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1.
Cureus ; 16(1): e51440, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38298283

ABSTRACT

Primary spinal tumors such as schwannomas are uncommon causes of back pain that can be easily missed during the initial workup. Delayed diagnosis is associated with further neurological impairment. A 46-year-old man presented with a six-month history of progressive lower back pain and left leg radiculopathy. Previous treatments failed, including medications, physical therapy, acupuncture, and chiropractic manipulations. Examination revealed weakness (4/5) in left knee extension and ankle dorsiflexion. Magnetic resonance imaging (MRI) revealed a 2-cm intraspinal schwannoma at the L4 level with nerve root compression. The patient underwent laminectomy and gross total resection without any complications. The patient had near-complete symptomatic resolution six weeks postoperatively and returned to normal functioning. After four months of postoperative rehabilitation, the patient remained asymptomatic. This case reinforces the urgent need for early MRI in the presence of neurological deficits and other symptoms, despite normal radiographs. An increased suspicion of spinal tumors can prevent delays in diagnosis and minimize adverse outcomes. Multidisciplinary care optimizes the treatment of complex cases.

2.
Explore (NY) ; 2023 Nov 21.
Article in English | MEDLINE | ID: mdl-38008591

ABSTRACT

OBJECTIVE: We identified the potential role of electroacupuncture (EA) as an alternative therapy to traditional Chinese medicine (TCM) in a rare case of postoperative gastroparesis after gastric schwannoma (GS). CLINICAL PRESENTATION: A 31-year-old woman presented with impaired gastric emptying after gastrectomy for GS and was diagnosed with postoperative gastroparesis syndrome (PGS). The symptoms were slightly relieved after routine placement of the jejunal tube; however, symptoms such as dietary intolerance and impaired gastric emptying persisted. After the consultation, the patient agreed to undergo EA therapy. INTERVENTION AND RESULTS: The patient was able to tolerate oral intake after seven days of EA treatment, and the frequency and amount of food intake increased. The jejunal tube was removed at the outpatient follow-up two weeks after discharge, and the patient resumed a semi-liquid diet and was able to eat small amounts of rice. Reexamination of the upper digestive tract angiography showed that part of the contrast agent passed through the pyloric sinus, which showed improvement. CONCLUSION: EA stimulation increased tolerance to transoral feeding in patients with postoperative gastroparesis and facilitated the passage of contrast agents through the pyloric sinus. No adverse effects were observed during treatment, and the treatment was well accepted and tolerated by patients. A review article noted the benefits of acupuncture for gastrointestinal disorders but lacked high-quality evidence to support this.1 Therefore, the therapeutic role of EA needs to be further elucidated to provide high-quality evidence-based medical evidence for its clinical use.

3.
Zh Vopr Neirokhir Im N N Burdenko ; 87(1): 104-110, 2023.
Article in English, Russian | MEDLINE | ID: mdl-36763561

ABSTRACT

Trigeminal neuroma (TN) is a benign neoplasm arising from trigeminal nerve sheath. The tumor can grow from any part of the nerve from the root in posterior cranial fossa to peripheral extracranial branches. Symptoms of trigeminal neuroma are variable and usually depend on location and dimensions of tumor. We present a review devoted to the problem of TN. Surgery was the only possible option in patients with TN for a long time. However, radiotherapy became one of the options and sometimes alternative to surgical treatment since the late 1980s. Besides active management of patients with TN, follow-up with regular radiographic control of small asymptomatic tumors also seems to be reasonable. When evaluating treatment outcomes, physicians consider quality of life and return to previous work and activity in addition to resection quality, neurological impairment, relapse-free period and tumor growth control. However, assessment of these indicators after certain treatment is rare. Thus, it is difficult to determine treatment strategy with maximum ratio of effectiveness and quality of life. Therefore, optimization of TN treatment is currently an urgent problem that requires further study.


Subject(s)
Cranial Nerve Neoplasms , Neuroma , Trigeminal Neuralgia , Humans , Cranial Nerve Neoplasms/diagnosis , Cranial Nerve Neoplasms/pathology , Cranial Nerve Neoplasms/surgery , Quality of Life , Trigeminal Nerve/pathology , Trigeminal Nerve/surgery , Neuroma/diagnostic imaging , Neuroma/surgery , Treatment Outcome , Trigeminal Neuralgia/surgery
4.
J Nippon Med Sch ; 89(6): 629-633, 2023 Feb 01.
Article in English | MEDLINE | ID: mdl-34840221

ABSTRACT

Schwannoma is an uncommon benign tumor in the oral and maxillofacial region, and development of schwannoma in the lower lip is rare. Herein, we present the case of a 68-year-old woman who visited Nihon University Itabashi Hospital complaining of a painless mass in the lower lip. The lesion was surgically resected under local anesthesia. On histopathological examination, the resected specimen was a mixture of Antoni types A and B schwannoma. No recurrence has been seen over a postoperative follow-up period of 58 months. In the schwannoma of the lower lip, the mean tumor volume was compared for type A and the mixed type, which tended to be larger in the mixed type. No previous reports have described the relationship between the size of schwannoma in the lower lip and Antoni classification. Therefore, this report discusses the possibility of a relationship between tumor size and Antoni classification for schwannomas in the lower lip.


