A DIFFICULT CASE
A mystery solved H Abbasi,1 S L Bell,2 W Stewart,2 Asha Neelakantan,3 Stewart Webb1 1
Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK 2 Department of Neuropathology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK 3 Department of Neuroradiology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK Correspondence to Dr Hina Naz Abbasi, Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, 1345 Govan Road, Glasgow G51 4TF, Scotland; [email protected] Published Online First 12 November 2013
CASE REPORT A 58-year-old man initially noticed an abnormal sensation around the abdomen and weakness of the legs, making it difficult for him to finish a round of golf. Two months later, he developed sudden weakness of the left face and hand, with abnormal sensation of the upper lip. He was admitted to the local hospital. An MR scan of brain showed an acute infarction in the right centrum semiovale; he was started on stroke prevention therapy. One month later, he was re-admitted with a 2-week history of gradual onset bilateral lower limb weakness, paraesthesia and urinary retention. MR scan of spine showed a hyperintense contrast-enhancing, intramedullary lesion between T6 and T10, thought to represent either ischaemia or demyelination (figure 1). Despite starting corticosteroids, he continued to deteriorate, with complete flaccid paraplegia, night sweats and pyrexia. His erythrocyte sedimentation rate and serum C reactive protein were significantly elevated, but tests for vasculitis, coagulation defects and infections (including HIV, syphilis, human Tlymphotropic virus type 1 (HTLV1), tuberculosis and Lyme disease) were negative. Other blood tests, including serum ACE, copper, zinc and vitamins, were also normal. Cerebrospinal fluid (CSF) showed a white cell count of 5/mL (≤5), protein 0.57 g/L (0.15–0.45) and glucose 3.2 mmol/L with a matched plasma glucose of 8.1 mmol/L (he was taking steroids). CSF pathology and flow cytometry were normal. 12-lead ECG, echocardiogram and CT cerebral angiogram were normal. Whole-body CT and positron emission tomography scan looking for malignancy were normal. Nerve conduction studies suggested a diffuse lower limb preganglionic abnormality. As he continued to deteriorate, we gave a further course of intravenous corticosteroids followed by
oral prednisolone 60 mg daily. We also started quadruple antituberculous treatment. Despite this, he did not improve. Repeat MR scan of spine showed a new hyperintense signal in conus medullaris (figure 2). During admission, his haemoglobin fell from 117 to 87 g/L (130–180); we found no source of acute blood loss. Rheumatological investigation found no evidence of connective tissue disorder or vasculitic process. The haematologist decided against a bone marrow biopsy as the diagnostic yield was likely to be low. His serum albumin fell gradually during admission and he was found to have a nephrotic syndrome. Renal biopsy showed a discohesive population of large atypical cells within the glomeruli and peritubular capillaries. These cells had large pleomorphic nuclei and prominent nucleoli with limited cytoplasm. Immunocytochemistry of the atypical cells was positive for CD20, CD79a, MUM-1, BCL2 and BCL6, but negative for CD3, CD5, CD10, CD30 and CD56. In situ hybridisation for Epstein–Barr virus was negative and there was no evidence of (14:18) translocation (figure 3). Thus, the morphological and immunocytochemical findings were in keeping with intravascular large B-cell lymphoma. He received five cycles of R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone); repeat biopsy showed no residual CD20 tumour cells. However, 5 months later he presented with acute confusion and drowsiness and MR scan of brain showed evidence of cerebral lymphoma (figures 4 and 5). We gave two cycles of intravenous iphosphamide, etoposide and epirubicin, two of high-dose methotrexate and one dose of rituximab. Following this, his confusion and drowsiness resolved and his cranial imaging improved (figure 6). Despite persistent weakness and sensory loss in the
To cite: Abbasi H, Bell SL, Stewart W, et al. Pract Neurol 2014;14:107–109.
