Sjogren’s Syndrome Affects the Brain and Spine

Did you hear about Sjogren’s syndrome  affecting your brain and your spine   today we’re gonna talk about how this syndrome  sjogren will affect your central nervous system   so if you’re newly diagnosed with sjogran or if  you have symptoms related to your brain or to  
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your spine that you could not understand this  might be the cause rheumatologist oncall.com   when we think about Sjogren’s syndrome we think  about sjogren that is primary not necessarily   caused by a disease or sjogren that is secondary  associated with another autoimmune disease such  
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as lupus rheumatoid arthritis or Scleroderma is  this syndrome frequent it is reported that about   0.1 up to 3 percent of the general population can  develop primary Sjogren’s syndrome and females are   much more affected than males and the ratio that  was reported is nine to one that means that nine  
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female will develop shogron versus only one male  and the age of onset or the age when we diagnose   this disease is between 40 and 60 years now what  are the most common signs and symptoms of sugar   if you want to learn more about sjogren and about  the multitude of symptoms that this disease can  
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cause you can watch this video in my channel  now you have to understand that children is   a systemic disease it’s not going to affect only  your eyes or your mouth it can affect your skin it   can affect your teeth it can affect your stomach  it can affect your vessels and it can also affect  
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your lungs or your kidneys if you look at this  whole list of children manifestation you’re gonna   see that patients with sjogren can develop fatigue  fever weight loss can develop joint pain or muscle   pain they can develop cough because of dryness  in their trachea they can develop difficulties  
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to swallow food again because they lack saliva or  they can have urinary tract infections vessels or   skin involvement but also neurological involvement  and that’s we are going to talk about today   what are the laboratory tests that  we order for patients with sugar  
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we do order a a SSA SSB antibodies chromative  Factor anti-centromere antibodies but not all   patients will have those antibodies present if you  look at these numbers only 80 percent of patients   will have positive DNA only 60 to 80 percent  of patients will have a positive SSA or SSB  
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antibodies and 74 percent of patients will have a  rheumatoid Factor present not only patients with   rheumatoid arthritis but also patients with  sjogren have rheumatoid Factor positive there   are other tests that we do to evaluate dryness  dryness of the eyes like Shimmer tests or dryness  
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of the mouth where we do measure the salivary  flow or we do a salivary gland biopsy how do we   diagnose Sjogren’s syndrome we use different type  of criteria like subjective criteria your dryness   in the eyes or your dryness in the mouth but we  also use objective criteria like measuring the  
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amount of lacrimal secretion that you have or  the salivary flow or we can do a salivary gland   biopsy that will tell us if you have infiltration  with certain immune cells in your salivary glands   we also test you for the antibodies like Ana SSA  SSB and we use both of these types of criteria  
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to make the diagnosis of children now we have  to consider certain situations that can also   cause dryness if you had for example lymphoma  or if you had head or neck radiation if you   had hepatitis C HIV sarcoidosis if you had a  transplant in your history or if you had IGG  
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4 disease or if you have any treatments  with anticholinergic drugs all of this   can cause dryness in your mouth and needs to be  excluded before we make the diagnosis of sjogren   how can shogren affect your nervous system when we  think about the nervous system we try to be more  
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specific and we stratify that into the peripheral  and the central nervous system the central nervous   system refers to your brain and your spine while  your peripheral nervous system refers to all the   nerves that come out of your spine and they can  control involuntary body function and regulate  
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your gland or muscles that controls your movement  how often was neurosogen reported the symptoms of   neurological involvement in sjogren were  reported in primary children since 1935.   if you look at different studies between 10 to  60 percent of patients with primary sjogren will  
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have some neurological complaints now peripheral  nervous system involvement is much well recognized   and documented compared to central nervous  system involvement which we will discuss today   as I said before central nervous system refers to  your brain and refers to your spine the central  
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nervous system was shown to be affected mostly  in females between 40 and 50 years of age and   sometimes the disease was there for about 10  to 11 years before those symptoms would appear   but not always and the prevalence or how often  it was reported was shown to be between three  
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percent up to 80 percent of patients as I already  mentioned the peripheral nervous system is well   documented to be affected in Shogun syndrome and  patients will complain of neuropathy which could   be sensory or sensory and motor neuropathy which  could involve one nerve or multiple nerves and can  
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cause also autonomic neuropathy some patients  can also develop myasthenia gravis the central   nervous system is affected in a diffuse way or  just focal way and you can have involvement of   the brain or involvement of the spine or both  there are many studies published that they show  
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that the central nervous system to your brain and  your spine can be involved in shogren and I just   listed a few here when the brain is affected  just in certain areas you can have sensory   loss you can have motor issues but you can also  have difficulties to talk difficulties to think  
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you may develop seizures movement disorder you  can develop Subacute transverse myelitis and   sometimes you can have involvement of the optical  nerve and sometimes sjogren can look like a tumor   but is not really a tumor there when the brain is  involved diffusely you you can have symptoms like  
