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ABSTRACT Introduction: Transcranial Doppler ultrasonography (TCD) is a technique that allows measurement of blood flow from the basal intracerebral vessels. It is relatively inexpensive, non-invasive, can be performed at the bedside, and allows monitoring in acute emergency settings and for prolonged periods with a high temporal resolution, making it ideal for studying the haemodynamics within the intracranial arteries in neuro-Behcet's disease (NBD) and neuro-psychiatric lupus (NPSLE). Our aim was to assess the cerebral haemodynamic patterns in patients with NBD and NPSLE using TCD, while brain lesions were examined using magnetic resonance imaging (MRI). Material and methods: Case-control prospective study of 30 neuro-Behcet's disease patients, 25 neuro-psychiatric lupus patients and 26 healthy age-matched volunteers. All patients and healthy controls were examined by TCD. Only the groups of patients underwent cranial magnetic resonance imaging (MRI). Results: Transcranial Doppler (TCD) values for middle cerebral artery (MCA), anterior cerebral artery (ACA), posterior cerebral artery (PCA), vertebral artery (VA) and basilar artery (BA) in NBD, NPSLE and control groups were measured. The results showed that there was a significant decrease in mean blood flow velocities in all the arteries examined in NBD and NPSLE patients. There was also a significant increase in the pulsatile index of PCA, VA and BA between NBD and NPSLE patients. The same results were obtained when comparing NBD versus controls. However, there was no significant difference between the NPSLE patients and the control group. The MRI lesions described were parenchymal lesions in 14 patients (46.7%), and vascular lesions in 4 patients (13.3%). Vascular lesions co-existed with parenchymal lesions (mixed lesion). Parenchymal lesions were in white matter (40%), thalamus (26.7%), brain stem (26.7%) and cerebellum (20%). While, in NPSLE, 23 patients were normal (92%) and only two patients had a vascular lesion (8%). Conclusion: There was a significant decrease in mean blood flow and a significant increase in the pulsatile index among both NBD and NPSLE patients, according to the TCD values.
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Humanos , Masculino , Femenino , Adulto , Infecciones , Enfermedades Estomatognáticas , Infecciones del Sistema Nervioso Central , Síndrome de Behçet , Vasculitis por Lupus del Sistema Nervioso Central , Enfermedades de la BocaRESUMEN
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.
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Humanos , Ganglios Basales , Encéfalo , Tronco Encefálico , Clasificación , Consenso , Diagnóstico , Diagnóstico Diferencial , Disartria , Fiebre , Cefalea , Corea (Geográfico) , Imagen por Resonancia Magnética , Registros Médicos , Neurología , Estudios Retrospectivos , Médula Espinal , TálamoRESUMEN
La enfermedad de Behçet puede afectar a varios órganos y tiene dentro de sus características la presencia de úlceras recurrentes genitales, orales y la uveítis. El neurobehçet es una de las manifestaciones clínicas más serias e infrecuente de la enfermedad de Behçet y debe ser considerado en el diagnóstico diferencial con enfermedades inflamatorias, infecciosas o desmielinizante del sistema nervioso central. Este reporte de caso describe a una paciente con manifestaciones neurológicas aguda severas, lo cual es inusual en esta enfermedad(AU)
Behçet's disease can affect several organs and the presence of recurrent genital, oral and uveitis ulcers characterize it. Neuro-Behçet's is one of the most serious and uncommon clinical manifestations of Behçet's disease, so it should be taken into account in the differential diagnosis with inflammatory, infectious or demyelinating diseases of the central nervous system. This case report describes a female patient with acute severe neurological manifestations, unusual in this disease(AU)
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Humanos , Femenino , Adulto , Síndrome de Behçet/complicaciones , Enfermedades del Sistema Nervioso/diagnóstico , Úlceras Bucales/diagnóstico , Enfermedades del Sistema Nervioso/complicacionesRESUMEN
Objective To explore the use of biological agents in Neuro-Beh(c)et's disease (NBD).Methods We retrospectively reviewed the clinical data of five NBD patients treated with biological agents who were in-patients at Peking Union Medical College Hospital between June 2009 and June 2016.The continuous variables were analyzed by t test.Results All five cases (4 male and 1 female) were severe and/or refractory patients with parenchymal involvement (pNBD).Their age at neurological onset was (31±12) years old.Four cases presented with multiple lesions.The brainstem,spinal cord,cerebral hemisphere and cerebellum involvement were presented in 4,3,3 and 2 patients,respectively.The Rankin score at the onset of NBD was (4.0±0.7).The biological agents were administrated when corticosteroids and immunosuppressant were ineffective.Three cases received tumor necrosis factor (TNF)-α inhibitors therapy,among whom one patient had gastrointestinal ulceration.One patient with refractory retinal vasculitis received interferon-α therapy.One patient treated with tocilizumab [interleukin (IL)-6R inhibitor] had high level IL-6 in the cerebrospinal fluid.All patients achievcd clinical improvements and the Rankin score significantly decreased to (2.2±0.8) when compared with the baseline (t=4.81,P<0.01) after the treatment with biological agents.The corticosteroid dose was tapered in all cases and the numbers of immunosuppressants were reduced in most cases,indicating a potential steroid and immunosup-pressant-sparing effect.No serious adverse events were observed during the follow-up.Conclusion Neurological involvement is a severe complication of Behqet's disease.We can take appropriate biological agents such as TNF-α inhibitors or interferon-α into consideration when patients have severe/refractory pNBD.
