1/11. Neurolymphomatosis in Waldenstrom's macroglobulinaemia. We report four patients with Waldenstrom's macroglobulinaemia with an unusual neurologic complication, neurolymphomatosis, characterized by meningeal and root nerve infiltration by lymphoplasmacytic cells. patients presented with rapidly progressive leg proximal weakness. Examination of cerebrospinal fluid disclosed lymphoplasmacytic cells. magnetic resonance imaging of the lumbar spine was suggestive of a tissular infiltration of leptomeninges and nerve roots. Chemotherapy and irradiation of involved tissues led to a remarkable and sustained neurological improvement. ( info) |
2/11. Solitary sciatic nerve lymphoma as an initial manifestation of diffuse neurolymphomatosis. Case report and review of the literature. Solitary peripheral nerve lymphomas are exceedingly rare primary manifestations of diffuse peripheral nervous system or central nervous system (CNS) lymphomatosis. A 52-year-old man presented with progressive weakness in gastrocnemius and anterior tibial muscle function, which was associated with radiating pain in the right leg. magnetic resonance imaging studies revealed a solitary fusiform tumor, extending from the sciatic nerve, at the level of the lesser trochanter of the femur, into the posterior tibial nerve below the popliteal fossa. Intraoperative gross examination found that the tumor diffusely expanded the nerve, but did not extend from or into surrounding muscle or tendons. The final histological diagnosis was a solitary extranodal lymphoma (Burkittlike high-grade B-cell lymphoma). Postoperative staging did not reveal evidence of lymphomatous involvement of other organs, but additional chemo- and radiotherapies were administered. Four months after the surgical biopsy, the patient presented with a right facial nerve palsy. The results of cytological examination of cerebrospinal fluid were positive for the presence of atypical lymphocytes, which was consistent with apparently progressive neurolymphomatosis; however, the results of radiological studies were negative for systemic progression. The patient underwent intrathecal chemotherapy followed by systemic myelosuppressive chemotherapy with bone marrow rescue, but died of respiratory failure while still receiving treatment. Postmortem examination revealed extensive lymphomatosis in the peripheral nerves and spinal nerve roots without evidence of cranial nerve, CNS, or other organ system involvement. The aggressive biological characteristics of these tumors, their management, and pertinent literature are reviewed. ( info) |
3/11. Clinicopathological and molecular biological studies in a patient with neurolymphomatosis. We describe a patient with a clinical disorder that resembled vasculitic neuropathy in which peripheral nerves were successively affected over several months, but without systemic involvement. An initial muscle biopsy near the involved nerves showed signs of nonspecific inflammation around the muscle and nerve fibers. Immunosuppressive treatment resulted in a dramatic reduction in pain, but relapses of the disease eventually occurred, and the patient died 22 months after onset of the first symptoms. Pathologically, a malignant non-Hodgkin's B-cell lymphoma, restricted to the intra- and extradural peripheral nervous system, was found. The demonstration by Southern blotting of immunoglobulin heavy chain gene rearrangement confirmed the monoclonal nature of the lymphomatous cells. in situ hybridization tests for Epstein-Barr and herpes virus subtypes were negative. Our case underlines i) how difficult diagnosis can be despite extensive investigations, ii) the usefulness of immunosuppressive treatment in the early stage of the disease, iii) the importance of immunostaining and genome analysis for distinguishing between different types of human neurolymphomatosis, and iv) the fact that the initial inflammatory process in the muscle biopsy may be interpreted either as a paraneoplastic effect of the lymphoma or as a viral inflammatory neuromyopathy that triggers the development of the malignant lymphoma. ( info) |
4/11. Neurolymphomatosis associated with muscle and cerebral involvement caused by natural killer cell lymphoma: a case report and review of literature. We report a biopsy-proven case of neurolymphomatosis (NL) presenting with sensory motor axonal polyneuropathy, polymyositis, and cerebral involvement. Ours is the second reported case of NL caused by natural killer-cell lymphoma defined by morphology and immunophenotyping. For 3 months, the patient developed stocking-glove distribution of hypesthesia, subacute progressive weakness and mental deterioration. EMG showed severe sensorimotor mixed axonal-demyelinating polyradiculoneuropathy. Lumbar puncture revealed mildly high protein level with normal glucose and cell count. sural nerve biopsy demonstrated lymphomatous axonal neuropathy and muscle biopsy was indicative of lymphomatous polymyositis. brain MRI revealed multiple white matter lesions, consistent either with progressive multifocal leukoencephalopathy or cerebral lymphoma. bone marrow biopsy showed neoplastic infiltrates. The patient died of multiple organ failure prior to initiation of chemotherapy. ( info) |
