Medical History Girish Phanje, M- 39 Yrs. March 21, 2014 1) Problem of sensory deficiency in left leg (on thigh areas) first reported in Oct-2008. a. MRI findings Oct-2008: Intramedullary mass lesion in spinal cord from C7-D3, ? Atrocytoma. b. Second opinion MRI (Lucid) Nov-2008: Intramedullary spinal cord lesion, involving posterior columns, extending between D1-D5 levels. ? Astrocytoma ??Ependymoma. c. Consulting Neuro physician : Dr. K. Bhaskar Rao (Dr.A.S. Rao Nagar, Practicing at Sri Krisna (SKS Hospital, Uppal) d. Medicines : ATT+ 5mg Wyselon on Dr. Deopujari (Bombay Hospital, Mumbai) advise, no relief. All blood reports (for TB) and chest X-ray : clear. e. Problem suddenly increased, difficult even to laugh as the same was giving extreme pain in chest & back. MRI repeated in Dec-2008, found with substantial increase in lesion. 2) Laminectomy with lax duraplasty with fascia from left thigh : Jan-2009 : By Dr. Chandrasekhar Deopujari, Bombay Hospital, Mumbai. HPR : Non necrotizing Granuloma. No culture has been done. Pre-op Body profile tests including Syr. ACE, X-ray :Normal Post-op Medicines : ATT + Tappered Steroid from 40mg. (R-cinex-600mg, PZA-750mg, ETM-800mg, Wysolone 40mg/tapered to 1 month, Shelcal-500mg) a. No relief. Sensory deficiency increased from legs to hand. b. April-2009 : MRI suggest : Significant increase in the extent of cord edema and enhancing mass lesion between C3-D5 c. May-2009 : ATT 2nd line drugs started ( EHRZ+ Ethionamide-250mg, Cyclocerene-250mg), Adv : CT-Chest, MRI Brain with contrast. d. July-2009 : MRI Brain with contrast: Findings : Megacisterna Magna-Congenital Variant. No other abnormally detected & overall normal report. e. No Relief and frustrated. f. April-2009 : ACE : Increased. Same (ATT) medicines continued based on several Dr. advise, X-ray chest : clear. g. All second opinion (from NIMS, TATA Memo) on biopsy paraffin block : Ganuloma, only Ranbaxy said its possibly TB but could not correlate with other findings. h. Went to Yashoda, Raj Bhavan Rd, Dr. Jaydip Ray Chaudhuri. 3) Aug-2009 : Dr. Jaydip Ray Chaudhari : Advised to continue same medicines, Also refered to Dr. Venkateshwara Rao, (general medicine) and Dr. Liza Raj sekhar, NIMS (Rheumatologist). a. Aug-2009 : CT Chest (without contrast) : Soft tissue density lesion in subcarnial region-Likely enlarged lympnodes. b. Ultrasound of neck and bilateral axilla : sonographically benign morphology lymphnodes in bilateral submandibular (SAD : 8mm), Left axilla (SAD : 6mm), ? Reactive. c. ATT : Continued till Jan-2010 (About 1 year). All these time, Liorisal (Baclofen) taken in varying quantity depending on plastisity. d. Went to KIMS : Dr. Sita Jaylakshmi : She confirmed Neuro sorcoidosis based on ACE, CT, MRI Lymphnodes findings. 4) Jan-2010 to June-2010 : Stopped all medicines, taken only baclofen. Searched many doctors, visited Dr. Sita Jay Lakshi (KIMS). a. June-2010 : MRI : Persistent intramedullary lesion within cord with increase in extent as compared to the previous imaging studies. Presence of hillar and mediastinal lymphadenopathy, Neurosorcoidosis needs to be ruled out. b. June-2010 : CT guided biopsy from subcarinal Lymphnodes : The histological features in correlation with clinical findings are of granulomatous lymphadentis. AFB and GMS stain : Negative. No culture done. c. June-2010 : Treatment for sorcoid started with 1gm X 5days Solumedrol injection, followed by tapered dose of Predisolone 30mg, Methotextrate (5mg+5mg once a week) with supplementary medicines. d. Aug-2010 : 1gm solumedrol repeat for 1 day. HCQS-200mg per day added to above. e. No improvement. f. Same continued till Dec-2010 with last 5mg dose in Dec-2010. From Jan-2011, steroids stopped. 5) Aug-2011 : HCQS Stopped. Continued with Methotextrate (5+5mg once a week) only with Mecalvit-500mg and Folonol-5mg. Dr. Sita of the opinion to go for Cyclophosphamide pulse theory also, but not taken so far as Rhematologist was not confident. 6) Aug-2011 : MRI with contrast : Spinal cord from D1-D3 shows linear hypertense signal along with posterior surface of the cord tethering of the cord posteriorly to the dura. Subtle hyperintensity along the posterior surface of the cord at D4-D5 levels., descrete nodular enhancement posterior surface of cord opposite C2-C3, C-5, C7-D1 level., plaque like intramedullary enhancing lesion along the posterior surface from D1-D-5, Screening of mediastinum shows enlarged mediasitnal lymphnodes., significant reduction of plaque burden compared to previous study., PET CT may help to look for the activity of disease. 7) March-2013 : Same medicines continued i.e., Methotextrate (5+5mg once a week), Folinal 5mg OD, Mecalvit-500mg OD without any relief. Symptoms are detorating with left leg almost dead, both legs shaking uncontrollably when stretched, extreme burning in back, strong belt like feeling on chest making impossible to breathe, urine extreme urgency, Sometimes pain in arm pits/mussles. Not able to run. Not able to stand on road devider as even a wind of the passing vehicle gives the feeling that I will fall down., Needs to watch the stairs while going down. 8) March-2013 : MRI with contrast repeated : Report enclosed with few images of the same. 9) Probably need to think beyond sorcoid... Tethering ? but why ??