Rare but Real: Severe Unilateral Macroglossia and Submandibular Sialoadenitis After Skull Base Surgery.

We present a 43-year-old patient with a left-sided cerebellopontine angle meningioma with extension to the internal acoustic meatus and jugular foramen. The patient underwent a resection using a retrosigmoid approach, which resulted in near-complete tumor removal. Postoperatively, the patient experienced tongue swelling, swallowing difficulties and right-sided subcutaneous swelling, caused by patient positioning and endotracheal tube placement. Imaging showed phlegmonous infiltration of subcutaneous fat tissue with submandibular gland enlargement. The patient's condition gradually improved with conservative management. This case highlights the rare occurrence of combined macroglossia and sialoadenitis after posterior fossa surgery, emphasizing the importance of patient positioning and tube placement.

Decompression of the internal auditory canal via the retrosigmoid approach in a patient with Camurati-Engelmann disease: illustrative case.

BACKGROUND: Camurati-Engelmann disease (CED) is a rare condition characterized by hyperostosis of the long bones and skull base. Symptoms include contractures and pain in affected extremities but can also include manifestations of cranial hyperostosis such as intracranial hypertension, Chiari malformation, exophthalmia, frontal bossing, and several cranial neuropathies due to cranial foraminal stenosis. OBSERVATIONS: This report describes a 27-year-old patient with suspected CED who developed progressive intermittent facial nerve paresis, hemifacial spasms, and a decrease in hearing. There were no symptoms of increased intracranial pressure or vertigo. Radiological evaluation showed a significant thickening of the skull base with serious bilateral internal auditory canal stenosis. Because of the progressive nature of the aforementioned cranial neuropathies in combination with the correlating severe radiological compression, a surgical decompression of the facial nerve and vestibulocochlear nerve was performed via a retrosigmoid approach with intraoperative monitoring. Postoperative facial nerve function was intact. Hearing and vestibular function were unchanged. There were no more episodes of facial nerve palsy or spasm. LESSONS: To the authors' knowledge, this is the first report to describe decompression of the internal auditory canal via a retrosigmoid approach for symptomatic facial and cochlear nerve compression in a patient with CED.

Intratemporal facial nerve schwannomas: multicenter experience of 80 cases.

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

Postoperative Infections Associated With Prolonged Spinal Cord Stimulation Trial Duration (PROMISE RCT).

INTRODUCTION: In the PROMISE study, a multinational randomized controlled trial (RCT) of the effectiveness of spinal cord stimulation (SCS) with multicolumn surgical leads as a treatment of low back pain, clinicians followed their usual practice. An early, unplanned safety analysis revealed that the infection rate in Belgium (5/23), where trial duration was a median 21.5 days, was significantly higher than the 1/64 rate observed in the other study countries (median 5.8 days, p < 0.01). This report reviews infections observed in the PROMISE study after study completion. MATERIALS AND METHODS: For all infections related to SCS, we used descriptive statistics and tests of independent variables to analyze potentially contributing factors (age, sex, coexisting medical conditions, tobacco use, lead type, and trial duration) between subjects with infections versus those without. Cumulative incidence curves were created using the Kaplan-Meier method and compared between the two strata using a log-rank test. RESULTS: Among nine (5.2%) infections in 174 subjects trialed, the only significant contributing factor to infection was trial duration: median 21 days (range 3-56) for those with infection vs. six days (1-41) for those without (p = 0.001; Wilcoxon rank-sum test). The cumulative incidence of infection for subjects trialed >10 days was 24.1% vs. 1.4% for subjects trialed ≤10 days (p < 0.001). After the protocol was amended to limit trial duration to 10 days, 14 infection-free trials were performed in Belgium. CONCLUSIONS: Although not part of the preplanned analysis, our observation supports the hypothesis of a cause-effect relationship between trial duration and the risk of infection and the conclusion that prolonged SCS trials should be avoided.

Complete Transection of Optic Nerve After Endovascular Coiling of a Large Ophthalmic Artery Aneurysm.

