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Background and Aims: This study aimed to identify the clinical characteristics and electrodiagnostic subtypes of Guillain-Barré syndrome (GBS) in Istanbul., Methods: Patients with GBS were prospectively recruited between April 2019 and March 2022 and two electrodiagnostic examinations were performed on each patient. The criteria of Ho et al., Hadden et al., Rajabally et al., and Uncini et al. were compared for the differentiation of demyelinating and axonal subtypes, and their relations with anti-ganglioside antibodies were analyzed., Results: One hundred seventy-seven patients were included, 69 before the coronavirus disease 2019 pandemic (April 2019-February 2020) and 108 during the pandemic (March 2020-March 2022), without substantial changes in monthly frequencies. As compared with the criteria of Uncini et al., demyelinating GBS subtype diagnosis was more frequent according to the Ho et al. and Hadden et al. criteria (95/162, 58.6% vs. 110/174, 63.2% and 121/174, 69.5%, respectively), and less frequent according to Rajabally et al.'s criteria (76/174, 43.7%). Fourteen patients' diagnoses made using Rajabally et al.'s criteria were shifted to the other subtype with the second electrodiagnostic examination. Of the 106 analyzed patients, 22 had immunoglobulin G anti-ganglioside antibodies (14 with the axonal subtype). They had less frequent sensory symptoms (54.5% vs. 83.1%, p = 0.009), a more frequent history of previous gastroenteritis (54.5% vs. 22.9%, p = 0.007), and a more severe disease as compared with those without antibodies., Interpretation: Serial electrodiagnostic examinations are more helpful for accurate subtype diagnosis of GBS because of the dynamic pathophysiology of the disease. We observed no significant increase in GBS frequency during the pandemic in this metropolis., (© 2024 Peripheral Nerve Society.)

Background: Despite the primary myelin-related pathophysiology, small fiber neuropathy (SFN) and axonal degeneration are also considered to be involved and associated with disabling symptoms and impaired quality of life in chronic inflammatory demyelinating polyneuropathy (CIDP). Demonstration of SFN usually requires complex or invasive investigations., Objects: In vivo corneal confocal microscopy (IVCCM) has evolved as a non-invasive, easily applied method for quantification of small fiber involvement in peripheral nerve disorders. We aimed to investigate the potential role of IVCCM in CIDP., Methods: In this cross-sectional study, 15 patients with CIDP underwent assessment with clinical disability scales, neuropathic pain (NP) and autonomic symptom questionnaires, nerve conduction studies, and IVCCM. IVCCM parameters were analyzed and compared to those from 32 healthy controls., Results: Corneal nerve fiber density (CNFD) and corneal nerve fiber length (CNFL) were significantly decreased in the CIDP group, compared to those in controls (p = 0.03 and p = 0.024, respectively). Langerhans cells and fiber tortuosity were increased in CIDP patients (p = 0.005 and p = 0.001, respectively). IVCCM parameters were significantly lower in patients with NP compared to those in patients without NP., Conclusion: IVCCM shows promise as a non-invasive complementary biomarker in the assessment of demyelinating polyneuropathies, providing insights into the potential pathophysiology of these non-length-dependent neuropathies., (© 2023. Fondazione Società Italiana di Neurologia.)

This consensus statement by a panel of neurology experts aimed to provide a practical and implementable guidance document to assist clinicians with the best clinical practice in terms of diagnosis, treatment, and monitoring of late-onset Pompe disease (LOPD). The participating experts consider the clinical suspicion of LOPD by the physician to be of utmost importance in the prevention of diagnostic and therapeutic delay in LOPD patients. A diagnostic algorithm is proposed to facilitate the diagnosis of LOPD in patients presenting with unexplained proximal/axial weakness (with or without respiratory symptoms) or restrictive respiratory insufficiency with hyperCKemia and/or exercise intolerance as the red flag symptoms/signs that raise the index of suspicion for LOPD diagnosis. The diagnosis is based on the subsequent use of dried blood spot (DBS) assay, and the DBS assay can be confirmed by acid alpha-glucosidase (GAA) tissue analysis in leukocytes, fibroblasts, or muscle fibers and/or genetic mutation analysis. Accordingly, experts consider increased awareness among physicians about potential presenting characteristics with a high index of suspicion for LOPD to be crucial to suspect and consider LOPD in the differential diagnosis, while strongly suggesting the use of a diagnostic algorithm combined with DBS assay and confirmatory tests in the timely diagnosis of LOPD and implementation of best practice patterns., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Erdem Ozdamar, Koc, Durmus Tekce, Kotan, Ekmekci, Sengun, Yuceyar and Uluc.)

