SOX-1 antibodies in a patient with Crohn’s disease: a case report

Abstract Background The anti-SOX-1 antibodies have been mainly associated with Lambert-Eaton Myasthenic Syndrome (LETMS) and Small-Cell Lung Cancer (SCLC). In this report, we describe the interesting case of a patient with serum anti-SOX-1 antibodies and Crohn’s Disease (CD) with ensuing neurologica...

Full description

Bibliographic Details
Main Authors: Ennio Polilli, Antonella Frattari, Jessica Elisabetta Esposito, Gilda Angelini, Annalisa Di Risio, Elena Mazzotta, Simona Coladonato, Giancarlo Di Iorio, Giustino Parruti, Pierluigi Tocco
Format: Article
Language:English
Published: BMC 2022-11-01
Series:BMC Neurology
Subjects:
Online Access:https://doi.org/10.1186/s12883-022-02923-8
_version_ 1811223349080096768
author Ennio Polilli
Antonella Frattari
Jessica Elisabetta Esposito
Gilda Angelini
Annalisa Di Risio
Elena Mazzotta
Simona Coladonato
Giancarlo Di Iorio
Giustino Parruti
Pierluigi Tocco
author_facet Ennio Polilli
Antonella Frattari
Jessica Elisabetta Esposito
Gilda Angelini
Annalisa Di Risio
Elena Mazzotta
Simona Coladonato
Giancarlo Di Iorio
Giustino Parruti
Pierluigi Tocco
author_sort Ennio Polilli
collection DOAJ
description Abstract Background The anti-SOX-1 antibodies have been mainly associated with Lambert-Eaton Myasthenic Syndrome (LETMS) and Small-Cell Lung Cancer (SCLC). In this report, we describe the interesting case of a patient with serum anti-SOX-1 antibodies and Crohn’s Disease (CD) with ensuing neurological symptoms. Case presentation A Caucasian 67-year-old female was admitted to the Emergency Department with seizures, vertigo, emesis, nausea, postural instability and recurrent falls, over a period of 10 days. She had been affected by Crohn’s Disease since 1991. A CT scan failed to detect any ischemic or haemorrhagic lesion. A brain MRI revealed signs of leukoencephalopathy. Western blot analysis of her serum revealed a high titre of the onconeural antibody anti-SOX1, consistent with a neurological, cerebellar type, paraneoplastic syndrome. In spite of multiple efforts to unmask a possible underlying malignancy, no neoplastic lesion cropped up during hospitalization. Her clinical conditions progressively deteriorated, up to respiratory failure; a few days later she died, due to ensuing septic shock and Multiple Organ Failure. Conclusions Our experience may usher and reveal a new role of anti-neural antibodies, so far reckoned an early indicator of associated malignancy, suggesting that neurological syndromes associated with such antibodies may complicate also chronic Gastrointestinal (GI) diseases. As of now, testing for anti-neuronal antibodies appeared unnecessary within the diagnostic assessment of gastroenterological disorders, which may lead to overlooking incident neurologic autoimmune diseases. Further exploration of such research hypothesis in clinical grounds appears intriguing.
first_indexed 2024-04-12T08:31:22Z
format Article
id doaj.art-f2ea971e08fc49c0b53d48b9ca6931ae
institution Directory Open Access Journal
issn 1471-2377
language English
last_indexed 2024-04-12T08:31:22Z
publishDate 2022-11-01
publisher BMC
record_format Article
series BMC Neurology
spelling doaj.art-f2ea971e08fc49c0b53d48b9ca6931ae2022-12-22T03:40:13ZengBMCBMC Neurology1471-23772022-11-012211510.1186/s12883-022-02923-8SOX-1 antibodies in a patient with Crohn’s disease: a case reportEnnio Polilli0Antonella Frattari1Jessica Elisabetta Esposito2Gilda Angelini3Annalisa Di Risio4Elena Mazzotta5Simona Coladonato6Giancarlo Di Iorio7Giustino Parruti8Pierluigi Tocco9Clinical Pathology Unit, Pescara General HospitalIntensive Care Unit, Pescara General HospitalClinical Pathology Unit, Pescara General HospitalClinical Pathology Unit, Pescara General HospitalClinical