✓Tethered cord syndrome (TCS) is a stretch-induced functional disorder of the spinal cord due to the fact that its caudal portion is anchored by an inelastic structure. The functional lesion of TCS is generally situated in the lumbosacral cord, and many authors have shown that the syndrome is reversible via surgery to untether the cord. To clarify the expressions relevant to TCS, such as “cord tethering” and “tethered cord,” the authors have formulated three categories. These categories include cases that show the anatomical appearance of spinal cord stretching. Among them, Category 1 is isolated to represent the “true TCS.” The authors focus their discussion of the pathophysiology of TCS on Category 1 to explain the impaired oxidative metabolism and electrophysiological derangements within the tethered spinal cord, which is the primary intrinsic cause of the dysfunction. Furthermore, they extend the discussion to the extrinsic (outside the spinal cord) factors and other complex conditions that mimic TCS.

Object Deep brain stimulation (DBS) for dystonic cerebral palsy (CP) has rarely been reported, and its efficacy, though modest when compared with that for primary dystonia, remains unclear, especially in the pediatric population. The authors present a small series of children with dystonic CP who underwent bilateral pallidal DBS, to evaluate the treatment's efficacy and safety in the pediatric dystonic CP population. Methods The authors conducted a retrospective review of patients (under the age of 18 years) with dystonic CP who had undergone DBS of the bilateral globus pallidus internus between 2010 and 2012. Two of the authors independently assessed outcomes using the Barry-Albright Dystonia Scale (BADS) and the Burke-Fahn-Marsden Dystonia Rating Scale–movement (BFMDRS-M). Results Five children were diagnosed with dystonic CP due to insults occurring before the age of 1 year. Mean age at surgery was 11 years (range 8–17 years), and the mean follow-up was 26.6 months (range 2–42 months). The mean target position was 20.6 mm lateral to the midcommissural point. The mean preoperative and postoperative BADS scores were 23.8 ± 4.9 (range 18.5–29.0) and 20.0 ± 5.5 (range 14.5–28.0), respectively, with a mean overall percent improvement of 16.0% (p = 0.14). The mean preoperative and postoperative BFMDRS-M scores were 73.3 ± 26.6 (range 38.5–102.0) and 52.4 ± 21.5 (range 34.0–80.0), respectively, with a mean overall percent improvement of 28.5% (p = 0.10). Those stimulated at least 23 months (4 patients) improved 18.3% (p = 0.14) on the BADS and 30.5% (p = 0.07) on the BFMDRS-M. The percentage improvement per body region yielded conflicting results between rating scales; however, BFMDRS-M scores for speech showed some of the greatest improvements. Two patients required hardware removal (1 complete system, 1 unilateral electrode) within 4 months after implantation because of infections that resolved with antibiotics. Conclusions All postoperative dystonia rating scale scores improved with pallidal stimulation, and the greatest improvements occurred in those stimulated the longest. The results were modest but comparable to findings in other similar series. Deep brain stimulation remains a viable treatment option for childhood dystonic CP, although young children may have an increased risk of infection. Of particular note, improvements in the BFMDRS-M subscores for speech were comparable to those for other muscle groups, a finding not previously reported.

The neurosurgical treatment of skull base temporal encephalocele for patients with epilepsy is variable. We describe two adult cases of temporal lobe epilepsy (TLE) with spheno-temporal encephalocele, currently seizure-free for more than two years after anterior temporal lobectomy (ATL) and lesionectomy sparing the hippocampus without long-term intracranial electroencephalogram (EEG) monitoring. Encephaloceles were detected by magnetic resonance imaging (MRI) and confirmed by maxillofacial head computed tomography (CT) scans. Seizures were captured by scalp video-EEG recording. One case underwent intraoperative electrocorticography (ECoG) with pathology demonstrating neuronal heterotopia. We propose that in some patients with skull base temporal encephaloceles, minimal surgical resection of herniated and adjacent temporal cortex (lesionectomy) is sufficient to render seizure freedom. In future cases, where an associated malformation of cortical development is suspected, newer techniques such as minimally invasive EEG monitoring with stereotactic-depth EEG electrodes should be considered to tailor the surrounding margins of the resected epileptogenic zone.

Bilateral convexity and interhemispheric subdural hematomas are common neuroimaging patterns seen in infants who have sustained nonaccidental head injuries (NAHIs). These collections often appear aschronic or acute-on-chronic on computerized tomography (CT) studies. To determine the nature of these extraaxial fluid collections and their relationship to cerebrospinal fluid (CSF) dynamics, the authors studied five infants with suspected NAHI in whom symptomatic bilateral mixed- or low-density subdural collections were revealed on imaging studies; the patients underwent burr hole evacuation of the hematoma and external drainage. Once decompression was achieved, radiotracer was injected into the lumbar subarachnoid space, and the subdural drainage system was monitored for appearance of the isotope. In all five cases, the radiotracer moved rapidly from the lumbar subarachnoid space into the convexity subdural space and then into the external drainage system. This indicated the possibility that some of these mixed-density subdural collections were acute blood mixed with CSF rather than acute-on-chronic collections arising from rebleeding subdural membranes. The authors propose that, during infancy, tears in the loosely adherent arachnoid envelope at the main arachnoid granulation site along the superior sagittal sinus may result in a considerable amount of CSF mixing with acute blood in the subdural space, creating a hematohygroma.

A 26-year-old woman with obstructive hydrocephalus caused by a cysticercal cyst blocking the left foramen of Monro was initially treated with an external ventriculostomy and subsequent endoscopically assisted cyst resection and septostomy. Postoperatively, the patient developed the clinical syndrome of akinetic mutism, but her condition improved after the administration of the dopaminergic agonist bromocriptine. This result indicates that a disruption in the ascending dopaminergic pathway involved in behavior formation was likely the cause of these neurological symptoms.