It Must Be Voodoo... and he was Far from Spineless (slideshow)

Maybe it is Voodoo. Patient is a 31 year old male with bilateral optic neuropathy, hearing loss which began following a bout of 'aseptic meningitis', presented with the new symptom of low back pain. Past skin, bone marrow, liver and a brain biopsy had been unrevealing. A past imaging study revealed bilateral optic nerve enhancement. A past lumbar puncture showed 270 white cells and a protein of 140. The rest of his work up had been unrevealing including a negative Gallium Scan, Pet Scan, ACE level and Lysozyme titer. An updated study of his axial skeleton radiologically finally clinched the diagnosis. Biopsy of the patient's L1 pedicle confirmed the radiologic diagnosis of non caseating granulomatous disease, that some may equate with Voodoo. Though sarcoidosis involving the axial skeleton is unusual, it is radiologically a classic finding and very important to recognize.

It must be Voodoo… and he was far from Spineless Norah Sydney Lincoff MD, Charles Chung MD, Lucia Balos MD SUNY of New York at Buffalo Jacobs Neurological Institute Walsh Society Meeting 2017 Washington DC No disclosures related to this presentation It must be Voodoo…  35 year old gentleman presents with new neck and back pain for 2 weeks  FOUR+ year hx of bilateral blindness, hearing loss and imbalance from an aseptic meningitic process  TWO years into his illness he escaped Tower One with the help of kind humans It all began following a Graveyard tour in New Orleans ….6 boys ran off without paying History     PMHX:  Splenectomy age 13 s/p trauma  Hypertension  Hypercholesterolemia  Obesity FMHx:  Father MI age 55 Social Hx:  Non smoker, social alcohol use, no illicit drug use  Employed as a banker Allergies: None History and treatment      Chronic relapsing optic neuropathy L>R x 4+ years Imbalance Hearing loss (bilateral) Weight gain and diabetes from chronic steroids Anemia from chronic immunosuppression       IV steroids and prednisone tapers IVIG Cyclosporine Imuran Cytoxan Chlorambucil Examination      Alert and Oriented VA:  20/40 with searching R  HM L EOMF  No nystagmus Slit lamp  Clear and deep with no inflammatory changes  Pupils poorly reactive  IOP 16/15 on glaucoma drops Fundi  Bilateral optic atrophy with vascular narrowing, no vascular sheathing or cells in vx Year 1 Year 4 Examination  Goldmann Visual fields 1st bout of optic neuropathy 4th bout of optic neuropathy ? Past work up retrieved from ‘paper charts'  Labs: Negative   MRI available:   CBC, Biochemical Profile, Sedimentation rate, ANA, CH50, C3/C4, Thyroid panel, SPEP, ANCA, ACE, RF, Lysosyme, Lyme, VDRL, FTA, HIV, COAGS, CSF Ace, Crypto, Herpes Virus, malignancy Vague perineural enhancement and optic atrophy LP1:      WBC 8 lymphocytes Protein 24 Glucose 88 -OB, MBP nl, IGG synth 4.1 nl, +IgG/Alb 0.32, +Index 2.4 Negative gram stain and culture Imaging Available   MRI Axial T1 fat suppression Post Gad Flair Past Biopsies  Brain Biopsy R temporal lobe non diagnostic: reactive perineuronal satellitosis, few focal perivascular collections of mononuclear cells, normal dura  Liver Biopsy non diagnostic: fatty liver  Skin Biopsy non diagnostic: dermal fibrosis with nonspecific inflammation Brain biopsy Skin biopsy Imaging  Chest X-ray  Normal  Gallium Scan:  Normal  Lumbar X-ray:  Normal ? more tests? more biopsies? more Voodoo? MRI of the Lumbar Spine T1 T2 A Biopsy was performed Core biopsy of R pedicle and body of L1 Sarcoid of the Axial Skeleton Hx of treatment of presumed CNS Sarcoid So was this Voodoo? Of course not!  We went years treating him for sarcoid, only to finally find diagnostic tissue in the bone  Did it make us feel better? Of course  Intraosseous sarcoid involving the spine is rare but can be suggestive of sarcoid in the appropriate scenario Late involvement of the axial skeleton with Sarcoid         Uncommon with only 1-13% of patients with sarcoidosis affected Present with joint or back pain Osteolytic lesions typical A lacelike honeycomb appearance More common in the phalanges of the hands and feet Pathologic fractures and bone collapse occur The articular spaces and disc spaces usually remain intact DDX : vertebral metastases, multiple myeloma Highest suspicion of Sarcoid was Year 2 Past Reports  LP2:       (during encephalitis) WBC: 270 Protein 140 Glucose 85 Cx, Gram stain, Ace, Flow cytometry negative -OB, -CSF kappa Chains Imaging 7: Only a drawing in chart available    ?Enhancement of the pituitary infundibulum Left >Right perineural optic nerve enhancement 1cm mass with contrast enhancement posterior R optic nerve and chiasm bordering the right ICA Bottom Line Treat… for often you will never find diagnostic tissue of this Voodoo Condition 