Increased plasmin-mediated proteolysis of L1CAM in a mouse model of idiopathic normal pressure hydrocephalus.

Idiopathic normal pressure hydrocephalus (iNPH) is a common neurological disorder that is characterized by enlarged cerebral ventricles, gait difficulty, incontinence, and dementia. iNPH usually develops after the sixth decade of life in previously asymptomatic individuals. We recently reported that loss-of-function deletions in CWH43 lead to the development of iNPH in a subgroup of patients, but how this occurs is poorly understood. Here, we show that deletions in CWH43 decrease expression of the cell adhesion molecule, L1CAM, in the brains of CWH43 mutant mice and in human HeLa cells harboring a CWH43 deletion. Loss-of-function mutations in L1CAM are a common cause of severe neurodevelopmental defects that include congenital X-linked hydrocephalus. Mechanistically, we find that CWH43 deletion leads to decreased N-glycosylation of L1CAM, decreased association of L1CAM with cell membrane lipid microdomains, increased L1CAM cleavage by plasmin, and increased shedding of cleaved L1CAM in the cerebrospinal fluid. CWH43 deletion also decreased L1CAM nuclear translocation, suggesting decreased L1CAM intracellular signaling. Importantly, the increase in L1CAM cleavage occurred primarily in the ventricular and subventricular zones where brain CWH43 is most highly expressed. Thus, CWH43 deletions may contribute to adult-onset iNPH by selectively downregulating L1CAM in the ventricular and subventricular zone.

Surgical considerations for spinal epidural hematoma evacuation in the setting of blue rubber bleb nevus syndrome in a child.

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition that presents with venous malformation blebs throughout the body, most commonly on the skin and gastrointestinal tract. There have only been a limited number of reports of benign BRBNS lesions involving the spine in children, which were detected after chronic symptomatology. We herein present a unique case of a ruptured BRBNS venous malformation into the epidural space of the lumbar spine in a child presenting with acute neurologic deficit and discuss the relevant surgical considerations for operating in the setting of BRBNS.

Bacterial translocation as a cause of ventriculoperitoneal shunt infection: A case report.

Bacterial translocation as a mechanism of distal catheter infection may play a larger role in ventriculoperitoneal shunt infections than previously recognized.

Spontaneous Propionibacterium Acnes abscess with intraventricular rupture in an immunocompetent adult without prior neurosurgical intervention.

Previously viewed as a culture contaminant, Propionibacterium Acnes can cause infection following neurosurgical intervention. Its role in brain abscess in the immunocompetent, surgically naïve population has been infrequently reported. Herein, we describe an immunocompetent 55-year-old man with no risk factors found to have a thalamic abscess with intraventricular rupture.

Utilization of anterior lumbar interbody fusion for severe kyphotic deformity secondary to Pott's disease: illustrative case.

BACKGROUND: Spinal tuberculosis may result in severe kyphotic deformity. Effective restoration of lordosis and correction of sagittal balance often requires invasive osteotomies associated with significant morbidity. The advantages of focusing on symptomatic management and staging in the initial treatment of these deformities have not been well reported to date. OBSERVATIONS: The authors reported the case of a 64-year-old Vietnamese woman with a history of spinal tuberculosis who underwent anterior lumbar interbody fusion (ALIF) for symptomatic treatment of L5-S1 radiculopathy resulting from fixed kyphotic deformity. Postoperatively, the patient experienced near immediate symptom improvement, and radiographic evidence at 1-year follow-up showed continued lordotic correction of 30° as well as stable sagittal balance. LESSONS: In this case, an L5-S1 ALIF was sufficient to treat the patient's acute symptoms and provided satisfactory correction of a tuberculosis-associated fixed kyphotic deformity while effectively delaying more invasive measures, such as a vertebral column resection. Patients with adult spinal deformity may benefit from less invasive staging procedures before treating these deformities with larger surgeries.

Evidence for increased intraabdominal pressure as a cause of recurrent migration of the distal catheter of a ventriculoperitoneal shunt: illustrative case.

BACKGROUND: Placement of a ventriculoperitoneal (VP) shunt is an effective treatment for several disorders of cerebrospinal fluid flow. A rare complication involves postoperative migration of the distal catheter out of the intraperitoneal compartment and into the subcutaneous space. Several theories attempt to explain this phenomenon, but the mechanism remains unclear. OBSERVATIONS: The authors report the case of a 37-year-old nonobese woman who underwent placement of a VP shunt for idiopathic intracranial hypertension. Postoperatively, the distal catheter of the VP shunt migrated into the subcutaneous space on three occasions despite the use of multiple surgical techniques, including open and laparoscopic methods of abdominal catheter placement. Notably, the patient repeatedly displayed radiographic evidence of chronic bowel distention consistent with increased intraperitoneal pressure. LESSONS: In this case, the mechanism of catheter migration into the subcutaneous space did not appear to be caused by pulling of the catheter from above but rather by expulsion of the catheter from the peritoneum. Space in the subcutaneous tissues caused by open surgical placement of the catheter was permissive for this process. Patients with chronic increased intraabdominal pressure, such as that caused by bowel distention, obesity, or Valsalva maneuvers, may be at increased risk for distal catheter migration.