RESUMEN
Primary pseudotumor cerebri syndrome (PPTS) is a rare disorder of elevated intracranial pressure (ICP) in the absence of an identifiable underlying etiology. Afflicted patients are usually obese women in their reproductive age presenting with symptoms of elevated ICP. Seldom, patients can present with an encephalocele. We reported a case of a 31-year-old female who initially presented to our center with complaints of headaches, foreign body sensation in the nasal cavity, and decreased ability to smell. Brain computed tomography (CT) scan showed a large intranasal encephalocele and defect along the frontal skull base, through which brain tissue was herniating. The patient was successfully treated surgically by implantation of a lumboperitoneal shunt to manage the high ICP caused by her PPTS. In combination, reconstruction of the frontal skull base defect for the encephalocele was performed. Currently, the patient is doing well despite some on-and-off headaches.
RESUMEN
Background Neurological diseases entail a broad spectrum of disorders. Among such ailments are epilepsy and neuromuscular disorders which impose a substantial burden on children and their families. Ensuring adequate access to outpatient services is crucial for these children regardless of the subclinical specialty, and clinicians can better comprehend the caregivers' perspectives by being aware of their backgrounds which can be aided using epidemiological studies. Methods In June 2023, a cross-sectional study was carried out in pediatric neurology clinics at a tertiary care center. The study included all families with a child or more (14 years and younger) diagnosed with neurological disorders. The study adopted a three-section survey delivered to participants recruited using a non-probability sampling technique to achieve a 95% confidence interval with a 5% margin of error. Results A total of 821 families participated in this study. The mean age of respondents was 40.46±8.72 years. Of the affected children, there were 600 (73.08%) children following up with the general neurology and epilepsy clinics, 164 (19.98%) were following up with the neuromuscular disorders clinics, and 57 (6.94%) were following up with the neurogenetic clinics. Familial status had no association with the type of clinic the patient was following up with p=0.0054. Single respondents had a significantly higher prevalence of children with epilepsy (p<0.0001). Parents with a high school level of education or lower had a significantly greater prevalence of epilepsy clinic follow-ups (p=0.0048). Conclusion The findings of this study contribute to the assessment of prevalent neurological disorders in children and shed light on the family dynamics surrounding these conditions. Through statistical analysis, the study establishes connections between certain demographic and clinical traits and specific neurological disorders among pediatric patients and their families. The study emphasizes the importance of socio-economic and socio-clinical support in promoting child health in such cases. Similar research would offer a more accurate portrayal of the challenges faced by families in these circumstances.
RESUMEN
Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition known to affect multiple organ systems. While its manifestations are diverse, pulmonary involvement, especially of the pleura, remains less common. We report the case of a 99-year-old Saudi male with a medical history of diabetes mellitus, chronic kidney disease, and hypertension. He presented with dyspnea and syncope, with radiological findings revealing pleural effusion and a mass in the right hemidiaphragm. Laboratory investigations highlighted elevated serum IgG4 levels, and histopathological evaluation confirmed the diagnosis of IgG4-RD. Notably, the patient's thoracic histopathology differed from typical IgG4-RD presentations, emphasizing the variability of the disease. This case underscores the significance of recognizing IgG4-RD as a potential cause of unexplained pleural effusion. It also highlights the need for a comprehensive diagnostic approach, integrating laboratory values, histopathological findings, and clinical context. Given the potential variability in thoracic IgG4-RD histopathology, clinicians should maintain a heightened awareness of this condition to avoid missed diagnoses.