Subject(s)
Lip , Neurilemmoma , Female , Humans , Aged , Lip/pathology , Neurilemmoma/diagnostic imaging , Neurilemmoma/surgery , Anesthesia, Local
5.
Anticancer Res ; 43(1): 1-6, 2023 Jan.
Article in English | MEDLINE | ID: mdl-36585175

ABSTRACT

BACKGROUND/AIM: This study evaluated the effect of enrofloxacin antibiotic treatment on the ability of an attenuated Salmonella typhimurium (S. typhimurium) strain VNP20009 to control schwannoma growth in a preclinical mouse schwannoma tumor model. MATERIALS AND METHODS: The antitumor efficacy of VNP20009 intratumoral (i.t.) injection was assessed in a syngeneic mouse-NF2 schwannoma model, with and without subcutaneous (s.c.) injection of enrofloxacin beginning on day-1 or day-8 post-VNP20009 injection. S. typhimurium colonization was assessed in excised tumor samples following antibiotic treatment. RESULTS: I.t. injection of the VNP20009 significantly decreased the growth of schwannoma tumors in mice compared to PBS-treated controls. Treatment of mice with enrofloxacin on day-1 post-VNP20009 injection resulted in abrogation of VNP20009-mediated tumor growth control. In contrast, tumor growth in i.t. VNP20009-injected mice infused with enrofloxacin beginning on day 8 was significantly decreased compared to i.t. PBS-injected controls. Enrofloxacin significantly reduced the number of viable VNP20009 bacteria in excised tumor samples within one day of antibiotic infusion. Viable bacteria were either few or essentially eliminated at the end of the experiment in antibiotic-treated animals compared to VNP20009-only. CONCLUSION: Viable VNP20009 can persist for as long as 2.5 weeks following intratumoral injection of schwannoma, during which time tumor growth is retarded. Antibiotic treatment starting 1-day following i.t. VNP20009 abrogated bacterial tumor growth control, whereas initiation of antibiotics 8-days following i.t. VNP20009 was associated with control of tumor growth, albeit less than seen in animals unexposed to antibiotics.


Subject(s)
Neurilemmoma , Salmonella typhimurium , Mice , Animals , Enrofloxacin , Neurilemmoma/drug therapy , Anti-Bacterial Agents/pharmacology
6.
Front Pharmacol ; 13: 941854, 2022.
Article in English | MEDLINE | ID: mdl-36059985

ABSTRACT

Background: Neurofibromatosis type 2 (NF2) is a rare genetic syndrome that predisposes individuals to develop bilateral vestibular schwannomas (VSs) causing a high risk of life-threatening neurological complications. Traditional treatment options for NF2-associated VS usually cause neurological damage, and to date, there are no FDA-approved pharmacotherapies for NF2. The aim of this study was to evaluate the antitumor efficacy of Qu-Du-San-Jie (QDSJ) decoction, a traditional Chinese medicine formula, on NF2-associated VS and to investigate the potential underlying mechanisms. Methods: Ultra high-performance liquid chromatography-mass spectroscopy (UHPLC-MS) analysis was performed to identify the components of QDSJ and their targets. To determine the relationships between the putative targets of QDSJ and the differential genes of NF2-associated VS, the drug-disease crossover genes were screened using the UHPLC-MS data combined with our previous gene expression profiling data. The differentially expressed genes were imported into the STRING database to generate a PPI network. Differentially expressed gene targets and pathways were identified using GO and KEGG pathway enrichment analyses. The in vitro and in vivo drug efficacy of QDSJ decoction was tested using a patient-derived schwannoma cell line and a patient-derived xenograft mouse model, respectively. H&E staining, immunochemistry, and immunofluorescence staining were used to evaluate the cell proliferation and tumor vessels. Results: A total of 133 compounds were identified in QDSJ decoction using UHPLC-MS analysis. Network pharmacology showed that the regulation of necroptosis, apoptosis, cell cycle, angiogenesis, adherens junction, and neuroactive ligand-receptor interaction could be associated with the efficacy of QDSJ in treating NF2-associated VS. Treatment with QDSJ induced necrotic cell death and apoptosis of schwannoma cells in vitro and suppressed the tumor growth in vivo. Histopathological analysis revealed areas of cell necrosis and enlarged tumor blood vessels in the QDSJ-treated tumors. The numbers of cells positive for Cyclin D1 and Ki-67 were significantly reduced in QDSJ-treated tumors compared to control tumors. Immunofluorescence staining of CD31 and αSMA showed a decreased number and density of tumor vessels and normalized vessel structure in QDSJ-treated tumors. Conclusion: Our study demonstrates that QDSJ decoction shows significant antitumor activity against NF2-associated schwannoma and is a possible candidate for future clinical trials.

