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diagnostic testsAtypical Cause of Functional Decline in Parkinson's Disease

Atypical Cause of Functional Decline in Parkinson’s Disease

Selected cuts from magnetic resonance imaging of the spine.
Selected cuts from magnetic resonance imaging of the spine.

A 74-year-old Chinese man with past medical history of Parkinson’s disease presented with rapid functional decline over 2 months. From being able to ambulate independently, he had become bedridden and was dependent in activities of daily living. On admission, he was also noted to be coughing on eating and had visual hallucinations for 2 weeks prior to admission.

He had been diagnosed with Parkinson’s disease in June 2015 and was on Madopar (levodopa/benserazide; Roche Products Limited, Welwyn Garden City, UK), which was gradually uptitrated from 62.5 mg 3 times a day to 125 mg 3 times a day over the past 2 years, with a recent increase 4 weeks prior to admission.

Physical examination showed marked rigidity and bradykinesia with resting tremor. He had no focal neurology and reflexes were normal. There was no lymphadenopathy, hepatosplenomegaly, or bony tenderness.

Blood investigations showed anemia with hemoglobin 9.7 g/dL (normal range, 13.1-16.6), previously 12.3 g/dL in 2016. In addition, it was noted that serum lactate dehydrogenase was 13,842 U/L, white cell count was normal at 4.64 × 109/L, and platelets were 228 × 109/L.

The initial working diagnosis was of delirium with possible cerebrovascular event. A magnetic resonance imaging (MRI) study of the brain showed generalized cerebral atrophy with no acute changes.

A computed tomography (CT) scan of the neck, thorax, abdomen, and pelvis, and MRI of the spine were requested to rule out any hematological causes. MRI of the spine reported widespread metastatic disease of the whole spine. The CT scan showed an ill-defined soft tissue nodular thickening in the right glutei and iliacus extending to the anterolateral abdominal wall and likely lower psoas, with mild cortical rarefaction of the underlying right ilium. No significant lymphadenopathy was detected (Figure).

A bone marrow study was carried out, and histology revealed a necrotic tumor with features suspicious for high-grade B-cell lymphoma. Flow cytometry was not performed, as it was a dry tap. Subsequently, an ultrasound-guided biopsy of the right pelvic mass showed a high-grade B-cell lymphoma with strong expression of MYC, BCL2, and BCL6 protein.

Discussion

Parkinson’s disease is a neurodegenerative disorder.1 At 15 years after diagnosis, more than 70% of patients will have died, and approximately half of those surviving will need institutional care.2 Functional decline in Parkinson’s disease can be due to a myriad of causes including stroke disease, cord compression, sepsis, and disease progression. A rapid decline should prompt a physician to look for alternative causes; in this case it was due to primary diffuse large B-cell lymphoma of the bone marrow.

Diffuse large B-cell lymphoma in the bone marrow is rare,3 unlike secondary involvement of malignant lymphomas in the bone marrow. The criteria used in diagnosing primary bone marrow diffuse large B-cell lymphoma are as follows:4 1) pathologically confirmed bone marrow involvement with diffuse large B-cell lymphoma; 2) pan-B-cell marker (CD19 or CD20) expression shown via immunohistochemistry or flow cytometry analysis; and 3) absence of lymph node involvement (defined as any lymphadenopathy with a size >1 cm) shown via whole-body CT scan or physical examination. The mean survival duration of patients with primary bone marrow diffuse large B-cell lymphoma was 14.9 months.5

For patients with Parkinson’s disease and rapidly progressing symptoms, alternative diagnoses should be considered, as some may be amenable to treatment if diagnosed early.

To read this article in its entirety please visit our website.

-Li Feng Tan, MBBS, MRCP (UK), M.Med (Int Med), ABIM (Int’l), Shu Ee Ng, MBBS, MRCP (UK), Reshma Merchant, MBChB(Edin), MRCP(UK), FRCP(Edin), FAMS

This article originally appeared in the June issue  of The American Journal of Medicine.

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