Clinical and Radiographic Characteristics of Neuro-Behçet's Disease in South Korea

BACKGROUND AND PURPOSE: Neurological involvement in Behçet's disease [neuro-Behçet's disease (NBD)] is uncommon, but it is worth investigating since it can cause substantial disability. However, difficulties exist in understanding the clinical features of NBD due to regional variations and the lack of studies utilizing well-established diagnostic criteria. We therefore analyzed the clinical features of patients with NBD based on the recent international consensus recommendation. METHODS: We retrospectively searched electronic databases for patients with Behçet's disease (BD) between 2000 and 2017, and reviewed their medical records. Based on the recent international consensus recommendation, patients with definite or probable NBD were included. RESULTS: Of 9,817 patients with the diagnosis code for BD, 1,682 (17.1%) visited the neurology clinic and 110 (1.1%) were classified as NBD. Ninety-eight patients exhibited parenchymal NBD and 12 exhibited nonparenchymal NBD. Their age at the onset of NBD was 37.6±10.6 years and the male-to-female ratio was 1.24:1. Brainstem syndrome (43.9%) was the most common condition in the 98 patients with parenchymal NBD, followed by multifocal (32.7%) and spinal cord (12.2%) syndromes. 72.4% exhibited acute NBD and 27.6% exhibited a progressive disease course. Frequent manifestations included pyramidal signs (52.0%), headache (45.9%), dysarthria (42.9%), and fever (31.6%). A frequent pattern in brain MRI was an upper brainstem lesion extending to the thalamus and basal ganglia. CONCLUSIONS: Approximately 1% of the patients with suspected BD exhibited NBD. Neurologists must understand the clinical characteristics of NBD in order to perform the differential diagnosis and management of these patients.

A Fatal Case of Full-Blown Neuro-Behcet Disease / 대한임상신경생리학회지

We reported a 48-year-old man with Behcet disease, who presented with right hemiparesis. His first brain MRI showed multiple enhanced lesions. During the recovery, he had an episode of left 6th nerve palsy without new lesions in a follow-up MRI. Third episode was cervical myelitis, resulting in respiratory difficulty and quadriplegia without any reflexes. The myelitis was not responsive to immunotherapy. He died of respiratory failure complicated with pneumonia. This is a rare case of full-blown neuro-Behcet disease.

Neuro-Behcet's Disease Presenting as Hypertrophic Pachymeningitis

A 25-year-old man presented with blurred vision and chronic headache. His brain MRI revealed bilateral frontal pachymeningeal enhancement with leptomeningeal enhancement. The patient had experienced recurrent oral ulcer and had anterior uveitis and papulopustules skin lesion. We diagnosed him with hypertrophic pachymeningitis (HP) associated with neuro-Behcet's disease (NBD). There have been few reports describing HP in patients with NBD. We report a case of NBD presenting as HP.

Fregoli Syndrome Due to Right Hippocampal Vasculitis in Neuro-Behcet's Disease

We report a 40-year-old man with known neuro-Behcet's disease who showed Fregoli syndrome after a seizure attack. A patient with Fregoli syndrome strongly believes that the psychological identity of a familiar person remains unchanged while at the same time there is a marked change in physical identity. The anatomical basis of Fregoli syndrome is still unclear; our patient showed right hippocampal vasculitis in MRI and frontal dysfunction in neuropsychological testing.

Successful Treatment of Child Neuro-Behcet's Disease with Infliximab

Behcet's disease is a systemic vasculitis, characterized by recurrent oral aphthous ulcers, recurrent genital ulcers, skin lesion, and ocular involvement. Monoclonal antibody to the tumor necrosis factor-alpha (TNF-alpha) is considered as a possible therapeutic approach to achieve clinical improvement, preventing relapse in Behcet's disease refractory to conventional anti-inflammatory drugs or immunosuppressive durgs. We report the use of infliximab, which is one of the TNF-alpha monoclonal antibodies, in a 17-year-old girl with Behcet's disease exhibiting severe mucocutaneous, ocular and neurological involvement refractory to standard treatment.

Neuro-Behcet disease presented diplopia with hemiparesis following minor head trauma / 소아과

Behcet disease (BD) is rare in childhood. We report a 9-year-old boy with neuro-Behcet disease who presented diplopia and weakness on the left side after a cerebral concussion. Brain magnetic resonance imaging (MRI) revealed hyperintensity of the right mesodiencephalic junction on T2-weighted and fluid attenuated inversion recovery images. Prednisolone administration resulted in complete remission and normalization of abnormal MRI finding. Brain MRI is a useful diagnostic tool when the neurological sign is the first symptom of subclinical BD.