BACKGROUND: We describe a patient who developed delayed blindness of the left eye at 5 weeks after endovascular coiling of a large ophthalmic aneurysm. CASE DESCRIPTION: A 44-year-old male was admitted with visual decline due to compression of the optic nerve by a large ophthalmic aneurysm. The aneurysm was treated by endovascular coiling, but visual function was unchanged. One month and 7 days later, the patient developed sudden blindness of the affected eye, despite complete angiographical occlusion of the aneurysm. Surgical exploration in an attempt to restore vision showed a fully thrombosed aneurysm but, surprisingly, complete transection of the optic nerve just proximal to its entry into the optic canal. CONCLUSIONS: This report describes a rare complication of a sudden increase in size of a large ophthalmic aneurysm despite successful endovascular occlusion.

Burst SCS Microdosing Is as Efficacious as Standard Burst SCS in Treating Chronic Back and Leg Pain: Results From a Randomized Controlled Trial.

INTRODUCTION: The burst waveform, a recent innovation in spinal cord stimulation (SCS), can achieve better outcomes than conventional tonic SCS, both for de novo implants and as a salvage therapy. Burst stimulation delivers more energy per second than tonic stimulation, which is a consideration for battery consumption. The clinical effectiveness of an energy-conserving strategy was investigated. METHODS: Subjects were experienced users of BurstDR SCS for back and leg pain. Three 2-week stimulation paradigms were presented in blinded random order: standard (continuously delivered) BurstDR, microdosing A: 5 sec of BurstDR alternating with 5 sec of no stimulation, and microdosing B: 5 sec of BurstDR alternating with 10 sec of no stimulation. The primary outcome for each paradigm was change in pain ratings, and secondary outcomes included changes in scores for quality of life, satisfaction, and preference. RESULTS: Twenty-five subjects assessed all three stimulation paradigms. There were no significant differences in pain (visual analog scale) or quality of life (EQ-5D) when comparing standard burst outcomes with those of microdosing A and, separately, microdosing B. Microdosing paradigms were graded with slightly higher level of satisfaction and were generally preferred above standard burst stimulation. DISCUSSION: These results suggest that the use of energy-efficient burst microdosing stimulation paradigms with alternating stimulation-on and stimulation-off periods can provide clinically equivalent results to standard burst stimulation. This is important for extending SCS battery life. Further research is needed to comprehensively characterize the clinical utility of this approach and the neurophysiological mechanisms for the maintenance of pain relief during stimulation-off periods.

What is the Required Frequency of MRI Scanning in the Wait and Scan Management?

The wait and scan policy is being increasingly used as the first measure after the diagnosis of a vestibular schwannoma (VS) using magnetic resonance imaging (MRI). As part of the European Academy of Otology and Neuro-Otology (EAONO) position statement on VS, the frequency of imaging has been studied in the literature. Among 163 studies, 29 fulfilled the inclusion criteria and were scored using the Grading of Recommendations, Assessment, Development, and Evaluation system. Because tumor growth rate during the first 5 years of follow-up is predictive of further growth during the upcoming years, a protocol for wait and scan is useful for centers dealing with this condition. The EAONO proposal is that after the initial diagnosis by MRI, a first new MRI would take place after 6 months, annually for 5 years, and then every other year for 4 years, followed by a lifelong MRI follow-up every 5 years. The first early MRI is to screen for fast-growing tumors, and the lifelong follow-up with tapered intervals is to detect late repeated growth.

Adding Prefrontal Transcranial Direct Current Stimulation Before Occipital Nerve Stimulation in Fibromyalgia.