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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.
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Humanos , Persona de Mediana Edad , Enfermedades del Nervio Abducens , Síndrome de Behçet , Encéfalo , Tronco Encefálico , Estudios de Seguimiento , Inmunoterapia , Imagen por Resonancia Magnética , Mielitis , Paresia , Neumonía , Cuadriplejía , Reflejo , Insuficiencia Respiratoria , Médula EspinalRESUMEN
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.
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Adulto , Humanos , Hipocampo , Imagen por Resonancia Magnética , Pruebas Neuropsicológicas , Convulsiones , VasculitisRESUMEN
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.
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Adulto , Humanos , Encéfalo , Trastornos de Cefalalgia , Imagen por Resonancia Magnética , Meningitis , Úlceras Bucales , Piel , Uveítis AnteriorRESUMEN
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.
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Adolescente , Niño , Femenino , Humanos , Anticuerpos Monoclonales , Recurrencia , Úlcera Cutánea , Estomatitis Aftosa , Vasculitis Sistémica , Factor de Necrosis Tumoral alfa , InfliximabRESUMEN
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.
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Humanos , Persona de Mediana Edad , Apatía , Ganglios Basales , Encéfalo , Tronco Encefálico , Núcleo Caudado , Eritema Nudoso , Imagen por Resonancia Magnética , Mesencéfalo , Enfermedades del Nervio Oculomotor , Úlceras Bucales , Tálamo , UveítisRESUMEN
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.
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Humanos , Persona de Mediana Edad , Apatía , Ganglios Basales , Encéfalo , Tronco Encefálico , Núcleo Caudado , Eritema Nudoso , Imagen por Resonancia Magnética , Mesencéfalo , Enfermedades del Nervio Oculomotor , Úlceras Bucales , Tálamo , UveítisRESUMEN
Behcet's disease (BD) is an inflammatory systemic disorder with oral and genital ulcers, as well as ophthalmologic and cutaneous symptoms. Neurological manifestations in BD represent between 2.2% to 50% of the cases. The 25-year-old male patient, diagnosed with BD three years earlier, was admitted to our clinic with complaints of recurrent headaches. Tumor-like-parenchimal involvement was detected on a cranial magnetic resonance imaging. The lesion was removed surgically and then he suffered from right hemiparesis and epilepsy. Pathological examination of the lesion noted a demyelinating non-tumoural etiology. A neuro-Behget's case with parenchymal involvement has been examined in light of the literature, in terms of a tumor and a demyelinating disease differential diagnosis.
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Humanos , Masculino , Enfermedades Desmielinizantes , Diagnóstico Diferencial , Errores Diagnósticos , Epilepsia , Cefalea , Luz , Imagen por Resonancia Magnética , Manifestaciones Neurológicas , Paresia , ÚlceraRESUMEN
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.
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Niño , Humanos , Síndrome de Behçet , Encéfalo , Conmoción Encefálica , Traumatismos Craneocerebrales , Diplopía , Cabeza , Imagen por Resonancia Magnética , Paresia , PrednisolonaRESUMEN
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.