Neuro-Behcet's Disease Presented with Progressive Disinhibition

We report a man who presented with progressive disinhibition and through clinicoradiologic correlation using magnetic resonance imaging (MRI), aim to investigate the pathomechanism of disinhibition in neuro-Behcet's disease (NBD). A 46-year-old man presented with progressive disinhibition and apathy for 4 months. One month after his visit, additionally, he developed left partial third nerve palsy. His brain MRI showed lesions in the ventral caudate nucleus as well as left midbrain and thalamus. Taking his recurrent oral ulcers, uveitis, and erythema nodosum into consideration, he was diagnosed with NBD. We found that progressive disinhibition could be one of presenting symptoms in BD and might be associated with the caudate nucleus. This finding suggests that involvement of the basal ganglia in BD prior to the involvement of the brainstem could result in unique clinical features such as behavioral changes without extrapyramidal signs.

Neuro-Behcet's Disease Presented with Progressive Disinhibition

We report a man who presented with progressive disinhibition and through clinicoradiologic correlation using magnetic resonance imaging (MRI), aim to investigate the pathomechanism of disinhibition in neuro-Behcet's disease (NBD). A 46-year-old man presented with progressive disinhibition and apathy for 4 months. One month after his visit, additionally, he developed left partial third nerve palsy. His brain MRI showed lesions in the ventral caudate nucleus as well as left midbrain and thalamus. Taking his recurrent oral ulcers, uveitis, and erythema nodosum into consideration, he was diagnosed with NBD. We found that progressive disinhibition could be one of presenting symptoms in BD and might be associated with the caudate nucleus. This finding suggests that involvement of the basal ganglia in BD prior to the involvement of the brainstem could result in unique clinical features such as behavioral changes without extrapyramidal signs.

Rapid Atypical Progression of Neuro-Behcet's Disease Involving Whole Brainstem and Bilateral Thalami

We present a case of Neuro-Behcet's disease with an unpredictable clinical course. A 47-year-old man was admitted to the neurosurgery department of our hospital with a mild headache. Three days after admission, his consciousness suddenly decreased and respiratory distress progressed rapidly. A brain MRI revealed that the previously observed abnormal signal had extended markedly to both the thalamic areas and the entire brain stem, and the surrounding brain parenchyma were compressed by cerebral edema. Based on the patient's symptoms of recurrent oral and genital ulcers, skin lesions, and uveitis, a rheumatologist made a diagnosis of Behcet's disease with CNS involvement. The patient was treated with high-dose methylprednisolone with respiratory assistance in the intensive care unit for 9 days and his neurologic symptoms improved remarkably. Neuro-Behcet's disease must be considered in the differential diagnosis in rapidly deteriorated young neurological patients along with a stroke, low-grade glioma, multiple sclerosis, and occlusive venous disease.

A Case of Recurrent Neuro-Behcet's Disease after Tooth Extraction

We report a 39-yr-old man with neuro-Behcet's disease (NBD) in remission who developed left-sided ataxia with a sensory deficit about 10 days after tooth extraction. Several years ago, he experienced a similar episode of relapse after tooth extraction. Brain magnetic resonance imaging showed a newly developed right thalamic lesion. In cerebrospinal fluid, lymphocyte-dominant pleocytosis and mild elevation of IgG were found. Immunologic factors may be important in the pathogenesis of NBD because of the time delay between tooth extraction and relapse. Careful observation and prevention are needed before dental procedures in patients with NBD.

Thrombosis of the Superior Sagittal Sinus in Behcet's Disease With Vascular and Enteric Involvements

We report superior sagittal sinus thrombosis as a central nervous system presentation of Behcet's disease in a patient with peripheral vascular and enteric involvement. The main symptoms were headache and horizontal diplopia due to intracranial hypertension. T1-weighted MRI with gadolinium enhancement revealed a similar pattern of the "empty delta sign" that is usually seen on brain CT. A magnetic resonance venogram revealed filling defects resulting from the thrombosis. These features are helpful for the noninvasive diagnosis of sagittal sinus thrombosis.

A Case of Neuro-Behcet's Disease Presenting as Anterograde Amnesia

Anterograde amnesia in Behcet's disease is a rare occurrence. A 50-year-old man presented with anterograde amnesia. He had been suffering multiple oral aphthous ulcers and genital ulcers with erythema nodosum. A neurological examination revealed prominent anterograde memory disturbance. Brain MRI revealed high signal intensity lesions involving the anterior thalamus, posterior part of the basal ganglia and the mesial temporal lobe. We report a rare case of Behcet's disease manifesting severe anterograde amnesia resulting from thalamic and mesial temporal lesions.

A Case of ReVersible Basilar Artery Stenosis in Neuro-Behcet's Disease

The reversibility of basilar artery stenosis in neuro-Behcet's disease (NB) has been rarely reported. We report a patient with NB who developed brainstem infarction related to severe stenosis in basilar artery. Initial MRA showed severe stenosis in the basilar artery, which was improved on follow-up MRA after immunosuppressive treatment. This case shows that arterial stenosis in NB can be reversed with proper management.