OBJECTIVES: Fibromyalgia (FM) is a type of chronic musculoskeletal pain without a clear peripheral origin of nociception, often associated with depression. The underlying pathophysiology involves changes in a functional network that is related to pain and emotional processing in the central nervous system. Transcranial direct current stimulation (tDCS) targeting the dorsolateral prefrontal cortex or the occipital nerve (ON) is a noninvasive neuromodulation technique capable of improving fibromyalgia symptoms. This study aims to test the effect of combining 2 targets of stimulation using tDCS. MATERIALS AND METHODS: We applied ON-tDCS in isolation or coupled with pre-ONS right-anode bifrontal tDCS and assessed its effect on fibromyalgia using the Fibromyalgia Impact Questionnaire, the Beck Depression Inventory, and Numeric Rating Scale for pain scores. These measures were compared with a sham control group using repeated measures analysis of variance. RESULTS: The interaction effect of stimulation trials and the protocols of sham versus ON-tDCS were significant for the impact, distress, and pain caused by fibromyalgia (P<0.05). The interaction effect of trials and protocols of sham versus ON-tDCS with bifrontal tDCS was significant for distress (P<0.01), and it showed a trend of improvement for impact and pain (P<0.1). On the basis of the nonsignificant interaction effect of ON-tDCS versus ON-tDCS with bifrontal tDCS (P>0.1), adding bifrontal tDCS was found not to improve the treatment effect of ON-tDCS in any of the tested clinical outcome measures. DISCUSSION: This study suggests that adding right-anode bifrontal tDCS to ONS has no added benefit in improving fibromyalgia-related symptoms.

Resting state electrical brain activity and connectivity in fibromyalgia.

The exact mechanism underlying fibromyalgia is unknown, but increased facilitatory modulation and/or dysfunctional descending inhibitory pathway activity are posited as possible mechanisms contributing to sensitization of the central nervous system. The primary goal of this study is to identify a fibromyalgia neural circuit that can account for these abnormalities in central pain. The second goal is to gain a better understanding of the functional connectivity between the default and the executive attention network (salience network plus dorsal lateral prefrontal cortex) in fibromyalgia. We examine neural activity associated with fibromyalgia (N = 44) and compare these with healthy controls (N = 44) using resting state source localized EEG. Our data support an important role of the pregenual anterior cingulate cortex but also suggest that the degree of activation and the degree of integration between different brain areas is important. The inhibition of the connectivity between the dorsal lateral prefrontal cortex and the posterior cingulate cortex on the pain inhibitory pathway seems to be limited by decreased functional connectivity with the pregenual anterior cingulate cortex. Our data highlight the functional dynamics of brain regions integrated in brain networks in fibromyalgia patients.

A Multidisciplinary Infection Control Bundle to Reduce the Number of Spinal Cord Stimulator Infections.

OBJECTIVE: To investigate the effect of a quality improvement project that resulted in an infection control bundle to reduce the number of spinal cord stimulator (SCS) infections. MATERIALS AND METHODS: The study was performed in a single center for neuromodulation from January 1, 2014, through May 31, 2016. In response to a high number of surgical site infections (SSIs) after SCS surgery, a multidisciplinary team analyzed the surgical process and developed an infection prevention bundle consisting of five items: 1) showering and decolonization for five days prior to surgery and showering in the hospital on the morning of surgery; 2) performing the SCS implantation as the first in the daily operating room (OR) program; 3) maintaining a minimal number of people in the OR; 4) providing home care nurses with a folder with SCS wound care instructions including pictures; 5) giving oral specific wound care instructions to patients. The number of infections was calculated for the baseline, implementation, and sustainability phases. RESULTS: A total of 410 SCS surgeries were performed during the study period. In the preintervention phase, 26/249 (10.4%) SCS surgeries were infected. During the implementation and sustainability phase, 2/59 (3.4%) and 1/102 (1.0%) SCS surgeries were infected, respectively. The reduction in the number of infections in pre and postintervention phase was statistically significant (p = 0.003). CONCLUSION: Multidisciplinary measures to reduce SSIs reduced the number of SCS associated infections in our study setting.

Spinal cord stimulation for the treatment of chronic back pain patients: 500-Hz vs. 1000-Hz burst stimulation.

