Cord Herniation through the Site of Undiagnosed Thoracic Dermoid Tumour during Spinal Anaesthesia; Report of a Case and Describing Ways to Avoid.

Spinal anaesthesia (SA) is one of the most prevalent types of anaesthetic procedures. There are very few reports of cord herniation through the site of spinal canal stenosis due to tumour. A 33-year-old female presented with acute paraparesis after spinal anaesthesia for caesarean section. Magnetic resonance imaging (MRI) revealed an intradural mass from posterior of T6 to T8-T9 interface. We operated the patient and after laminectomy of T6 to T9, dermoid tumour containing hairs was totally resected and cord was completely decompressed. After 6 months, the patient is without any neurological deficit. Puncturing the dura with cerebrospinal fluid (CSF) in the presence of an extramedullary mass could cause cord herniation through the blockade. In these cases, awareness about related signs even in absence of symptoms or complaints could help us to prevent post-SA neurological deficit.

Clinical Presentation of Foramen Magnum Meningioma Masqueraded by Carpal Tunnel Syndrome: A Report of 2 Cases.

CASE: Meningioma is the second most common intracranial tumor. We present 2 cases of foramen magnum meningioma (FMM) that was first operated on with the diagnosis of carpal tunnel syndrome (CTS). CONCLUSION: During the diagnostic assessment of CTS and recalcitrant CTS, a more proximal etiology of nerve compression should be considered, including FMM. If a more proximal cause of nerve dysfunction is suspected, cervical spine magnetic resonance imaging may be beneficial to evaluate a patient for spinal etiology.

Long term follow-up results of deep brain stimulation of the Globus pallidus interna in pediatric patients with DYT1-positive dystonia.

OBJECTIVES: Primary generalized dystonia (PGD) due to heterozygous torsin 1A (TOR1A) gene mutation (DYT1) is a childhood onset dystonia with rapid deterioration of symptoms, leading to severe disability in adolescence. Globus pallidus interna deep brain stimulation (GPi-DBS) has been shown to provide significant improvement in these cases. METHODS: This was a retrospective study of TOR1A mutation positive dystonia patients, conducted at a university hospital from 2006 to 2018. Burke-Fahn-Marsden Dystonia Rating Scale (BFM-DRS) was used to evaluate dystonia severity before and after surgery. Emergence of postsurgical parkinsonian symptoms was evaluated using the Unified Parkinson Disease Rating Scale (UPDRS) part III. Montreal Cognitive Assessment (MOCA) was applied to assess cognitive dysfunction. SPSS version 18 was used for data analysis. RESULTS: Eleven patients entered for analysis with an average age of 22.36 (±3.35) years (range: 18-28). Seven patients (63.6 %) were female. Mean follow-up period was 8.72 (±0.87). Difference between baseline and most recent BFM scores was significant (disability: 10.5 ±4.52 versus 2.09 (±3.20), P: 0.001; severity: 48.45 (±17.88) versus 9.36 (±10.47), P<0.001). The mean MOCA and UPDRS III scores after 7-9 years of DBS were 27.18 (±2.99), and 6.09 (±4.15), respectively. CONCLUSION: Our experience confirms that GPi-DBS in pediatric patients with DYT1 dystonia is overall successful, with significant and long-lasting positive effects on motor and cognitive functions. There was no prominent side effect in long-term follow up.

An Experience with Frame-Based Stereotactic Biopsy of Posterior Fossa Lesions via Transcerebellar Route.

BACKGROUND: Tissue diagnosis for lesions in the posterior fossa, such as the brainstem, cerebellar peduncle, and cerebellum, is an important determinant of the next treatment option. Herein, we present our 10-year experience with magnetic resonance imaging (MRI)-guided frame-based stereotactic biopsy for 39 patients with posterior fossa lesions, the largest case series in this matter. METHODS: We performed a retrospective cross-sectional study on all patients with posterior fossa lesion admitted to 2 referral centers between 2006 and 2016. We used Leksell Frame G for stereotactic biopsy of all patients. MRI systems of both hospitals were 1.5 T. RESULTS: We performed analysis on the 39 cases (21 men and 18 women). Age of the patients ranged between 9 and 73 years (mean, 35.4 ± 15.7 years). Localization success rate was 100%. For 38 patients (97.4%), tissue sample size was enough for tissue diagnosis. For 1 case, it was insufficient and nondiagnostic. In this series, we had no surgery-related complications. CONCLUSIONS: We present the largest reported series of MRI-guided frame-based stereotactic biopsy of the posterior fossa lesions via a transcerebellar route. We prefer oblique positioning of the frame on the skull and use a transcerebellar route to reduce surgical complications and achieve a greater localization success rate.

