Introduction: Little is known about the feasibility and effectiveness of rehabilitative treatment for systemic lupus erythematosus (SLE) in individuals with progressive multifocal leukoencephalopathy (PML). We describ...Introduction: Little is known about the feasibility and effectiveness of rehabilitative treatment for systemic lupus erythematosus (SLE) in individuals with progressive multifocal leukoencephalopathy (PML). We describe a patient with SLE complicated by PML and ameliorated by comprehensive rehabilitation. We also review the epidemiology, pathology, imaging characteristics, and treatment of PML. Patient Concerns: We found a patient with SLE with PML improved by multidisciplinary rehabilitation techniques. Diagnoses, Interventions, and Outcomes: We diagnosed a PML with a 13-year history of SLE and lupus nephritis after longtime immunosuppressive therapy. The patient underwent a comprehensive, multifaceted rehabilitation program, including drug therapy, integrated physical therapy, occupational therapy, acupuncture, music therapy, computer-aided cognitive rehabilitation training, and behavioral management training. This rehabilitation program improved her motor function and activities of daily living. Conclusions: Her condition improved in the short term through comprehensive rehabilitation, including physical, speech, and cognitive therapy. Therefore, we recommend comprehensive rehabilitation to improve the function and activities of daily living in patients with PML.展开更多
Objective: To describe the radiological, histological and immunohistochemical findings in a case of Progressive Multifocal Leukoencephalopathy (PML) affecting the cerebellar peduncles in a patient with chronic lymphoc...Objective: To describe the radiological, histological and immunohistochemical findings in a case of Progressive Multifocal Leukoencephalopathy (PML) affecting the cerebellar peduncles in a patient with chronic lymphocytic leukemia. Patient and Methods: Magnetic Resonance Imaging (MRI), histological picture (H.E., Kluver-Barrera) and immunohistochemical picture (GFAP, neurofilaments, CD68, JC virus) were obtained. Results: 1) Magnetic resonance imaging: Asymmetric and progressive lesions on middle cerebellar peduncles, that were hyperintense in T2/FLAIR, extended towards the pons, had no mass effect and were unmodified after intravenous contrast. 2) Histology: Marked reactive gliosis with cytopathic changes suggesting viral infection, plus demyelination areas with axonal preservation. 3) Immunohistochemistry: Marked positivity for viral (polyoma and JC virus) markers in glial cells showing cytopathic changes. Conclusions: The importance of histological and immunohistochemical diagnosis in everyday assistance;of the collaboration between clinicians, radiologists and pathologists;and the validity of postmortem studies as a key element for research and clinical quality assessment must be stressed.展开更多
AIM: To investigate the neuropathology of the brain in a rare case of remission following diagnosis of progressive multifocal leukoencephalopathy(PML).METHODS: Consent from the family for an autopsy was obtained, clin...AIM: To investigate the neuropathology of the brain in a rare case of remission following diagnosis of progressive multifocal leukoencephalopathy(PML).METHODS: Consent from the family for an autopsy was obtained, clinical records and radiograms were retrieved. A complete autopsy was performed, with brain examination after fixation and coronal sectioning at 1 cm intervals. Fourteen regions were collected for paraffin embedding and staining for microscopic analysis. Histologic sections were stained with Luxol blue, hematoxylin/eosin, and immunostained for myelin basic protein, neurofilament, SV40 T antigen and p53. The biopsy material was also retrieved and sections were stained with hematoxylin/eosin and immunostained for SV40 and p53. Sections were examined by American Board of Pathology certified pathologists and images captured digitally.RESULTS: Review of the clinical records was notable for a history of ulcerative colitis resulting in total colectomy in 1977 and a liver transplant in 1998 followed by immune-suppressive therapy. Neurological symptoms presented immediately, therefore a biopsy was obtained which was diagnosed as PML. Immunotherapy was adjusted and clinical improvement was noted. No subsequent progression was reported. Review of the biopsy demonstrated atypical astrocytes and enlarged hyperchromatic oligodendroglial cells consistent with JC virus infection. Strong SV40 and p53 staining was found in glial cells and regions of dense macrophage infiltration were present. On gross examination of the post-mortem brain, a lesion in the same site as the original biopsy in the cerebellum was identified but no other lesions in the brain were found. Microscopic analysis of this cerebellar lesion revealed a loss of myelin and axons, and evidence of axonal damage. This single burned-out lesion was equivocally positive for SV40 antigen with little p53 staining. Examination of thirteen other brain regions found no other occult sites.CONCLUSION: Our study reveals residual damage, rare macrophages or other inflammation and minimal evidence of persistent virus. This case demonstrates the possibility of complete remission of PML.展开更多
