Ring-enhanced Brainstem Lesion in a Patient with Neuro-Behcet's Disease

Yasuda, Chiharu; Okada, Kazumasa; Takechi, Utako; Tsuji, Sadatoshi

doi:10.2169/internalmedicine.51.6789

Cited by 4 publications

(3 citation statements)

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(1 reference statement)

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“…Varying patterns were observed on MRI throughout the patient history: (a) hyper-intensity on T2WI at the globus pallidus, (b) ring-enhancing lesion with central hyper-intensity on DWI, and (c) multiple patchy hyper-intense areas on DWI, T2WI, FLAIR, and Gad-T1WI. The findings of (a) and (c) correspond to the presentation of A-P-NBD, whereas those of (b) may correspond to NBD, as some case reports described NBD causing ring-enhancing lesions [26–29]. Nishino et al reported that GPA can cause cerebritis, but it is rare among the other neurological manifestations in GPA (approximately 4%) [30].…”

Section: Discussionmentioning

confidence: 97%

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

et al. 2019

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Patient: Male, 67 Final Diagnosis: Unclassified vasculitis Symptoms: Fever • ocular pain • erythema • chest pain • headache Medication: — Clinical Procedure: Biopsy Specialty: Rheumatology Objective: Rare disease Background: Systemic vasculitides constitute heterogenous conditions affecting many organs and systems through blood vessel inflammation. Although there are some classifications for vasculitis, several vasculitides are “unclassified” because they cannot be clearly assigned to one of the known entities. Case Report: We report an autopsy case of a 67-year-old Japanese man who presented with fever, ocular pain, erythema, chest pain, and headache. The disease caused episcleritis, thrombophlebitis, extensive deep vein thrombosis, multiple pulmonary nodules and masses, hypertrophic pachymeningitis, and hyper-intensity areas in brain parenchyma on magnetic resonance images. Histopathology of the pulmonary nodule confirmed vasculitis affecting medium-to-small veins and arteries without necrotizing vasculitis or granulomatous inflammation. We diagnosed the patient with unclassified vasculitis based on the clinicopathological characteristics. Steroids in combination with immunosuppressants were used, but the disease was refractory and relapsing. The disease activity was eventually controlled with rituximab, but the patient died of bronchopneumonia. On autopsy, lung and brain findings indicated healed vascular inflammation. Conclusions: This is the first case report of unclassified vasculitis, which is characterized as medium-to-small-sized arteritis and phlebitis, causing episcleritis, thrombophlebitis, deep vein thrombosis, pulmonary vasculitis, and intracranial vasculitis. The clinical conditions share some similarities with granulomatosis with polyangiitis and Behçet’s disease; however, they meet no classification criteria of any specific vasculitis. More cases need to be analyzed to confirm our findings.

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Section: Discussionmentioning

confidence: 97%

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

et al. 2019

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“…, lower images). A few cases of ring‐enhanced lesions in patients with acute NBD have been reported . Because the magnetic resonance imaging findings for acute NBD and brain abscesses look similar, it is difficult to distinguish these two conditions.…”

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confidence: 99%

“…A few cases of ring-enhanced lesions in patients with acute NBD have been reported. 1,2 Because the magnetic resonance imaging findings for acute NBD and brain abscesses look similar, it is difficult to distinguish these two conditions. It should be noted that multiple ring enhancement lesions in the magnetic resonance imaging might occur in patients with acute NBD, and that steroid therapy should be started as soon as the brain abscess is distinguished.…”

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confidence: 99%

Neuro‐Behcet's disease with multiple ring‐enhanced brain lesions

Tsugawa

Takeshita

Ouma

et al. 2014

Neurology & Clinical Neurosc

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Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome

Kidd

2023

Pract Neurol

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Neurological involvement in Behçet’s syndrome arises predominately through an inflammatory meningoencephalitis characterised by perivenular inflammation due to activation of Th-17 immunological pathways. The brainstem is involved in 50% of cases, the diencephalon and other areas of the brain in 30%, and the spinal cord in 10%. Movement disorders and epilepsy may occur. Psychiatric syndromes may arise with brain and brainstem involvement, and cognitive disorders relate to the brain disease, to circulating inflammatory factors, and to fatigue and despondency. Eighty per cent of cases begin with a relapsing disease course, of whom 70% have only one attack, and 30% have a progressive disease course either from onset or following an initially relapsing course. Venous thrombosis leading to intracranial hypertension and cerebral venous infarction is less common and caused by inflammation in affected veins and a circulating prothrombotic state. Arterial involvement is rare and relates to an arteritis affecting large-sized and medium-sized vessels within the brain leading to infarction, subarachnoid and parenchymal haemorrhage, aneurysm formation and arterial dissection. There is a newly recognised disorder of cerebral cortical hypoperfusion. Cranial neuropathy, peripheral neuropathy and myositis are rare. There has been significant progress in understanding the pathophysiology and treatment of the systemic disease, leading to improved outcomes, but there has been no randomised trial of treatment in the neurological disorder.

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Ring-enhanced Brainstem Lesion in a Patient with Neuro-Behcet's Disease

Cited by 4 publications

References 1 publication

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

Neuro‐Behcet's disease with multiple ring‐enhanced brain lesions

Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome

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