7.
Trials ; 22(1): 475, 2021 Jul 22.
Article in English | MEDLINE | ID: mdl-34294114

ABSTRACT

BACKGROUND: A previously performed phase III trial on 112 subjects investigating prophylactic nimodipine treatment in vestibular schwannoma (VS) surgery showed no clear beneficial effects on preservation of facial and cochlear nerve functions, though it should be considered that protection of facial nerve function was the primary outcome. However, the risk for postoperative hearing loss was halved in the nimodipine group compared to the control group (OR 0.49; 95% CI 0.18-1.30; p = 0.15). Accordingly, this phase III extension trial investigates the efficacy and safety of prophylactic nimodipine for hearing preservation in VS surgery. METHODS: This is a randomized, multi-center, two-armed, open-label phase III trial with blinded expert review and two-stage with interim analysis. Three hundred thirty-six adults with the indication for microsurgical removal of VS (Koos I-IV) and serviceable preoperative hearing (Gardner-Robertson scale (GR) 1-3) are assigned to either the therapy (intravenous nimodipine 1-2 mg/h from the day before surgery until the fifth postoperative day and standard of care) or the control group (surgery only and standard of care). The primary endpoint of the trial is postoperative cochlear nerve function measured before discharge according to GR 1-3 versus GR 4-5 (binary). Hearing function will be determined by pre- and postoperative audiometry with speech discrimination, which will be evaluated by a blinded expert reviewer. Furthermore, patient-reported outcomes using standardized questionnaires will be analyzed. DISCUSSION: Prophylactic parenteral nimodipine treatment may have a positive effect on hearing preservation in VS surgery and would improve patient's quality of life. Further secondary analyses are planned. Except for dose-depending hypotension, nimodipine is known as a safe drug. In the future, prophylactic nimodipine treatment may be recommended as a routine medication in VS surgery. VS can be considered as an ideal model for clinical evaluation of neuroprotection, since hearing outcome can be classified by well-recognized criteria. The beneficial effect of nimodipine may be transferable to other surgical procedures with nerves at risk and may have impact on basic research. TRIAL REGISTRATION: EudraCT 2019-002317-19, DRKS00019107 . 8th May 2020.


Subject(s)
Neuroma, Acoustic , Nimodipine , Adult , Clinical Trials, Phase III as Topic , Hearing , Humans , Multicenter Studies as Topic , Neuroma, Acoustic/diagnosis , Neuroma, Acoustic/surgery , Nimodipine/adverse effects , Postoperative Complications , Quality of Life , Randomized Controlled Trials as Topic , Retrospective Studies , Treatment Outcome
8.
Neurosurg Rev ; 44(3): 1729-1735, 2021 Jun.
Article in English | MEDLINE | ID: mdl-32827307

ABSTRACT

A 2016 published randomized multicenter phase III trial of prophylactic nimodipine treatment in vestibular schwannoma surgery showed only a tendency for higher hearing preservation rates in the treatment group. Gender was not included in statistical analysis at that time. A retrospective analysis of the trial considering gender, preoperative hearing, and nimodipine treatment was performed. The treatment group received parenteral nimodipine from the day before surgery until the seventh postoperative day. The control group was not treated prophylactically. Cochlear nerve function was determined by pure-tone audiometry with speech discrimination preoperatively, during in-patient care, and 1 year after surgery and classified according to the Gardner-Robertson grading scale (GR). Logistic regression analysis showed a statistically significant effect for higher hearing preservation rates (pre- and postoperative GR 1-4) in 40 men comparing the treatment (n = 21) and the control (n = 19) groups (p = 0.028), but not in 54 women comparing 27 women in both groups (p = 0.077). The results were also statistically significant for preservation of postoperative hearing with pre- and postoperative GR 1-3 (p = 0.024). There were no differences in tumor sizes between the treatment and the control groups in men, whereas statistically significant larger tumors were observed in the female treatment group compared with the female control group. Prophylactic nimodipine is safe, and an effect for hearing preservation in 40 men with preoperative hearing ability of GR 1-4 was shown in this retrospective investigation. The imbalance in tumor size with larger tumors in females of the treatment group may falsely suggest a gender-related effect. Further investigations are recommended to clarify whether gender has impact on nimodipine's efficacy.


