require more invasive procedures like a nerve biopsy, which isn't always available.

What diagnostic tools are used to assess neurological manifestations in Sjögren's syndrome?

Diagnosing neurological issues involves recording symptoms, performing physical exams, and conducting tests such as nerve conduction studies. For conditions like small fiber neuropathy, a nerve biopsy may be required, but access to this diagnostic test can be limited. Imaging studies like MRI can also help detect central nervous system abnormalities.

What is the difference between large fiber neuropathy and small fiber neuropathy in Sjögren's syndrome?

Large fiber neuropathy primarily affects sensory nerves in the extremities, leading to symptoms like reduced temperature perception and vibration sensitivity, with no effect on strength. Small fiber neuropathy affects both sensory and autonomic fibers, leading to broader symptoms that include sensory abnormalities, pain, and autonomic dysfunction like digestive issues and sweating problems.

What are the current treatment options for neurological manifestations in Sjögren's syndrome?

Treatment for neurological manifestations in Sjögren's syndrome is limited. Immunosuppressive drugs, such as glucocorticoids and methotrexate, may be used based on their efficacy in other autoimmune diseases, though their effectiveness in SS-related neurological symptoms is not well-established. Symptomatic treatments are available for autonomic dysfunction, but no specific therapies for neurological issues in SS have proven efficacy.

What role do non-pharmacological treatments play in managing neurological symptoms of Sjögren's syndrome?

Non-pharmacological treatments, such as physical therapy, cognitive behavioral therapy, and vitamin supplementation, play a crucial role in managing symptoms. These treatments can help alleviate some symptoms and improve the quality of life for patients, especially since pharmacological options remain limited.

What are the challenges in distinguishing neurological manifestations of Sjögren's syndrome from other conditions?

One of the main challenges in diagnosing neurological manifestations of Sjögren's syndrome is that symptoms often overlap with those of other conditions like diabetes, vitamin deficiencies, and fibromyalgia. Additionally, some conditions mimic peripheral neuropathy symptoms but are not due to actual nerve damage, complicating the diagnostic process.