View Full Version : CNS Disease in Sjogren's from the Sjogren's Conference

04-22-2010, 09:18 PM
Hi Everyone,
I'm back, and at last, I have another report ready to post. This one might be of interest to many.

CNS Disease in Sjogren’s Elaine L. Alexander, MD, PhD

Dr. Alexander is from the Johns Hopkins Medical Institutions and has researched the potential causes and treatment of autoimmune, inflammatory, rheumatologic and neurologic disorders, emphasizing Sjogren’s.

CNS in SJS is probably inflammation of the brain and/or spinal cord. It can present with symptoms from brain fog to dementia. Can be focal and/or nonfocal. May involve more than one neuroanatomical region. May be recurrent. MRI lesions tend to localize to subcortical/periventricular areas, but the MRI does not have to be positive for CNS symptoms to be present.

May involve any region of brain/spinal cord/optic nerves:
Cerebral cortex
• Frontal/prefrontal
• Parietal
• Temporal (hippocampus, amygdala)
• Occipital

Basal ganglia, thalamus, midbrain
Medulla/brain stem

Vascular-Neurovascular Syndromes
• Intracerebral hemorrhage
• Subarachnoid hemorrhage
• Spinal artery rupture

Clinical Manifestations of focal CNS SJS:
• Motor deficits
• Sensory deficits
• MS-like syndromes
• Aphasia/dysarthria
• Gaze disturbances
• Cortical blindness
• Seizure disorders
• Movement disorders ( PD-like or Chorea)
• Cerebellar syndromes
• Ataxia, gait disturbances
• Brainstem disturbances

Clinical Manifestations of non-focal CNS SJS:
• Aseptic meningitis
• Subacute/acute encephalopathy
• Attention/concentration, working memory, executive function deficits
• Brain fog
• Mild cognitive impairment
• Dementia
• Affective neuropsychiatric disorders (anxiety, depression)
• Sleep disturbances

There is increasing evidence that inflammation is a cause for CNS SJS
Cerebrospinal fluid shows active inflammation:
• Increased IgG index
• Oligocioncal bands
• Autoantibodies
• Increased numbers of inflammatory cells
• Complement activation

There are sometimes MRI abnormalities in CNS SJS – multiple areas of increased signal intensity in subcortical/periventricular regions – predominantly white matter distribution.

SPECT is a new way of testing which measures regional cerebral blood flow.

There is also a specific pattern in the lip biopsy in CNS SJS.

Inflamed meninges can mimic MS, but without the demylenization.

There is current research concerning brain pathology in CNS SJS and the characterization of cognitive dysfunction in Sjogren’s patients. This research is showing that SJS patients have B cell activation and increased levels of the pro-inflammatory cytokine BAFF. The anti-BAFF molecules which are currently being tested for treating Lupus may also be of use in CNS SJS.

1. Memory problems, brain fog, dx of Alzheimer’s in later years – all SJS? See above.
2. Is my Meniere’s Syndrome related? (AIED) yes
3. Could the SJS have led to my Spasmodic Dysphonia? yes
4. Sweating on one side of body only – is this a CNS issue? This could be.

Marla’s notes – This report is full of a lot of difficult terms, but I think that many of us will recognize ourselves in some of it. It might give you a starting point for continued research or be something that you can share with your doctor for him/her to explain.
Some of these symptoms sound very scary, and are most likely worst-case scenarios, but I'm sure that a knowledge of all of this will be helpful.

04-23-2010, 11:45 AM
Thank You, again, Marla for providing this information!!!!

Peace and Blessings