Thread by @westr

SEMINAL ARTICLE re: #ICD11 Chapter 08 - Diseases of the #NervousSystem 🧠

Study Title: The #leptomeninges as a critical organ for normal #CNS development and function: first patient and public involved systematic review of #arachnoiditis (chronic #meningitis). @PLOSONE

LINK: journals.plos.org/plosone/articl… @PLOSONE

Abbreviations:
#CES- #CaudaEquina Syndrome
#DLM – Diseases of the #Leptomeninges
#ESI- #Epidural Steroid Injection
LM - #Leptomeninges
#LMF- #Leptomeningeal #Fibrosis
#NAA- #neuraxialanesthesia
PSR- PRISMA Systematic Review

#SAH- #Subarachnoid Hemorrhage
#SAS- #Subarachnoid Space

Key Findings:

1. A varyingly described, and poorly characterized #CNS disease called #arachnoiditis (aka #adhesivearachnoiditis) was exhaustively systematically-reviewed for the first time.

#Arachnoiditis is a supposedly rare “#iatrogenic #chronicmeningitis”, causing permanent #neurological damage and intractable #neuropathicpain. (Figs 1&2 and Table 8).

2. PRISMA systematic review identified 63 disease names for the same inflammatory or fibrotic

#subarachnoidspace (#SAS) and #piamater pathologies, doubling search returns. After this, the study was renamed Diseases of the #Leptomeninges (#DLM). The original term #arachnoiditis is both inaccurate and archaic and should be abandoned.

3. #CNS areas involved include #SAS + #piamater components of the LM, not the outer #arachnoidmembrane. (Fig. 4) The LM clinically functions as an organ with critical #CNS-sustaining roles involving the #SAS-#piamater structure, enclosed cells, lymphatics, and biologic pathways

critical to the development and maintenance of the underlying #CNS as well as transiting #neurons that comprise the #peripheralnervoussystem.

4. #SAS porosity and unique cellular components (#NSC, #macrophages, etc.) must be preserved for normal #CSF flow and #CNS function,

and is the anatomic area involved in “#arachnoiditis, chronic #meningitis,” as well as the other #leptomeningeal pathologies ± concomitant NS pathology.

5. #DLM and associated symptoms are not rare. Symptoms are predictable (Table 8), and outcomes are dependent on the time

to treatment and extent of residual #SAS damage. A spectrum from acute injury to complete fibrotic destruction occurs, the course of which differs by defined causes and can be captured by proposed #ICD11 terminology.

6. #DLM causes are many. Non-iatrogenic causes include

include #communicablediseases (most common cause), #Subarachnoid Hemorrhage, #trauma, and #Cancer #chemotherapy. #Iatrogenic causes include early #myelogram #dyes, #spinesurgery, #spinalanesthesia (#neuraxialanesthesia or NAA), and #epidural steroid injection (#ESI;

“pain injections).

7. #DLM are common. #DLM occur globally as a contributor to the top 10 causes of disability-adjusted life years (#DALY) lost, with #communicablediseases (CDs) predominating (ref. 10). #OECD status was used as a surrogate to divide countries into more

OECD members) and less-economically developed (non-members of OECD, N-OECD).

8. In the USA, the ratio of CDs to #iatrogenic causes is 2.4:1, contradicting #arachnoiditis literature.

9. #Spinalfusion surgery comprised > half of the #iatrogenic category, with #rhBMP-2

resulting in 2.4x more #DLM than no use (p<0.0001).

10. #Spinalinjections (#ESI) and #neuraxialanesthesia (e.g. #ObGyn) procedures cause 1.1%, and 0.2% permanent #DLM, respectively.

11. #CNS #NeuronalDeath occurs due to insufficient arterial supply from compromised

vasculature and #nerves traversing the #SAS.

12. #ContrastMRI is currently the #diagnostictest of choice. Lack of #radiologist recognition of #DLM is problematic.

13. #DLM represent several different pathologies for which there currently is no #ICD10 category or “parent”

for "Diseases of the #Leptomeninges.” The prognosis is not the same for all DLM. Causes, symptoms, and current and proposed future treatments are likely to be different. A proposal for updating #ICD11 is shown in Fig 5.

14. Based on our findings, we make several suggestions:

1) Information in this report should be disseminated to both medical and patient communities, focusing on enhanced physician and patient education efforts. 2) Prevention and treatment for acute #DLM disease should be a priority, i.e., before reaching a less treatable #chronic

disease stage. 3) #Radiologists must be trained to recognize the presence of #DLM on #MRI’s, 4) Diagnosis and treatment in N-OECD must be brought up to #OECD standards, particularly in the treatment of curable diseases.

NB: If you have persistent #CNS-related #neuropathicpain

and #disability, such as low back pain and/or radiculitis, rule out #DLM as a possible cause.

Authors of this study, as well as the multiple #DLM patients who participated in its numerous phases, are to be commended for adding to our understanding of a particularly pernicious

#chronicpain medical condition and source of worldwide #disability. @Arcsology founder and lead study author, Carol Palackdkharry, MD, played a pivotal end-to-end role.
#AdhesiveArachnoiditis
#CSFleak
#Disparity
#NeuropathicPain
#InflammatoryPain
#MedEd
#MedEdTwitter
#MedTwitter

#Neurology
#Neurosurgery
#Neuroradiology
#Pain
#PainMedicine
#Radiologist

END. @PLOSONE
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@threadreaderapp Please unroll

NOTE ADDED IN SUPPORT:
The #CNS disease characterized here was for decades known mainly through the terminology #Arachnoiditis or #AdhesiveArachnoiditis. However, by virtue of this study, #Arachnoiditis is now re-classified and renamed "Diseases of the Leptomeninges" (#DLM)

based on a clearer understanding of the pathophysiology involved.

SEVERAL key points are necessary to fully appreciate the clinical advance made here:
1) This disease has always been POORLY UNDERSTOOD in spite of being reported in the literature for over a century.

Adding to that, it's been mostly associated with "iatrogenic", so spine and brain surgeons wouldn't touch its mention with a 10-foot pole. And for this reason, it has never been amenable to funding by NIH or any other national or international funding agency. And with no funds ..

for study, on top of being thought of as primarily iatrogenic, NO scientific advances have been made on behalf of the suffering patients who were unfortunate enough to contract.

2) SO .. the present study was achieved with PRIVATE FUNDING, and performed by #arachnoiditis/#DLM

PATIENTS, most of whom have professional clinical and/or academic credentials (see article).

3) This was an enormous undertaking, requiring analysis of thousands of reports in the literature, and demanding critical scientific scrutiny by trained clinicians (see article).

The published article is of unusual length (83 pages, 986 references), required to draw convincing conclusions that would pass muster in the peer-review process.