5/11. Fusion PET-CT imaging of neurolymphomatosis. In a patient suffering from peripheral neuropathy due to neurolymphomatosis, fused PET-CT imaging, performed on a novel in-line PET-CT system, showed multiple small nodular lesions extending along the peripheral nerves corresponding to an early relapse of a transformed B-cell non-Hodgkin's lymphoma. ( info) |
6/11. Neurolymphomatosis: a clinicopathologic syndrome re-emerges. We describe a patient with sensorimotor peripheral neuropathy and cranial neuropathy due to autopsy-proven neurolymphomatosis defined by infiltration of peripheral nerves by tumor cells and review the findings in 39 previously reported patients. The cause of the neuropathy is not known. The association with immune-deficient states suggests virally mediated pathogenesis, possibly a retrovirus. ( info) |
7/11. High-dose intravenous methotrexate followed by autologous stem cell transplantation as a potentially effective therapy for neurolymphomatosis. BACKGROUND: Neurolymphomatosis (NL) is a rare neurologic manifestation of systemic lymphoma characterized by lymphomatous infiltration of the peripheral nervous system. The diagnosis of NL is difficult and requires a multidisciplinary approach for obtaining an adequate biopsy specimen of the suspected nerve. The prognosis of patients with NL has been poor because adequate penetration of chemotherapy into the nervous system is difficult. methods: The authors presented the case of a 37-year-old man who was treated for Ann Arbor Stage IVB diffuse large B-cell lymphoma. The patient developed disease recurrence in the sciatic nerve without systemic involvement. RESULTS: The patient achieved a clinical response after receipt of high-dose intravenous methotrexate followed by high-dose chemotherapy and autologous stem cell transplant. CONCLUSIONS: The authors reported this case to highlight the effectiveness of this regimen in a rare and fatal disorder. In the current study they also reviewed the literature regarding the diagnosis, prognosis, and treatment of NL. ( info) |
8/11. Assessment of neurolymphomatosis by brachial plexus biopsy and PET/CT. Report of a case. OBJECTIVE: To report a biopsy-proven neurolymphomatosis in a young woman with previous non-Hodgkin's lymphoma (NHL) of uterine cervix. PATIENT: The patient presented with a painful brachial plexopathy and developed multiple cranial and spinal nerve palsies. methods AND RESULTS: The diagnosis was achieved by an open brachial plexus biopsy. A PET/CT study was used to assess the full extent of the disease and showed involvement of additional cranial nerves and spinal nerve roots. A complete although short lasting clinical and radiological response was achieved by means of systemic high dose methotrexate treatment combined with rituximab and intra-CSF injections of cytarabine. ( info) |
9/11. Human neurolymphomatosis by adult T-cell leukemia. We report a case of adult T-cell leukemia presenting with sensorimotor polyneuropathy with persistent severe pain. The patient died without treatment after 4 months. autopsy showed infiltration of peripheral nerves by leukemia with no involvement of the CNS. This case closely resembles Marek's disease of fowls, a virus-induced T-cell lymphoma associated with neurolymphomatosis. ( info) |
10/11. Human neurolymphomatosis. A patient with a chronically progressive fatal sensorimotor neuropathy showed, at autopsy, extensive and selective lymphocytic infiltration of peripheral and cranial nerves and a segmental demyelinative process. A clinically occult retroperitoneal lymphoma without spread to other systemic organs was also present. The possibility of a selective infiltration of the peripheral nervous system by the retroperitoneal malignancy is rejected as unlikely. Our case bears a strong similarity to 3 other cases previously reported which may be grouped together under the heading "human neurolymphomatosis". The clinical and pathological features of this rare entity are discussed. It bears a resemblance to Marek's disease of chickens and seems to represent an unusual inflammatory neuropathy or form of malignancy. ( info) |