Background: Central neurological involvement is the most frequent extra hematological manifestation of chronic lymphocytic leukemia;it is multifactorial and rarely due to a cerebral localization of the disease. We rep...Background: Central neurological involvement is the most frequent extra hematological manifestation of chronic lymphocytic leukemia;it is multifactorial and rarely due to a cerebral localization of the disease. We report a case of cerebral localization of chronic lymphoid leukemia whose clinical and radiological aspects were very suggestive of progressive multifocal leukoencephalopathy. Case Presentation: A 65-year-old patient who was HIV-negative (human immunodeficiency virus), had consulted for bilateral axillary, cervical and inguinal lymphadenopathy associated with major asthenia and hyper lymphocytosis (lymphocyte count was 11 giga/l). Chronic lymphocyticleukemia with TP53 mutation was diagnosed and treatment with Ibrutinib 420 mg/day was initiated. After 2 months of treatment, the evolution was marked by the onset of neurological disorders whose clinical-radiological presentation and temporal evolution had led to the diagnosis of progressive multifocal leukoencephalopathy. In the absence of virological evidence in the cerebrospinal fluid analysis, a stereotactic biopsy of the brain lesions had been performed, making it possible to formally rule out this infectious hypothesis and to demonstrate cerebral invasion by tumour cells. Immuno-chemotherapy combining Rituximab-Cyclophosphamide-Doxorubicin-Vincristine-Prednisone-Ibrutinib (RCHOP-Ibrutinib) with intrathecal chemotherapy resulted in a very good clinical-radiological response. Conclusion: The appearance of neurological manifestations in the context of chronic lymphocytic leukemia must systematically lead to a search for a cerebral localization of the disease. In the absence of virological evidence in the cerebrospinal fluid, any suspicion of progressive multifocal leukoencephalopathy in this context should lead to the histological study of brain lesions.展开更多
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by reactivation of JC virus in immunocompromised patients. To date, PML with discrete involvement of the pyramidal tract has been desc...Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by reactivation of JC virus in immunocompromised patients. To date, PML with discrete involvement of the pyramidal tract has been described in only two patients. This report describes an additional case with PML showing discrete involvement of the pyramidal tract on T2-weighted images and FLAIR images.展开更多
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system due to the reactivation of the JC virus, which usually occurs in immunocompromised patients and is a major oppo...Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system due to the reactivation of the JC virus, which usually occurs in immunocompromised patients and is a major opportunistic infection associated with HIV infection. We report a case of a previously healthy patient who was diagnosed with PML.展开更多
Progressive multifocal encephalopathy (PML) is a rare but often fatal infectious brain disease caused by the reactivation of John Cunningham polyomavirus. Reactivation occurs in immunocompromised individuals with AIDS...Progressive multifocal encephalopathy (PML) is a rare but often fatal infectious brain disease caused by the reactivation of John Cunningham polyomavirus. Reactivation occurs in immunocompromised individuals with AIDS and leukemia, on chemotherapy or being treated with immunosuppressant drugs (e.g. monoclonal antibodies). Cases of PML have been described in patients treated with natalizumab, efalizumab and rituximab used, respectively, for the treatment of (1) multiple sclerosis, (2) psoriasis and (3) haematological malignancies or systemic autoimmune diseases (rheumatoid arthritis and systemic lupus erythematosus). The authors describe an unusual case of acute brainstem and cerebellar PML following chemotherapy for chronic lymphatic leukemia diagnosed 4 years before the onset of PML in a 75-year-old man. The patient was treated with high dose chemotherapy and rituximab with complete response. The onset of symptoms of PML was very rapid and occurred after more than two years from last rituximab infusion;patient had a sudden neurological deterioration, with rapid progression to death in about a month from the onset of symptoms. Lesions were localized in the cerebellum, brainstem and such pattern has been reported in very few cases in the literature.展开更多