Parkinsonism management issues in a patient with exhausted subthalamic deep brain stimulation battery, stricken by severe acute respiratory syndrome coronavirus 2.

Background: Nowadays, many neurological conditions, including Parkinson's disease (PD), are treated with deep brain stimulation (DBS). Life-threatening consequences can occur from DBS hardware failure or sudden implantable pulse generator (IPG) battery depletion. This issue may potentially worsen in concomitance with medical or infectious conditions, requiring stronger emergency management. Methods: We present here a 58 year-old PD patient with DBS, whose IPG replacement surgery was complicated by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and we report management of this patient along with recommendations for patients with similar situation. Results: As the newly-emerged coronavirus disease 2019 (COVID-19) is now announced to be pandemic, new protocols and specific measures for each individual group of patients with chronic diseases seem obligatory. Regarding our recent experience with a patient suffering from PD, on DBS treatment, who needed hospitalization, we felt useful to share our experience as a recommended protocol for similar patients in the time of current pandemic. Conclusion: Close monitoring of laboratory and clinical signs should be warranted in patients with PD awaiting IPG replacement in order to be prepared in these novel conditions that may precipitate an akinetic crisis/dystonic storm and to prevent life-threatening complications during the current pandemic.

A Case Study on Aneurysmal Bone Cyst of T2 Vertebral Body in a 7-Year-Old Girl: Anterior and Posterior Surgical Approach for Tumor Resection and Spinal Reconstruction.

Aneurysmal bone cyst (ABC) is defined as a benign lesion of bone containing blood-filled channels divided by soft-tissue septa and mostly diagnosed in children and adolescents. In the present study, the case of a 7-year-old girl with spastic paraparesia and left foot drop was investigated. Spinal imaging revealed a large lytic mass in the posterior elements of the T2 vertebral body, which resulted in the partial collapse of the T2 vertebral body, a T1 to T3 dislocation, and a spinal sagittal imbalance. The patient underwent a two-stage surgical procedure including an anterior T2 corpectomy, T1/T2 and T2/T3 discectomy, anterior instrumentation, posterior total tumor resection, and posterior fixation with laminar hooks. Postoperative imaging demonstrated cord decompression and good spinal alignment. Although a spinal ABC is a rare entity, it can occur in any segment of the spinal column and cause cord compression and spinal instability. Thus, proper treatment planning is crucial for total tumor resection to avoid tumor recurrence and correct spinal imbalance.

Anteromedial GPi deep brain stimulation in Tourette syndrome: The first case series from Iran.

OBJECTIVES: Tourette syndrome (TS) is a neuropsychiatric disorder characterized by childhood onset motor and phonic tics. In refractory cases, deep brain stimulation (DBS) with different targets including anteromedial Globus pallidus (AM-GPi) looks promising. PATIENTS AND METHODS: Patients with TS diagnosed according to DSM-IV TR criteria with severe medication-recalcitrant disease referred to our DBS clinic, were recruited for this study. They underwent bilateral AM-GPi DBS with Model 3389, Medtronic electrodes. Patients were assessed using Yale Global Tic Severity Scale (YGTSS) and Gilles de la Touretts syndrome-quality of life (GTS-QOL) questionnaire before and one year after DBS. RESULTS: Six patients (four men and two women) with severe medication-recalcitrant TS, mean age of 26.33 ±â€¯7.25 years fulfilled the follow up visits. All patients revealed significant improvement in tics severity one year after surgery. Based on YGTSS, total tic severity score decreased from 75.66 ±â€¯16.54 to 28.33 ±â€¯13.95, P-value:0.005. Quality of life improved significantly after DBS (26.66 ±â€¯20.65 before and 70.00 ±â€¯17.88 one year after surgery, P-value:0.02). CONCLUSIONS: Results of our study in accordance to previous ones suggest AM-GPi DBS as an effective and well-tolerated therapeutic modality for patients with medication refractory TS.