OBJECTIVE: Spinal cord stimulation is a commonly used, safe, and effective procedure applied for medically intractable failed back surgery syndrome, as well as other neuropathic pain syndromes. Recently, a novel stimulation paradigm called burst stimulation has been developed that is paresthesia-free and has a more pronounced suppressive effect on neuropathic pain. MATERIALS AND METHODS: Fifteen patients who were being treated with burst spinal cord stimulation for failed back surgery syndrome participated in an open-label trial to verify whether their pain suppression could be further ameliorated by changing the burst pattern. Burst stimulation with packets of five electrical pulses delivered at 500 Hz with 1000-µsec pulse width 40 times per second was changed to burst mode delivering five spikes at 1000 Hz with 500-µsec pulse width 40 times a second. As the amplitudes did not differ between the two groups, the total delivery of current to the spinal cord was not different between the two modes of burst stimulation. Scores on visual analog scales for pain and paresthesia, the Pain Catastrophizing Scale, the Pain Vigilance and Awareness Questionnaire, and the Short Form 36 quality of life measurement were compared between the two modes of burst stimulation. [Correction added on 06 Feb 2015, after first online publication: this paragraph has been revised to signify the comparison of amplitudes between two groups] RESULTS: No statistically significant differences were found between the two modes of stimulation. CONCLUSION: The results suggest that increasing the frequency from 500 to 1000 Hz while keeping the pulse width constant does not add any extra benefit in suppressing pain. Further studies should verify whether increasing the frequency above 1000 Hz has a similar lack of effect.

A 2-center comparative study on tonic versus burst spinal cord stimulation: amount of responders and amount of pain suppression.

INTRODUCTION: Spinal cord stimulation is a safe and effective procedure applied for medically intractable neuropathic pain and failed back surgery syndrome. Recently, a novel stimulation paradigm was developed, called burst stimulation consisting of intermittent packets of closely spaced high-frequency stimuli. The design consists of 40 Hz burst mode with 5 spikes at 500 Hz per burst, with a pulse width of 1 ms and 1 ms interspike interval delivered in constant current mode. METHODS AND MATERIALS: A retrospective analysis is performed looking at 102 patients from 2 neuromodulation centers, 1 in Belgium and 1 in the Netherlands. This consisted of 2 groups, 1 group who had become failures to tonic (conventional) stimulation and 1 group who still responded to tonic stimulation. All patients were switched from tonic to burst stimulation and the amount of responders as well as the amount of pain suppression was assessed. RESULTS: Overall burst stimulation was significantly better than tonic stimulation and baseline. On average the pain on numeric rating scale (NRS) improved from 7.8 at baseline to 4.9 with tonic to 3.2 with burst stimulation. For the Belgian and Dutch centers combined, 62.5% of nonresponders to tonic stimulation did respond to burst stimulation, on average, with 43% pain suppression. Most responders to tonic further improved with burst stimulation; on average, pain suppression improved from 50.6% to 73.6.3%. The results (from both centers) did not differ for the amount of obtained pain suppression, only for the amount of responders, which could be related to the different profile of the 2 participating centers. CONCLUSIONS: Burst seems to be significantly better than tonic stimulation. It can rescue an important amount of nonresponders to tonic stimulation and can further improve pain suppression in responders to tonic stimulation.

Development of acute schmorl nodes after discography.

We report the development of acute Schmorl nodes at the L3-L4 intervertebral disc level after discography in a 36-year-old man. Although a few cases of acute Schmorl nodes have been reported in the literature, they have not been described because of discography. We surmise that the herniation of disc material through the vertebral endplates, with the ensuing formation of Schmorl nodes, should be regarded as a potential, but fortunately rare, complication of discography.

Late onset subgaleal hemorrhage infection with Streptococcus pneumoniae?

We report a case of an infected subgaleal hematoma caused by an unusual micro-organism in a previously healthy 11-month-old girl. Our patient presented at the emergency department with an increasing scalp swelling for 2 weeks, and culture of the evacuated fluid yielded Streptococcus pneumoniae. Although she was born after vacuum delivery and a scalp swelling was noticed from the third day of life, this swelling disappeared completely at the age of 3 months. Parents were thoroughly questioned but we could not find out a new traumatic head event. We postulate that in our patient, a subgaleal hemorrhage developed after vacuum delivery and possibly infected 11 months later, presumably from hematogenous seeding of an acute otitis media. The patient recovered well after surgical drainage and antimicrobial therapy.

Opisthotonus and intrathecal treatment with baclofen (ITB) in children.