Acute posterior multifocal placoidpigment epitheliopathy (APMPPE),origi-nally described by Gass in 1968,is general-ly recognized as having a self-limited natu-ral course and good prognosis,thoughchronic progression ca...Acute posterior multifocal placoidpigment epitheliopathy (APMPPE),origi-nally described by Gass in 1968,is general-ly recognized as having a self-limited natu-ral course and good prognosis,thoughchronic progression can lead to severe visualimpairment.A case associated with fatalcerebral vasculitis has been reported in re-展开更多
Purpose: The purpose of this study was to evaluate the change in corneal wavefront aberrations in young adults who have been fit with multifocal soft contact lenses for myopia progression control. Findings have been a...Purpose: The purpose of this study was to evaluate the change in corneal wavefront aberrations in young adults who have been fit with multifocal soft contact lenses for myopia progression control. Findings have been analyzed for statistical significance and clinical relevance and compared to reportedly successful Orthokeratology outcomes. Methods: The dominant eye of 40 participants (27 women, 13 men;mean age 27.3 ± 3.2 years;range 23 to 39 years) was fit with Proclear Multifocal center distance lenses (Coopervision, Pleasanton, USA) having a variety of distance powers and reading additions. Refractive errors were limited to a range of –6.00 D up to +1.00 D of sphere, and no greater than –1.00 D of cylinder. Corneal wavefront measurements were performed over 6 mm diameters with a Zeiss Atlas 9000 corneal topographer (Zeiss Meditec, Dublin, USA) prior to, and following lens fitting. Data were converted into rectangular Fourier optics terms M, J0, J45 and RMS values for each reading addition were statistically analyzed. Following evaluation of statistical significance and clinical relevance, results were compared to published data from successful Orthokeratology treatments. Results: Statistically significant changes in higher order aberrations were detected for lenses of all reading additions. Lens groups with higher Add-powers demonstrated stronger changes with increased significance. Final RMS values relating to 2nd, 3rd and 4th Zernike Orders reached clinical significance with a wavefront error of 0.10 μm, the equivalent of 0.25D. Moreover, as Add-powers increased, 3rd and 4th order aberrations likewise showed an increase. Pre-fitting astigmatism values accounted for the highest recorded aberrations and remained predominantly unchanged. Conclusion: Proclear Multifocal center-distance contact lenses were found to increase higher order wavefront aberrations in a manner dependent on their Add-power. In comparison to successful Orthokeratology outcomes, the amounts of resulting aberrations are notably different.展开更多
文摘Introduction: Little is known about the feasibility and effectiveness of rehabilitative treatment for systemic lupus erythematosus (SLE) in individuals with progressive multifocal leukoencephalopathy (PML). We describe a patient with SLE complicated by PML and ameliorated by comprehensive rehabilitation. We also review the epidemiology, pathology, imaging characteristics, and treatment of PML. Patient Concerns: We found a patient with SLE with PML improved by multidisciplinary rehabilitation techniques. Diagnoses, Interventions, and Outcomes: We diagnosed a PML with a 13-year history of SLE and lupus nephritis after longtime immunosuppressive therapy. The patient underwent a comprehensive, multifaceted rehabilitation program, including drug therapy, integrated physical therapy, occupational therapy, acupuncture, music therapy, computer-aided cognitive rehabilitation training, and behavioral management training. This rehabilitation program improved her motor function and activities of daily living. Conclusions: Her condition improved in the short term through comprehensive rehabilitation, including physical, speech, and cognitive therapy. Therefore, we recommend comprehensive rehabilitation to improve the function and activities of daily living in patients with PML.