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encephalopathy or dementia you can develop  psychiatric problems or you can have what it   looks like a meningo Encephalitis but it’s  without any infectious cause there you can   also develop an intellectual decline you can have  difficulties with your memories or difficulties  
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to formulate ideas and you can have also mood  changes let me introduce you to some of the   symptoms that you can encounter if you have  your brain affected by chogren headaches that   looks like migraine or tension type headaches  are very frequent in patients with sjogren  
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spinal cord involvement are also frequent seizures  motor and sensory deficits or neuromyelitis Optica   as I mentioned headaches looks to be a very  frequent complaint of patients with neurosogram   this is a table with many studies that prove that  headaches neuromyelitis Optical seizures are very  
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frequent in patients with involvement of their  brain now this is another study that shows that   headaches can be seen in 47 percent of patients  cognitive dysfunction can be seen in 44 percent of   patients and mood disorders are also very frequent  depression as I said is one of the mood disorders  
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that can be seen very frequently in patients with  sjogren shogren can involve your central nervous   system and also the peripheral nervous system  at the same time but in about 40 percent of   cases we do see only involvement of the central  nervous system meaning the brain and the spine  
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now what are the other symptoms that can accompany  the Brain and Spine involvement here is a study   that shows that neurological involvement is often  associated with dryness with pain in the joints   with pain in the muscles Raynaud’s phenomenon  severe fatigue autoimmune thyroiditis and less  
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frequently with pulmonary involvement or lung  involvement this is another study that shows that   patients will complain about dryness will complain  about fatigue weight loss joint pain and Raynaud’s   phenomenon in conclusion neurological involvement  is also associated with dryness in the eyes in the  
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mouth reynard’s phenomenon which is a change in  your hands or feet color when you expose to them   to cold weather most of the time it can associate  with thyroiditis or autoimmune thyroiditis fatigue   lung issues and arthritis now what is coming first  the dryness or the neurological symptoms in 46  
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of patients with neurological involvement this is  actually the first symptom of the disease and that   is more frequent in patients that have brain and  spine involvement compared to peripheral nervous   system involvement how do we diagnose neurosogram  or sjogren involving the central nervous system  
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cerebrospinal fluid or the spinal fluid analysis  is very important for the diagnosis of neurosogram   we can see certain changes like your lymphocytes  being elevated there your IGG index being elevated   but less oligochloral bands and we’ll talk about  that in a little bit and we also do blood work the  
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blood work that I mentioned before we test for  your Ana SSA and SSB antibody we look at your   number of white blood cells we look to see if  you have more gamma globulins we look to see if   you have Rheumatic Factor complement levels all  of these are important to be tested and what it  
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was seen in patients that have CNS involvement  or central nervous system involvement is that   not all of them they have a positive DNA and  only 38 percent will will have a positive early   about 48 percent have a positive SSA and only  six percent will have a positive SSB antibody  
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as I just mentioned in shogron that affect your  brain and your spine only 40 to 50 percent of   them will have a positive SSA antibody and only  six percent will have a positive SSB antibody   and this is important because that makes  the diagnosis very challenging this type  
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of antibodies SSA antibodies they actually have  been associated with a more aggressive disease of   the brain what does it mean it means that if you  have SSA antibodies with neurological involvement   that could mean that you have a more aggressive  disease towards your brain or your spine we do  
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use other tests like visual evoked potential which  are abnormal in 61 percent of patients we use EEG   or Electro as a pallogram which sometimes has  a limited value but it can be useful to detect   subclinical signs of neurological involvement we  also use MRI and I’m going to take the time to  
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explain to you the value of MRI in neurological  involvement of shogron MRIs are more sensitive   that CAT scans to detect anatomical abnormalities  in primary CNS or primary neurological shogran   there are multiple areas of increased signal that  will show inflammation specifically located in the  
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subcortical and periventricular white matter  and those lesions are found more frequently   in patients that have involvement of their brain  let me talk to you more about brain MRI findings   in sjogren I mentioned to you about white matter  lesions and I made the comment that they have to  
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be located in certain areas like periventricular  areas and this makes the diagnosis challenging   because some patients with multiple sclerosis they  can have the lesions in the same areas we’ll talk   more about that in a little bit in some patients  we do see signs of cerebral Venous Thrombosis and  
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in other patients we do see two more like lesions  that are actually not tumor but there is a sign of   program syndrome let’s talk about spinal MRIS  spinal MRIs are ordered to evaluate the spinal   cord involvement and they can show in patients  with sjogren intensities or hyperintensities in  
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the cervical area most of the time 82 percent of  patients will have that problem or they can have   extended lesions in cases of acute myelopathy  this is an MRI of a patient with Hyper signal   in the cord that is suggestive of acute myelopathy  this is also an MRI from a patient with extensive  
