What a Pain in the Neck?

Dr Lisa Yeh, DO Maine Medical Center, Internal Medicine Resident ACP Annual Chapter Meeting, Bar Harbor, ME Clinical Vignette 2016

Overview of Initial Presentation • 89 year old female • Presented from home to MidCoast hospital • 1 day of LUQ pain radiating to her back along with emesis and diarrhea. • ED work-up included: – CT abdomen and pelvis – stone protocol • No hydronephrosis or renal stones, moderate narrowing of L2-3 and L4-5. There is a vacuum disc phenomenon at L2-3 through L5-S1 levels.

– UA with >182 WBCs, 4 RBCs, 3+ leuk esterase. Nitrite neg, no bacteria seen.

• Diagnosed with Pyelonephritis – Prescribed Ciprofloxacin for 7 days

Case Continued 9 days later • Patient presented to MMC from home with – severe acute on chronic back pain.

• Completed course of Ciprofloxacin for presumed pyelonephritis – Review of Urine Culture from MidCoast showed: No Growth

• Back pain progressive over 3 days. Started in her lumbar spine and traveled up to her cervical spine and right scapula • + History of back pain but this is more severe • No recent trauma or falls, fevers, chills, headache or altered mental status

Past Medical History Past Medical History •Degenerative Joint Disease •Spinal Stenosis – lumbar •S1 radiculopathy •OA •Hypothyroidism •Hypertension •Shingles •GERD

Surgical History • Oophorectomy, left 1966 • Appendenctomy 1966 • L3-4, L4-5 left mirco lamina foraminotomies 2009 • Left L5-S1 extended laminoforaminotomy for nerve root decompression 1/2016 • Multiple epidural injections, last was 6 months ago

Medications • Gabapentin, amlodipine, levothyroxine, Multivitamin, Fish Oil, Vitamin D, potassium, omeprazole, PRN Oxycodone, PRN acetaminophen

History Family History •Diabetes in parents and sister •Rheumatic Fever – father

Allergies •Penicillin – rash •Lisinopril

Social History Lives with her husband in a house in Georgetown. Completely independent with ADLS and IADLs. • Never smoker • One glass of wine 5 nights/week • No illicit drugs • Born in Germany, moved to US in 1952 • Recent travel to Bahamas 2 months ago

Physical Exam

VS: BP 137/80| Pulse 82 | Temp 98.6 °F (Oral) | Resp 16 | BMI 27.90 kg/m2 | SpO2 94% General: Awake, alert, oriented x 3. No acute distress at rest in bed. Appears stated age HEENT: Mucous membranes moist. No oral lesions. No conjunctival injection Neck: Very Limited, painful neck ROM. Neck pain not worse with palpation. No lymphadenopathy, masses or thyromegaly Cardiovascular: Regular rhythm, normal rate. II/VI systolic murmur, no clicks or rubs. 2+ pulses. No JVD. Chest: Clear to auscultation bilateral with good air entry. No accessory muscle use. Abdomen: Soft, TTP LUQ, non-distended. Normoactive bowel sounds. No hepatosplenomegaly. Extremities: Full ROM. No joint swelling or erythema. Neurological: AAOx3, CN II-XII intact. Strength 5/5. Sensation intact. Reflexes 2+. No point tenderness along spine Skin: No rashes. Raised, pedunculated 5 mm papule on R low back.

Investigations 136 3.5

100 26

14 0.49

15.1 142

18.6 81% PMNs

189 44.1

CRP = 107.8 (0-8 mg/L) Sedimentation Rate = 42 (0-30m/h) Urine Analysis Clear, 1.021 Leuk esterase – neg Nitrite – neg pH 6.5 Ketones 2+ Hemoglobin 10 Erythrocytes 3-5 Leukocytes 10-20 Bacteria 2+ Epithelial cells 1+

Xray Lumbar Spine FINDINGS: A left-sided curvature is unchanged. There are no acute compression fractures. Multilevel disc degeneration with endplate spondylosis is unchanged. L5-S1 facet arthrosis is stable. The sacroiliac joints are normal. IMPRESSION: Stable multilevel disc degeneration and facet arthrosis.

