23 March 2023: Articles
Unusual Cause of Cord Compression in a Patient with Chronic Lymphocytic Leukemia
Unusual clinical course, Challenging differential diagnosis, Unusual or unexpected effect of treatment, Educational Purpose (only if useful for a systematic review or synthesis), Rare coexistence of disease or pathologyKirubel Z. Gebreselassie1ABCDEF*, Daniel Valancius1C, Lara Millikan Ranic2B, Christopher Kligora3E
Am J Case Rep 2023; 24:e938852
BACKGROUND: Spinal gout is not so uncommon. The numbers of case reports are low because of underdiagnosis. Nevertheless, the presentation of spinal gout in a patient who was never diagnosed with gout or hyperuricemia is rare. In addition, the probability of occurrence of spinal gout at the thoracic spine is low, making this case unique. Late or missed diagnosis causes treatment delays with grave outcomes.
CASE REPORT: A 65-year-old man with a past medical history of chronic lymphocytic leukemia presented with rapid and progressive weakness of the lower extremities that led to complete paraplegia over 2 weeks. Magnetic resonance imaging showed severe central canal stenosis with disc protrusion and a T4-5 ventral mass with abnormal marrow signaling. The margins at the erosion were sclerotic, and malignancy was top on the differential diagnosis. Open minimally invasive laminectomy with discectomy at T4-5 was done. Sections on the biopsy specimen demonstrated fragments of benign cartilage and bone and multiple granulomas palisading around areas of crystalline material, consistent with tophaceous gout. He was started on a urate-lowering treatment per the American College of Rheumatology guideline. Unfortunately, due to the late patient presentation, neurologic recovery was not possible.
CONCLUSIONS: This case report illustrates an unusual presentation of gout with a catastrophic complication that could have been prevented with early diagnosis and treatment. Some reports have described patients with tophaceous deposits in the absence of gout flare among patients with urate overproduction due to myeloproliferative disorders. Further studies are needed to explore such relationships.
Keywords: gout, Paraplegia, hyperuricemia
Spinal gout is not so uncommon. A good number of case reports mention gout causing myelopathy secondary to cord compression; the first case was reported by Koskoff et al in 1953 . There are at least 68 published cases of spinal gout between 2000 and 2014, with the most common clinical picture reported as localized back or neck pain, spinal nerve root, and spinal cord compression, with weakness mostly at the lumbar spine . Despite traditional beliefs that spinal gout is rare, recent studies show it is more prevalent than reported because of underdiagnosis . Nevertheless, such presentation in a patient who was never diagnosed with gout or hyperuricemia is rare . This causes diagnostic challenges and potential treatment delays. In addition, the probability of occurrence of spinal gout at the thoracic spine is low , making our case unique. We present a 65-year-old man with a past medical history of chronic lymphocytic leukemia (CLL) who came to our facility with 3 weeks of progressive weakness of the lower extremities that progressively became complete paraplegia. Thoracic magnetic resonance imaging with and without contrast showed severe central canal stenosis with disc protrusion and T4–5 ventral mass with abnormal marrow signaling. Open minimally invasive laminectomy, with discectomy at T4–5, was done, and a biopsy specimen was sent for pathological analysis. Sections demonstrated fragments of benign cartilage and bone and multiple granulomas palisading around areas of crystalline material, consistent with tophaceous gout. There was no evidence of malignancy.
The patient was a pleasant 65-year-old man with a past medical history of atrial fibrillation, chronic kidney disease stage 3, deep venous thrombosis, chronic diastolic heart failure, hypertension, hyperlipidemia, and CLL, which was not on treatment. He was admitted to the hospital for neurosurgical management of his compressive myelopathy, secondary to spinal cord compression. He reported that he was very active before he experienced progressive weakness for the 3 weeks leading up to his hospitalization, which worsened to the point of his being non-ambulatory for 2 weeks before hospitalization. The patient reported ongoing weakness in his bilateral lower extremities and diffuse thoracic and lumbar back pain. He denied shortness of breath, chest pain, abdominal pain, nausea or vomiting, and fever or chills. He denied tobacco smoking, alcohol consumption, and illicit drug use. He lived at home with his wife. He has no known history of gout or previous gout treatment. He had swelling on the right elbow, which was previously diagnosed as bursitis, a potentially missed diagnosis of gout tophi. His past surgical history was relevant for carpal tunnel surgery approximately a year ago.
On physical examination, a man who appeared to be his stated age was in no acute distress and was alert and cooperative. A surgical dressing on the thoracic spine was clean, dry, and intact. He had a regular heart rate and rhythm, S1 and S2 were well heard, and there was no murmur or gallop rhythm. The lungs were clear, with symmetric air entry. He had +2 peripheral edema on the musculoskeletal system and a small swelling on his left knuckles. On neurologic examination, he was oriented to time, place, and person; there was paraplegia of the lower extremities, with a power of 0/5, and a sensory level around T7–8. The power on the upper extremities was 0/5. He had diminished reflexes on the lower extremities, with no muscle fasciculations.
