Cognitive impairment in Brazilian patients with Behçet's disease occurs independently of neurologic manifestation.

UNLABELLED: Neuro-Behçet's disease (NBD) presents cognitive and behavioral symptoms possibly explained by secondary dysfunction of frontal and temporal cortices due to subcortical damage, as NBD commonly involves the brainstem and basal ganglia. Nonetheless, there are reports of cognitive impairment in patients without neurological manifestations. OBJECTIVE: To evaluate cognitive function in Behçet's disease (BD) patients with and without neurological manifestations and to analyze clinical variables associated with cognitive deficits. METHODS: This is a cross-sectional study that compared healthy controls, BD patients without neurological manifestations and NBD patients. Each group comprised 24 participants. All participants underwent neuropsychological evaluation, Hamilton Anxiety Rating Scale (HAM-A), Beck Depression Inventory (BDI) application and brain MRI. Cumulative prednisone dose, years of education, and presence of white-matter lesions in brain MRI were recorded. RESULTS: 41.6% of BD and 41.6% of NBD patients showed impaired language and executive function, whereas visual memory was impaired only in NBD patients. Multiple logistic regression revealed that anxiety (OR 1.09 95% CI 1.03-1.16, p=0.003) and lower educational level (OR 0.62 95% CI 0.48-0.80, p<0.0001) were independently associated with cognitive impairment. CONCLUSION: Cognitive impairment occurs frequently in patients with BD independently of neurological manifestation. Low educational level and anxiety are risk factors for cognitive impairment in BD.

Epilepsy and Behçet's disease: cortical and hippocampal involvement in Brazilian patients.

OBJECTIVE: To describe clinical, radiological and electrophysiological findings in epileptic neuro-Behçet's (NBD) patients. METHODS: A retrospective review of 178 medical records of Behçet's disease patients was conducted in Brazil. Information on gender, ethnicity/skin color, age at symptom onset and age at onset of neurologic manifestations, type of seizures, clinical manifestation of the disease, use of antiepileptic drugs and immunosupressors was collected from medical records of all epileptic NBD patients. Brain MRI, cerebrospinal fluid (CSF) analysis and electroencephalograms (EEG) were assessed. RESULTS: Forty NBD cases were identified, of which seven patients (17%) presented epilepsy. In five patients seizures occurred during an acute exacerbation of the disease, and in one patient they occurred six months after meningoencephalitis. One patient presented seizures in the progressive form of the disease and five patients had complex partial seizures. The EEG showed temporal involvement in three patients and frontal in one. Hippocampal lesions were identified in three patients and cortical lesions in five. All patients had good response to antiepileptic drugs and are seizure-free, except for one who developed refractory seizures. CONCLUSIONS: Brazilian NBD patients showed a high prevalence of epilepsy, mainly complex partial seizures, occurring at any phase of the disease. Epileptic NBD patients may have cortical and hippocampal lesions that could explain the occurrence of epilepsy.

A rare association of tension pneumocephalus and a large frontoethmoidal osteoma: imaging features and surgical treatment.

Osteomas are the most common benign paranasal sinus tumors and are often found in the frontal and ethmoidal sinus. Intracranial complications such as pneumocephalus have occasionally been reported in association with osteomas. We describe a 33-year-old man with a large frontoethmoidal osteoma complicated by tension pneumocephalus and neurological disturbances, and we discuss the clinical and imaging resolutions after surgical management. We emphasize the imaging features and neuroendoscopic approach to avoid life-threatening conditions.

Lipomyelocele with osseous dysraphic hamartoma in a child: a case report.

Lipomyelocele is a well-known vertebral malformation that affects intraspinal and extraspinal tissues and compartments. However, other tissues can be associated with the lipomatous component, such as well-developed bones in unusual sites. This association is consistent with dysraphic hamartoma, one of the rarest malformations of the vertebral canal closure. We describe an 8-year-old girl without neurological deficit with a voluminous lipomyelocele associated with a well-developed flat bone articulated to the left iliac wing. We reviewed the role of magnetic resonance and computed tomography with tridimensional reconstruction in this rare form of spinal dysraphism and described the imaging features.