Opisthotonus is a relatively rare, but challenging neurological symptom of spasticity or dystonia that most often results from a dramatic event such as near-drowning. The classic treatment option for opisthotonus is the oral administration of medication such as benzodiazepines and baclofen. However, results with these medications are usually not very beneficial. Numerous studies however have shown that intrathecal treatment with baclofen (ITB) is an efficient and safe treatment for generalized therapy-resistant spasticity, even in children. In this retrospective study, we describe 11 children (mean age 9 years) with pronounced opisthotonus and quadriplegia caused by different types of acquired lesions who were treated with intrathecal baclofen. Results show that in addition to an expected decrease in muscle tonus, there was also a clear improvement in patient comfort and nursing. A remarkable weight gain was observed in most patients, even when calorie intake did not change. This increase in weight might be due to a reduction in energy expenditure as a result of the decrease in spasticity. Intrathecal treatment with baclofen should be considered in every child with opisthotonus.

Treatment of intraventricular hemorrhage with intraventricular administration of recombinant tissue plasminogen activator A clinical study of 18 cases.

OBJECTIVE: Intraventricular hemorrhage is associated with a very poor outcome. Simple external ventricular drainage alone has not resulted in a decline of mortality. The aim was to study the effect of direct intraventricular administration of recombinant tissue plasminogen activator (rtPA). PATIENTS AND METHODS: A retrospective series of eighteen adult patients with severe intraventricular hemorrhage, admitted to our university hospital, was studied for the effect of direct intraventricular administration of recombinant tissue plasminogen activator (rtPA). rtPA was administered in a dosage of 2mg. The injection was repeated at 12h intervals until serial CT scans showed a substantial reduction of intraventricular blood. RESULTS: The total of rtPA doses per patient ranged from 2 to 32mg. Seven out of 18 patients showed good neurological recovery, 4 died. Only one patient had a complication which could be directly attributed to the intraventricular thrombolytic therapy. CONCLUSION: We conclude that the procedure of intraventricular administration of a thrombolytic agent, i.e. rtPA, seems effective in lysis of the intraventricular hematoma and may, therefore, improve outcome.

Basilar artery thrombosis in a trauma patient. Case report and review of the literature.

Thrombotic disease of the vertebrobasilar circulation is associated with a poor prognosis. It may occur in trauma patients, especially those with neck injuries and even several months after the initial insult. We report on the case of a young polytrauma patient, victim of a traffic accident, with associated cervical and thoracic spinal injuries resulting in paraplegia. Consciousness was not impaired initially, but during transfer to our hospital he became suddenly unconscious. An occluded basilar artery was found on angiography, but unfortunately we were unable to reopen the vessel with thrombolytic therapy. This case again proves that lesions of the vertebro-basilar system must always be suspected in neck injuries. Even after minor whiplash injuries, fatal basilar thrombosis may occur. A review of all reported cases of traumatic basilar artery thrombosis is given and the use of thrombolytic therapy is discussed.

Natural history of petroclival meningiomas.

OBJECTIVE: For evaluation of the natural history of petroclival meningiomas, a cooperative retrospective study of 21 conservatively treated patients is reported. METHODS: All patients had petroclival meningiomas that were observed for at least 4 years, with regular clinical and radiological control examinations. The follow-up periods ranged from 48 to 120 months (mean, 82 mo; median, 85 mo). Functional evaluations were performed by using the Karnofsky index. Individual growth curves and rates were defined. RESULTS: Age and sex distributions and presenting symptoms were comparable to those of other studies. During follow-up monitoring, radiological tumor growth was observed in 76% of the cases. With 63% of the growing tumors, there was functional deterioration. We performed statistical analyses of demographic features, radiological findings, and functional deterioration. Severe functional deterioration was observed to be statistically significantly associated with infratentorial growth and increased growth rates. A change in the growth pattern often preceded functional deterioration. CONCLUSION: This study provides a better understanding of the natural course of petroclival meningiomas. The growth patterns of these tumors are unpredictable and variable. The exact factors influencing growth remain unclear. This study can contribute to the optimization of individual management of these tumors.