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Discussion Starter · #1 ·
Hi all,

I've been asked by some to list the 19 possible presentations of Central Nervous System involvement of Lupus. These are recognised by the ACR (American College of Rheumatology) as part of their different criteria used to help diagnosis of CNS Lupus.

Many of you will have read about the 2 official ones that are included in the ACR Criteria which are Psychosis and Central Nervous System Vasculitis (this one comes under the umbrella of Cerebrovascular Disease in the list below).

Because of the variation in criteria the College have case definitions, reporting standards, and diagnostic and testing recommendations of the 19 neuropsychiatric SLE syndromes.
Good Neuro's should be aware of these if they have kept up to date.

The 19 possibles surprised me as some I would never have thought of as being possibly linked to Lupus. I had a fairly obscure first presentation of a movement disorder and an unusual one at that. Chorea is the more likely movement disorder found with SLE, but I first manifested with Dystonia as one of my symptoms.

I think it's helpful to be aware of these presentations especially if you are seeing a Neuro and they are unsure what the reason for your presentation may be.


Acute Inflammatory Demylinating Polyradiculoneuropathy (Guillain-Barre' Syndrome)

Aseptic Meningitis

Autonomic Disorder

Cerebrovascular Disease -. Neurologic deficits from arterial occlusion, venous occlusive disease or haemorrhage, e.g. stroke syndrome, TIA, crhonic multifocal disease, subarachnoid and intracranial haemorrhage, sinus thrombosis

Demylinating Syndrome

Headache : Migraine, tension, cluster, headache from intracranial hypertension, intractable headache nonspecific

Mononeuropathy (single/multiplex)

Movement disorder - most common is Chorea

Myasthenia Gravis

Myelopathy

Neuropathy - Cranial

Plexopathy

Polyneuropathy

Seizures and Seizure disorders

Acute Confusional State

Anxiety Disorder

Cognitive Dysfunction

Mood disorders

Psychosis

The Neuropsychiatric manifestations of SLE are varied and may be classified as primary neurologic & psychiatric disease. Primary (e.g. related to direct involvement of the central nervous system), and secondary disease (e.g. related to complications of the disease and it's treatment). The latter are much more common and can be produced by a variety of mechanisms.



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Lily
 

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Extremely useful Lily. Bearing in mind that there are only 2 NP criteria on the famous ACR list


Thank you :)

Clare
 

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Question: There are only 2 of these on the "official" ACR list of 11 criteria. But I had heard that the ACR was considering including all 19 of these. Is it official, or are they still considerations?
 

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Discussion Starter · #5 ·
Is it official? Good question. I'd stick my neck out and say yes but then I'm no doc :lol: They do stress that the diagnosis of Lupus must be confirmed first before moving on and considering the possibility of it involving the central nervous system directly.

I'd say they'd be reluctant to publish the full list because so many other things can cause these presentations. But maybe I'm wrong and maybe they will make it public. This is obviously the criteria they go by and no wonder for those of us with these problems our best asset is a good Neuro to liase with the Rheumy. It's pretty mind boggling stuff some of it :wacko:

But the diagnosis still remains a very complex issue for several reasons. Some of them may be on the list but not directly related to an organic problem but as a part of living with a chronic disease and the meds we take and all that entails. I'm thinking particularly some of the psychological presentations would be hard to pin down.

Meds can have side effects on the CNS, Lupus can also attack muscles directly - you can see where the lines get skewed. It's not to be confused with a problem directly related to Lupus attacking the central nervous system, i.e. a Primary presentation. A Primary presentation would require more aggressive treatment with immune modulating drugs, just treating the symptom would not be effective. But if it's a Secondarypresentation treating the symptom may be enough. Or at least bringing Lupus under control and improving lifestyle issues could help. I'm confusing myself now does any of this make any sense :wacko:

Working out which one is which especially as some of us have multi-presentations would be a pretty tough job I'd imagine, depending on our particular presentation or clusters of them, the meds we are taking etc. Maybe that's why they have those two main criteria standing now the Psychosis and the CNS Vasculitis. They are more tangible and the diagnosis is more cut and dried, easier to arrive at. The evidence is staring them in the face.