Subject(s)
Hearing/drug effects , Neuroma, Acoustic/drug therapy , Neuroma, Acoustic/surgery , Nimodipine/administration & dosage , Pre-Exposure Prophylaxis/trends , Adult , Aged , Female , Hearing/physiology , Hearing Tests/trends , Humans , Male , Middle Aged , Neuroma, Acoustic/diagnosis , Prospective Studies , Radiosurgery/methods , Retrospective Studies , Single-Blind Method , Treatment Outcome
9.
Neuroimage Clin ; 24: 101969, 2019.
Article in English | MEDLINE | ID: mdl-31419767

ABSTRACT

OBJECTIVE: Our main aim was to investigate the blood oxygenation level dependent (BOLD) response to monaural and binaural speech- and non-speech stimuli as measured with fMRI in subjects with single-sided deafness and in normal hearing controls. We hypothesised that the response to monaural stimulation in both normal hearing subjects and persons with single-sided deafness would vary with the complexity and nature of the stimuli and the side of stimulation. DESIGN: Patients with left- and right single-sided deafness and controls with normal hearing receiving either binaural or monaural stimuli were tested using speech and non-speech auditory stimuli in an event-related fMRI experiment. STUDY SAMPLE: Twenty-two patients with single-sided deafness after treatment for vestibular schwannoma and 50 normal hearing controls. RESULTS: Normal hearing persons receiving right side monaural stimuli activate bilateral temporal regions. Activation following left side monaural stimulation is more right lateralized. Persons with single-sided deafness respond similarly to controls to monaural stimulation. Persons with right side single-sided deafness show activation of frontal cortical regions not seen in persons with left side single-sided deafness following speech stimuli. This is possibly related to increased effort and more frequently reported problems with communication. Right side single-sided deafness is related to increased activation of areas usually related to processing of degraded input, including the thalamus. CONCLUSION: Hemispheric dominance following monaural auditory stimulation is modulated by the spectral-temporal properties of the stimuli and by which ear is stimulated. Differences between patients with right- and left side deafness suggests that right side deafness is related to increased activation of areas involved in processing of degraded input.


Subject(s)
Brain/physiopathology , Functional Laterality/physiology , Hearing Loss, Unilateral/physiopathology , Acoustic Stimulation , Adult , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged
10.
Handb Clin Neurol ; 160: 437-449, 2019.
Article in English | MEDLINE | ID: mdl-31277867

ABSTRACT

The transduction process in the cochlea requires patent hair cells. Population responses that reflect this patency are the cochlear microphonic (CM) and summating potential (SP). They can be measured using electrocochleography (ECochG). The CM reflects the sound waveform in the form of outer hair cell (OHC) depolarization and hyperpolarization, and the SP reflects the average voltage difference of the OHC membrane potential for depolarization and hyperpolarization. The CM can be measured using ECochG or via the so-called otoacoustic emissions, using a sensitive microphone in the ear canal. Neural population responses are called the compound action potentials (CAPs), which by frequency selective masking can be decomposed into narrow-band action potentials (NAPs) reflecting CAPs evoked by activity from small cochlear regions. Presence of CM and absence of CAPs are the diagnostic hallmarks of auditory neuropathy. Increased and prolonged SPs are often found in Ménière's disease but are too often in the normal range to be diagnostic. When including NAP waveforms, Ménière's disease can be differentiated from vestibular schwannomas, which often feature overlapping symptoms such as dizziness, hearing loss, and tinnitus. The patency of the efferent system, particularly the olivocochlear bundle, can be tested using the suppressive effect of contralateral stimulation on the otoacoustic emission amplitude.


Subject(s)
Action Potentials/physiology , Cochlea/physiology , Cochlear Nerve/physiology , Hearing Loss/physiopathology , Hearing Tests/methods , Acoustic Stimulation/methods , Efferent Pathways/physiology , Hearing Loss/diagnosis , Humans
11.
Handb Clin Neurol ; 160: 451-464, 2019.
Article in English | MEDLINE | ID: mdl-31277868

ABSTRACT

The auditory brainstem response (ABR), consisting of five to six vertex-positive peaks with separation of about 0.8ms, is very sensitive to factors that affect conduction velocity and hence ABR wave latencies in the brainstem auditory pathways. In addition, disorders causing dissynchronization of neural activity result in an amplitude decrease or disappearance of ABR peaks. The opposite effects occur in the maturation process, which takes about 2 years postterm; here conduction velocity increases quickly to its adult value, but synaptic delays being sensitive to synchronous release of transmitter substance take considerably longer. In neurological disorders, those that cause dissynchrony, such as auditory neuropathy and vestibular schwannoma, Gaucher disease, and Krabbe disease, the (longer latency) ABR peaks are reduced or absent. Effects on neural conduction, resulting in increased ABR interwave latencies, are found in vestibular schwannomas, Bell's palsy, Duane retraction syndrome, Marcus Gunn ptosis, and various encephalomyopathies. These measures allow an assessment of the parts of the brainstem that are involved.


