Cerebral vein thrombosis in Behçet’s disease

doi:10.1016/S0887-8994(01)00314-9

Pediatric Neurology

Volume 25, Issue 4, October 2001, Pages 332-335

https://doi.org/10.1016/S0887-8994(01)00314-9 Get rights and content

Abstract

Behçet’s disease is a chronic, relapsing multisystem disorder, and nervous system involvement is one of the serious manifestations. Neuro-Behçet is rarely reported in children and may present with a wide variety of symptoms because the entire neuraxis may be affected. A case of cerebral vein thrombosis secondary to Behçet’s disease is presented. The patient has recovered without any visual loss and had no complaints at 1-year follow-up.

Introduction

Behçet’s disease is a heterogeneous, multisystem inflammatory disorder first described by Turkish ophthalmologist, Dr Hulusi Behçet, in 1937. The classic triad consists of recurrent oral and genital ulcerations and uveitis. Other systemic features, including skin lesions, arthritis, and neurologic and pulmonary involvement, have been reported in association with Behçet’s disease [1]. The pathogenesis of the disease has not been understood, although some agents, such as streptococci or herpes virus, are thought to be responsible [1]. Various immunologic abnormalities induced by particular microbial agents or other environmental factors are possible in genetically susceptible subjects. The major pathology of Behçet’s disease is vasculitis, and all sizes of arteries and veins are affected. Superficial or deep vein thrombosis is seen in approximately 30% of Behçet’s disease patients [1]. There have been many reports of cerebral vein thrombosis in Behçet’s disease, most of them in adult patients [2], [3], [4], [5], [6], [7], [8].

This study reports a case of neuro-Behçet’s disease with central vein thrombosis that had not been diagnosed as Behçet’s disease before reporting to our hospital.

Section snippets

Case report

A 15-year-old female reported to the emergency room with a 3-day history of headache, nausea, and vomiting. There was no fever or history of recent infection. She had photophobia and phonophobia. Past medical history was remarkable for a 2-year history of recurrent oral and genital ulcers. No history of arthralgia or arthritis was present.

The patient’s family history revealed multiple family members, including mother, a 23-year-old sister, and maternal grandfather, with histories of recurrent

Discussion

Behçet’s disease is uncommon in childhood, and the clinical presentation of the disease in children is different from that of an adult. It has been reported that, although mucocutaneous findings are common in childhood presentation, ocular involvement is relatively less frequent than in adult patients [12], [13], [14]. Neuro-Behçet’s disease is usually observed after a long duration of the disease. Occurrence of neurologic involvement as a first presentation is quite exceptional in pediatric

Conclusion

The diagnosis of Behçet’s disease in children can be difficult because of the rarity of the condition and nonspecific manifestations. Although it is a multisystem disorder, it may involve only a single organ during childhood, which could delay the diagnosis. Behçet’s disease should be considered in the differential diagnosis of intracranial hypertension due to cerebral vein thrombosis, especially in silk route countries, even in the absence of the cardinal findings of Behçet’s disease.

