RESUMO
Uveitis is occasionally encountered in multiple sclerosis (MS) patients. The objective of this report is to investigate whether uveitis has a prognostic impact on the clinical course of MS. Several clinical and demographic features were compared between 41 MS patients with uveitis and 100 randomly selected MS patients without uveitis. While there were no significant differences by means of gender, age of MS onset, oligoclonal band positivity and disease duration, EDSS and progression index (PI) scores of MS patients with uveitis were significantly lower than those without uveitis (p = 0.004 and <0.001, respectively). Our results suggest that uveitis might be used as a good prognostic factor.
Assuntos
Esclerose Múltipla/diagnóstico , Uveíte/diagnóstico , Adolescente , Adulto , Idade de Início , Comorbidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/epidemiologia , Prognóstico , Uveíte/epidemiologia , Adulto JovemRESUMO
OBJECTIVES: Behçet's disease (BD) is a systemic auto-inflammatory disorder of unknown cause, which may affect the central nervous system in around 5% of the patients [neuro-BD (NBD)], usually causing large lesions encompassing brainstem, diencephalon and basal ganglia regions. Occasionally NBD patients present with white matter lesions necessitating differential diagnosis from multiple sclerosis (MS). In this study, the efficacy of Barkhof criteria was tested in diagnostic differentiation of NBD and MS. METHODS: Charts and MRIs of 84 NBD patients were retrospectively evaluated. Clinical and radiological features of NBD patients fulfilling (Barkhof+) and not fulfilling Barkhof criteria (Barkhof-) were compared. RESULTS: While the Barkhof- patients (n=73) mostly displayed typical large lesions covering brainstem, diencephalon and basal ganglia regions and neurological findings consistent with brainstem involvement, all Barkhof+ (n=11) patients demonstrated MS-like white matter lesions, fulfilled McDonald's criteria and showed reduced frequency of brainstem symptoms and increased frequency of hemiparesis, hemihypesthesia and spinal cord symptoms. Moreover, the Barkhof+ group had more female patients, increased number of attacks, higher rate of oligoclonal band positivity and less patients with cerebrospinal fluid pleocytosis. CONCLUSIONS: A subgroup of BD patients with neurological complaints displays MS-like lesions, fulfills the clinical and radiological criteria of MS and presents with clinical and laboratory features resembling those of MS rather than NBD. These results suggest that Barkhof+ patients are either an overlapping group between NBD and MS, or they represent MS patients with concomitant systemic findings of BD, rather than NBD. Barkhof criteria appear to be effective in discriminating these patients.
Assuntos
Síndrome de Behçet/diagnóstico , Tronco Encefálico/patologia , Leucoencefalopatias/diagnóstico , Imageamento por Ressonância Magnética , Esclerose Múltipla/diagnóstico , Substância Branca/patologia , Adolescente , Adulto , Idoso , Síndrome de Behçet/complicações , Síndrome de Behçet/patologia , Síndrome de Behçet/fisiopatologia , Tronco Encefálico/fisiopatologia , Diagnóstico Diferencial , Feminino , Humanos , Leucoencefalopatias/etiologia , Leucoencefalopatias/patologia , Leucoencefalopatias/fisiopatologia , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/complicações , Esclerose Múltipla/patologia , Esclerose Múltipla/fisiopatologia , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Substância Branca/fisiopatologia , Adulto JovemRESUMO
Behçet's disease (BD) is a multisystemic, recurrent and inflammatory disorder. Neurological involvement is rare and affects mainly the central nervous system (CNS) in the form of brainstem meningoencephalitis or dural sinus thrombosis. Peripheral neuropathy is usually not observed during the course of BD but some reports have shown electrophysiologic evidence of subclinical neuropathy, mononeuritis multiplex and cranial neuropathy in BD patients. The co-occurrence of Guillain-Barré syndrome (GBS), an acute inflammatory demyelinating neuropathy, with other autoimmune or systemic diseases is rare. We present a case of BD with clinical and electrophysiological diagnosis of GBS. The findings of the patient were discussed with reference to literature.