Subject(s)
Brain Stem/physiology , Evoked Potentials, Auditory, Brain Stem/physiology , Nervous System Diseases/physiopathology , Acoustic Stimulation/methods , Action Potentials/physiology , Brain Stem/physiopathology , Humans , Nervous System Diseases/diagnosis , Neuroma, Acoustic/diagnosis , Neuroma, Acoustic/physiopathology
12.
J Biol Chem ; 294(30): 11354-11368, 2019 07 26.
Article in English | MEDLINE | ID: mdl-31171721

ABSTRACT

Neurofibromatosis type 2 (NF2) is an autosomal-dominant disorder characterized by the development of bilateral vestibular schwannomas. The NF2 gene encodes the tumor suppressor merlin, and loss of merlin activity promotes tumorigenesis and causes NF2. Cellular redox signaling has been implicated in different stages of tumor development. Among reactive nitrogen species, peroxynitrite is the most powerful oxidant produced by cells. We recently showed that peroxynitrite-mediated tyrosine nitration down-regulates mitochondrial metabolism in tumor cells. However, whether peroxynitrite supports a metabolic shift that could be exploited for therapeutic development is unknown. Here, we show that vestibular schwannomas from NF2 patients and human, merlin-deficient (MD) Schwann cells have high levels of endogenous tyrosine nitration, indicating production of peroxynitrite. Furthermore, scavenging or inhibiting peroxynitrite formation significantly and selectively decreased survival of human and mouse MD-Schwann cells. Using multiple complementary methods, we also found that merlin deficiency leads to a reprogramming of energy metabolism characterized by a peroxynitrite-dependent decrease of oxidative phosphorylation and increased glycolysis and glutaminolysis. In MD-Schwann cells, scavenging of peroxynitrite increased mitochondrial oxygen consumption and membrane potential, mediated by the up-regulation of the levels and activity of mitochondrial complex IV. This increase in mitochondrial activity correlated with a decrease in the glycolytic rate and glutamine dependence. This is the first demonstration of a peroxynitrite-dependent reprogramming of energy metabolism in tumor cells. Oxidized proteins constitute a novel target for therapeutic development not only for the treatment of NF2 schwannomas but also other tumors in which peroxynitrite plays a regulatory role.


Subject(s)
Cell Survival/physiology , Genes, Tumor Suppressor , Peroxynitrous Acid/physiology , Schwann Cells/metabolism , Animals , Cells, Cultured , Glutamine/metabolism , Glycolysis , Humans , Mice , Mitochondria/metabolism , Neurofibromatosis 2/genetics , Oxidative Phosphorylation , Oxygen Consumption
13.
Arch Soc Esp Oftalmol (Engl Ed) ; 94(2): 100-104, 2019 Feb.
Article in English, Spanish | MEDLINE | ID: mdl-30025986

ABSTRACT

CASE REPORT: A patient with a history of surgical resection of an acoustic neuroma presented with involvement of both the left facial nerve and the left trigeminal nerve. She initially consulted for exposure keratitis, but two weeks later presented with an infectious keratitis. After taking the corneal sample, she presented with persistent epithelial defect, which did not respond to medical management. Topical insulin was indicated, and a decrease in the area of the lesion was seen in the following 5 days. A therapeutic contact lens was also placed at that time and finally, two weeks after the initiation of insulin, the epithelial defect completely closed. DISCUSSION: This was a complex case due to the confluence of facial paralysis, neurotrophic keratitis, and infectious keratitis, which finally had a successful outcome. Topical insulin can be an effective adjuvant therapy in cases of neurotrophic ulcers that do not respond to standard therapy.


Subject(s)
Facial Nerve Injuries/complications , Insulin/therapeutic use , Keratitis/etiology , Neuroma, Acoustic/surgery , Postoperative Complications/etiology , Trigeminal Nerve Injuries/complications , Administration, Ophthalmic , Anti-Bacterial Agents/therapeutic use , Combined Modality Therapy , Contact Lenses, Hydrophilic , Corneal Ulcer/drug therapy , Corneal Ulcer/etiology , Corneal Ulcer/therapy , Facial Paralysis/etiology , Female , Humans , Insulin/administration & dosage , Keratitis/drug therapy , Keratitis/microbiology , Keratitis/therapy , Middle Aged , Moxifloxacin/therapeutic use , Postoperative Complications/drug therapy , Staphylococcal Infections/drug therapy , Staphylococcal Infections/etiology , Vancomycin/therapeutic use
14.
Am J Health Syst Pharm ; 75(18): 1369-1377, 2018 Sep 15.
Article in English | MEDLINE | ID: mdl-30190294