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The mean duration of medical treatment was 2 months.1–11 The mean follow-up period was 6.6 months.4–22 None of the patients had a recurrence and the visual acuity was normal in all patients at the end of the follow-up period.
To investigate the etiological and clinical features of pseudotumour cerebri (PTC) in children, features of 12 children with PTC were documented. The etiology could be clarified in 6 patients. The other 6 patients were accepted as idiopathic intracranial hypertension (primary PTC). Acetazolamide was used as the first drug and was effective in only 4 patients who had no underlying cause. Repeated lumbar punctures (LP) were performed in 6 patients and 5 of them were unresponsive. Prednisone was used in 3 patients and was effective in only one patient. The CSF pressure continued to be high in five patients and could be normalized only by withdrawing of the precipitating drug in three patients and by ventricular-peritoneal shunting in two patients with cerebral venous thrombosis. Although, medical treatment and repeated LP are usually effective in children with PTC, clarifying and solving the underlying cause can be crucial
Suppurative intracranial thrombophlebitis
2010, Handbook of Clinical Neurology
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Rare cases of intracranial venous sinus thrombosis have been associated with Trichinella infestation (el Koussa et al., 1994). Aseptic venous sinus thrombosis, in particular involving the superior sagittal sinus, may occur in patients with Behçet's disease (Alper et al., 2001; Saltik et al., 2004; Bir et al., 2005; Yazici et al., 2007). Drainage of venous blood from the central nervous system occurs via a complex network of cerebral veins.
This chapter discusses the epidemiology, etiology, pathogenesis, clinical features, diagnosis, and management of suppurative intracranial thrombophlebitis. Septic intracranial venous sinus thrombosis arises as a consequence of infection in the paranasal sinuses and less frequently following infections of the middle ear or mastoid. The most common isolate from the cases of septic intracranial venous thrombosis associated with pericranial infections is Staphylococcus aureus. Suppurative intracranial thrombophlebitis is most frequently the result of pericranial infection. The local infection causes septic thrombophlebitis of veins draining the infected area, with subsequent intracranial extension of the infected thrombus along emissary veins. The cavernous sinuses are the structures most frequently involved in septic venous sinus thrombosis. The transverse sinuses are the second most frequent intracranial venous sinuses to be involved by septic processes. The advent of magnetic resonance imaging (MRI) and then of computed tomography (CT) angiography and venography revolutionized diagnosis of both venous sinus and cortical vein thrombosis, such that MRI and CT venography are currently the diagnostic procedures of choice in detecting venous sinus thrombosis.
Neurological manifestations of Behcet's disease: 22 cases among 170 patients
2009, Presse Medicale
Présenter les diverses manifestations neurologiques de la maladie de Behçet d’une série libanaise, leur pronostic et leur traitement.
Cette étude rétrospective a porté sur les dossiers des malades hospitalisés entre 1980 et 2005 et ceux de la consultation externe en médecine interne. Tous ces malades répondaient aux critères diagnostiques du Groupe d’étude international sur la maladie de Behçet.
L’atteinte neurologique était observée chez 13 % (22/170) de nos malades avec une légère prédominance masculine (sexe-ratio : 1.75). L’âge moyen lors du diagnostic de la maladie de Behçet était de 26 ± 6 ans et lors de l’apparition des signes neurologiques de 30 ± 8 ans avec un délai moyen de 9 ans. Vingt et un patients avaient une atteinte centrale, un seul avait une atteinte cochléovestibulaire. Les atteintes centrales comportaient une méningo-encéphalite et/ou une myélite transverse (57 % des cas), associées dans 2 cas à une atteinte du tronc cérébral, une atteinte du tronc cérébral sans méningo-encéphalite (5 cas), un aspect clinique évoquant une tumeur cérébrale (2 cas), des accidents ischémiques à répétition (1 cas) et une thrombose veineuse cérébrale (1 cas). Le principal aspect clinique était une atteinte motrice (syndrome pyramidal avec ou sans déficit focalisé) (16 cas) et le nerf moteur oculaire externe était le plus fréquemment atteint (4 cas). Le LCR était anormal dans 56 % des cas avec une pléiocytose à prédominance lymphocytaire. La tomodensitométrie cérébrale, réalisée dans 6 cas, était normale pour 2 d’entre eux. L’IRM cérébrale, réalisée dans 9 cas, était anormale dans 6, dont des lésions multiples du tronc cérébral en hypersignal T2 (4 cas), des signes en faveur d’une thrombose du sinus latéral gauche (1 cas) et un aspect pseudotumoral hémisphérique (1 cas). Les corticoïdes étaient habituellement efficaces mais, lorsqu’ils étaient utilisés seuls, une rechute tardive était observée dans 31 % des cas après arrêt du traitement. Un patient ayant eu une atteinte du tronc cérébral est décédé après une corticothérapie tardive.