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transverse myelitis this is also another MRI of  a patient with neuromyelitis Optica a patient   that also has shogron this is another case of  neuromyelitis optica where you can see involvement   of the dorsal midbrain and and the point in  lesion in a patient with sjogren that develop  
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neuromyelitis Optica we also use combinations of  tests like MRI and a voxel-based morphometry and   this is a method commonly used to quantitate and  objectively evaluate the differences in Regional   cerebral volumes this type of test was able to  shown that patients with sjogren had certain  
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areas of white matter hyper intensities and those  areas were also associated with more atrophy these   studies show that patients with primary children  that have this white matter intensities and gray   and white matter atrophy those are probably  related to a sort of cerebral vasculitis or  
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inflammation in the vessels of the brain there  are other tests like single Photon emission CT or   pet scans that can evaluate the blood flow in the  brain and it was shown that patients with children   they have reduced cerebral blood flow they have  brain atrophy and decreased glucose metabolism in  
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the brain in certain patients neuropsychological  testing it’s also very important to evaluate   symptoms that are very subtle in affecting the  brain cerebral angiography is used more rarely in   patients with primary sjogren but when it was used  in 45 percent of Highly selected patients with  
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children and active CNS involvement they actually  shown to have small vessel vasculitis in the brain   it is important to differentiate children from  other diseases and let’s talk about shogron   versus multiple sclerosis the brain involvement  in sjogran can mimic multiple sclerosis and it  
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is very hard to differentiate even for the most  experienced clinicians Sienna shogron or brain   involvement in the sjogran can mimic a type  of multiple sclerosis that we call relapsing   remitting Multiple Sclerosis but these two  diseases they have features in common like they  
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both tend to involve the brain they both tend to  involve the spinal cord and the optical tract let   me show you some differences between neurological  sjogran and multiple sclerosis shogran with brain   involvement tends to affect people over the age of  40. many of them are females and not many of them  
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will have clinical criteria for multiple sclerosis  when we do the spinal fluid analysis only 30   percent of them will have molecular coronal bands  when we do the MRI of the brain only 40 percent   of these people will meet criteria for multiple  sclerosis SSA SSB antibodies are more frequently  
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seen in patients with brain involvement in sjogran  compared to Multiple Sclerosis about 50 percent of   patients with sjogran and brain involvement will  have these antibodies positive while only five   percent of people with multiple sclerosis will  have an SSA or SSB antibody positive multiple  
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sclerosis tends to appear at younger age between  20 and 40 years old and only 65 percent of these   people will be female now when we look at the  spinal analysis 90 of them will have oligo coronal   bands in multiple sclerosis eighty percent  of them will meet MRI criteria for multiple  
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sclerosis and only five percent as I said will  have an SSA or SSB antibody positive there are   some signs and symptoms that goes against multiple  sclerosis and goes towards more primary children   for example when both peripheral and cranial nerve  involvement are affected then that goes against  
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multiple sclerosis when we see less oligroclonal  bands in the spinal fluid that again goes towards   shogren unless the words multiple sclerosis when  we see more Ana positive SSA and SSB and positive   motive factor that goes against multiple sclerosis  and when we see zika symptoms like dryness in  
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the mouth and dryness in the eyes that goes  against multiple sclerosis and more towards   Sjogren’s syndrome how do we treat neurological  involvement in shogran when it comes to treatment   of brain or spine involvement in sjogren  there is no consensus between us we can  
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use corticosteroids usually we use them in high  doses when we see neurological involvement that   can put the patient in danger but sometimes  corticosteroids are not effective especially   in patients with spinal cord involvement in some  patients we do consider to use immunosuppressive  
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therapy like cyclophosphamide or rituximal and  there are also reports about using il-6 inhibitor   like tocilizumab however the results are variable  and we have to adjust that to the patient needs   however that could result in partially recovery  or just to stabilize the disease plasmapheresis in  
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combination with prednisone was also reported to  be affected in some patients especially patients   with acute transverse myelopathy in severe  cases IVIG was also used successfully in a   small group of patients however the role of IVIG  is more recognized towards small fiber neuropathy  
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there are multiple reports that show that IVIG  is helpful for patients with peripheral nervous   system involvement in conclusion neurological  involvement of the spine and the brain is common   in patients with primary sjogren sometimes it can  come before the dryness of the eyes or dryness  
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of the mouth now how often does it happen we  still don’t have very clear idea because of the   heterogeneity of these studies that I presented  it could be disabling and you have to know about   this type of disease for you to call your doctor  and discuss the symptoms that you have if you have  
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any symptoms that I presented now we need more  studies more prospective control studies with   larger number of patients to assess the treatment  and the efficacy and safety of the treatment for   brain involvement in sjogren and with that I would  like to thank you and wish you a very good day see