Outcome of subthalamic nucleus deep brain stimulation on long-term motor function of patients with advanced Parkinson disease.

Background: The objective of our study was to assess Unified Parkinson Disease Rating Scale (UPDRS) score in Parkinson disease (PD) patients who underwent subthalamic nucleus (STN) deep brain stimulation (DBS) 6 years after their surgery and to compare their UPDRS score 6 years after DBS with their score before surgery and 6 months after their operation. Methods: In this cross sectional study which was carried out at Neurology Department of Rasool-e Akram Hospital, Tehran, Iran, affiliated to Iran University of Medical Sciences between 2008 and 2014, 37 patients with advanced PD were enrolled using non-randomized sampling method. All of the patients underwent STN DBS surgery and one patient died before being discharged, therefore; we started our study with 36 patients. The UPDRS III total score at preoperative state, 6-month follow-up and 6-year follow-up state were compared using repeated-measure analysis of variance. Results: Thirty-seven patients (26 men and 10 women) with mean age of 50 ± 3 ranging from 32 to 72 years underwent STN DBS surgery. All patients were suffering from advanced PD with mean period of 11.3 ± 1.9 years. All patients except one were followed up for six months. And 14 patients (8 men and 6 women) were included in a six-year follow-up. The UPDRS score measurements before surgery, at 6-month follow-up and 6-year follow-up were 18.22 ± 2.88, 12.80 ± 3.14, 25.0 ± 11.8, respectively. Significant increase in UPDRS score was observed between the preoperative and six-year follow-up period (P < 0.001). Conclusion: In conclusion, this study suggests that total UPDRS score will increase at 5 years following STN DBS and also showed that resting tremor, one of UPDRS sub-scores, will improve over time and the benefit of DBS will be persistent even after 6 years.

The Role of Protein Kinase B Signaling Pathway in Anti-Cancer Effect of Rolipram on Glioblastoma Multiforme: An In Vitro Study.

INTRODUCTION: The mechanism of putative cytotoxicity of 4-[3-(cyclopentyloxy)-4-methoxyphenyl]-2-pyrrolidone (rolipram), a specific phosphodiesterase-4 (PDE4) inhibitor, on glioblastoma multiforme (GBM) is almost unknown. This study aimed to investigate the role of protein kinase B (Akt) pathway in the cytotoxic effect of rolipram on human GBM U87 MG cell line and Tumor-Initiating Cells (TICs) isolated from patient's GBM specimen. METHODS: TICs were characterized by using flow cytometry and quantitative real-time PCR. The cells were treated with rolipram at inhibitory concentration of 50% (IC50) in the presence or absence of SC79 (4µg/mL), a specific AKT activator, for 48 hours. The cell viability and apoptosis were measured by MTT assay and TUNEL staining, respectively. The relative expression of Phospho-Akt (Ser473), matrix metalloproteinase 2 (MMP2), and vascular endothelial growth factor A (VEGFA) were detected using Western blotting. RESULTS: The findings showed that rolipram could suppress cell viability in both U87MG and TICs, dose-dependently. Interestingly, the rolipram-induced cytotoxicity was significantly reduced in the presence of SC79. Nevertheless, in rolipram-treated cells, the pretreatment with SC79 significantly led to increase in U87 MG cells and TICs apoptosis and decrease in viability of U87 MG cells but not TICs relative to corresponding control. In U87 MG and TICs, rolipram-induced reduction of Phospho-Akt (Ser473) and MMP2 levels were significantly suppressed by SC79. CONCLUSION: There is a cell type-specific mechanism of anti-proliferative action of rolipram on GBM cells. The reduction of intracellular level of MMP2 but not VEGFA by rolipram is conducted through the inhibition of Akt signal. Rolipram-induced apoptosis is mediated via Akt dependent/independent mechanisms.

Parkinsonism: a rare manifestation of craniopharyngioma.

