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Unmasking of anti-Ma2/Ta paraneoplastic rhombencephalitis: case report A 69-year-old man exhibited unmasking of anti-Ma2/Ta paraneoplastic rhombencephalitis during treatment with pembrolizumab for lung cancer. The man was diagnosed with metastatic non-small cell lung cancer. Initial work-up revealed two pulmonary nodular lesions located in the right inferior and in the apical segment of the left inferior lobes. They were associated with mediastinal adenopathies and a solitary left cerebellar metastasis (stage IV, N2-M1b). ALK and EGFR mutations were absent and 100% of the neoplastic cells expressed PDL-1. He started receiving pembrolizumab every 3 weeks [route and dose not stated]. Following administration of 10 cycles of pembrolizumab, PET scan and MRI revealed a dramatic volume reduction of both the cerebellar and the pulmonary tumoural lesions. However, 1 month later, he experienced subacute cerebellar gait ataxia with left gaze oculomotor palsy and upbeat vertical nystagmus. He did not have any concurrent nutritional disorder. An MRI demonstrated T2 and Flair hyperintense lesion within the pontine tegmentum. High serum titers of anti-Ma2/Ta antibodies confirmed the diagnosis of anti-Ma2 paraneoplastic rhomboencephalitis. The rhomboencephalitis It was speculated that T-cell activation induced by pembrolizumab probably revealed a latent anti-Ma2/Ta paraneoplastic neurological disorders (PND) [time to reaction onset not stated]. Pembrolizumab was withdrawn. The man received a 5 day-course of IV methylprednisolone 1g daily, followed by a slow tapering down of oral prednisolone. however, he did not achieve any beneficial effect. His neurological condition continued to worsen, despite 3 cycles of rituximab. He eventually developed an acute respiratory distress, and died 3 months after the onset of the neurological symptoms, without recurrence of the underlying lung cancer [immediate cause of death not stated].
Unmasking of anti-Ma2/Ta paraneoplastic rhombencephalitis: case report A 69-year-old man exhibited unmasking of anti-Ma2/Ta paraneoplastic rhombencephalitis during treatment with pembrolizumab for lung cancer. The man was diagnosed with metastatic non-small cell lung cancer. Initial work-up revealed two pulmonary nodular lesions located in the right inferior and in the apical segment of the left inferior lobes. They were associated with mediastinal adenopathies and a solitary left cerebellar metastasis (stage IV, N2-M1b). ALK and EGFR mutations were absent and 100% of the neoplastic cells expressed PDL-1. He started receiving pembrolizumab every 3 weeks [route and dose not stated]. Following administration of 10 cycles of pembrolizumab, PET scan and MRI revealed a dramatic volume reduction of both the cerebellar and the pulmonary tumoural lesions. However, 1 month later, he experienced subacute cerebellar gait ataxia with left gaze oculomotor palsy and upbeat vertical nystagmus. He did not have any concurrent nutritional disorder. An MRI demonstrated T2 and Flair hyperintense lesion within the pontine tegmentum. High serum titers of anti-Ma2/Ta antibodies confirmed the diagnosis of anti-Ma2 paraneoplastic rhomboencephalitis. The rhomboencephalitis It was speculated that T-cell activation induced by pembrolizumab probably revealed a latent anti-Ma2/Ta paraneoplastic neurological disorders (PND) [time to reaction onset not stated]. Pembrolizumab was withdrawn. The man received a 5 day-course of IV methylprednisolone 1g daily, followed by a slow tapering down of oral prednisolone. however, he did not achieve any beneficial effect. His neurological condition continued to worsen, despite 3 cycles of rituximab. He eventually developed an acute respiratory distress, and died 3 months after the onset of the neurological symptoms, without recurrence of the underlying lung cancer [immediate cause of death not stated].
No abstract
Anti-Ma2 encephalitis is a rare neurological disorder with a predominant involvement of brainstem, limbic and diencephalic structures. Although an unspecific encephalopathy is the usual form of presentation, acute-onset neurologic symptoms and other atypical manifestations have been described and account for the challenging diagnosis of this entity. Despite being usually detected as a paraneoplastic syndrome in patients with early-stage tumors or without a previous history of malignancy, a growing concern has arisen from several cases reported in metastatic patients under treatment with immune checkpoint inhibitors. We report what to our knowledge is the first known case of anti-Ma2 encephalitis associated to pembrolizumab and presenting as an acute-onset focal neurological syndrome, consisting on acute global aphasia, right upper limb paresia, hypoacusia, sleep disorder, decreased conscious level and a motor focal status that was refractory to anticonvulsant therapy. A brain MRI scan showed a focal alteration of the cortical-subcortical signal on the left parietal lobe. CSF study found a significant hyperproteinorrhachia and electroencephalography showed lateralized periodic discharges (LPDs), suggestive of a diffuse encephalopathy. A positive result for anti-Ma2 antibodies was obtained both in blood and CSF samples through indirect immune-fluorescence (IFI) and later confirmed by western-blot technique. Our patient obtained a mild response to steroid therapy and a significant improvement after the administration of intravenous immunoglobulins. The hypothesis that checkpoint inhibitors may trigger the expression of previously subclinical paraneoplastic events, through the strengthening of cytotoxic T cells-mediated immune response, is supported by our finding of preexisting anti-Ma2 antibodies in preserved blood samples obtained before the initiation of pembrolizumab in our patient. Further research is needed to reveal if the detection of onconeural antibodies prior to a treatment with checkpoint inhibitors may be used as a predictive biomarker of neurologic immune-related high-grade toxicity.
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