ABSTRACT

PURPOSE: The uses of nimodipine for otolaryngic indications are reviewed, and recommendations for its use in clinical practice are provided. SUMMARY: Nimodipine is currently indicated for the improvement of neurologic outcomes in adult patients with aneurysmal subarachnoid hemorrhage (aSAH). However, other oral and i.v. calcium channel blockers have not exhibited the same beneficial effects in patients with aSAH, leading clinicians to believe that nimodipine possesses unique neuroprotective effects in addition to its calcium channel-blocking and vasodilatory properties. Consequently, clinical investigations of nimodipine have been conducted for cochlear and facial nerve preservation after vestibular schwannoma (VS) surgery, symptomatic management of Ménière's disease and peripheral vertigo, and recovery of vocal cord paralysis after laryngeal nerve injury. Three prospective randomized studies have investigated nimodipine for hearing and/or nerve preservation in patients undergoing VS resection, the results of which have suggested a potential benefit of initiating nimodipine during the perioperative period. Several studies of Ménière's disease and/or peripheral vertigo have reported improved symptom control with nimodipine. For vocal fold paralysis associated with recurrent laryngeal nerve (RLN) injury, nimodipine may increase the recovery rate based on the results of 1 nonrandomized prospective study that used nimodipine in a protocolized manner. One small pilot study found that nimodipine improved facial nerve function after maxillofacial surgery. CONCLUSION: Due to its proposed vasoactive and neuroprotective effects, nimodipine may play a role in the treatment of a number of otolaryngic pathologies including VS, Ménière's disease, peripheral vertigo, RLN injury, and facial weakness after maxillofacial surgery. Small studies have shown improved symptom control and recovery after surgery. Since all of the aforementioned indications are still considered off label, clinicians and patients should collaboratively assess the risks and benefits before initiating treatment.


Subject(s)
Calcium Channel Blockers/therapeutic use , Neuroprotective Agents/therapeutic use , Nimodipine/therapeutic use , Otorhinolaryngologic Diseases/drug therapy , Humans , Meniere Disease/drug therapy , Vasodilator Agents/therapeutic use , Vertigo/drug therapy
15.
Scand J Pain ; 18(1): 125-127, 2018 01 26.
Article in English | MEDLINE | ID: mdl-29794280

ABSTRACT

Schwannoma is a common neoplasm in the peripheral and central nervous systems. Sciatic nerve schwanommas are rare. We report the case of a 50-year-old woman who was referred for treatment of persistent neuropathic pain in the left lower limb after resection of a schwannoma on the left S1 nerve root. The patient's history goes back when she was 27 years old and started to have electric-like pain in her lower left limb upon intercourse. Examination revealed a left ovarian cyst which was surgically removed. Her pain persisted despite taking nonsteroidal anti inflammatory drugs (NSAIDs). Several years later a schwannoma on the left S1 nerve root was detected. The patient had surgical excision of the left S1 nerve root at the plexus along with the schwannoma. Following the surgery, she experienced pain upon sitting and touch, and had a limp in her left leg. She was prescribed NSAIDs, antidepressant and pregabalin. Despite the pharmacological treatment, the patient had persistent mild pain. Upon physical examination, the incision from her previous surgery was 4 cm away from the sacral midline and parallel to S1 and S2. The length of the incision was 3 cm. The patient had severe allodynia upon palpation at the area between S1 and L5 and the visual analog scale (VAS) score increased from 3 to 10. She had severe pain at rest and movement. Her neurologic exam revealed that the left lower extremity motor power showed mild weakness in the leg abduction, foot eversion, plantar and toes flexion, and in the hip extension. The sensory exam showed severe reduction in pinprick and temperature sensation in the lateral aspect of foot, lower leg and dorsolateral thigh and buttocks. Nerve stimulator guided injection was performed at the pain trigger point being 1 cm above the midline of the incision. Upon nerve stimulation the contraction of the gluteal muscle was observed. Then, 20 mL of the anesthetic mixture were injected. The patient had immediate pain relief after the block (VAS 1/10). She remained pain free for 15 days after which pain reappeared but with less severity (3/10). Repetitive sciatic nerve block was performed in a progressive manner and was shown to be effective in managing neuropathic pain.


Subject(s)
Nerve Block , Neuralgia/drug therapy , Female , Humans , Lower Extremity , Middle Aged , Nerve Block/methods , Neurilemmoma/surgery , Pain Management/methods , Pain, Postoperative/drug therapy , Peripheral Nervous System Neoplasms/surgery , Sciatic Nerve , Sciatic Neuropathy/surgery
16.
Am J Med Genet A ; 176(5): 1258-1269, 2018 05.
Article in English | MEDLINE | ID: mdl-29681099

