Altered Circulating Immune Cell Distribution in Traumatic Spinal Cord Injury Patients in Relation to Clinical Parameters.

Following a spinal cord injury (SCI), an inflammatory immune reaction is triggered which results in advanced secondary tissue damage. The systemic post-SCI immune response is poorly understood. This study aimed to extensively analyse the circulating immune cell composition in traumatic SCI patients in relation to clinical parameters. High-dimensional flow cytometry was performed on peripheral blood mononuclear cells of 18 traumatic SCI patients and 18 healthy controls to determine immune cell subsets. SCI blood samples were collected at multiple time points in the (sub)acute (0 days to 3 weeks post-SCI, (s)aSCI) and chronic (6 to >18 weeks post-SCI, cSCI) disease phase. Total and CD4+ T cell frequencies were increased in cSCI patients. Both CD4+ T cells and B cells were shifted towards memory phenotypes in (s)aSCI patients and cSCI patients, respectively. Most profound changes were observed in the B cell compartment. Decreased immunoglobulin (Ig)G+ and increased IgM+ B cell frequencies reflected disease severity, as these correlated with American Spinal Injury Association (ASIA) impairment scale (AIS) scores. Post-SCI B cell responses consisted of an increased frequency of CD74+ cells and CD74 expression level within total B cells and B cell subsets. Findings from this study suggest that post-SCI inflammation is driven by memory immune cell subsets. The increased CD74 expression on post-SCI B cells could suggest the involvement of CD74-related pathways in neuroinflammation following SCI. In addition, the clinical and prognostic value of monitoring circulating IgM+ and IgG+ B cell levels in SCI patients should be further evaluated.

T3-T4 Disc Herniations: Clinical Presentation, Imaging, and Transaxillary Approach.

OBJECTIVE: To describe a cohort of T3-T4 thoracic disc herniations (TDHs), their clinical and radiologic characteristics, and unique thoracoscopic transaxillary approach (TAA). METHODS: All patients operated on for a T3-T4 TDH with minimal follow-up of 1 year were selected. RESULTS: Eight TAA procedures (6 males and 2 females) were included (1.4%). Six patients reported axial pain, irradiating in 2, 4 sensory changes, 1 objective and 1 merely subjective motor weakness. Only 1 TDH was calcified, none was giant, 2 were accompanied by myelomalacia, and 2 by a small segmental syrinx. A cardiothoracic surgeon helped with exposure through a curved axillary incision using anterior cervical and more recently double-ring wound retractors. All patients were operated on using a 10-mm 30° rigid (three-dimensional) high-definition scope. There were no major complications and a good outcome with symptomatic relief in 7 of 8 patients. CONCLUSIONS: T3-T4 TDHs are infrequent but may be underdiagnosed because they tend to be small and their signs and symptoms may mimic a cervical problem involving the shoulders and even the arms. There may be a male predominance. The TAA is straightforward, safe, efficacious, and well tolerated despite the supposed vulnerability of the upper thoracic spinal cord. Dissection between large crowded subpleural veins characteristic for the upper thoracic spine and ensuring adequate dura decompression when the steep angle may partially obscure the tip of the instruments does require some extra time. Thorough knowledge of the unique anatomy of the upper thorax is mandatory and the assistance of a cardiothoracic surgeon is highly recommended.

Short-term discontinuation of vagal nerve stimulation alters 18F-FDG blood pool activity: an exploratory interventional study in epilepsy patients.