À part l’hypertension intracrânienne par thrombose veineuse cérébrale, il est possible, dans les atteintes centrales du neuroBehçet, d’individualiser 3 aspects cliniques majeurs : la méningomyélo-encéphalite, l’atteinte du tronc cérébral et les pseudotumeurs cérébrales. Les anomalies observées au scanner et à l’IRM, par leur localisation notamment au niveau du tronc cérébral et leurs aspects multiples, doivent faire évoquer le diagnostic. La corticothérapie, débutée précocement, améliore le pronostic mais son association aux immunosuppresseurs est à envisager dans les atteintes parenchymateuses.
To present neurological involvement in Behçet's disease, its prognosis and treatment.
Our study was retrospective and was done at Hotel-Dieu Hospital of Beirut between 1980 and 2005. All these patients fulfilled the International Study Group criteria for diagnosis of Behçet's disease.
Neurological involvement was observed in 13% (22/170) of our patients and was more frequent in men (sex-ratio: 1,75). The mean age of onset for Behçet's disease and NeuroBehçet's syndrome was 26 ± 6 and 30 ± 8 years respectively. Central nervous system involvement was found in 21 patients and peripheral nervous system involvement in one. Meningoencephalitis and/or transverse myelitis were found in 57% (12/21) of cases (in association with brainstem syndrome in 2 of these cases), brainstem syndrome without meningoencephalitis in 5 cases, tumor-like syndrome in 2 cases, repetitive ischemic attacks in 1 case and cerebral venous thrombosis in one. Focal deficits were the major presenting signs (16 cases) and external oculomotor nerve paralysis was observed in 4 patients. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level. CT Scan, performed in 6 patients, was normal in 33% of cases. MRI, performed in 9 patients, was abnormal in 6 and showed abnormal signals distributed over the brainstem and the thalamus in 4, a tumor-like lesion and thrombosis of the left lateral sinus one each. Corticosteroids were usually efficacious but, when used alone, relapse was observed in 31% of patients. One patient who had brainstem syndrome died within 18 months because of a delayed corticosteroid treatment.
Within central neurological involvement in Behçet's disease, we can individualize 4 clinical aspects: meningoencephalitis (and/or myelitis), brainstem syndrome, tumor-like features and cerebral venous thrombosis. Abnormalities, observed on CT Scan and MRI, by their brainstem localization and their multiplicity, should evoke the diagnosis. Corticosteroids, when prescribed early, are useful and are associated with better prognosis; their association to immunosuppressant agents should be considered in the parenchymatous forms.
Cerebrovascular manifestations of Behçet's disease
2009, Journal of Clinical Neuroscience
Behçet’s disease (BD) is a systemic inflammatory disease commonly characterized by oral and genital ulcerations, with skin and eye involvement. Its cause is still unknown, but vasculitis is the major pathologic feature. BD is a rare condition, seen more commonly in Turkey, as well as in Middle Eastern, Mediterranean and Far Eastern countries. Neurological involvement is reported to manifest as brainstem or corticospinal tract syndromes, increased intracranial pressure mostly related to venous sinus thrombosis or aseptic meningitis, isolated behavioral symptoms or isolated headache. This report presents a 34-year-old BD patient with secondary cerebral infarction due to head trauma. The clinical and central nervous system findings of BD are discussed.
Cerebral Venous Thrombosis
2009, Stroke in Children and Young Adults: Expert Consult - Online and Print
Cerebral Venous Thrombosis
2009, Stroke in Children and Young Adults

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Cerebral vein thrombosis in Behçet’s disease

Abstract

Introduction

Section snippets

Case report

Discussion

Conclusion

J Pediatr

Mayo Clin Proc

J Pediatr

Cerebral venous thrombosis in Behçet’s diseaseClinical study and long-term follow-up of 25 cases

Neurology

Seven-year follow-up of neurologic involvement in Behçet syndrome

Arch Neurol

Raised intracranial pressure in a 16-year-old boy

S Afr Med J

Papilloedema in Behçet’s diseaseValue of MRI in diagnosis of dural sinus thrombosis

J Neurol Neurosurg Psychiatr

Case of month

Eur J Pediatr

Neurological presentation of Neuro-Behçet’s syndromeClinical categories

Eur Neurol

Criteria for diagnosis of Behçet’s disease

Lancet