Neuro-Behcet's Disease Presented with Cerebral Venous Sinus Thrombosis: A Case Report / 영남의대학술지

Behcet's disease is a chronic, relapsing multisystem disorder, that may develop into variable neurological manifestations. They include vascular and parenchymal involvement. Vascular involvement is dominated by cerebral venous sinus thrombosis marked by benign intracranial hypertension. Cerebral venous sinus thrombosis can present with all the classical criteria for idiopathic intracranial hypertension, including normal brain CT findings with normal CSF content. But brain MRI is a useful diagnostic method in this situation to confirm the presence of cerebral venous sinus thrombosis. We experienced a case of raised intracranial pressure in a 21-year-old man, caused by cerebral venous sinus thrombosis. We disclosed his symptoms and signs thus fulfilling the diagnostic criteria for Behcet's disease.

Neuro-Behcet's Disease Presenting with Foville Syndrome : A Case Report

A Foville syndrome is rare. A 46-year-old man with a 3-year history of Behcet's disease presented with a right facial palsy and a limitation in the rightward movements of the eyes. On neurologic examination, there was no movement of eyes in the right hemifield during all kinds of eye movements, including saccade, smooth pursuit, vestibulo-ocular reflex, and optokinetic nystagmus. However, all the eye movements were normal in the left hemifiled except for slowed saccades toward the right. Additional features were right infranuclear facial paresis and extensor plantar response on left side. Brain MRI revealed high signal intensity lesions in the right pons both in the dorsal tegmentum and in the basis. Neurological signs resolved gradually with steroid treatment. Different clinical features between abducens nucleus syndrome and paramedian pontine reticular formation syndrome are briefly discussed. To the authors' knowledge, this is the first case report of a Foville syndrome caused by neuro-Behcet's disease.

A Case of Behcet's Disease Presenting with Transient Global Amnesia and Perimesencephalic Subarachnoid Hemorrhage

A 55-year-old man was admitted due to transient global amnesia. A similar episode recurred. He also presented with uveitis and oral ulcers. Brain MRI showed lesions in the brainstem and medial thalamus. One year later he was readmit-ted due to subarachnoid hemorrhage. Cerebral angiography revealed normal. The last two episodes were treated with a high dose of prednisolone and resolved completely. Vasculitis related to neuro-Behcet's disease is presumed to play a role in the development of transient global amnesia and subarachnoid hemorrhage. (J Korean Neurol Assoc 19(4):396~399, 2001)

Proton Magnetic Resonance Spectroscopy in Patients with Neuro-Behcet's Disease / 대한류마티스학회지

Magnetic resonance imaging (MRI)and computed tomography had been used to detect the neurologic lesions of the Behcet's disease.However,there are a number of cases which can not be detected by conventional imaging studies in neuro-Behcet's disease.To overcome this problem,magnetic resonance spectroscopy (MRS)has been introduced in several autoimmune diseases involving central nervous system such as neuropsychiatric lupus,but which was rarely performed in Behcet's disease.In the present study,we report two cases of neuro-Behcet's disease that showed normal MRI but abnormal proton MRS (1HMRS)findings.MRS in two cases revealed a reduction of the N-acetyl aspartate (NAA)/creatine (Cr)ratio in the substantia nigra and thalamus,respectively, which were the areas without abnormality on MRI,suggesting a functional defect of neurons in these areas.In a case with thalamic dysfunction,abnormal NAA/Cr ratio was normalized in parallel with clinical improvement after treatment with high-dose steroids and immuno-suppressive agents.These observa-tions suggest that MRS may be useful to early detect the functional abnormality in neuro-Behcet's disease and to monitor neurologic status after treatment with immuno-suppressive agents.

Neuro-Behcet's disease presenting with isolated unilateral lateral rectus muscle palsy

The authors present the clinical findings of a 30-year-old female and a 29-year-old male who both had isolated unilateral lateral rectus muscle palsy in neuro-Behcet's disease. The clinical feature related to isolated abduscens nerve palsy was identified by CT, systemic assessment and extraocular examination. These patients' constellation of findings appear to be unique: it does not follow any previously reported pattern of ocular manifestations of neuro-Behcet's disease.

A case of Neuro-Behcet's disease with CNS lesions confined to multiple cortical areas

We report a patient diagnosed with neuro-Behcet's disease showing multiple enhancing lesions on a brain MRI that were confined to the cerebral cortex without any involvement of other brain regions. The patient, a 30-year-old man, was admitted because of seizure attacks. He had the symptom triad of recurrent oral ulcer, genital ulcer, and uveitis. Neurologic symptoms included ataxia, dysarthria, and dementia which had progressed over several years. During the hospital course, he underwent a right hemicolectomy due to bowel perforation. A pathological examination showed multiple discrete ulcerations from the cecum to the mid-transverse colon, consistent with Behcet's colitis. A brain MRI revealed multiple small enhancing lesions distributed over wide areas of the cerebral cortex. To our knowledge, this pattern of lesion distribution has not yet been reported in neuro-Behcet's disease.