BACKGROUND: Vagus nerve activation impacts inflammation. Therefore, we hypothesized that vagal nerve stimulation (VNS) influenced arterial wall inflammation as measured by 18F-FDG uptake. RESULTS: Ten patients with left-sided VNS for refractory epilepsy were studied during stimulation (VNS-on) and in the hours after stimulation was switched off (VNS-off). In nine patients, 18F-FDG uptake was measured in the right carotid artery, aorta, bone marrow, spleen, and adipose tissue. Target-to-background ratios (TBRs) were calculated to normalize the respective standardized uptake values (SUVs) for venous blood pool activity. Median values are shown with interquartile range and compared using the Wilcoxon signed-rank test. Arterial SUVs tended to be higher during VNS-off than VNS-on [SUVmax all vessels 1.8 (1.5-2.2) vs. 1.7 (1.2-2.0), p = 0.051]. However, a larger difference was found for the venous blood pool at this time point, reaching statistical significance in the vena cava superior [meanSUVmean 1.3 (1.1-1.4) vs. 1.0 (0.8-1.1); p = 0.011], resulting in non-significant lower arterial TBRs during VNS-off than VNS-on. Differences in the remaining tissues were not significant. Insulin levels increased after VNS was switched off [55.0 pmol/L (45.9-96.8) vs. 48.1 pmol/L (36.9-61.8); p = 0.047]. The concurrent increase in glucose levels was not statistically significant [4.8 mmol/L (4.7-5.3) vs. 4.6 mmol/L (4.5-5.2); p = 0.075]. CONCLUSIONS: Short-term discontinuation of VNS did not show a consistent change in arterial wall 18F-FDG-uptake. However, VNS did alter insulin and 18F-FDG blood levels, possibly as a result of sympathetic activation.

Elevated inflammatory cytokine expression in CSF from patients with symptomatic thoracic disc herniation correlates with increased pain scores.

BACKGROUND: The pathophysiology of pain in patients with symptomatic thoracic disc herniation (TDH) remains poorly understood. Mere mechanical compression of the spinal cord and/or the exiting nerve root by a prolapsed disc cannot explain the pathogenesis of pain in all cases. Previous studies report a direct correlation between the levels of proinflammatory cytokines in disc biopsies and the severity of leg pain in patients with lumbar disc herniation. A similar correlation in patients with TDH has not been investigated. PURPOSE: To correlate the cerebrospinal fluid (CSF) expression of cytokines and pain-related amino acids with preoperative pain scores in patients with symptomatic TDH. STUDY DESIGN: A prospective human study of CSF samples and clinical outcome scores. METHODS: Using enzyme-linked immunosorbent assay (ELISA) and high-performance liquid chromatography (HPLC), we determined inflammatory cytokine levels (TNF-α, IL-1ß, and IL-10) and amino acid levels (glutamate, aspartate, gamma-aminobutyric acid, glycine, and arginine) in CSF samples from 10 patients with TDH and 10 control subjects who did not suffer an inflammatory disease nor pain related to spinal cord compression and subsequently correlated these levels with preoperative pain scores. Differences between both groups were evaluated by a Mann-Whitney U test. In order to estimate the correlation between cytokine or amino acid expression and pain scores, data were analyzed using a linear regression analysis. RESULTS: No inflammatory cytokines were found in CSF samples from control subjects, whereas TNF-α, IL-1ß, and IL-10 were detectable by ELISA in all CSF samples from patients with TDH. TNF-α and IL-10 but not IL-1ß levels moderately correlated with preoperative pain scores. Elevated TNF-αlevels positively correlated with high pain scores; elevated IL-10 levels negatively correlated with high pain scores. Amino acids were detectable in all samples from both groups. There were no significant differences between the groups in any of the amino acids measured with HPLC. CONCLUSION: Increased proinflammatory cytokine expression is associated with elevated pain scores in patients with symptomatic TDH. On the other hand, there is no conclusive correlation between the intensity of pain and the local or systemic presence of amino acids associated with pain transmission.

Multilayer Dura Reconstruction After Thoracoscopic Microdiscectomy: Technique and Results.