Assuntos
Síndrome de Behçet/complicações , Síndrome de Guillain-Barré/complicações , Adulto , Azatioprina/uso terapêutico , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamento farmacológico , Síndrome de Behçet/imunologia , Colchicina/uso terapêutico , Feminino , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/tratamento farmacológico , Síndrome de Guillain-Barré/imunologia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Exame Neurológico , Resultado do TratamentoRESUMO
OBJECTIVE: This study was conducted to characterize the prevalence and clinical features of kleptomania, an impulse control disorder, in patients with Behçet's disease involving the central nervous system. SUBJECTS AND METHODS: Medical records of 350 patients with neuro-Behçet's disease were evaluated, and clinical and neuropsychological features of patients with kleptomania were noted. RESULTS: Of the 350 neuro-Behçet's disease patients 6 (1.7%) had presented with symptoms that fulfilled the criteria of kleptomania according to the revised 4th version of the Diagnostic and Statistical Manual of Mental Disorders. The 6 patients (5 men, 1 woman) had parenchymal lesions and had developed kleptomania during remission. Magnetic resonance imaging done on the 6 patients before the onset of kleptomania mostly revealed brainstem lesions. Psychiatric assessment did not show any comorbid psychiatric disorders and neuropsychological evaluation showed executive dysfunction in all patients. CONCLUSION: The 6 patients with kleptomania had developed a frontal lobe syndrome.
Assuntos
Síndrome de Behçet/psicologia , Transtornos Disruptivos, de Controle do Impulso e da Conduta/psicologia , Adulto , Síndrome de Behçet/patologia , Síndrome de Behçet/terapia , Tronco Encefálico/patologia , Transtornos Disruptivos, de Controle do Impulso e da Conduta/epidemiologia , Transtornos Disruptivos, de Controle do Impulso e da Conduta/patologia , Feminino , Lobo Frontal , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Prevalência , Estudos RetrospectivosRESUMO
OBJECTIVES: Behçet's disease is a multisystemic, relapsing, inflammatory disorder of unknown origin. Among Turkish cohorts, 5-15% of patients show involvement of the central nervous system (CNS) at some time during their disease. There are mainly two types of clinical presentation: parenchymal CNS inflammation manifesting mainly as meningoencephalitis of the brainstem, or dural sinus thrombosis. Several drugs like high-dose steroids or immunosuppressive agents, mainly azathioprine, are used in the treatment. For patients who do not respond sufficiently to these agents or are not able tolerate them, other options are needed. PATIENTS: We are presenting 4 cases with parenchymal neuro-Behçet's disease, where commonly used immunosuppressive drugs could not be continued due to intolerance or inefficacy. However, the patients benefited well from mycophenolate mofetil. The benefit was sustained during 3-7 years of follow-up (median 6.5 years). CONCLUSIONS: Mycophenolate mofetil seems to be an alternative drug in parenchymal neuro-Behçet's disease; however, large controlled studies should be performed for verification of our results.
Assuntos
Síndrome de Behçet/tratamento farmacológico , Imunossupressores/uso terapêutico , Meningoencefalite/tratamento farmacológico , Ácido Micofenólico/análogos & derivados , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/imunologia , Feminino , Seguimentos , Humanos , Imageamento por Ressonância Magnética , Masculino , Meningoencefalite/etiologia , Meningoencefalite/imunologia , Mesencéfalo/patologia , Ácido Micofenólico/uso terapêutico , Ponte/patologia , Trombose dos Seios Intracranianos/tratamento farmacológico , Trombose dos Seios Intracranianos/etiologia , Trombose dos Seios Intracranianos/imunologia , Resultado do Tratamento , Uveíte/tratamento farmacológico , Uveíte/etiologia , Uveíte/imunologiaRESUMO
Neurological involvement is observed in 5%-25% of patients with lymphoma being either the first presentation of the disease or emerging during its course. However, Guillain-Barré syndrome is rarely reported. In this article, we present a case with intestinal lymphoma developing Guillain-Barré syndrome during the course of the disease. A 66-year-old male patient with primary extranodal intestinal lymphoma developed quadriparesis, sensory deficits and autonomic dysfunction while receiving chemotherapy. The findings of clinical, electrophysiological and laboratory examinations were consistent with Guillain-Barré syndrome. Guillain-Barré syndrome can potentially be fatal and mimic chemotherapy-induced neurotoxicity, especially in patients with lymphoma, and therefore, must be considered in the differential diagnosis.