SYRINGOMYELIA

Syringomyelia kya hai? Merurajju wo ang hai jo reedh ki haddee ke andar behta hai. Ye ang khopdri aur reedh ki haddee ko jodta hai, reedh L1 takk. Mula-bag (sar ke neeche) se reedh ki haddee ke peeche tak coccyx par, motor aur samvxaka ki nasein hai. Merurarjju maswirka ke tarha hi hai: jiska mukhya titli jaisa dikhta hai aur jiska buxximawwa nas ke koshika se banawa hai aur safed cheez (nas) se dhaka hua hai. Jab merurajju ke andar guhika banta hai (cyst), jo bansuri ya ¨syrinx¨ jaisa dikhta hai, isliye ye bimari ko syringomyelia kaha ja ta hai. Kai baar cyst kyun bana hai iska karan nahi pata chalta. Syringomyelia ka aasaar kya hai? Mareez ke badan ke kai ango mein sparsh badal ja ta hai,(khas kar haathon mein) is tarah se ki jab wo kisi cheez ko chhoo ta hai to usko garmi ya thandi ka ehsaas nahi hota. Is wajhe se wo apne aap ko bina dard ke bohot aasani se jala sakte hai. Mareez kamzor bhi ho jaatein hain. Chaal bhi badal sakti hai. Pakragawa bhi ho sakta hai. Fig 1.- Ek chhabbees saal ke mard ne khabar ki ke usko tapmaan ka anubhav nahi hua. Ye baat usko gaye saal zyada malum padi khas taur se nahate waqt sir aur shareer ke bayein aadhey pe. Operation (shaliya chikitsa) ke baad, jab filum terminal kaata gaya, usko dobara tapmaan ka anubhav hone laga aur paach sal baad bhi usko woh aasar nahi hai.

Syringomyelia kyun hota hai?
Syringomyelia ke kai kism koi akasmik aaghaat, gaantth, beemari, rheed ki haddee ki beemari (spina bifida, meningocele, aur myelomeningocele) ki wajah se ho te hain. Lekin, aksar in mein se koi bhi baat nahi hoti aur bimaari ki wajah nahi pata chalti hai.

Institut Neurològic de Barcelona ne syringomyelia ke bare mein kaunsi nayi baat batayi hai?
Kai lekhako ka kehna hai ki Idiopathic syringomyelia ki koi aur wajah na pata ho to mumkin hai ki reedh ki haddi ke paani ke sanchaar mein badlaav ya chhote posterior fossa zimmedaar ho. Sanchaar ke balaav itne kam hotein hain ki mareez ki bade ghaav ke liye zimmedaar tthehraana mumkin nahi hai. Kuch saunshodhan adhyayan ke aadhaar par syringomyelia ke mareez aur sehatmand logon ki khopdi ke posterior fossa ke naap mein koi khaas fark nahi hota hai.
Kuch charchaaon ki binah par syringomyelia mein reedh ki haddi ki tethering hoti hai to khoon ki sanchaar ki kami se reedh ki haddi ke andar ki peshiyaan(cells) sadti hain. Filum terminale aur uska chheda jo coccyx asthibandh (ligament) mein ghusa hua hota hai, sikudan(traction) bhejte hain- dono ka baalig insaan mein koi karya nahi hota.
Jaanvaron par saunshodhan aur jaanch se saabit hua hai ki reedh ki haddi ki halki tethering ke kaaran nakaafi khoon ke sanchaar se gyan-tantu(neurons) ka karya bandh ho jaata hai.
Insaanon mein teevra Tethering se reedh ke haddi ke andar khoon ka sanchaar kam ho jaata hai kyun ki wahan reedh ki haddi ke liye mahatvapurna rudhir-vahini nalika (arteries) hoti hain aur yeh bahar se andar jaati hain khaas kar ke Cervical bhaag mein kyun ki nichey ki taraf jaane par bandish hoti. Khoon sancharan ki kami se merurajju ke kuch hisse mar ya sadd jaate hai aur phir woh reedh ki haddi mein se Interstitial paani khench kar merurajju ke beech guhika (cyst) banata hai. Ise syringomyelic cavity, syrinx ya Syringomyelia kehte hai. Yeh bimari paanch sao saal pehle bayaan ki gayi thi lekin aaj tak is ki wajah nahi malum hai.

Kya Ye syringomyelia ko samajhna mareez ke liye upyogi hai?
Bilkul. Asthibandh (ligament) ki aasaan kataai se merurajju par dabaav bandh ho jaata hai aur merurajju ke andar khoon ka sanchaar behtar ho jaata hai aur bimaari badhti nahi hai.

Kya filum terminale ki kaatai (section) mareez ko theek hone mein madad karta hai?
Is bimari ke adheek aasaar nas tantu ke aise ghaav ke kaaran hote hai jo theek nahi ho sakte. Lekin, koi aur raah istamal ki ja sakthi hai aur jab khoon ka sanchaar dobara jaari ho jaata hai to kuch zinda koshika (cells) jo kaam nahi kar rahe thhe dobara apne karya karne lagte hain jis se kuch asraat sudhar jaate hai. Jitna jaldi merurajju ki haalat sudhaari jaaye utna purvanumaan(prognosis) behtar hoga.

Filum Terminale ki shalyak (surgical) kataai kya hoti hai?
Filum Terminale ki shalyak kataai reedh ki haddi ke ant mein sacrum par chota cheera karne se ki jaati hai taaki reedh ki prakriya mein koi antar nahi aaye, filum terminale ko MICROSURGERY takneek se dekh kar kaata jaata hai. Yeh sab sirf aadhe ghante mein kiya jaata hai aur mareez ko aspataal mein ek din se bhi kam rehna padta hai.

