Intracranial Hemorrhage in Neuro-Behcet's Syndrome.

Kikuchi, Seiji; Niino, Masaaki; Shinpo, Kazuyoshi; Terae, Satoshi; Tashiro, Kunio

doi:10.2169/internalmedicine.41.692

Cited by 10 publications

(3 citation statements)

References 14 publications

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“…Observations in cases with bilateral internal carotid artery occlusion, vertebral artery thrombosis, vertebral artery dissection, intracranial aneurysms and intracranial arteritis with their corresponding neurological consequences [4,10,29,44,46,50,69,75] suggested that arterial involvement may be a subgroup of NBS [92]. Intracranial hemorrhages may occur but are extremely rare, with most occurring within ischemic lesions [42,46].…”

Section: ■ Arterial-nbsmentioning

confidence: 99%

The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis

2009

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Behçet's Syndrome (BS) is a multi-system, vascular-inflammatory disease of unknown origin, involving the nervous system in a subgroup of patients. The growing clinical and imaging evidence suggests that primary neurological involvement in BS may be subclassified into two major forms: the first one, which is seen in the majority of patients, may be characterized as a vascular-inflammatory central nervous system (CNS) disease, with focal or multifocal parenchymal involvement mostly presenting with a subacute brainstem syndrome and hemiparesis; the other, which has few symptoms and a better neurological prognosis, may be caused by isolated cerebral venous sinus thrombosis and intracranial hypertension. These two types rarely occur in the same individual, and their pathogenesis is likely to be different. Isolated behavioral syndromes and peripheral nervous system involvement are rare, whereas a nonstructural vascular type headache is relatively common and independent from neurological involvement. Neurologic complications secondary to systemic involvement of BS such as cerebral emboli from cardiac complications of BS and increased intracranial pressure due to superior vena cava syndrome, as well as neurologic complications related to BS treatments such as CNS neurotoxicity with cyclosporine and peripheral neuropathy with the use of thalidomide or colchisin are considered as secondary neurological complications of this syndrome. As the neurological involvement in this syndrome is so heterogeneous, it is difficult to predict its course and prognosis, and response to treatment. Currently, treatment options are limited to attack and symptomatic therapies with no evidence for the efficacy of any long term preventive treatment.

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Section: ■ Arterial-nbsmentioning

confidence: 99%

The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis

2009

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“…The angiographic features suggest an intimal tear during the development of an aneurysm. [133][134][135] There are also reports of parenchymal haemorrhage [136][137][138][139][140] ; in one series, there was one haemorrhage in the brainstem and two in the caudate 137 ; another involved the cerebellum. 138 An inflammatory lesion may be complicated by haemorrhage within; in one a lesion evolved to become haemorrhagic and the second had a series of haemorrhagic lesions associated with inflammation within the cortices on both sides.…”

Section: Aneurysmmentioning

confidence: 99%

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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“…Hemorrhages in venular vasculitis are reported in the pulmonary, mucocutaneous involvement of BD [ 3 , 9 ], and other types of systemic small vessel vasculitis [ 6 ]. Regarding NBD, while a large observational study of 200 patients did not report any hemorrhagic manifestations [ 1 ], one report described two cases of hematomas detected in peripheral lesion at subacute phases [ 8 ]. However, the clinical significance of a delayed hematoma development in NBD has not been demonstrated.…”

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confidence: 99%

Delayed symptomatic intracerebral hemorrhages in neuro-Behcet’s disease

Lee¹,

Choi

Kim

et al. 2016

J Neurol

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Intracranial Hemorrhage in Neuro-Behcet's Syndrome.

Abstract: Objective Most cerebrovascular disturbances in Behcet's syndrome are occlusive in nature, while hemorrhage is rare.

Cited by 10 publications

References 14 publications

The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis

The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis

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

Delayed symptomatic intracerebral hemorrhages in neuro-Behcet’s disease

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