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Humanos , Persona de Mediana Edad , Encéfalo , Edema Encefálico , Tronco Encefálico , Estado de Conciencia , Diagnóstico Diferencial , Glioma , Cefalea , Unidades de Cuidados Intensivos , Metilprednisolona , Esclerosis Múltiple , Manifestaciones Neurológicas , Neurocirugia , Úlcera Cutánea , Accidente Cerebrovascular , UveítisRESUMEN
La enfermedad de Behçet (EB) es una patología inflamatoria vascular, multisistémica y recurrente. El fenómeno subyacente es una perivasculitis que afecta el territorio arterial y venoso. La mayor prevalencia de la enfermedad se encuentra en el Oriente y cuenca del Mediterráneo, en la llamada Ruta de la Seda. La prevalencia en Chile es desconocida. Las manifestaciones clínicas más frecuentes son úlceras orales y genitales recurrentes, uveítis, artritis, compromiso de sistema nervioso y vascular. El compromiso de SNC (Neuro-Behçet) varía entre 5 por ciento y 13 por ciento, y se divide en dos tipos: Intra-axial o parenquimatoso, más frecuente, más grave, con lesiones inflamatorias en tronco del encéfalo, en unión meso-diencefálica y región pontobulbar. Puede extenderse hacia diencéfalo, bulbo y médula espinal. El compromiso hemisférico es menos frecuente. Extra-axial, que se presenta como trombosis de senos venosos, y aneurismas, estenosis y disección arterial. El tratamiento del Neuro-Behçet intra-axial se basa en esteroides en dosis altas asociados a inmunosupresores, entre ellos, y dependiendo de la severidad, Metotrexato, Azatioprina, Ciclofosfamida, Micofenolato, y Anti-TNFα en casos refractarios o severos.
The highest prevalence of the disease is found in the East and the Mediterranean basin, in the so called Silk Road. The prevalence in Chile is unknown. The most frequent clinical manifestations are recurrent oral and genital ulcers, uveitis, arthritis, and CNS involvement (Neuro-Behçet). Neurological disease varies between 5 percent and 13 percent, and it can be divided into two types: Intra-axial or parenchymatous: more frequent, more severe, with inflammatory lesions involving the brainstem, midbrain, diencephalon and pontobulbar regions. It may extend into the spinal cord. Involvement of the brain hemispheres is less common. Extra-axial: also called Neurovasculo Behçet, less common and with better prognosis. It can manifests as venous sinus thrombosis, and aneurysm, stenosis, or arterial dissection of intracerebral or extracranial arteries. Treatment of Intra-axial Neuro-Behçet is based on high-dose steroids associated with immunosuppressive agents like: Methotrexate, Azathioprine, Cyclophosphamide, Mycophenolate, and anti α-TNF in refractory or severe disease.
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Humanos , Masculino , Adulto , Enfermedades del Sistema Nervioso/diagnóstico , Síndrome de Behçet/diagnóstico , Diagnóstico Diferencial , Encéfalo/patología , Enfermedades del Sistema Nervioso/tratamiento farmacológico , Esteroides/uso terapéutico , Inmunosupresores/uso terapéutico , Síndrome de Behçet/clasificación , Síndrome de Behçet/tratamiento farmacológico , Resultado del Tratamiento , Tronco Encefálico/lesionesRESUMEN
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.
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Adulto , Humanos , Masculino , Síndrome de Behçet/diagnóstico , Encéfalo/diagnóstico por imagen , Inmunoglobulina G/análisis , Leucocitosis/líquido cefalorraquídeo , Imagen por Resonancia Magnética , Metilprednisolona/uso terapéutico , Fármacos Neuroprotectores/uso terapéutico , Recurrencia , Extracción DentalRESUMEN
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.
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Humanos , Encéfalo , Sistema Nervioso Central , Diplopía , Gadolinio , Cefalea , Hipertensión Intracraneal , Espectroscopía de Resonancia Magnética , Trombosis del Seno Sagital , Seno Sagital Superior , TrombosisRESUMEN
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.
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Humanos , Persona de Mediana Edad , Amnesia Anterógrada , Ganglios Basales , Encéfalo , Eritema Nudoso , Imagen por Resonancia Magnética , Memoria , Examen Neurológico , Estomatitis Aftosa , Lóbulo Temporal , Tálamo , ÚlceraRESUMEN
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.
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Humanos , Arteria Basilar , Infartos del Tronco Encefálico , Constricción Patológica , Estudios de Seguimiento , Insuficiencia VertebrobasilarRESUMEN
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.
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Humanos , Adulto Joven , Encéfalo , Hipertensión Intracraneal , Presión Intracraneal , Imagen por Resonancia Magnética , Manifestaciones Neurológicas , Seudotumor Cerebral , Trombosis de los Senos IntracranealesRESUMEN
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.