Breast cancer-associated opsoclonus-myoclonus syndrome: a case report

Abstract Background Paraneoplastic neurological syndromes constitute rare neurological complications of malignant disease, manifesting in <1% of patients with cancer. Opsoclonus-myoclonus syndrome (OMS) presents with chaotic ocular saccades (opsoclonus), spontaneous muscular jerking (myoclonus) t...

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Autores principales: Aikaterini Kostoglou, Dimitrios Vlastos, Athanasios Bakalis, Debashis Ghosh
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Publicado: BMC 2021
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spelling oai:doaj.org-article:0b61d1953e3c4dc1b417b1c26a17783d2021-11-21T12:33:37ZBreast cancer-associated opsoclonus-myoclonus syndrome: a case report10.1186/s12957-021-02436-71477-7819https://doaj.org/article/0b61d1953e3c4dc1b417b1c26a17783d2021-11-01T00:00:00Zhttps://doi.org/10.1186/s12957-021-02436-7https://doaj.org/toc/1477-7819Abstract Background Paraneoplastic neurological syndromes constitute rare neurological complications of malignant disease, manifesting in <1% of patients with cancer. Opsoclonus-myoclonus syndrome (OMS) presents with chaotic ocular saccades (opsoclonus), spontaneous muscular jerking (myoclonus) that may be accompanied by ataxia, strabismus, aphasia, or mutism. Its paraneoplastic variant in the adult is most commonly associated with small-cell lung cancer, followed by breast cancer. Importantly, neurological symptoms usually precede the diagnosis of breast cancer and tend to recure after its treatment. Case presentation A 43-year-old premenopausal Caucasian woman with a medical history of hypertension was admitted following an episode of focal seizure. This progressed to generalised tonic-clonic seizures and she was subsequently loaded with phenytoin, valproate, and levetiracetam. Initial workup included whole body CT scan, viral and autoimmune serology. The CT scan revealed an enhancing right axillary lymph node, which in combination with Anti-Ri antibody positivity raised the spectre of paraneoplastic OMS. MRI of the head revealed subtle nonspecific white matter signal change within the centrum semiovale without any mass lesions, while MRI of the spine was unremarkable. An uncomplicated right mastectomy and axillary lymph node clearance was performed: histopathology revealed a 9-mm, grade 2, oestrogen receptor-positive, progesterone receptor-negative (ER8, PR0), Her2-negative invasive ductal carcinoma, and 4/6 positive lymph nodes (T1b N2 M0). Two months later, she was readmitted with vertigo, diplopia, facial weakness, and ataxia, setting the diagnosis anti-Ri syndrome recurrence. MDT recommended mammogram and ultrasound of the left breast, which were normal. Subsequently, four months after initial discharge, she suffered another neurological recurrence; due to concomitant abdominal pain, PET-CT was performed demonstrating a hypermetabolic right ovarian focus. Bilateral salpingo-oophorectomy was performed as per gynaecology MDT and final histology showed normal tubes and ovaries. She has remained on remission since then, with a negative annual mammogram follow-up. Conclusions In conclusion, we report a case of OMS associated with breast cancer anti-Ri onconeural antibody. Its manifestations preceded the diagnosis of malignancy and it persisted after cancer treatment, underlining the importance for high clinical suspicion in cases of classical paraneoplastic neurological syndromes as well as the need for long-term clinical follow-up.Aikaterini KostoglouDimitrios VlastosAthanasios BakalisDebashis GhoshBMCarticleParaneoplastic syndromeBreast cancerAnti-RiOpsoclonus-myoclonus syndromeSurgeryRD1-811Neoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENWorld Journal of Surgical Oncology, Vol 19, Iss 1, Pp 1-5 (2021)
institution DOAJ
collection DOAJ
language EN
topic Paraneoplastic syndrome
Breast cancer
Anti-Ri
Opsoclonus-myoclonus syndrome
Surgery
RD1-811
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
