An atypical presentation of diffuse midline pontine glioma in a middle age patient: Case report

S Floro, D Belvedere, C Rosci, M Secchi, C Casellato, G Oggioni, C Gambini, L Campiglio, M Zardoni, R Altavilla, F Bianchi, A D'Arrigo, F Bartesaghi, M Egidi, C Uggetti, G Giaccone, G Marucci, A Mastronuzzi, M Vinci, A Priori

Research output: Contribution to journalArticle

Abstract

INTRODUCTION: Diffuse midline glioma is a newly WHO defined entity (grade IV) (Louis et al., 2016) which includes diffuse intrinsic pontine glioma (DIPG) reported in pediatric population and, occasionally, in young adults. Here, we present a detailed description of an atypical case of diffuse midline glioma in a 53 years old woman.

CASE REPORT: A caucasian woman aged 53 from Ukraine, was referred to another neurological department complaining of 3 months history of progressive postural instability and gait impairment with frequent falling. Magnetic resonance demonstrated two brainstem lesions, hyperintense in FLAIR with "patchy" peripheral enhancement, leptomeningeal and cranial nerves enhancement. CSF was normal. Due to positive antinuclear antibodies test (ANA 1:360), intravenous steroid treatment was administered and reported to initially improve the patient condition. However, the following weeks the lady worsened. Imaging features were unchanged. Because quantiferon test resulted positive, MRI-Spectroscopy showed an inflammatory pattern and MRI perfusion study and brain FDG-PET, were normal, tubercolar granulomatous hypothesis was initially favored. Antitubercular therapy with isoniazid, pyrazinamide, ethambutol and rifampicin was started without any clinical improvement. Hence, the biopsy was proposed. The procedure revealed a diffuse midline pontine glioma. Considering the advanced stage of the disease, radiotherapy was not indicated. Patient died after eight months from the onset of neurological disturbances.

CONCLUSION: Our case shows that diffuse midline glioma is a CNS tumor not limited to young population but occurring also in middle aged patients with an insidious pattern. We therefore recommend to perform biopsy at very early stages in patients with atypical brainstem lesions.

Original languageEnglish
JournalJournal of Clinical Neuroscience
DOIs
Publication statusE-pub ahead of print - Sep 20 2019

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Glioma
Brain Stem
Accidental Falls
Biopsy
Pyrazinamide
Ukraine
Ethambutol
Cranial Nerves
Antinuclear Antibodies
Isoniazid
Rifampin
Gait
Peripheral Nerves
Population
Young Adult
Spectrum Analysis
Magnetic Resonance Spectroscopy
Radiotherapy
Perfusion
Steroids

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An atypical presentation of diffuse midline pontine glioma in a middle age patient : Case report. / Floro, S; Belvedere, D; Rosci, C; Secchi, M; Casellato, C; Oggioni, G; Gambini, C; Campiglio, L; Zardoni, M; Altavilla, R; Bianchi, F; D'Arrigo, A; Bartesaghi, F; Egidi, M; Uggetti, C; Giaccone, G; Marucci, G; Mastronuzzi, A; Vinci, M; Priori, A.

In: Journal of Clinical Neuroscience, 20.09.2019.

Research output: Contribution to journalArticle

Floro, S, Belvedere, D, Rosci, C, Secchi, M, Casellato, C, Oggioni, G, Gambini, C, Campiglio, L, Zardoni, M, Altavilla, R, Bianchi, F, D'Arrigo, A, Bartesaghi, F, Egidi, M, Uggetti, C, Giaccone, G, Marucci, G, Mastronuzzi, A, Vinci, M & Priori, A 2019, 'An atypical presentation of diffuse midline pontine glioma in a middle age patient: Case report', Journal of Clinical Neuroscience. https://doi.org/10.1016/j.jocn.2019.08.096
Floro, S ; Belvedere, D ; Rosci, C ; Secchi, M ; Casellato, C ; Oggioni, G ; Gambini, C ; Campiglio, L ; Zardoni, M ; Altavilla, R ; Bianchi, F ; D'Arrigo, A ; Bartesaghi, F ; Egidi, M ; Uggetti, C ; Giaccone, G ; Marucci, G ; Mastronuzzi, A ; Vinci, M ; Priori, A. / An atypical presentation of diffuse midline pontine glioma in a middle age patient : Case report. In: Journal of Clinical Neuroscience. 2019.
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abstract = "INTRODUCTION: Diffuse midline glioma is a newly WHO defined entity (grade IV) (Louis et al., 2016) which includes diffuse intrinsic pontine glioma (DIPG) reported in pediatric population and, occasionally, in young adults. Here, we present a detailed description of an atypical case of diffuse midline glioma in a 53 years old woman.CASE REPORT: A caucasian woman aged 53 from Ukraine, was referred to another neurological department complaining of 3 months history of progressive postural instability and gait impairment with frequent falling. Magnetic resonance demonstrated two brainstem lesions, hyperintense in FLAIR with {"}patchy{"} peripheral enhancement, leptomeningeal and cranial nerves enhancement. CSF was normal. Due to positive antinuclear antibodies test (ANA 1:360), intravenous steroid treatment was administered and reported to initially improve the patient condition. However, the following weeks the lady worsened. Imaging features were unchanged. Because quantiferon test resulted positive, MRI-Spectroscopy showed an inflammatory pattern and MRI perfusion study and brain FDG-PET, were normal, tubercolar granulomatous hypothesis was initially favored. Antitubercular therapy with isoniazid, pyrazinamide, ethambutol and rifampicin was started without any clinical improvement. Hence, the biopsy was proposed. The procedure revealed a diffuse midline pontine glioma. Considering the advanced stage of the disease, radiotherapy was not indicated. Patient died after eight months from the onset of neurological disturbances.CONCLUSION: Our case shows that diffuse midline glioma is a CNS tumor not limited to young population but occurring also in middle aged patients with an insidious pattern. We therefore recommend to perform biopsy at very early stages in patients with atypical brainstem lesions.",
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T1 - An atypical presentation of diffuse midline pontine glioma in a middle age patient

