“…In some circumstances, a particular neurological disorder is encountered frequently with a specific autoantibody (eg, cerebellar ataxia in patients with PCA-1 [anti-yo]) 17 However, the neurological manifestations associated with any 1 paraneoplastic antibody are protean (in one series 60% of PCA-1-seropositive patients had neurological features in addition to ataxia, 23% did not have ataxia at presentation, and 11% never developed ataxia). 11 Thus, testing for a single antibody (individually rare) on the premise of a particular neurological syndrome is likely to have low diagnostic yield, and comprehensive serological evaluations are usually preferred. 12 An example of an exception to this rule is the NMDA receptor antibody encephalitis (described below), which has a fairly stereotyped sequence of neuropsychiatric and neurological events that characterize its course.…”
Section: Some Common Neurological Disordersmentioning
confidence: 99%
“…9 The neurological deficits usually do not improve with treatment, although sometimes the clinical picture stabilizes post oncological therapy. 10,11 Deaths from the complications of neurological disease (rather than from metastatic cancer) are common.…”
As a result of the burgeoning growth of disease-specific neural autoantibody markers available for diagnostic patient evaluation, there has been increasing awareness of autoimmune central nervous system (CNS) disorders in hospital practice. Hospital-based neurologists have also taken great interest in these disorders since many occur in the setting of an occult systemic cancer which can be detected and treated at an early stage, and many affected patients are responsive to immunotherapy. Associated neurological disorders are typically subacute in onset, some are common or classic (eg, limbic encephalitis, cerebellar degeneration), but others have atypical or multifocal presentations. For patients with a suspected paraneoplastic disorder, many and costly oncological evaluations may be required for diagnosis. Comprehensive serological and cerebrospinal fluid (CSF) evaluation for neural autoantibodies may permit a focused cancer evaluation (eg, antineuronal nuclear antibody type 1 [ANNA-1] is associated with small cell lung carcinoma), and in some circumstances may indicate the likelihood of a good response to therapy (eg, voltagegated potassium channel complex antibody) or poor neurological prognosis (eg, purkinje cell cytoplasmic antibody type 1 [antiYo]). Positron-emission tomography-computed tomography (PET-CT) imaging of trunk may increase the diagnostic yield for certain cancers where other modalities have been negative. For some patients, rapid treatment with immunotherapy may facilitate marked improvement, or full recovery; multiple sequential trials of one or more of steroids, intravenous immunoglobulin or plasma exchange, or combination therapy are often required. For patients with N-methyl-D-aspartate receptor antibody encephalitis, early treatment with immunosuppressants and weeks or months of supportive intensive care may additionally be required. One or more of clinical examination, electroencephalogram (including video telemetry), and imaging provide objective parameters to which posttreatment outcomes can be compared.
“…In some circumstances, a particular neurological disorder is encountered frequently with a specific autoantibody (eg, cerebellar ataxia in patients with PCA-1 [anti-yo]) 17 However, the neurological manifestations associated with any 1 paraneoplastic antibody are protean (in one series 60% of PCA-1-seropositive patients had neurological features in addition to ataxia, 23% did not have ataxia at presentation, and 11% never developed ataxia). 11 Thus, testing for a single antibody (individually rare) on the premise of a particular neurological syndrome is likely to have low diagnostic yield, and comprehensive serological evaluations are usually preferred. 12 An example of an exception to this rule is the NMDA receptor antibody encephalitis (described below), which has a fairly stereotyped sequence of neuropsychiatric and neurological events that characterize its course.…”
Section: Some Common Neurological Disordersmentioning
confidence: 99%
“…9 The neurological deficits usually do not improve with treatment, although sometimes the clinical picture stabilizes post oncological therapy. 10,11 Deaths from the complications of neurological disease (rather than from metastatic cancer) are common.…”
As a result of the burgeoning growth of disease-specific neural autoantibody markers available for diagnostic patient evaluation, there has been increasing awareness of autoimmune central nervous system (CNS) disorders in hospital practice. Hospital-based neurologists have also taken great interest in these disorders since many occur in the setting of an occult systemic cancer which can be detected and treated at an early stage, and many affected patients are responsive to immunotherapy. Associated neurological disorders are typically subacute in onset, some are common or classic (eg, limbic encephalitis, cerebellar degeneration), but others have atypical or multifocal presentations. For patients with a suspected paraneoplastic disorder, many and costly oncological evaluations may be required for diagnosis. Comprehensive serological and cerebrospinal fluid (CSF) evaluation for neural autoantibodies may permit a focused cancer evaluation (eg, antineuronal nuclear antibody type 1 [ANNA-1] is associated with small cell lung carcinoma), and in some circumstances may indicate the likelihood of a good response to therapy (eg, voltagegated potassium channel complex antibody) or poor neurological prognosis (eg, purkinje cell cytoplasmic antibody type 1 [antiYo]). Positron-emission tomography-computed tomography (PET-CT) imaging of trunk may increase the diagnostic yield for certain cancers where other modalities have been negative. For some patients, rapid treatment with immunotherapy may facilitate marked improvement, or full recovery; multiple sequential trials of one or more of steroids, intravenous immunoglobulin or plasma exchange, or combination therapy are often required. For patients with N-methyl-D-aspartate receptor antibody encephalitis, early treatment with immunosuppressants and weeks or months of supportive intensive care may additionally be required. One or more of clinical examination, electroencephalogram (including video telemetry), and imaging provide objective parameters to which posttreatment outcomes can be compared.
“…The prototypic example of this disorder is autoimmunity targeting Yo (CDR2) in patients seropositive for Purkinje cell cytoplasmic antibody type 1 (PCA-1, also known as anti-Yo antibody). 2,3 Pathophysiologic and pathologic studies 4,5 have suggested that this autoimmunity targeting is mediated by cytotoxic (CD8 + ) T lymphocytes rather than by PCA-1 antibody. Ninety percent of affected women develop cerebellar ataxia, and 90% have accompanying gynecologic or breast adenocarcinomas.…”
mentioning
confidence: 99%
“…Ninety percent of affected women develop cerebellar ataxia, and 90% have accompanying gynecologic or breast adenocarcinomas. 5 Reported neurologic improvements attributable to immunotherapy (corticosteroids, intravenous immunoglobulin, plasma exchange, and immunosuppressants) are rare, and the median survival from symptom onset is just 2 years, with death due to cancer or neurologic decline. Little has been published regarding treatment responses and neurologic outcomes among patients with autoimmune cerebellar ataxia in general.…”
cell cytoplasmic antibody type 1 (PCA-1, or anti-Yo) paraneoplastic cerebellar ataxia has a poor prognosis, yet little has been published otherwise regarding treatment responses and outcomes among patients with autoimmune cerebellar ataxia.OBJECTIVES To investigate treatment responses and outcomes in adults with autoimmune cerebellar ataxia.
“…It may present with or be preceded by other signs of neurologic autoimmune reaction by affecting the pyramidal tract, brainstem, anterior horn cell, and peripheral nerves. 3 Cognitive impairment and personality change have also been reported.…”
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