BACKGROUND: Anterior transthoracic approaches, including thoracoscopic microdiscectomy (TMD), are the preferred techniques for central, broad-based, calcified thoracic disc herniations (TDHs). Dural defects due to manipulation or transdural extension may create a potentially life-threatening subarachnoid-pleural fistula. We evaluate a fast, sutureless technique for reconstructing the dura. METHODS: We analyzed 476 anterior transthoracic approaches for TDHs (437 TMDs, 31 mini-thoracotomies, 7 transaxillary approaches, and 1 sternotomy). Dural defects were closed using an onlay dura substitute, fibrin glue, an autologous fat graft, and another layer of fibrin glue topped by an absorbable gelatin sponge. A chest tube was used to provide a water seal, and the use of external lumbar drains (ELDs) was discontinued in recent years. RESULTS: We encountered 35 dural defects (7.35%), including 27 in 279 procedures (9.67%) in the first 10 years and 8 in 197 (4.06%) in the past 6 years. Five defects were large, 23 were relatively small, and 5 had an intact arachnoid layer, likely preventing cerebrospinal fluid (CSF) egress. Two CSF leaks in the early series were overlooked intraoperatively; 1 patient died following widespread cortical venous thrombosis, whereas the other merely had a headache that disappeared within 1 week. Overall, an ELD was inserted in 3 of 5 large defects and in 17 of 23 small defects; however, in the past 6 years, an ELD was inserted in no large defects and in only 1 of 5 small defects. The technique was successful in all but 1 patient, who underwent reoperation using a larger fat graft. CONCLUSIONS: Dural defects encountered during anterior transthoracic approaches to TDHs can be reconstructed endoscopically, while avoiding the use of an ELD in most cases.

Vagus nerve stimulation lead removal or replacement: surgical technique, institutional experience, and literature overview.

BACKGROUND: With the growing use of vagus nerve stimulation (VNS) as a treatment for refractory epilepsy, there is a growing demand for complete removal or replacement of the VNS system. We evaluate the safety and efficacy of complete removal or replacement of the VNS system and provide an extensive description of our surgical technique. METHODS: We retrospectively reviewed our patient registry for all VNS surgeries performed between January 2007 (the year of our first complete removal) and May 2014. In order to assess patient satisfaction, a written questionnaire was sent to patients or their caregivers. Additionally, we reviewed all literature on this topic. RESULTS: The VNS system was completely removed in 22 patients and completely replaced in 13 patients. There were no incomplete removals. Revision surgery was complicated by a small laceration of the jugular vein in two patients and by vocal cord paralysis in one patient. Seizure frequency was unaltered or improved after revision surgery. Electrode-related side effects all improved after revision surgery. Twenty-one studies reported a total of 131 patients in whom the VNS system was completely removed. In 95 patients, the system was subsequently replaced. The most frequently reported side effect was vocal cord paresis, which occurred in four patients. CONCLUSIONS: Complete removal or replacement of the VNS system including lead and coils is feasible and safe. Although initial results seem promising, further research and longer follow-up are needed to assess whether lead replacement may affect VNS effectiveness.

Crippling upper back pain after whiplash and other motor vehicle collisions caused by thoracic disc herniations: report of 10 cases.

STUDY DESIGN: Retrospective analysis of a prospectively collected database of thoracoscopic microdiscectomies performed at the Maastricht University Medical Center. OBJECTIVE: Many victims of a motor vehicle collision (MVC) report crippling upper back pain resistant to conservative treatment. Although this pain is often regarded as nonspecific or related to a whiplash type of cervical spine injury, this study demonstrates it may be caused by a thoracic disc herniation. SUMMARY OF BACKGROUND DATA: Recent literature on bodily pain after whiplash and other MVCs has shown that most patients rather than pain confined to the posterior neck area (0.4%) report pain in multiple body areas, the most frequently affected region being the posterior trunk region, including the posterior neck, posterior shoulder, upper back, lumbar, and buttock areas. Although several patterns determining most variance in pain localization in these patients have been identified, different pathoanatomical and pathophysiological substrates underlying these patterns have not been identified. However, a high incidence of posterior shoulder pain (75%) and upper back pain (66%) is striking. METHODS: In a series of 326 thoracoscopic microdiscectomies for one or more symptomatic TDHs, we identified 10 patients whose symptoms had started after an MVC. We analyzed their clinical and radiological presentation, intraoperative findings, and postoperative outcome. RESULTS: All patients (7 females, 3 males; age, 26-58 yr, including 4 with typical whiplash complaints) had reported substantial improvement of their complaints except for their upper back pain. Most hernias were small or medium sized (n = 8), at the apex of the kyphotic curvature (n = 6), and to some extent calcified (n = 7). One year postoperatively, results were excellent in 7, good in 2, and poor in 1. CONCLUSION: Crippling upper back pain after MVCs may be caused by a (previously asymptomatic) thoracic disc herniation. Although the exact pathophysiological mechanism has not been elucidated, results after thoracoscopic microdiscectomy are quite encouraging.