ABSTRACT

Organized and hosted by the Children's Tumor Foundation (CTF), the Neurofibromatosis (NF) conference is the premier annual gathering for clinicians and researchers interested in neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN). The 2016 edition constituted a blend of clinical and basic aspects of NF research that helped in clarifying different advances in the field. The incorporation of next generation sequencing is changing the way genetic diagnostics is performed for NF and related disorders, providing solutions to problems like genetic heterogeneity, overlapping clinical manifestations, or the presence of mosaicism. The transformation from plexiform neurofibroma (PNF) to malignant peripheral nerve sheath tumor (MPNST) is being clarified, along with new management and treatments for benign and premalignant tumors. Promising new cellular and in vivo models for understanding the musculoskeletal abnormalities in NF1, the development of NF2 or SWN associated schwannomas, and clarifying the cells that give rise to NF1-associated optic pathway glioma were presented. The interaction of neurofibromin and SPRED1 was described comprehensively, providing functional insight that will help in the interpretation of pathogenicity of certain missense variants identified in NF1 and Legius syndrome patients. Novel promising imaging techniques are being developed, as well as new integrative and holistic management models for patients that take into account psychological, social, and biological factors. Importantly, new therapeutic approaches for schwannomas, meningiomas, ependymomas, PNF, and MPNST are being pursued. This report highlights the major advances that were presented at the 2016 CTF NF conference.


Subject(s)
Neurilemmoma/diagnosis , Neurilemmoma/etiology , Neurofibromatoses/diagnosis , Neurofibromatoses/etiology , Neurofibromatosis 1/diagnosis , Neurofibromatosis 1/etiology , Neurofibromatosis 2/diagnosis , Neurofibromatosis 2/etiology , Skin Neoplasms/diagnosis , Skin Neoplasms/etiology , Animals , Disease Management , Disease Models, Animal , Genetic Association Studies , Genetic Predisposition to Disease , Humans , Molecular Diagnostic Techniques , Neurilemmoma/therapy , Neurofibromatoses/therapy , Neurofibromatosis 1/therapy , Neurofibromatosis 2/therapy , Skin Neoplasms/therapy , Translational Research, Biomedical
17.
Adv Clin Exp Med ; 27(3): 335-341, 2018 Mar.
Article in English | MEDLINE | ID: mdl-29533538

ABSTRACT

BACKGROUND: Air-conducted and skull-tap cervical vestibular evoked myogenic potentials (AC-cVEMP and Tap-cVEMP) have been shown to be very promising tools in clinical practice. They are noninvasive, easy to obtain and - importantly - they require little time and the cost of the instruments is low. OBJECTIVES: The aim of this study was to evaluate the usefulness of the combined use of ACand Tap-cVEMPs as a diagnostic tool for advanced assessment of vestibular schwannoma in determining tumor origin, and to investigate whether the results are helpful for a surgeon as an additional source of information about the tumor before surgery. MATERIAL AND METHODS: ACand Tap-cVEMPs were acquired (with EMG-based biofeedback) from the sternocleidomastoid muscles (SCM) of 30 vestibular schwannoma patients just before surgery. The results were compared to the surgical information about nerve bundle involvement in the tumor and the size of the tumor obtained from magnetic resonance imaging (MRI). RESULTS: On the tumor side, abnormal corrected amplitude asymmetry ratios were detected in 73.33% of the patients, abnormalities in P1-latencies in 70% of the patients, and both in 90% of the patients. The cervical vestibular evoked myogenic potential (cVEMP) results indicated the affected nerve division to be the inferior in 23.33% of the patients, the superior in 20% of the patients, and both in 46.67% of the patients. No cVEMP abnormalities were found in 10% of cases. The combined results of both ACand Tap-cVEMP were significantly compatible with the surgical information about the tumor origin. The number of abnormalities was significantly correlated with the tumor size. CONCLUSIONS: The information provided by the combined application of ACand Tap-cVEMPs might be useful for a surgeon in presurgical planning, providing more detailed information about the tumor and the affected nerve division in the internal auditory canal. It is not a diagnostic replacement for MRI in vestibular schwannoma patients; however, in our opinion, ACand Tap-cVEMPs may serve as additional sources of information about the tumor before the surgery.


Subject(s)
Neck Muscles , Neuroma, Acoustic/diagnosis , Vestibular Evoked Myogenic Potentials/physiology , Acoustic Stimulation , Audiometry, Pure-Tone , Humans , Magnetic Resonance Imaging , Neuroma, Acoustic/pathology , Neuroma, Acoustic/physiopathology , Skull
18.
Free Radic Biol Med ; 115: 43-56, 2018 02 01.
Article in English | MEDLINE | ID: mdl-29138018

ABSTRACT

Vestibular schwannoma (VS), although a benign intracranial tumor, causes morbidities by brainstem compression. Since chemotherapy is not very effective in most Nf2-negative schwannomas, surgical removal or radiation therapy is required. However, depending on the size and site of the tumor, these approaches may cause loss of auditory or vestibular functions, and severely decrease the post-surgical wellbeing. Here, we examined the feasibility of cold atmospheric pressure plasma (CAP) as an intra-operative adjuvant treatment for VS after surgery. Cell death was efficiently induced in both human HEI-193 and mouse SC4 VS cell lines upon exposure to CAP for seven minutes. Interestingly, both apoptosis and necroptosis were simultaneously induced by CAP treatment, and cell death was not completely inhibited by pan-caspase and receptor-interacting serine/threonine-protein kinase 1 (RIK1) inhibitors. Upon CAP exposure, cell death phenotype was similarly observed in patient-derived primary VS cells and tumor mass. In addition, CAP exposure after the surgical removal of primary tumor efficiently inhibited tumor recurrence in SC4-grafted mouse models. Collectively, these results strongly suggest that CAP should be developed as an efficient adjuvant treatment for VS after surgery to eliminate the possible remnant tumor cells, and to minimize the surgical area in the brain for post-surgical wellbeing.