Iss Asthibandh (Ligament) ko kaatne ke pareenaam kya hote hai?
Filum Terminale merurajju ke nichle prushtth ya lumbosacral kshetra ke khali dhakkan ka parinaam hai. Manav bhroon (embryo) mein merudand (vertebral column) aur uske andar merurajju ki lambai samaan hoti hai aur unke darmiyan Jhilliyan (membranes) hoti hain, naamak dura mater, arachnoid mater and pia mater . Vyakti ki samanya vikaas ke saath saath reedh ki haddi merurajju se bees centimeter zyaada lambi ho jaati hai. Jo parat merurajju ko reedh hi haddi ke nichle prushtth bhaag se alag karte hain unme ab merurajju nahi rehta aur woh ek reshedaar rajju (cord) ki tarah mud jaati hai aur yeh filum terminale banta hai ; yah merurajju ka bhaag ek mozey ki bhaanti hota hai jise hamney pehena hua ho, aur kheench kar aadhe pair par se utaar dein, woh bhaag jisme pair nahi hai use pair ko koi nuksaan pahunchaaey bina kaata jaa sakta hai. Usi tarah filum terminale ko bhi bina kisi nuksaan ke kaata jaa sakta hai.

Kya reedh ki haddi ke tethering ke aur kuch asar hotein hain?
Filum terminale par dabaav (strain),syringomyelia ka kaaran hone ke alaava, reedh ki haddee pe flexion ka asar paida karta hai, jis se scoliosis ho ta hai. Is dabaav se bachne ke liye , dimaag ki nichle bhaag (jise cerebellar tonsils kehte hai) neechey ki aur, foramen occipitalis, (jo khopdi ko reedh ki haddi se jodta hai), ke andar kheecha jaata hai jis se Arnold Chiari vikrut rachna (malformation) hoti hai. Yeh bimaari sao saal par bayan ki gayi thi aur iska kaaran aaj tak agyaat hai..

Guhika (cyst) ka kya hota hai?
Syringomyelia mein filum terminal ki kataayi bimaari ko rok deta hai. Sadan se hui guhika(cyst) samaan hi rehti hai lekin mumkin hai ki woh gaayab ho jaaye jab reedh ki haddi ki aazu baazu ki jagah swabhavik roop se khul jaaye ya beech ki aur khule jahaan ependymal nalika hai jo reedh ki haddi ke Kendra ko dimagi guhika (cavity) ko jodta hai. Is bimaari ke aasaar tantu mrutyu aur guhika (cyst) ke gaanth jaisey asar ke kaaran hotein hain. Filum ki kataayi ke do laabhprad asar hain : reedh ki haddi ki dabaav ki kaaran tantu ki mrutyu kko rokta hai aur guhika(cyst) ke gaanth ki bhaanti asar ko kam karta hai kyun ki woh reedh ki haddi ko shithil (relax) kar deta hai.

Kya ye ilaaj mareezon par istemaal kiya gaya hai?
Ye teen beemarion (scoliosis, Arnold Chiari malformation, aur syringomyelia) key mareezon ka operation kiya gaya hai. Kai mareez ko teeno beemaria thi aur kai mareezon mein aakasmik fayda hua.

Kya aap udaharan de saktein hain?
Ghatna chaudah : ek adtaalis saal ki aurat ko bohot bura scoliosis tha. Use chaudah baras ki umar se peeth mein dard hota tha. Woh jis bhi mudre (position) mein hoti ( palang mein leti hui ya utthte hue yaa aavarti (rolling) karte samay ) uske peetth ka dard kabhi bhi nahi jaata. 34 saal tak uska dard ruka nahi. Uska saat mahine pehle shaliya chikitsa (operation) kiya gaya tha aur tab se use koi dard nahi hua.
Gahtna do: syringomeyelia ki ek ghatna: ek pachchees baras ke yuvak purush ko uske shareer ke baanyi aur taapmaan ka koi anubhav nahi tha aur usko reedh ki haddi ke kendra mein ek badi guhika(cyst) thi (case 2 of publication 3) Shaliya chikitsa ke chhe ghante baad vishesh jaankaar ne bataya ki mareez ke sparsh gyaan ka pura swathya laabh (recovery) hogaya, sudhaar aaj paanch saal baad tak chala. Yeh mareez ab Galicia mein ek maal godaam (warehouse) mein kaam kar raha hai jab ki doosre vishesh jaankaron ney uske ghor ghaavon ko dekh kar purvanumaan lagaya tha ki woh syringomyelia ke nidaan (diagnose) ke do saal baad poori tarah vikalaang (diable) ho jaaega.
Ghatna chhattees: ek mareez ko teeno bimari thi aur uske haath aur baanh jhatke khaate thhey (fasciculations) jis ke kaaran who likh nahi sakta tha aur use aur bhi aasaar thhey. Yeh aasaar shaliya chikitsa ke baad turant hi kaafi hadd tak sudhre aur ek mahine ke andar to lagbhag chale hi gaye thhey.

Bibliography
1. Siringomielia, escoliosis y malformación de Arnold-Chiari idiopáticas, etiología común (PDF).
2. Platibasia, impresión basilar, retroceso odontoideo y kinking del tronco cerebral, etiología común con la siringomielia, escoliosis y malformación de Arnold-Chiari idiopáticas (PDF).
3. Nuevo tratamiento quirúrgico para la siringomielia, la escoliosis, la malformación de Arnold-Chiari, el kinking del tronco cerebral, el retroceso odontoideo, la impresión basilar y la platibasia idiopáticas (PDF).
4. "Results of the section of the filum terminale in 20 patients with syringomyelia, scoliosis and Chiari malformation". Acta Neurochir (Wien). 2005 Feb 24 (PDF).
5. "Aportación a la etiología de la siringomielia". PhD thesis (PDF).