spellingShingle Paraneoplastic syndrome
Breast cancer
Anti-Ri
Opsoclonus-myoclonus syndrome
Surgery
RD1-811
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
RC254-282
Aikaterini Kostoglou
Dimitrios Vlastos
Athanasios Bakalis
Debashis Ghosh
Breast cancer-associated opsoclonus-myoclonus syndrome: a case report
description Abstract Background Paraneoplastic neurological syndromes constitute rare neurological complications of malignant disease, manifesting in <1% of patients with cancer. Opsoclonus-myoclonus syndrome (OMS) presents with chaotic ocular saccades (opsoclonus), spontaneous muscular jerking (myoclonus) that may be accompanied by ataxia, strabismus, aphasia, or mutism. Its paraneoplastic variant in the adult is most commonly associated with small-cell lung cancer, followed by breast cancer. Importantly, neurological symptoms usually precede the diagnosis of breast cancer and tend to recure after its treatment. Case presentation A 43-year-old premenopausal Caucasian woman with a medical history of hypertension was admitted following an episode of focal seizure. This progressed to generalised tonic-clonic seizures and she was subsequently loaded with phenytoin, valproate, and levetiracetam. Initial workup included whole body CT scan, viral and autoimmune serology. The CT scan revealed an enhancing right axillary lymph node, which in combination with Anti-Ri antibody positivity raised the spectre of paraneoplastic OMS. MRI of the head revealed subtle nonspecific white matter signal change within the centrum semiovale without any mass lesions, while MRI of the spine was unremarkable. An uncomplicated right mastectomy and axillary lymph node clearance was performed: histopathology revealed a 9-mm, grade 2, oestrogen receptor-positive, progesterone receptor-negative (ER8, PR0), Her2-negative invasive ductal carcinoma, and 4/6 positive lymph nodes (T1b N2 M0). Two months later, she was readmitted with vertigo, diplopia, facial weakness, and ataxia, setting the diagnosis anti-Ri syndrome recurrence. MDT recommended mammogram and ultrasound of the left breast, which were normal. Subsequently, four months after initial discharge, she suffered another neurological recurrence; due to concomitant abdominal pain, PET-CT was performed demonstrating a hypermetabolic right ovarian focus. Bilateral salpingo-oophorectomy was performed as per gynaecology MDT and final histology showed normal tubes and ovaries. She has remained on remission since then, with a negative annual mammogram follow-up. Conclusions In conclusion, we report a case of OMS associated with breast cancer anti-Ri onconeural antibody. Its manifestations preceded the diagnosis of malignancy and it persisted after cancer treatment, underlining the importance for high clinical suspicion in cases of classical paraneoplastic neurological syndromes as well as the need for long-term clinical follow-up.
format article
author Aikaterini Kostoglou
Dimitrios Vlastos
Athanasios Bakalis
Debashis Ghosh
author_facet Aikaterini Kostoglou
Dimitrios Vlastos
Athanasios Bakalis
Debashis Ghosh
author_sort Aikaterini Kostoglou
title Breast cancer-associated opsoclonus-myoclonus syndrome: a case report
title_short Breast cancer-associated opsoclonus-myoclonus syndrome: a case report
title_full Breast cancer-associated opsoclonus-myoclonus syndrome: a case report
title_fullStr Breast cancer-associated opsoclonus-myoclonus syndrome: a case report
title_full_unstemmed Breast cancer-associated opsoclonus-myoclonus syndrome: a case report
title_sort breast cancer-associated opsoclonus-myoclonus syndrome: a case report
publisher BMC
publishDate 2021
url https://doaj.org/article/0b61d1953e3c4dc1b417b1c26a17783d
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AT dimitriosvlastos breastcancerassociatedopsoclonusmyoclonussyndromeacasereport
AT athanasiosbakalis breastcancerassociatedopsoclonusmyoclonussyndromeacasereport
AT debashisghosh breastcancerassociatedopsoclonusmyoclonussyndromeacasereport
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