文摘Objective: To describe the radiological, histological and immunohistochemical findings in a case of Progressive Multifocal Leukoencephalopathy (PML) affecting the cerebellar peduncles in a patient with chronic lymphocytic leukemia. Patient and Methods: Magnetic Resonance Imaging (MRI), histological picture (H.E., Kluver-Barrera) and immunohistochemical picture (GFAP, neurofilaments, CD68, JC virus) were obtained. Results: 1) Magnetic resonance imaging: Asymmetric and progressive lesions on middle cerebellar peduncles, that were hyperintense in T2/FLAIR, extended towards the pons, had no mass effect and were unmodified after intravenous contrast. 2) Histology: Marked reactive gliosis with cytopathic changes suggesting viral infection, plus demyelination areas with axonal preservation. 3) Immunohistochemistry: Marked positivity for viral (polyoma and JC virus) markers in glial cells showing cytopathic changes. Conclusions: The importance of histological and immunohistochemical diagnosis in everyday assistance;of the collaboration between clinicians, radiologists and pathologists;and the validity of postmortem studies as a key element for research and clinical quality assessment must be stressed.
基金Supported by The Harvey Family Endowment(ELB)National Institutes of Health RO1 MH096093,RO1 NS062184,and RO1 NS046810
文摘AIM: To investigate the neuropathology of the brain in a rare case of remission following diagnosis of progressive multifocal leukoencephalopathy(PML).METHODS: Consent from the family for an autopsy was obtained, clinical records and radiograms were retrieved. A complete autopsy was performed, with brain examination after fixation and coronal sectioning at 1 cm intervals. Fourteen regions were collected for paraffin embedding and staining for microscopic analysis. Histologic sections were stained with Luxol blue, hematoxylin/eosin, and immunostained for myelin basic protein, neurofilament, SV40 T antigen and p53. The biopsy material was also retrieved and sections were stained with hematoxylin/eosin and immunostained for SV40 and p53. Sections were examined by American Board of Pathology certified pathologists and images captured digitally.RESULTS: Review of the clinical records was notable for a history of ulcerative colitis resulting in total colectomy in 1977 and a liver transplant in 1998 followed by immune-suppressive therapy. Neurological symptoms presented immediately, therefore a biopsy was obtained which was diagnosed as PML. Immunotherapy was adjusted and clinical improvement was noted. No subsequent progression was reported. Review of the biopsy demonstrated atypical astrocytes and enlarged hyperchromatic oligodendroglial cells consistent with JC virus infection. Strong SV40 and p53 staining was found in glial cells and regions of dense macrophage infiltration were present. On gross examination of the post-mortem brain, a lesion in the same site as the original biopsy in the cerebellum was identified but no other lesions in the brain were found. Microscopic analysis of this cerebellar lesion revealed a loss of myelin and axons, and evidence of axonal damage. This single burned-out lesion was equivocally positive for SV40 antigen with little p53 staining. Examination of thirteen other brain regions found no other occult sites.CONCLUSION: Our study reveals residual damage, rare macrophages or other inflammation and minimal evidence of persistent virus. This case demonstrates the possibility of complete remission of PML.
文摘Background: Central neurological involvement is the most frequent extra hematological manifestation of chronic lymphocytic leukemia;it is multifactorial and rarely due to a cerebral localization of the disease. We report a case of cerebral localization of chronic lymphoid leukemia whose clinical and radiological aspects were very suggestive of progressive multifocal leukoencephalopathy. Case Presentation: A 65-year-old patient who was HIV-negative (human immunodeficiency virus), had consulted for bilateral axillary, cervical and inguinal lymphadenopathy associated with major asthenia and hyper lymphocytosis (lymphocyte count was 11 giga/l). Chronic lymphocyticleukemia with TP53 mutation was diagnosed and treatment with Ibrutinib 420 mg/day was initiated. After 2 months of treatment, the evolution was marked by the onset of neurological disorders whose clinical-radiological presentation and temporal evolution had led to the diagnosis of progressive multifocal leukoencephalopathy. In the absence of virological evidence in the cerebrospinal fluid analysis, a stereotactic biopsy of the brain lesions had been performed, making it possible to formally rule out this infectious hypothesis and to demonstrate cerebral invasion by tumour cells. Immuno-chemotherapy combining Rituximab-Cyclophosphamide-Doxorubicin-Vincristine-Prednisone-Ibrutinib (RCHOP-Ibrutinib) with intrathecal chemotherapy resulted in a very good clinical-radiological response. Conclusion: The appearance of neurological manifestations in the context of chronic lymphocytic leukemia must systematically lead to a search for a cerebral localization of the disease. In the absence of virological evidence in the cerebrospinal fluid, any suspicion of progressive multifocal leukoencephalopathy in this context should lead to the histological study of brain lesions.
