Bilateral Frontal Lobe Vasogenic Edema Resulting from Hypertrophic Pachymeningitis due to Granulomatosis with Polyangiitis

Itaya, Sakiko; Ueda, Yasuhiro; Kobayashi, Zen; Tomimitsu, Hiroyuki; Kobayashi, Daisuke; Sato, Shuzo

doi:10.2169/internalmedicine.8832-17

Cited by 6 publications

(6 citation statements)

References 4 publications

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“…It is notable that posterior fossa HP can cause acute non-communicating hydrocephalus ( 9 ). In addition to the posterior fossa, thickened falx cerebri ( 10 – 14 ), anterior cranial fossa ( 15 ), cerebellar tentorium ( 10 , 16 ), frontal lobes ( 10 , 11 , 17 ), sphenoid wings ( 18 ), cavernous sinuses ( 19 ), and paranasal maxillary sinuses ( 19 , 20 ) were also involved in HP patients. With enhancements in the cerebral falx and tentorium cerebelli in coronal scanning, a typical “Eiffel Tower” ( 21 , 22 ) or “Benz” sign emerged ( 23 ).…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Xiao

2020

Front. Neurol.

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Aims: To investigate the causes, clinical characteristics, imaging features, and therapeutic implications of hypertrophic pachymeningitis (HP) in a southern Chinese population. Methods: We retrospectively analyzed 48 patients with HP with different causes from 1 January 2006 to 31 December 2018. Clinical manifestation, laboratory findings, and neuroimaging results were evaluated in all HP patients. Results: The mean age at onset was 50 ± 12 years. The most common diagnosis was idiopathic HP (67%), followed by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). Headache was the most common symptom. The most frequently changed laboratory finding was elevated erythrocyte sedimentation rate (ESR). Imaging was characterized by cerebral or spinal dura mater enhancement in MRI scan with contrast. Enhancements were mainly located in the posterior fossa for idiopathic HP; frontal, parietal, and occipital lobes for ANCA-related HP; and posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment was applied in most cases. Conclusions: The etiology of HP varied among patients, with idiopathic HP being the most common. MRI showed enhancement of the dura mater, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.

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Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Xiao

2020

Front. Neurol.

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“…It is notable that posterior fossa HP can cause acute noncommunicating hydrocephalus [9]. In addition to the posterior fossa, thickened falx cerebri [10][11][12][13][14], anterior cranial fossa [15], cerebellar tentorium [10,16], frontal lobes [10,11,17], sphenoid wings [18], cavernous sinuses [19], and paranasal maxillary sinuses [19,20] were also involved in HP patients.…”

Section: Imaging Featuresmentioning

confidence: 99%

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

Xiao

Feng

2020

Preprint

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Background Hypertrophic pachymeningitis (HP) is a fibrotic disorder featuring a thickening of the dura matter. Most HP studies were from Caucasian population and only a few studies of HP are available in China. In this study, we investigated the causes, clinical and imaging features and therapeutic implications of HP in a southern Chinese population.Methods We retrospectively analyzed 48 patients with HP with different causes from 1 January 2006 to 31 December 2018. Clinical manifestation, laboratory findings, neuroimaging results, and clinical course were evaluated in all HP patients.Results The mean age at onset was 50±12 years. The most common diagnosis was idiopathic HP (67%), followed by ANCA-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). The main clinical manifestations were headache and cranial nerve deficits. The most frequently changed laboratory finding was elevated ESR. Imaging was characterized by cerebral or spinal dura matter enhancement in MRI scan with contrast. Enhancements were mainly located in the posterior fossa for idiopathic HP; frontal, parietal and occipital lobes for ANCA-related HP; and the posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment were applied in most cases.Conclusions HP was diagnosed based on clinical manifestations, imaging and laboratory results. Etiology varied among patients, with idiopathic HP being the most common. MRI with contrast showed enhancement of the dura matter, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.

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“…At no time was she treated with steroids during her hospital admission. and often warrant an expansive evaluation [4]. This case presentation amplifies the need for consensus diagnosis in difficult cases, complemented by exhaustive investigation, including brain biopsy.…”

Section: Case Reportmentioning

confidence: 85%

“…Vasogenic oedema, resulting from breakdown of the blood–brain barrier, is often seen in malignant processes [ 1 ]. Several auto-immune and para-neoplastic phenomena also present with cerebral oedema and often warrant an expansive evaluation [ 4 ]. This case presentation amplifies the need for consensus diagnosis in difficult cases, complemented by exhaustive investigation, including brain biopsy.…”

Section: Introductionmentioning

confidence: 99%

A Diagnostic Dilemma of White Matter Lesions and Cerebral Oedema without Identifiable Cause—A Neurological Conundrum

et al. 2021

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Introduction: This paper describes a case of bi-frontal vasogenic oedema associated with bilateral frontal lobe and left parietal lobe white matter lesions where extensive investigations, including brain biopsy, failed to establish a diagnosis. Case Report: A 67-year-old female presented with three weeks’ history of memory loss, fatigue, insomnia, nausea, and occasional dysphasia. Physical examination was unremarkable, yet cerebral CT and MRI showed bilateral frontal lobe vasogenic oedema. Extensive investigations, including: biochemical; radiological; immunological; microbiological; haematological; histopathological; and cytological, failed to establish a confirmed diagnosis. A multidisciplinary team could not achieve a consensus for this atypical presentation. Brain biopsy was unusual, showing destructive inflammatory and subtly granulomatous disease, but an exhaustive list of auxiliary tests could not confirm a cause, and consensus favoured glial fibrillary acidic protein (GFAP) autoimmune encephalopathy. Discussion: A definitive diagnosis could not be established for this patient despite a gamut of investigations. Although some of the presenting features were consistent with GFAP astrocytopathy, initial staining of the patient’s CSF for neuronal antibodies was negative. Her symptoms and radiological changes of brain imaging improved without any corticosteroid therapy. Conclusions: Through this case report, the aim is to add to the repository of neurological sciences in the hope that future similar presentations could potentially lead to discovery of a new aetiology or contribute towards better understanding of an existing disease process.

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Bilateral Frontal Lobe Vasogenic Edema Resulting from Hypertrophic Pachymeningitis due to Granulomatosis with Polyangiitis

Cited by 6 publications

References 4 publications

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

A Diagnostic Dilemma of White Matter Lesions and Cerebral Oedema without Identifiable Cause—A Neurological Conundrum

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