Recurrent bacterial meningitis (≥2 episodes with complete recovery between episodes) warrants evaluation for skull-base defects with cerebrospinal fluid (CSF) leak and for immune deficiencies. In adults, Streptococcus pneumoniae often indicates an anatomic breach (e.g., post-traumatic encephalocele/CSF rhinorrhea), whereas Neisseria suggests complement deficiency. We report a case of a 22-year-old man with his second attack of pneumococcal meningitis within a one-year timeframe after sustaining a road traffic accident about four years ago. The patient sustained a left frontal bone fracture communicating with the left frontal sinus, along with a left frontal encephalocele. His current complaint was fever and cough of one week duration; they were associated with headache, rhinorrhea, vomiting, sore throat, fatigue, and generalized body pain. The patient denied having any past history of recurrent or severe infections, especially during childhood and adulthood. Upon assessment, his temperature was 37.6 °C, and his vitals were within normal limits. Physical examination was remarkable for positive neck stiffness. Otherwise, no skin rashes were noted; neurological assessment included a Glasgow Coma Scale of 15/15, no focal neurological deficits, and intact cranial nerves. His abdomen was soft, lax, and non-tender; his spleen was not palpable. Labs were significant for leukocytosis, elevated inflammatory markers, hypoglycorrhachia, and elevated CSF protein. Both blood and CSF cultures were positive for S. pneumoniae. His HIV test was negative. He was empirically treated with IV ceftriaxone and vancomycin, and later tailored to ceftriaxone after the cultures' susceptibility results were out. In adults with recurrent pneumococcal meningitis, prompt skull-base imaging for occult CSF leak/encephalocele, and parallel immune evaluation are essential. Definitive endoscopic repair plus pneumococcal vaccination can prevent further episodes. Structured pathways that trigger beta-2 transferrin testing and high-resolution CT/MRI after a second episode may reduce diagnostic delay.

An encephalocele is a pathological brain herniation caused by osseous dural defects. Encephaloceles are known to be regions of epileptogenic foci. We describe the case of a 44-year-old woman with refractory epilepsy associated with a frontal skull base encephalocele. Epilepsy surgery for encephalocele resection was performed; however, the epilepsy was refractory. A second epilepsy surgery for frontal lobectomy using intraoperative electroencephalography was required to achieve adequate seizure control. Previous reports have shown that only encephalocele resection can result in good seizure control, and refractory epilepsy due to frontal lobe encephalocele has rarely been reported. To the best of our knowledge, this is the first report of frontal encephalocele plus epilepsy in which good seizure control using only encephalocele resection was difficult to achieve. Herein, we describe the possible mechanisms of encephalocele plus epilepsy and the surgical strategy for refractory epilepsy with encephalocele, including a literature review.

Amniotic band syndrome (ABS) is a rare condition of controversial etiology that is associated with varying degrees of anomalies. This study reports a case of a newborn with ABS associated with double encephalocele in the frontal region. A 29-year-old primiparous woman with no history of prenatal infection or consanguineous marriage had a cesarean section at gestational week 38, giving birth to a newborn who was well but had limb anomalies (constriction rings, amputations, and syndactyly) and craniofacial anomalies, mainly double frontal encephalocele. The patient underwent surgical repair and subsequent placement of a ventriculoperitoneal shunt. Studies clarifying this uncommon association with double encephalocele are limited. ABS associated with double encephalocele is rare and even more complex when associated with other anomalies. Thus, the conditions in such children are severe and require multidisciplinary monitoring.

Encephalocele is a rare congenital form of neural tube defect characterized by a protrusion of the meninges and cerebral tissue through a skull defect. These defects are classified according to their location: frontal, parietal and occipital, the last one being the most common form of presentation. The prognosis is related to the anatomical site, the volume of the neural contents and the presence of coexisting abnormalities. Most pregnancies are terminated, since the prognosis is poor. We report a case of an isolated fetal frontal encephalocele diagnosed at 21 weeks of gestation.