文摘Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by reactivation of JC virus in immunocompromised patients. To date, PML with discrete involvement of the pyramidal tract has been described in only two patients. This report describes an additional case with PML showing discrete involvement of the pyramidal tract on T2-weighted images and FLAIR images.
文摘Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system due to the reactivation of the JC virus, which usually occurs in immunocompromised patients and is a major opportunistic infection associated with HIV infection. We report a case of a previously healthy patient who was diagnosed with PML.
文摘Progressive multifocal encephalopathy (PML) is a rare but often fatal infectious brain disease caused by the reactivation of John Cunningham polyomavirus. Reactivation occurs in immunocompromised individuals with AIDS and leukemia, on chemotherapy or being treated with immunosuppressant drugs (e.g. monoclonal antibodies). Cases of PML have been described in patients treated with natalizumab, efalizumab and rituximab used, respectively, for the treatment of (1) multiple sclerosis, (2) psoriasis and (3) haematological malignancies or systemic autoimmune diseases (rheumatoid arthritis and systemic lupus erythematosus). The authors describe an unusual case of acute brainstem and cerebellar PML following chemotherapy for chronic lymphatic leukemia diagnosed 4 years before the onset of PML in a 75-year-old man. The patient was treated with high dose chemotherapy and rituximab with complete response. The onset of symptoms of PML was very rapid and occurred after more than two years from last rituximab infusion;patient had a sudden neurological deterioration, with rapid progression to death in about a month from the onset of symptoms. Lesions were localized in the cerebellum, brainstem and such pattern has been reported in very few cases in the literature.
文摘Acute posterior multifocal placoidpigment epitheliopathy (APMPPE),origi-nally described by Gass in 1968,is general-ly recognized as having a self-limited natu-ral course and good prognosis,thoughchronic progression can lead to severe visualimpairment.A case associated with fatalcerebral vasculitis has been reported in re-
文摘Purpose: The purpose of this study was to evaluate the change in corneal wavefront aberrations in young adults who have been fit with multifocal soft contact lenses for myopia progression control. Findings have been analyzed for statistical significance and clinical relevance and compared to reportedly successful Orthokeratology outcomes. Methods: The dominant eye of 40 participants (27 women, 13 men;mean age 27.3 ± 3.2 years;range 23 to 39 years) was fit with Proclear Multifocal center distance lenses (Coopervision, Pleasanton, USA) having a variety of distance powers and reading additions. Refractive errors were limited to a range of –6.00 D up to +1.00 D of sphere, and no greater than –1.00 D of cylinder. Corneal wavefront measurements were performed over 6 mm diameters with a Zeiss Atlas 9000 corneal topographer (Zeiss Meditec, Dublin, USA) prior to, and following lens fitting. Data were converted into rectangular Fourier optics terms M, J0, J45 and RMS values for each reading addition were statistically analyzed. Following evaluation of statistical significance and clinical relevance, results were compared to published data from successful Orthokeratology treatments. Results: Statistically significant changes in higher order aberrations were detected for lenses of all reading additions. Lens groups with higher Add-powers demonstrated stronger changes with increased significance. Final RMS values relating to 2nd, 3rd and 4th Zernike Orders reached clinical significance with a wavefront error of 0.10 μm, the equivalent of 0.25D. Moreover, as Add-powers increased, 3rd and 4th order aberrations likewise showed an increase. Pre-fitting astigmatism values accounted for the highest recorded aberrations and remained predominantly unchanged. Conclusion: Proclear Multifocal center-distance contact lenses were found to increase higher order wavefront aberrations in a manner dependent on their Add-power. In comparison to successful Orthokeratology outcomes, the amounts of resulting aberrations are notably different.