T2 - Case report

AU - Floro, S

AU - Belvedere, D

AU - Rosci, C

AU - Secchi, M

AU - Casellato, C

AU - Oggioni, G

AU - Gambini, C

AU - Campiglio, L

AU - Zardoni, M

AU - Altavilla, R

AU - Bianchi, F

AU - D'Arrigo, A

AU - Bartesaghi, F

AU - Egidi, M

AU - Uggetti, C

AU - Giaccone, G

AU - Marucci, G

AU - Mastronuzzi, A

AU - Vinci, M

AU - Priori, A

N1 - Copyright © 2019. Published by Elsevier Ltd.

PY - 2019/9/20

Y1 - 2019/9/20

N2 - INTRODUCTION: Diffuse midline glioma is a newly WHO defined entity (grade IV) (Louis et al., 2016) which includes diffuse intrinsic pontine glioma (DIPG) reported in pediatric population and, occasionally, in young adults. Here, we present a detailed description of an atypical case of diffuse midline glioma in a 53 years old woman.CASE REPORT: A caucasian woman aged 53 from Ukraine, was referred to another neurological department complaining of 3 months history of progressive postural instability and gait impairment with frequent falling. Magnetic resonance demonstrated two brainstem lesions, hyperintense in FLAIR with "patchy" peripheral enhancement, leptomeningeal and cranial nerves enhancement. CSF was normal. Due to positive antinuclear antibodies test (ANA 1:360), intravenous steroid treatment was administered and reported to initially improve the patient condition. However, the following weeks the lady worsened. Imaging features were unchanged. Because quantiferon test resulted positive, MRI-Spectroscopy showed an inflammatory pattern and MRI perfusion study and brain FDG-PET, were normal, tubercolar granulomatous hypothesis was initially favored. Antitubercular therapy with isoniazid, pyrazinamide, ethambutol and rifampicin was started without any clinical improvement. Hence, the biopsy was proposed. The procedure revealed a diffuse midline pontine glioma. Considering the advanced stage of the disease, radiotherapy was not indicated. Patient died after eight months from the onset of neurological disturbances.CONCLUSION: Our case shows that diffuse midline glioma is a CNS tumor not limited to young population but occurring also in middle aged patients with an insidious pattern. We therefore recommend to perform biopsy at very early stages in patients with atypical brainstem lesions.

AB - INTRODUCTION: Diffuse midline glioma is a newly WHO defined entity (grade IV) (Louis et al., 2016) which includes diffuse intrinsic pontine glioma (DIPG) reported in pediatric population and, occasionally, in young adults. Here, we present a detailed description of an atypical case of diffuse midline glioma in a 53 years old woman.CASE REPORT: A caucasian woman aged 53 from Ukraine, was referred to another neurological department complaining of 3 months history of progressive postural instability and gait impairment with frequent falling. Magnetic resonance demonstrated two brainstem lesions, hyperintense in FLAIR with "patchy" peripheral enhancement, leptomeningeal and cranial nerves enhancement. CSF was normal. Due to positive antinuclear antibodies test (ANA 1:360), intravenous steroid treatment was administered and reported to initially improve the patient condition. However, the following weeks the lady worsened. Imaging features were unchanged. Because quantiferon test resulted positive, MRI-Spectroscopy showed an inflammatory pattern and MRI perfusion study and brain FDG-PET, were normal, tubercolar granulomatous hypothesis was initially favored. Antitubercular therapy with isoniazid, pyrazinamide, ethambutol and rifampicin was started without any clinical improvement. Hence, the biopsy was proposed. The procedure revealed a diffuse midline pontine glioma. Considering the advanced stage of the disease, radiotherapy was not indicated. Patient died after eight months from the onset of neurological disturbances.CONCLUSION: Our case shows that diffuse midline glioma is a CNS tumor not limited to young population but occurring also in middle aged patients with an insidious pattern. We therefore recommend to perform biopsy at very early stages in patients with atypical brainstem lesions.

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SN - 0967-5868

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