Pathology Unit, Pescara General HospitalInfectious Diseases Unit, Pescara General HospitalInfectious Diseases Unit, Pescara General HospitalClinical Pathology Unit, Pescara General HospitalInfectious Diseases Unit, Pescara General HospitalNeurology and Stroke Unit, Pescara General HospitalAbstract Background The anti-SOX-1 antibodies have been mainly associated with Lambert-Eaton Myasthenic Syndrome (LETMS) and Small-Cell Lung Cancer (SCLC). In this report, we describe the interesting case of a patient with serum anti-SOX-1 antibodies and Crohn’s Disease (CD) with ensuing neurological symptoms. Case presentation A Caucasian 67-year-old female was admitted to the Emergency Department with seizures, vertigo, emesis, nausea, postural instability and recurrent falls, over a period of 10 days. She had been affected by Crohn’s Disease since 1991. A CT scan failed to detect any ischemic or haemorrhagic lesion. A brain MRI revealed signs of leukoencephalopathy. Western blot analysis of her serum revealed a high titre of the onconeural antibody anti-SOX1, consistent with a neurological, cerebellar type, paraneoplastic syndrome. In spite of multiple efforts to unmask a possible underlying malignancy, no neoplastic lesion cropped up during hospitalization. Her clinical conditions progressively deteriorated, up to respiratory failure; a few days later she died, due to ensuing septic shock and Multiple Organ Failure. Conclusions Our experience may usher and reveal a new role of anti-neural antibodies, so far reckoned an early indicator of associated malignancy, suggesting that neurological syndromes associated with such antibodies may complicate also chronic Gastrointestinal (GI) diseases. As of now, testing for anti-neuronal antibodies appeared unnecessary within the diagnostic assessment of gastroenterological disorders, which may lead to overlooking incident neurologic autoimmune diseases. Further exploration of such research hypothesis in clinical grounds appears intriguing.https://doi.org/10.1186/s12883-022-02923-8Anti-SOX1 antibodiesCrohn’s diseaseParaneoplastic neurological syndromes
spellingShingle Ennio Polilli
Antonella Frattari
Jessica Elisabetta Esposito
Gilda Angelini
Annalisa Di Risio
Elena Mazzotta
Simona Coladonato
Giancarlo Di Iorio
Giustino Parruti
Pierluigi Tocco
SOX-1 antibodies in a patient with Crohn’s disease: a case report
BMC Neurology
Anti-SOX1 antibodies
Crohn’s disease
Paraneoplastic neurological syndromes
title SOX-1 antibodies in a patient with Crohn’s disease: a case report
title_full SOX-1 antibodies in a patient with Crohn’s disease: a case report
title_fullStr SOX-1 antibodies in a patient with Crohn’s disease: a case report
title_full_unstemmed SOX-1 antibodies in a patient with Crohn’s disease: a case report
title_short SOX-1 antibodies in a patient with Crohn’s disease: a case report
title_sort sox 1 antibodies in a patient with crohn s disease a case report
topic Anti-SOX1 antibodies
Crohn’s disease
Paraneoplastic neurological syndromes
url https://doi.org/10.1186/s12883-022-02923-8
work_keys_str_mv AT enniopolilli sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT antonellafrattari sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT jessicaelisabettaesposito sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT gildaangelini sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT annalisadirisio sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT elenamazzotta sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT simonacoladonato sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT giancarlodiiorio sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT giustinoparruti sox1antibodiesinapatientwithcrohnsdiseaseacasereport
AT pierluigitocco sox1antibodiesinapatientwithcrohnsdiseaseacasereport