Anti-Hu-associated Paraneoplastic Encephalomyelitis with Esophageal Small Cell Carcinoma

Shirafuji, Toshihiko; Kanda, Fumio; Sekiguchi, Kenji; Higuchi, Masatsugu; Yokozaki, Hiroshi; Tanaka, Keiko; Takahashi, Hitoshi; Toda, Tatsushi

doi:10.2169/internalmedicine.51.6884

Cited by 7 publications

(2 citation statements)

References 12 publications

(13 reference statements)

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“…In this case, lung adenocarcinoma was suspected clinically because an adenocarcinoma component was identified on bronchial brush cytology. 8 In several studies, neuron loss, gliosis, microglial cell activation and the presence of infiltrating leukocytes have been reported as neuropathologic changes in this condition [9][10][11][12][13][14][15][16][17][18] However, neurons were preserved and levels of infiltrating leukocytes were relatively mild in our patient. 6 This poorly differentiated NSCLC is characterized by having at least 10% spindle and/or giant cells or a spindle and giant cell alone.…”

Section: Discussionmentioning

confidence: 46%

Paraneoplastic autoimmune encephalitis associated with pleomorphic lung carcinoma: An autopsy case report

et al. 2018

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A 64-year-old man was admitted with acute onset disturbed consciousness. Cerebrospinal fluid analysis revealed pleocytosis and elevated protein, with negative cultures and PCR. Serum antibodies for autoimmune encephalitis were also negative. Brain magnetic resonance imaging (MRI) was unremarkable, but whole-body CT scan showed a tumor in the left lower lung lobe. Bronchial brush cytology demonstrated clusters of malignant cells, and F-fluorodeoxyglucose positron emission tomography showed multiple lesions and increased uptake in the lung tumor. Clinically the patient had a stage IV lung carcinoma, graded as T3N3M1b (OSS). Steroid therapy had limited efficacy, but chemotherapy dramatically improved his neurological symptoms. Therefore, he was diagnosed with paraneoplastic autoimmune encephalitis based on the diagnostic criteria for paraneoplastic neurological syndromes. He died due to disease progression 14 months later. Subsequent postmortem examination revealed white ill-defined nodules in the left lung, with similar nodules in other organs. The brain weighed 1500 g before fixation, and a nodule was observed in the right precentral gyrus. Microscopically, the lung tumor was a pleomorphic carcinoma with an adenocarcinoma component. Multiple areas of micro-softening (≤500 μm) were identified in the cerebral cortex, gray-white matter junction and basal ganglia, and were distributed diffusely in both the limbic and non-limbic systems. Mild lymphocytic infiltrates were observed involving few intraparenchymal vessels. Few tumor metastases were observed in the right precentral gyrus. The multiple micro-softenings may reflect a chronic neuropathologic change of paraneoplastic autoimmune encephalitis. They were too small to be detected by brain MRI. However, these lesions may have the potential to cause the neurological symptoms in the acute phase because they were observed in many anatomical regions. We should pay attention to subtle findings such as micro-softenings when estimating the neuropathology of autoimmune encephalitis. Further investigations are needed to understand the characteristic neuropathology of this condition.

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Section: Discussionmentioning

confidence: 46%

Paraneoplastic autoimmune encephalitis associated with pleomorphic lung carcinoma: An autopsy case report

et al. 2018

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“…These paraneoplastic-associated antibodies often cause LE. It has been shown that LE usually refers to a tumor anti-neuron antibody (such as anti-Hu) and cell surface antibody [alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic antibody (AMPAR)] ( 43 ). The clinical features of LE are short-term memory loss, seizures, irritability, depression, and cognitive decline in subacute onset.…”

Section: Discussionmentioning

confidence: 99%

PD-1-induced encephalopathy: a report of 2 cases on neurological toxicities with immune checkpoint inhibitors

Chen,

Ye,

et al. 2024

Transl Cancer Res

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Background Immune-related adverse effects (irAEs) often occur during immune checkpoint inhibitor (ICI) therapy. In the nervous system, the incidence of irAEs ranges from 0.1–12%, with 80% occurring within the first 4 months of ICI application. For complications of the nervous system, adequate diagnosis is made by signs, symptoms, imaging and cerebrospinal fluid. If severe irAEs occur, ICIs should be discontinued and patients should be treated with high-dose glucocorticoids, immunoglobulins, or immunosorbent therapy with systemic support. Patients who develop severe neurologic irAEs have a poorer prognosis. Case Description In this article, we report 2 cases of encephalopathy induced by anti-programmed cell death protein 1 (PD-1) monoclonal antibodies at the initial diagnoses. Our findings may help clinicians to differentiate between encephalopathy caused by immunotherapy and other neurological disorders. Case 1 was a 24-year-old male patient who had undergone PD-1 immunotherapy to treat olfactory neuroblastoma. After the 6th course of therapy, he began to develop persistent epilepsy, which decreased significantly after high doses of glucocorticoid and immunosorbent therapy were administered. Based on his medical history and laboratory examination results, PD-1-induced encephalopathy was the most likely diagnosis. Case 2 was a 67-year-old female patient who had been treated with PD-1/programmed death ligand-1 therapy for lung adenocarcinoma. She began to have headaches after 1 cycle of treatment, and her cognitive function gradually decreased with the continuation of immunotherapy. Conclusions These case reports show the difficulty in distinguishing PD-1-induced encephalopathy from other neurological disorders, especially paraneoplastic neurological syndromes. If not treated properly, patients’ lives may be endangered. Thus, early identification and early treatment are very important.

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Neurologic Complications of Gastrointestinal Cancer

Magge¹,

Diamond

2017

Cancer Neurology in Clinical Practice

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Anti-Hu-associated Paraneoplastic Encephalomyelitis with Esophageal Small Cell Carcinoma

Cited by 7 publications

References 12 publications

Paraneoplastic autoimmune encephalitis associated with pleomorphic lung carcinoma: An autopsy case report

Paraneoplastic autoimmune encephalitis associated with pleomorphic lung carcinoma: An autopsy case report

PD-1-induced encephalopathy: a report of 2 cases on neurological toxicities with immune checkpoint inhibitors

Neurologic Complications of Gastrointestinal Cancer

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