Diagnostic radiation exposure in children with spinal dysraphism: an estimation of the cumulative effective dose in a cohort of 135 children from The Netherlands.

OBJECTIVE: Based on the assumption that children with spinal dysraphism are exposed to a large amount of ionising radiation for diagnostic purposes, our objective was to estimate this exposure, expressed in cumulative effective dose. DESIGN: Retrospective cohort study. SETTINGS: The Netherlands. PATIENTS: 135 patients with spinal dysraphism and under 18 years of age treated at our institution between 1991 and 2010. RESULTS: A total of 5874 radiological procedures were assessed of which 2916 (49.6%) involved ionising radiation. Mean cumulative effective dose of a child with spinal dysraphism during childhood was 23 mSv, while the individual cumulative effective dose ranged from 0.1 to 103 mSv. Although direct radiography accounted for 81.7% of examinations, the largest contributors to the cumulative effective dose were fluoroscopic examinations (40.4% of total cumulative effective dose). CONCLUSIONS: Exposure to ionising radiation and associated cancer risk were lower than expected. Nevertheless, the use of ionising radiation should always be justified and the medical benefits should outweigh the risk of health detriment, especially in children.

Behavioural and cognitive effects during vagus nerve stimulation in children with intractable epilepsy - a randomized controlled trial.

BACKGROUND/AIMS: In addition to effects on seizure frequency in intractable epilepsy, multiple studies report benefits of vagus nerve stimulation (VNS) on behavioural outcomes and quality of life. The present study aims to investigate the effects of VNS on cognition, mood in general, depression, epilepsy-related restrictions and psychosocial adjustment in children with intractable epilepsy, as well as the relation between these effects and seizure reduction. METHODS: We conducted a randomized, active-controlled, double-blinded, add-on study in 41 children (age 4-18) with medically refractory epilepsy. We performed cognitive and behavioural testing at baseline (12 weeks), at the end of the blinded phase (20 weeks) in children receiving either high-output or low-output (active control) stimulation, and at the end of the open label phase (19 weeks) with all children receiving high-output stimulation. Seizure frequency was recorded using seizure diaries. RESULTS: VNS did not have a negative effect on cognition nor on psychosocial adjustment. At the end of the follow-up phase we noted an improvement of mood in general and the depression subscale for the entire group, unrelated to a reduction of seizure frequency. At the end of the blinded phase a ≥50% reduction of seizure frequency occurred in 16% of the high-stimulation group and 21% of the low-stimulation group. At the end of the open-label follow-up phase, 26% of the children experienced a seizure frequency reduction of 50% or more (responders). CONCLUSIONS: VNS has additional beneficial effects in children with intractable epilepsy. As opposed to anti-epileptic drugs, there are no negative effects on cognition. Moreover, we observed an improvement of mood in general and depressed feelings in particular, irrespective of a reduction in seizure frequency. These beneficial effects should be taken into account when deciding whether to initiate or continue VNS treatment in these children.

Vagus nerve stimulation in children with intractable epilepsy: a randomized controlled trial.