Abbasi H, et al. Pract Neurol 2014;14:107–109. doi:10.1136/practneurol-2013-000638
107
A DIFFICULT CASE
Figure 1 MR scan of spine (sagittal T2W) showing subtle focal anterior cord hyperintensity.
legs, his condition remained stable over the next 3 years with no evidence of relapse. Intravascular (angiotropic) lymphoma is very rare, accounting for
A mystery solved H Abbasi,1 S L Bell,2 W Stewart,2 Asha Neelakantan,3 Stewart Webb1 1
Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK 2 Department of Neuropathology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK 3 Department of Neuroradiology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK Correspondence to Dr Hina Naz Abbasi, Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, 1345 Govan Road, Glasgow G51 4TF, Scotland; [email protected] Published Online First 12 November 2013
CASE REPORT A 58-year-old man initially noticed an abnormal sensation around the abdomen and weakness of the legs, making it difficult for him to finish a round of golf. Two months later, he developed sudden weakness of the left face and hand, with abnormal sensation of the upper lip. He was admitted to the local hospital. An MR scan of brain showed an acute infarction in the right centrum semiovale; he was started on stroke prevention therapy. One month later, he was re-admitted with a 2-week history of gradual onset bilateral lower limb weakness, paraesthesia and urinary retention. MR scan of spine showed a hyperintense contrast-enhancing, intramedullary lesion between T6 and T10, thought to represent either ischaemia or demyelination (figure 1). Despite starting corticosteroids, he continued to deteriorate, with complete flaccid paraplegia, night sweats and pyrexia. His erythrocyte sedimentation rate and serum C reactive protein were significantly elevated, but tests for vasculitis, coagulation defects and infections (including HIV, syphilis, human Tlymphotropic virus type 1 (HTLV1), tuberculosis and Lyme disease) were negative. Other blood tests, including serum ACE, copper, zinc and vitamins, were also normal. Cerebrospinal fluid (CSF) showed a white cell count of 5/mL (≤5), protein 0.57 g/L (0.15–0.45) and glucose 3.2 mmol/L with a matched plasma glucose of 8.1 mmol/L (he was taking steroids). CSF pathology and flow cytometry were normal. 12-lead ECG, echocardiogram and CT cerebral angiogram were normal. Whole-body CT and positron emission tomography scan looking for malignancy were normal. Nerve conduction studies suggested a diffuse lower limb preganglionic abnormality. As he continued to deteriorate, we gave a further course of intravenous corticosteroids followed by
oral prednisolone 60 mg daily. We also started quadruple antituberculous treatment. Despite this, he did not improve. Repeat MR scan of spine showed a new hyperintense signal in conus medullaris (figure 2). During admission, his haemoglobin fell from 117 to 87 g/L (130–180); we found no source of acute blood loss. Rheumatological investigation found no evidence of connective tissue disorder or vasculitic process. The haematologist decided against a bone marrow biopsy as the diagnostic yield was likely to be low. His serum albumin fell gradually during admission and he was found to have a nephrotic syndrome. Renal biopsy showed a discohesive population of large atypical cells within the glomeruli and peritubular capillaries. These cells had large pleomorphic nuclei and prominent nucleoli with limited cytoplasm. Immunocytochemistry of the atypical cells was positive for CD20, CD79a, MUM-1, BCL2 and BCL6, but negative for CD3, CD5, CD10, CD30 and CD56. In situ hybridisation for Epstein–Barr virus was negative and there was no evidence of (14:18) translocation (figure 3). Thus, the morphological and immunocytochemical findings were in keeping with intravascular large B-cell lymphoma. He received five cycles of R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone); repeat biopsy showed no residual CD20 tumour cells. However, 5 months later he presented with acute confusion and drowsiness and MR scan of brain showed evidence of cerebral lymphoma (figures 4 and 5). We gave two cycles of intravenous iphosphamide, etoposide and epirubicin, two of high-dose methotrexate and one dose of rituximab. Following this, his confusion and drowsiness resolved and his cranial imaging improved (figure 6). Despite persistent weakness and sensory loss in the
To cite: Abbasi H, Bell SL, Stewart W, et al. Pract Neurol 2014;14:107–109.
Abbasi H, et al. Pract Neurol 2014;14:107–109. doi:10.1136/practneurol-2013-000638
107
A DIFFICULT CASE
Figure 1 MR scan of spine (sagittal T2W) showing subtle focal anterior cord hyperintensity.
legs, his condition remained stable over the next 3 years with no evidence of relapse. Intravascular (angiotropic) lymphoma is very rare, accounting for