Craniopharyngioma is a non-glial, non-malignant intracranial tumor of ectodermal origin, which arises from a remnant of Rathke's pouch. This tumor accounts for 5.6 to 13% of intracranial tumors in children. This paper discusses a case of craniopharyngioma in a five-year-old boy. An MRI scan of his brain showed a huge sella and supra sella cystic-solid lesion that had invaded the prepontine and interpeduncular cisterns, filling of 3rd ventricle and hydrocephalus. The patient operated via interhemispheric subfrontal through lamina terminalis and the tumor dissected from all part of brain stem and total resection achieved. After surgery Parkinsonism was worse for 3 days and levodopa started for 3 days. Parkinsonism was gone and after one week levodopa discontinued. This case practically implied that decompression of mass effect of tumor on brain stem and short-term management with levodopa can improve Parkinsonism due to midline compressive brain tumors without basal ganglia involvement.

Extra-axial medulloblastoma in cerebello-pontine angle: A report of a rare case with literature review.

Medulloblastoma is quite uncommon in the adult population and even rarer in extra-axial site in cerebello-pontine (CP) angle. In this report, a 23-year-old male patient with a two month history of deafness, nausea, vomiting and ataxia is presented. Clinical and radiological findings demonstrated a heterogeneously enhanced extra-axial lesion in the right CP angle. Total excision was performed and the histopathological features of medulloblastoma were confirmed. After surgery, the patient had no neurological deficit and the audiometric findings were improved. In addition, he underwent adjuant radiotherapy and no sign of metastatic mass was observed in follow-up spinal cord MRI. Although extremely rare, medulloblastoma must be considered in the differential diagnosis of extra-axial CP angle lesions.

Deep brain stimulation of globus pallidus internus for DYT1 positive primary generalized dystonia.

BACKGROUND: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is recommended as a promising technique for the management of the primary generalized dystonia (PGD) with DYT1 gene mutation. We present the first report of DBS results in Iranian patients with DYT1 positive PGD. METHODS: Nine patients who suffered from severely disabling DYT1 positive PGD consecutively were recruited for the study between 2008 and 2010. The patients underwent bilateral deep brain stimulation of the GPi in a single procedure. The mean follow up duration was 8.8 ± 2.2 months. The efficacy of the intervention was evaluated by comparing pre- and post operative scores of patients with Burke-Fahn-Marsden's dystonia Scale (BFMDS). Statistical analysis was performed using SPSS 11.0 software. RESULTS: Of 9 patients six were female with the mean age of 15.2 ± 5.5 years old (range: 8- 25 years old). The mean for Burke-Fahn-Marsden's Dystonia Rating Scale (BFMDRS) score was 47.22 ± 14.1 before surgery and 12.3 ± 8.2 after follow up, which significantly improved (P=0.0001).The mean stimulation parameters at the last visit were at a frequency of 152.2 ± 32.4 Hz (range 130- 230 Hz), a voltage of 2.6 ± 0.7 V (range 1.1- 4), and a pulse width of 60 µsec. No complication was observed during follow up. CONCLUSION: Bilateral DBS of the GPi has an encouraging result for the management of DYT1 positive PGD and is recommended as a safe technique for the treatment of these patients. Shorter pulse width in stimulation parameters is suggested for DYT1 dystonia patients.

Pallidal deep brain stimulation for the treatment of DYT6 dystonia: a case report and review of literature.

Little is known about the results of pallidal deep brain stimulation (DBS) in DYT6 dystonia. This will be the first report of DYT6 dystonia treated with pallidal DBS from Iran. A 21 years old male patient with DYT6 dystonia underwent bilateral deep brain stimulation. The target of DBS was the sensorimotor region of the posteroventral globus pallidus internus (GPi). DBS parameters included an amplitude of 2.7 V, frequency of 160 Hz, and pulse width of 90 µs which were adjusted according to the patient's response 12 months after surgery. Treatment outcome was measured by the patient's Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) score. Before surgery, the patient's BFMDRS score was 32. However, BFMDRS score reduced to 7 at one year follow up after surgery (78% improvement of total score). Dystonic symptoms of extremities and mouth completely resolved. Also speech and swallowing function significantly improved. Although previous observations reported a poor to moderate response in speech, we found DBS as an effective treatment not only for dystonic features, but also for speech improvement of DYT6 dystonia.