AIM: The aim of this study was to evaluate the effects of vagus nerve stimulation (VNS) in children with intractable epilepsy on seizure frequency and severity and in terms of tolerability and safety. METHOD: In this study, the first randomized active controlled trial of its kind in children, 41 children (23 males; 18 females; mean age at implantation 11y 2mo, SD 4y 2mo, range 3y 10mo-17y 8mo) were included. Thirty-five participants had localization-related epilepsy (25 symptomatic; 10 cryptogenic), while six participants had generalized epilepsy (four symptomatic; two idiopathic). During a baseline period of 12 weeks, seizure frequency and severity were recorded using seizure diaries and the adapted Chalfont Seizure Severity Scale (NHS3), after which the participants entered a blinded active controlled phase of 20 weeks. During this phase, half of the participants received high-output VNS (maximally 1.75mA) and the other half received low-output stimulation (0.25mA). Finally, all participants received high-output stimulation for 19 weeks. For both phases, seizure frequency and severity were assessed as during the baseline period. Overall satisfaction and adverse events were assessed by semi-structured interviews. RESULTS: At the end of the randomized controlled blinded phase, seizure frequency reduction of 50% or more occurred in 16% of the high-output stimulation group and in 21% of the low-output stimulation group (p=1.00). There was no significant difference in the decrease in seizure severity between participants in the stimulation groups. Overall, VNS reduced seizure frequency by 50% or more in 26% of participants at the end of the add-on phase The overall seizure severity also improved (p<0.001). INTERPRETATION: VNS is a safe and well-tolerated adjunctive treatment of epilepsy in children. Our results suggest that the effect of VNS on seizure frequency in children is limited. However, the possible reduction in seizure severity and improvement in well-being makes this treatment worth considering in individual children with intractable epilepsy.

Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome.

OBJECTIVE: Tight filum syndrome (TFS) is caused by a thick (abnormal T1 MRI), shortened (low-lying conus), or non-elastic filum (strictly normal MRI). We carefully analyzed children treated for suspect TFS with or without radiological abnormalities. METHODS: Twenty-five children, operated between 2002 and 2009, were retrospectively identified. All children had been evaluated by a multidisciplinary team preoperatively. Symptoms, signs and diagnostic test results were categorized (neurologic, urologic, orthopedic, dermatologic) and compared pre- and one year postoperatively. Normal MR was defined as conus medullaris (CM) at or above mid-body L2 and filum diameter less than 2 mm. Occult TFS (OTFS) was defined as TFS with normal MR. DEMOGRAPHICS: 17 girls, 8 boys, age 2-18 years, including 11 syndromal children. CLINICAL PRESENTATION: all children had problems in the neurologic category and at least one other category: urologic (n = 17), orthopedic (n = 21), and dermatologic (n = 11). MR findings: low-lying CM (n = 14) including 2 with thick filum, normal CM but fatty filum (n = 2), strictly normal (n = 9). Clinical outcome one year postoperatively: neurologic 20 improved, 5 stabilized; urologic 13 improved, 3 stabilized, 1 worsened; orthopedic (8 children presenting with scoliosis) 3 improved, 4 stabilized, 1 worsened. All children with OTFS (n = 9) improved in at least one and 8 improved in all affected categories. CONCLUSIONS: Children with strong clinical suspicion for TFS (≥ 2 affected categories) with or without abnormal MR findings will likely benefit from surgery. In such cases we suggest a detailed full spine MR, a multidisciplinary diagnostic work-up, and eventual untethering through an interlaminar microsurgical approach.

Thoracic disc herniation and acute myelopathy: clinical presentation, neuroimaging findings, surgical considerations, and outcome.

