Severe hyperglycorrhachia and status epilepticus after endoscopic aqueductoplasty: illustrative case.

BACKGROUND: While hypoglycorrhachia is observed and managed frequently, there are few reports in the literature of clinically significant hyperglycorrhachia after neurosurgery. Understanding the effects and management of severe hyperglycorrhachia is important to the neurosurgeon and neurocritical care teams who care for patients in these rare scenarios. OBSERVATIONS: The authors present the case of a 3-month-old male with congenital hydrocephalus who faced profound hyperglycorrhachia and status epilepticus after an endoscopic aqueductoplasty using an irrigant composed of lactated Ringer's solution with dextrose 5% in water. A multidisciplinary approach was developed to monitor and treat the patient's seizures and cerebrospinal fluid (CSF) osmolytes. LESSONS: This case provides several learning opportunities for understanding CSF physiology, pathogenesis of common brain injuries related to osmotic shifts and inflammatory states, as well as clinical management of hyperglycorrhachia. It also reiterates the significance of meticulous intraoperative assessment to avoid preventable medical errors.

Fulminant non-infectious peri-electrode edema after deep brain stimulation system implantation in a pediatric patient.

Non-infectious peri-electrode edema is a rare complication after implantation of a deep brain stimulation (DBS) electrode. DBS is frequently used in the management of movement disorders with increasing interest surrounding its value in more rare disorders associated with movement abnormalities. This is the report of a 10-year-old male with Cockayne syndrome who acutely developed symptomatic non-infectious, non-hemorrhagic peri-electrode edema 18 days postoperatively from implantation of a DBS system targeting the bilateral globus pallidus internus. CT head confirmed extensive vasogenic edema along the entire length of the left electrode, and infectious workup was negative. The patient required admission to the pediatric intensive care unit for management utilizing steroid, hypertonic, and hyperosmolar therapy due to the amount of mass effect. Symptoms reduced over a 7-day hospital stay and were completely resolved at 1 month without removal of the DBS system. Management of this rare entity remains controversial and often involves the use of steroids and anti-epileptic prophylaxis. This represents the first case of non-infectious peri-electrode edema reported in a pediatric patient and is especially notable for its fulminant nature.

Impact of epilepsy surgery on seizure control and quality of life: a 26-year follow-up study.

PURPOSE: The short-term efficacy and safety of epilepsy surgery relative to medical therapy has been established, but it remains underutilized. There is a lack of data regarding the long-term seizure-control rates and quality of life outcomes after epilepsy surgery. This study represents the longest follow-up study to date, with a mean follow-up duration of 26 years. METHODS: We studied the seizure and health-related quality of life outcomes of patients who underwent epilepsy surgery by Dr. Sidney Goldring from 1967 to 1990. Retrospective clinical chart reviews gathered perioperative data and surveys obtained follow-up data. Seizure outcome was evaluated using the Engel classification system. KEY FINDINGS: Of 361 patients, 117 (32.4%) completed follow-up interviews. Fifty-six patients (48%) were Engel class I. Mean overall Quality of Life in Epilepsy (QOLIE-31) questionnaire score for the cohort was 68.2 ± 16. Eighty percent of patients reported their overall quality of life now as being better than before surgery. Seizure freedom was associated with better quality of life. We did not observe a statistically significant association between postoperative complications and long-term outcome. Patients who underwent temporal lobe resection achieved better seizure outcomes than those who underwent other types of procedures. Astatic seizures and bilateral surgery were associated with a worse Engel class outcome. SIGNIFICANCE: Our study demonstrates that the beneficial effects of epilepsy surgery are sustained over decades, and that these beneficial effects are correlated with an improved quality of life. The confirmation of its durability makes us optimistic that the outcomes from modern epilepsy surgery will be even better and that our present enthusiasm for this treatment modality is not misplaced.

Bilateral deep brain stimulation for treatment of medically refractory paroxysmal nonkinesigenic dyskinesia.

The authors present the case of a 26-year-old man with a 10-15-year history of worsening bilateral dystonia and baseline chorea occurring up to 20 times per day that was exacerbated by stress and anxiety and was refractory to medical management. Paroxysmal nonkinesigenic dyskinesia was diagnosed, which is a rare, hyperkinetic movement disorder that is episodic and does not respond to nonbenzodiazepine antiepileptics. The patient was significantly debilitated by his disease, lived in a group home, and suffered from frequent falls, necessitating the wearing of a protective helmet and face mask at all times. The patient underwent implantation of bilateral deep brain stimulation quadripolar electrodes in the globus pallidus internus with the aid of image-guided stereotactic neurosurgery and microelectrode recording without complication. At his 1-month postoperative follow-up, the patient reported a subjective 90% improvement in his symptoms; the only notable side effect was a slight increased slurring in his baseline dysarthria. Objective reporting and recording forms maintained by the patient's caretakers over the following 18 months suggested a significant and sustained improvement in his overall balance, ambulation, and gross motor function with a substantial decrease in the incidence of reported falls. The authors conclude that pallidal deep brain stimulation may be successfully applied to patients suffering from refractory paroxysmal nonkinesigenic dyskinesia with promising results. This treatment strategy deserves further prospective investigation, clinical consideration, and refinement.

The "clamshell" craniotomy technique in treating sagittal craniosynostosis in older children.

OBJECT: Although most patients with sagittal craniosynostosis are recognized and treated in infancy, some children are not referred to craniofacial centers until later in childhood. In this paper the authors describe a novel operative technique for calvarial reconstruction in older children with previously untreated sagittal craniosynostosis. METHODS: The authors report a clinical series of eight patients who were treated using novel single-stage calvarial reconstruction, and they assess the complications and outcomes. The patient is placed supine for the procedure, which consists of a coronal incision, bifrontal craniotomy without orbital osteotomy, and multiple interlocking midline parietooccipital osteotomies and recontouring. Fixation is achieved using a bioabsorbable plate system. Cranial indices were calculated from measurements obtained before and after the reconstructive procedures. Preoperative, intraoperative, and postoperative photographs and three-dimensional computed tomography scans are presented for review. Between November 2003 and April 2005, the authors treated seven boys (age range approximately 1-10 years, mean age 4.2 years) with uncorrected sagittal craniosynostosis and one with bicoronal and sagittal synostosis. The mean operating time was 5.13 hours (range 4.3-8 hours), with a mean blood loss of 425 ml (range 200-800 ml). As a percentage of the estimated circulating blood volume, the mean operative blood loss was 33.5% (range 17-57%). The mean hospital stay was 4.9 days. The cranial index significantly improved from a mean of 65.6 to 71.3% (p = 0.001). No acute or delayed complications have been noted. Follow-up examinations performed at an average of 12 months (range 1-17 months) have confirmed early patient and family satisfaction. CONCLUSIONS: An approach of aggressive calvarial reconstruction with multiple interleaving osteotomies crossing the midline achieves improvements in biparietal narrowing. Combined with a bifrontal reconstruction, early outcomes are excellent, with an acceptable amount of intraoperative blood loss and no significant complications.