Anti-C2 monoclonal antibody, clone 3D22

Objective: Rare disease Background: Spontaneous intracranial hypotension (SIH) is a rare cause of postural headache. In most patients, the site of cerebrospinal fluid (CSF) leak is at the cervical or thoracic spinal level. The imaging modalities to establish the diagnosis of SIH include computed tomography (CT) and magnetic resonance imaging (MRI) of the brain, CT, and MRI myelography, and radionuclide cisternography. Treatment usually consists of conservative measures, but patients unresponsive to these treatments can be treated by epidural blood patch (EBP) administration at the site of CSF leak. Case Report: A 25-year-old-man presented with headache aggravated upon sitting or standing and relieved by lying supine or consuming coffee. There was no history of recent trauma, lumbar puncture, or spinal anesthesia. His neurological examination was unremarkable. MRI of his head and entire spine showed features of intracranial hypotension with no obvious CSF leak. He was treated conservatively but his symptoms persisted. CT spinal myelography showed significant leakage of contrast medium at the retrospinal region between C1 and C2 spinous processes. The patient underwent cervical EBP administration under fluoroscopic guidance. His symptoms resolved completely and he remains asymptomatic more than 6 months later. Conclusions: SIH is an important cause of postural headache. In patients with non-resolving symptoms, further investigations are warranted to identify potential CSF leak. Patients found to have a CSF leak at the level of the cervical spine can be safely and effectively treated by cervical EBP administration.

Objective: To evaluate sociodemographic and clinical aspects of children with sickle cell disease (SCD) and their behavioral characteristics. Methods: Interview with parents of patients with SCD from four to ten years old, addressing socioeconomic aspects and other health conditions, and using the Strengths and Difficulties Questionnaire (SDQ). Clinical data were obtained from medical records. Exclusion criteria were the use of hydroxyurea, previous diagnosis of stroke, chronic encephalopathy and/or intellectual disability. Results: 45 patients (19 girls and 26 boys) were assessed. The median age was seven years. Diagnosis of SCD: 26 hemoglobinopathy SC; 19 hemoglobinopathy SS. Socioeconomic class: D: 24.4%; C2: 44.4%; C1: 28.9%; B2: 2.2%. Clinical history: acute chest syndrome: 40%; transfusions: 66.7%; hospitalizations: 82.2%. SDQ findings: 88.9% clinical impact (emotional subscale: 68.9%); total score: impact in 48.9%. It was not possible to establish a relation between the severity of the disease and the results of the SDQ. Regarding socioeconomic class: among individuals of classes B2 and C1, 21.4% had impact at the total score; in classes C2 and D, this percentage was 61.3%. Regarding the schooling of the head of the family, with Elementary School at least, 39.3% of the children had impacts; for fewer education, this percentage was 64.7%. Conclusions: Behavioral impacts are highly prevalent in children with SCD. Individuals in socioeconomic classes C2 and D suffered more behavioral impacts than individuals in classes B2 and C1.