OBJECT: Thoracic disc herniations (TDHs) may occasionally present with an acute myelopathy, defined as a variable degree of motor, sensory, and sphincter disturbances developing in less than 24 hours, and resulting in a Frankel Grade C or worse. Confronted with such a patient, the surgeon has to decide whether to perform an emergency operation and whether to use an anterior or posterior approach. The authors analyze their own experience and the pertinent literature, focusing on clinical presentation, imaging findings, surgical timing, technique, and outcome. METHODS: Among 250 patients who underwent surgery for symptomatic TDH, 209 had at least 1 year of follow-up at the time of writing, including 8 patients who presented with an acute myelopathy. They were surgically treated using standard thoracoscopic microdiscectomy, careful blood pressure monitoring, and intravenous methylprednisolone. The authors analyzed pre- and postoperative neuroimaging, and Frankel scores preoperatively, at discharge, and 1 year postoperatively. RESULTS: Although 5 patients had multiple TDHs, the symptomatic TDH was invariably situated between T9-10 and T11-12. Seven TDHs were giant, 6 were calcified, 6 were accompanied by myelomalacia, and 4 were accompanied by segmental stenosis. Although sudden dorsalgia was the initial symptom in 6, a precipitating event was noted in only 1. All patients had severe neurological deficits by the time they underwent surgery. Frankel grades improved from B to D in 2 patients, from C to E in 4, and from C to D and B to E in 1 patient each. All patients regained continence and ambulation. Transient complications were CSF leak (in 2 patients), and intraoperative blood loss greater than 1000 ml, reversible ischemic neurological deficit, and subileus (in 1 patient each). CONCLUSIONS: Approximately 4% of TDHs present with an acute myelopathy. They are often situated between T9-10 and T11-12, large or giant, and even calcified. They almost invariably cause important cord compression (sometimes aggravated by an associated segmental stenosis) and myelomalacia. Their clinical presentation may be misleading, and diagnosis may be delayed until other causes (especially vascular) have been excluded and the clinical picture has become more complete. Interestingly, whereas a precipitating event or trauma is rarely present, dorsalgia frequently precedes profound myelopathy and may help to make an early diagnosis. Remarkable recovery is possible even with profound neurological deficit, a delay of several days, in the elderly, and in the presence of myelomalacia, provided the spinal cord is adequately decompressed and intraoperative hypotension is strictly avoided. Although alternative approaches more familiar to most neurosurgeons may be used, the anterior transthoracic approach has the advantage of reaching the TDH in front of the compromised spinal cord, avoiding any manipulation. In experienced hands, thoracoscopic microdiscectomy combines the advantage and versatility of an anterior approach with minimal postoperative discomfort. The authors conclude that TDH-related acute myelopathy may have a favorable outcome when managed correctly, and they strongly recommend that every single patient should undergo surgical treatment.

The lumbosacral angle does not reflect progressive tethered cord syndrome in children with spinal dysraphism.

PURPOSE: Our goal was to validate the hypothesis that the lumbosacral angle (LSA) increases in children with spinal dysraphism who present with progressive symptoms and signs of tethered cord syndrome (TCS), and if so, to determine for which different types and/or levels the LSA would be a valid indicator of progressive TCS. Moreover, we studied the influence of surgical untethering and eventual retethering on the LSA. METHODS: We retrospectively analyzed the data of 33 children with spinal dysraphism and 33 controls with medulloblastoma. We measured the LSA at different moments during follow-up and correlated this with progression in symptomatology. RESULTS: LSA measurements had an acceptable intra- and interobserver variability, however, some children with severe deformity of the caudal part of the spinal column, and for obvious reasons those with caudal regression syndrome were excluded. LSA measurements in children with spinal dysraphism were significantly different from the control group (mean LSA change, 21.0° and 3.1° respectively). However, both groups were not age-matched, and when dividing both groups into comparable age categories, we no longer observed a significant difference. Moreover, we did not observe a significant difference between 26 children with progressive TCS as opposed to seven children with stable TCS (mean LSA change, 20.6° and 22.4° respectively). CONCLUSIONS: We did not observe significant differences in LSA measurements for children with clinically progressive TCS as opposed to clinically stable TCS. Therefore, the LSA does not help the clinician to determine if there is significant spinal cord tethering, nor if surgical untethering is needed.