Hospital Course Admitted for; • Axial pain, neck > lumbar and leukocytosis/inflammation • STAT MRI - rule out epidural abscess or other infectious etiology • Blood cultures ordered • CT Chest, Ab, Pelvis

Investigations CT Chest Abdomen Pelvis w Contrast Old fracture deformity of the left inferior pubic ramus. Degenerative changes of the spine. Diverticulosis. Subcentimeter thyroid lesions. No pyelonephritis MRI cervical, thoracic and lumbar Cervical: No signal abnormality in the vertebral bodies or intervertebral disks. Degenerative changes at multiple levels with mild acquired canal stenosis at C5-6 and C6-7 but no evidence of an infectious process. Thoracic: Vertebral body marrow signal is normal. No signal changes in the intervertebral disks that would suggest an infectious process. Thoracic cord demonstrates normal signal intensity. There were no epidural fluid collections. Small bilateral pleural effusions are noted Lumbar: There are postoperative changes as described above including an L5-S1 laminectomy and decompression of spinal canal and lateral recess when compared to 09/03/2015. There is enhancing granulation tissue at the site of prior laminectomies on the left, L3-4 and L4-5 as before as well as at the L5-S1 level at this time

Hospital Course continued Neurosurgery consulted – Difficult to pin-point a specific pain generator. – No specific neurosurgical intervention planned,

• however thought she may benefit from ESI and a multi-modal pain management regimen in the future.

PT/OT evaluation Mobility

– Total assist - patient < 25% (for boosting/repositioning in bed) – Moderate assist - patient 50-74% from supine to sit and sit to supine

• Inflammatory markers rise over next 24 hours from CRP 107 • Pt remained afebrile however was on scheduled Tylenol

281

Differential Diagnosis: •Flare of chronic degenerative process •Inflammatory arthritis –Ankylosing spondylitis or psoriatic, reactive arthritis •Infectious - Meningitis, discitis, abscess •Malignancy

– Unclear etiology for this severe pain with no acute findings on MRI or CT Chest, Abdomen, Pelvis. Considering pulse steroids, toradol and Rheumatology consult.

Hospital Course Hospital Day 4 • CRP 350 • Refusing to work with PT due to severity of neck pain • LP attempted by Neurologist • LP done under fluoroscopy (3 attempts; L4-5, L5-S1, and L2-3 levels)

– Findings: Clear CSF which turned bloody due to likely traumatic tap – Specimen: 1cc obtained 0 leukocytes 1400 erythrocytes Negative Gram stain

• Developed right wrist tenderness, erythema, swelling

Thoughts? • • • •

severe neck pain/rigidity elevated inflammatory markers negative imaging negative LP

What would be your next step?

Axial (Neck) Pain Mechanical

Non-Mechanical

Cervical strain

Neoplasm

Degenerative process – disc, facet age-related

Infection – osteomyelitis, discitis, abscess, meningitis, Shingles

Herniated Disc

Inflammatory Arthritis – ankylosing, psoriatic, reactive, PMR, crystal arthropathy

Spinal stenosis

Vasculitis - GCA

compression/traumatic fracture

Hospital Course Hospital Day 5 • Rheumatology Consulted

– trial of prednisone 40mg for concern of Crowned Dens Syndrome (as well as right wrist synovitis)

• CT cervical and right wrist xray obtained for examination of chondrocalcinosis. • Seen by Rheumatologist later that day with mild improvement in neck and wrist pain after receiving 40mg prednisone.

CT findings

Abnormal vs Normal CT C-spine

Discussion • Calcium pyrophosphate deposition (CPPD) disease is arthritis caused from calcium pyrophosphate crystals. • Clinical manifestations of CPPD include; – – – –

pseudogout pseudo-RA pseudo-OA pseudo-Charcot

• Disease of aging – rare in patients < 60 years old – Prevalence doubles with each decade over 60

CPPD - Presentation • Peripheral Joints: warmth, erythema, swelling in and around affected joint – most commonly knee and wrist (acute podagra is rare)

• Axial Involvement: various presentations – Crowned Dens – cervical pain, stiffness of nape of neck and occipital headache – spinal tissue involvement, intervertebral disks, spinal ligaments as well as tumoral soft tissue deposits that can be mistaken for cancers

• Often associated with systemic symptoms: fevers, chills and elevated inflammatory markers

Pathogenesis • •

•

Crystal formation in pericellular matrix Pyrophosphate generated from extracellular ATP – forms complexes with calcium CPP crystals induce inflammation & direct catabolic effects on joint tissues → prostaglandin E2 and matrix metalloproteinases

Ann Rosenthal et al NEJM

Rick Factors/Associations • Age • Hypophosphatasia (congenital syndrome, low functional alkaline phosphatase) • Hyperparathyroidism (CPPD ds can persist for yrs even after correction of calcium levels) • Hemochromatosis – inhibitory action of iron on pyrophosphatases, high levels of PTH in cartilage • Hypomagnesemia – Mg increases solubility of CPP crystals, cofactor for pyrophosphatases • Familial CPPD – two genetic loci associated, CCAL2 chromosome 5, CCAL1 chromosome 8 Recommendations; if presenting with CPPD disease