It's because of this confusion they should pull out all stops and do the correct scanning and a full Neuro work-up regardless. And if things arent demonstrated on first Neuro work up then go back and back until they do see what you are on about. But I really think the key is the MRI's, MRA's, EEG's, EMG's and Lumbar Punctures. Thats difficult for presentations that lean more on the Psych side but there are things like PET and SPECT scans that could probably help there, especially in the hands of an experienced technician. They do have a whole array of stuff they use to prove or disprove this and mostly something will show up.

As with any diagnosis the symptoms + the tests will reveal the correct diagnosis.

This originally came from a Neurologist here who was addressing a Lupus Association meeting - the ACR is his quoted source. They often have guest speakers at these meetings a couple of times a year. The full text and more detailed descriptions are on the ACR site somewhere but it requires subscription and fees.

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Lily
 

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Hello,

Lily this is excellent information and laid out fairly easy to read.
I have pinned this topic so it remains easily available if someone thinks they could have cns/np lupus.

Karen
 

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This site might be useful but I don't know how up to date it is and haven't compared it with other info. All grist to the mill I suppose :)


http://www.emedicine.com/neuro/topic360.htm

Sue
I haven't any up to date information about the Criteria Revision that's supposed to be underway

Clare
 

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Interesting, but very complicated I think! Boy, glad I am not a doctor :lol: No wonder they have so much trouble sorting everything out.
 

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Hi, Lily and everyone,


Please help my mom! She has had severe body or muscle pain for one year. She was just diagnosed with SLE last month. Then she rapidly started to have cognitive dysfunction. She is having confusion and memory problem. She is in China and I am in the USA right now. My relatives in China have told me she might die soon because the lupus starts attacking her brain. I think the doctors are giving her corticosteroids , maybe 60mg a day, by oral. Her weight is only about 85 pounds now. Please suggest me what else she should take? I am desperately needing your help !
 

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I fianlly read today that the 19 descriptions of NP lupus are just that--descriptions. They are not meant to be used for diagnosis. They MAY be used for categorization in studies, though. Maybe one day... :blink:
 

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Discussion Starter · #11 ·
Hi Hong,

I replied to you about your mother on another thread you posted :)

Sue, yes they are descriptions i.e. presentations of what sorts of things can be found and what to be on the lookout for. At least most good doctors would be aware of these possibles now and the fact that they could be linked with central nervous system disease in a Lupus patient. Emphasis on could as all things need to be considered as we talked about before.

One day they will work us out :D :p

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Lily
 

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After being diagnosed a couple of weeks ago, I've been doing research and have found that pseudotumor cerebri ("idiopathic intercranial hypertension") is also connected to lupus. Main symptoms are headaches and visual disturbances (which can lead to permanent blindness), but can also cause hearing loss, tingling or pain in extremities, balance problems, etc.

Just thought I'd add it to the list :)
 

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Discussion Starter · #13 ·
Thanks Tandarat, our list is growing ;)

My curiosity got the better of me and I went searching, here's an excellent article on Pseudotumor cerebri:

http://www.emedicine.com/NEURO/topic329.htm

and it does mention SLE, some people with this have undiagnosed SLE. I know we've had a few come through here with the condition, maybe 3 ladies. I also found interesting this section of the article:


Rule out systemic lupus erythematosus or other collagen-vascular disease, since these have been reported as underlying conditions in some patients who present with IIH (Nampoory et al, 1997).

An increased incidence of anti-cardiolipin antibodies has been reported in patients with IIH. Some authors advocate anti-cardiolipin antibody assessment in IIH patients regardless of prior history of thrombosis (Leker and Steiner, 1998). Some authors advocate screening for anti-cardiolipin antibodies and other procoagulant states in all patients with IIH who are either male or nonobese (Sussman et al, 1997).

Cases of IIH associated with Lyme disease have been reported (Bachman and Srivastava, 1998).
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Lily
 

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There seem to be MANY different forms of Lupus? Is it a wonder why Specialists cant diagnose it. I am just thinking *Ouch* if we need to go thru all of the tests before finding the right one.