The clinical spectrum of Blake's pouch cyst: report of six illustrative cases.

INTRODUCTION: Although Blake's pouch cyst (BPC) is frequently mentioned in the spectrum of posterior fossa cysts and cystlike malformations since its first description in 1996, its natural history, clinical presentation, specific imaging characteristics, optimal treatment, and outcome are relatively unknown. Consequently, BPC may still be underdiagnosed. We therefore report six cases ranging from a fatal hydrocephalus in a young boy, over an increasing head circumference with or without impaired neurological development in two infants, to a decompensating hydrocephalus at an advanced age. DISCUSSION: We focus on their radiological uniformity, which should help making the correct diagnosis, and widely variable clinical presentation, which includes adult cases as well. Differentiating BPC from other posterior fossa cysts and cystlike malformations and recognizing the accompanying hydrocephalus are essentially noncommunicating, not only have important implications on clinical management but also on genetic counseling, which is unnecessary in case of BPC. In our experience, endoscopic third ventriculostomy is a safe and effective treatment option, avoiding the risks and added morbidity of open surgery, as well as many shunt-related problems.

Intramedullary cavernoma presenting with hematomyelia: report of two girls.

INTRODUCTION: Less than 20 children with intramedullary cavernoma (ImC) have been reported in the English literature; however, cases with an unfavorable outcome may be underreported. Whereas these are predominantly boys, we report two girls who presented with hematomyelia (one cervical, one thoracic) and an acute, severe neurological deficit. CASE MATERIAL: A 10-year-old girl complaining about lower thoracic pain for several days suddenly developed lower body dysesthesias and paraparesis. Magnetic resonance (MR) demonstrated hematomyelia (T8-T11), intramedullary edema (T6-L1), and an ImC at T9-T10. Within an hour, she progressed to paraplegia and was therefore operated immediately. She slowly recovered regaining independent ambulation and continence. MR after 2 years shows no recurrence. A 7-year-old girl suddenly developed cervicalgia and paresis of her left arm and leg. MR demonstrated hematomyelia and an ImC at C4-C6. She gradually recovered with minimal residual deficit at 3 months and was subsequently operated uneventfully. Multiple cerebral cavernomas and a familial autosomal cavernous malformation syndrome were diagnosed. The following 1.5 years, she complained of intermittent cervicalgia and left brachial dysesthesias, with MR suggesting active residual cavernoma. Interestingly, her complaints gradually disappeared, and she is currently asymptomatic. MR after 3.5 years shows minimal cord swelling no longer suggesting active residual cavernoma. CONCLUSION: With adequate surgical treatment either in the acute phase in case of dramatic deterioration or after clinical recuperation, prognosis of symptomatic ImC may be surprisingly good. However, subtotally resected lesions and/or syndromal cases may recur, requiring further treatment. Definitive answers await more cases with longer follow-up.

Solitary juvenile xanthogranuloma of the temporal muscle and bone penetrating the dura mater in a 2-month-old boy.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily observed during the first 2 years of life. Most patients present with a solitary cutaneous lesion; however, others present with extracutaneous manifestations or even with systemic involvement. The authors describe a 2-month-old boy in whom was diagnosed a unifocal extracutaneous JXG involving the temporal bone. Unlike 3 other cases of solitary JXGs of the temporal bone in the literature, the present case involved destruction of the dura mater and leptomeningeal enhancement surrounding the entire temporal lobe. The lesion did not regress after an initial biopsy procedure and had to be removed more radically because of progressive mass effect on the brain. The child recently underwent a reconstructive skull procedure and is doing well almost 2 years postoperatively without evidence of disease. This case demonstrates that even in instances of extensive disease a favorable outcome is possible without chemotherapy.