Laboratory investigations were remarkable for an increased white blood cell count of 24.6 K due to CLL, serum uric acid level of 11.4 mg/dL, and serum sodium level of 134 meq/L. A thoracic spine magnetic resonance imaging study revealed severe T4–5 central stenosis, diffuse abnormal vertebral body marrow, and a T4–5 ventral mass lesion measuring 37×34 mm, concerning for malignancy (Figures 1, 2) given his history of hematopoietic malignancy. The margins at the erosion were sclerotic, and marrow-replacing processes, such as hematopoietic malignancy, multiple myeloma, or other metastatic disease, were highly considered. He underwent a laminectomy/ discectomy of T4–5, with a biopsy specimen taken and sent for pathological analysis, which turned out to be tophaceous gout (Figures 3, 4). Urate-lowering therapy with allopurinol was started at 100 mg per day, with a plan to increase the dose by 100 mg every few weeks per the American College of Rheumatology guideline. He was also given colchicine 1.2 mg followed by 0.6 mg daily to prevent exacerbation of gout attacks. However, there was no neurologic recovery, owing to the late presentation.
Tophaceous gout is characterized by solid urate collection, chronic inflammation, and often destructive changes in the surrounding connective tissue . The most common etiologies for acute to subacute cord compression are trauma, tumors, infections leading to an abscess, and herniated discs . The natural history of gout consists of a self-limited acute phase characterized by recurrent attacks of synovitis, an intercritical period in which patients are asymptomatic; however, the deposition of monosodium crystal deposition continues, and finally, a chronic stage in which inflammatory and structural changes of the joints and periarticular tissues occurs can lead to persistent symptoms and tophi formation . The development of tophaceous gout in the absence of a prior episode of gouty arthritis is not common . Some reports have described patients with tophaceous deposits in the lack of, or before, gout flare in patients with urate overproduction due to myeloproliferative disorders or hereditary enzyme defects .
Our patient had CLL, and it may have been the case that he was producing excessive urate production leading to a secondary gout. He may have been unaware of the initial episodes of acute gouty attacks that probably would have given an opportunity for earlier diagnosis and treatment. In addition, since most tophi are painless, he may have overlooked the swelling on his knuckles. The importance of early administration of allopurinol to prevent extreme hyperuricemia and excessive hyper-uricosuria in blood dyscrasias even before the onset of gouty arthritis should not be overlooked . Even though the association between elevated serum uric acid levels and the development of chronic gouty arthritis and tophi formation is not well established, screening those patients with high risk appears to be a reasonable approach to prevent a grave complication . The most common types of myeloproliferative disorders to cause secondary gout are mostly myeloid series metaplasia and polycythemia . Our patient had CLL, and further studies are needed to study the association between CLL and tophaceous gout.
Urate-lowering therapy is indicated in a patient with frequent gout flares, tophi deposits, and urate nephropathy . The target goal is to keep serum uric acid levels below 6 mg/dL, substantially below the urate solubility limit, and the most used agent in the United States is allopurinol . The allopurinol dose was planned to be increased by 100 mg every 2 weeks until target levels were reached in the subsequent weeks, according to the American College of Rheumatology guideline. The improvement in serum uric acid levels was not adequate, and a glucose-6-phosphate dehydrogenase test was done in anticipation of treatment with pegloticase infusion. The US Food and Drug Advisory approves using pegloti-case infusions for refractory cases and selected patients who need rapid reduction of gouty flares and tophi ; however, this needs further studies. Treatment was considered but not used in our patient because of financial constraints and lack of easy availability. On follow-up, the serum uric acid level reached 7.9 mg/dL the following month, still above desired levels. The patient’s neurologic deficit remained the same because of permanent neurologic damage, and unfortunately, treatment was unable to change the outcome.
This case presents an unusual presentation of gout with a catastrophic complication that could have been prevented with early diagnosis and treatment. High index of suspicion, early recognition, and initiation of treatment in patients with multiple risk factors for gout can prevent a catastrophic complication. The association between gout and underlying myeloproliferative disorders is beyond the scope of this case report. Further studies are needed to explore such relationships.
FiguresFigure 1.. Magnetic resonance imaging of thoracic spine without contrast. Severe T4–5 central stenosis is likely associated with a disc bulge and protrusion. Focally abnormal cord signal can be seen with myelomalacia or contusion. Figure 2.. Magnetic resonance imaging of thoracic spine without contrast. Diffuse abnormal vertebral body marrow and T4-5 ventral mass lesion. Marrow replacing process such as hematopoietic malignancy, multiple leiomyoma, profound anemia, myelofibrosis, or other metastatic disease may be considered. Mass lesion is likely associated and is destructive of the ventral T4-5 level. Margins at the erosion are sclerotic and chronicity may be considered. Mass lesion may be seen in the setting of hematopoietic malignancy, extra medullary hematopoiesis, plasmacytoma, or other metastatic lesion. Figure 3.. Pathology slide, hematoxylin and eosin stain. Low power view (50×) of bone (red arrow), cartilage (blue arrow), and gout (black arrow). Figure 4.. Pathology slide, hematoxylin and eosin stain. High power view (200×) of a tophaceous gout granuloma (big blue arrow), with foreign body giant cells and histocytes surrounding the uric acid crystals (small black arrows).
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