TOO MUCH INFOMRATION, scares me - Why cant life be simple as in Lupus or NOT? The information is good and I am trying to copy some of it so I am able to explain, to my consultant some of the different things that are happening in my body - before Fear & The Brain Fog Stops Me. ((THANKS)) donna

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Ha, Intractable Headache, intern eat that! I just got out of the hospital after a 5 day stay for an intractable headache and she said it wasn't lupus related because my MRI was normal. A rheumy wasn't even consulted. Sad. I already have PNS disease... why not CNS disease?
Julia
 

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Discussion Starter · #16 ·
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16380747&dopt=Abstract

[Neuropsychiatric systemic lupus erythematosus: where are we now?]

[Article in Italian]

Govoni G, Rizzo N, Castellino G, Padovan M, Trotta F.

Sezione di Reumatologia, Dip. Medicina Clinica e Sperimentale, Universita di Ferrara, Azienda Ospedaliera-Universitaria S. Anna, C.so Giovecca 203 - 44100 Ferrara, Italia. [email protected].

When dealing with Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) there are still many controversial topics. In 1999 the American College of Rheumatology gave classification criteria for 19 clinical syndromes. However major problems are still related to low specificity of some of them such as headache, cognitive impairment or mood disorders. Even though a frequency of CNS involvement from 14 to 75% has been described, depending on both the population studied and the methodology of assessment, a lower frequency ranging from 21 to 28 % derived by larger case series seems more realistic. The introduction of the concept of "borderline cases", proposed by Italian Study Group for NP-SLE, is based both on clinical and instrumental evaluation and could represent a useful tool when dealing with conditions which do not fulfil ACR classification. Also the relationship between SLE activity and NP involvement is a debated issue. Concerning pathogenesis, it seems reasonable to consider multifactorial mechanisms related to antibody-mediated damage, antiphospholipid pro-thrombotic effect, non-inflammatory vasculopathy and cytokines mediated cytotoxycity. However, direct and unequivocal evidence for the implication of any of the above-mentioned mechanisms is still lacking. Although a wide range of neuroimaging tools have been used to evaluate CNS involvement, no single technique has proven to be definitive and, when dealing with a patient with suspected NPSLE, it is important to combine different diagnostic techniques. Due to the lack of effective imaging along with limitation in knowledge of underlying pathogenetic mechanisms and paucity of histopathologic findings, therapeutic approach in NPSLE remains a difficult issue and is currently based on personal experience. Italian Study Group for NP-SLE proposes the creation of a national registry on NPSLE to validate ACR classification criteria. Furthermore, the possibility to collect large series and stratifying them for each of the included neuro-psychiatric syndromes seems a good strategy for planning multicentric controlled therapeutic trials in the near future.

Publication Types:
  • Editorial
PMID: 16380747 [PubMed - in process]
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Lily
 

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Discussion Starter · #17 ·
Hi everyone,

I've found a slide show on CNS Lupus some of you might be interested in, its very similar to the one presented at the lecture the Lupus Association here in Australia sent me. It contains the information in this post about the different presentations along with diagnostic methods and treatment.

Click to download, doesnt take that long:

www.cerebel.com/lupus/CNScourse.ppt

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Lily
 

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you know it wasnt until i came down with aseptic meningitis that i really started looking back at my medical history. Has anyone else had this illness???

sunsetgirl
 

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i'm still waiting for the preliminary results from my labwork..but this would explain all the weird things that heve been going on with me over the last several years...unexplained hives..started on my arm..off and on for a few days..then suddenly was all over my body..even down my throat..went to E.R...but by then they were almost all vanished
..bilateral carpal tunnel..came on after only a few months of repetitive employment..
...sinus headaches..
..memory problems..
..sudden developement of dyslexia..
..other symptoms..ringing in the ears..IBS..excema/discoid lesions..joint pain..
..and on..and on..
 

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Hi Lily,

My daughter experienced most of these definitions, and still does have many of the symptoms. I am worried to day as she is having increased visual hallucinations and have put out a call to her Rheum. nurse who is contacted the Rheumy. They have increased which worries me. She seems like she is getting sicker than better. I am not sure if it this 2 steps forward and 1 step back theory.

I can only hope the Cyclo. starts to works with future treatments next one is in hospital Oct. 10th

Thanks for the info

Sharon
 
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