Subject(s)
Adjuvants, Anesthesia , Atmospheric Pressure , Brain Neoplasms/therapy , Brain Stem/physiology , Cell Death/radiation effects , Electric Stimulation Therapy , Neuroma, Acoustic/therapy , Animals , Brain Neoplasms/surgery , Brain Stem/surgery , Cell Line, Tumor , Disease Models, Animal , Feasibility Studies , Humans , Intraoperative Care , Mice , Neuroma, Acoustic/surgery , Neurosurgical Procedures , Recurrence , Signal Transduction
19.
Article in Russian | MEDLINE | ID: mdl-30721216

ABSTRACT

INTRODUCTION: Radiation therapy of small vestibular schwannomas is quite often used as an effective alternative to surgical treatment. At the same time, 2-10% of patients are detected with radioresistant tumors progressing to varying degrees, which is associated with continued tumor growth. In these cases, a decision on surgical resection or re-irradiation of the tumor is made depending on the neurological symptoms, patient's somatic status, and neuroimaging data. Surgical outcomes and intraoperative findings in pre-irradiated patients have been poorly represented in the literature, for which reason we decided to conduct this study. The paper presents a series of patients with vestibular schwannomas who underwent surgical removal of the tumor after radiotherapy. MATERIAL AND METHODS: A total of 39 patients with vestibular schwannomas after radiotherapy underwent surgery at the Burdenko Neurosurgical Institute in 2007-2017. Of these, 22 patients had a tumor removed after a previously performed combined surgical and radiotherapy treatment (group I), and 17 patients underwent tumor resection after previous radiological treatment (group II). The surgical outcomes were studied depending on various factors, and an analysis of the morphological changes in vestibular schwannomas after radiological treatment was carried out. RESULTS: In group I, the tumor was resected totally in 18% of patients, almost totally in 5% of patients, subtotally in 68% of patients, and partially in 9% of patients. In group II, the tumor was resected totally in 6% of patients, almost totally in 12% of patients, subtotally in 76% of patients, and partially in 6% of patients. We found that post-radiation changes in patients undergoing surgery led to an increase in the response of neurovascular structures to surgical intervention, development of pronounced fibrosis around tumors, and changes in the structure of tumors that became more solid. As a result, surgical morbidity increased, and the patient's quality of life after surgery deteriorated.


Subject(s)
Neuroma, Acoustic , Humans , Neuroma, Acoustic/therapy , Quality of Life , Radiography , Retrospective Studies , Treatment Outcome
20.
Neuropsychologia ; 102: 135-143, 2017 Jul 28.
Article in English | MEDLINE | ID: mdl-28623107

ABSTRACT

We investigated speech recognition in noise in subjects with mild to profound levels of unilateral hearing loss. Thirty-five adults were evaluated using an adaptive signal-to-noise ratio (SNR50) sentence recognition threshold test in three spatial configurations. The results revealed a significant correlation between pure-tone average audiometric thresholds in the poorer ear and SNR thresholds in the two conditions where speech and noise were spatially separated: dichotic - with speech presented to the poorer ear and reverse dichotic - with speech presented to the better ear. This first result suggested that standard pure-tone air-conduction thresholds can be a reliable predictor of speech recognition in noise for binaural conditions. However, a subgroup of 14 subjects was found to have poorer-than-expected speech recognition scores, especially in the reverse dichotic listening condition. In this subgroup 9 subjects had been diagnosed with vestibular schwannoma at stage III or IV likely affecting the lower brainstem function. These subjects showed SNR thresholds in the reverse dichotic condition on average 4dB poorer (higher) than for the other 21 normally-performing subjects. For the 7 of 9 subjects whose vestibular schwannoma was removed, the deficit was no longer apparent on average 5 months following the surgical procedure. These results suggest that following unilateral hearing loss the capacity to use monaural spectral information is supported by the lower brainstem.


Subject(s)
Auditory Threshold/physiology , Brain Stem/physiopathology , Functional Laterality/physiology , Hearing Loss/pathology , Hearing Loss/physiopathology , Neuronal Plasticity/physiology , Speech Perception/physiology , Acoustic Stimulation , Adult , Aged , Audiometry, Pure-Tone , Female , Humans , Male , Middle Aged , Noise
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