Vascular compression of neural tissue causing neurological symptoms is a wellknown phenomenon. This is commonly seen in trigeminal neuralgia and, less commonly, in hemifacial spasm by small arteries, which can be treated by microvascular decompression. Rarely, larger arteries, such as the vertebral arteries, may compress the brainstem. This can lead to symptoms of pontine or medullary distress like hemiparesis, dysphagia, or respiratory distress. This is treated by macrovascular decompression. Due to the rare and heterogenous nature of this disease, there is no standardized approach. We describe a novel technique whereby the vertebrobasilar system is mobilized anterolaterally towards the occipital condyle with a sling to decompress the brainstem.
We report two cases of vertebrobasilar dolichoectasia causing brainstem compression. A carotid patch graft sling with anterolateral mobilization to the occipital condyle is described as a surgical nuance to macrovascular decompressive surgery. Briefly, the vertebral artery was identified and dissected away from the brainstem and the bulbar cranial nerves. Bovine pericardium graft was used to create a sling around the artery by suturing the two ends together. The sling was then fixed either to the occipital condyle using cranial plating screws or suturing to the dura of the occipital condyle.
A novel surgical technique for management of vertebrobasilar dolichoectasia causing brainstem compression with progressive neurological deterioration is reported. Anatomical location and the offending vessel should guide neurosurgeons to select the best surgical option to achieve complete decompression of the involved neural structures.

Background: Intra-arterial administration of chemotherapy with or without osmotic blood-brain barrier disruption enhances delivery of therapeutic agents to brain tumors. The aim of this study is to evaluate the safety of these procedures., Methods: Retrospectively collected data from a prospective database of consecutive patients with primary and metastatic brain tumors who received intra-arterial chemotherapy without osmotic blood-brain barrier disruption (IA) or intra-arterial chemotherapy with osmotic blood-brain barrier disruption (IA/OBBBD) at Oregon Health and Science University (OHSU) between December 1997 and November 2018 is reported. Chemotherapy-related complications are detailed per Common Terminology Criteria for Adverse Events (CTCAE) guidelines. Procedure-related complications are grouped as major and minor., Results: 4939 procedures (1102 IA; 3837 IA/OBBBD) were performed on 436 patients with various pathologies (primary central nervous system lymphoma [26.4%], glioblastoma [18.1%], and oligodendroglioma [14.7%]). Major procedure-related complications (IA: 12, 1%; IA/OBBBD: 27, 0.7%; P = .292) occurred in 39 procedures including 3 arterial dissections requiring intervention, 21 symptomatic strokes, 3 myocardial infarctions, 6 cervical cord injuries, and 6 deaths within 3 days. Minor procedure-related complications occurred in 330 procedures (IA: 41, 3.7%; IA/OBBBD: 289, 7.5%; P = .001). Chemotherapy-related complications with a CTCAE attribution and grade higher than 3 was seen in 359 (82.3%) patients., Conclusions: We provide safety and tolerability data from the largest cohort of consecutive patients who received IA or IA/OBBBD. Our data demonstrate that IA or IA/OBBBD safely enhance drug delivery to brain tumors and brain around the tumor., (© The Author(s) 2022. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Chemotherapeutics play a significant role in the management of most brain tumors. First pass effect, systemic toxicity, and more importantly, the blood-brain barrier pose significant challenges to the success of chemotherapy. Over the last 80 years, different techniques of intraarterial chemotherapy delivery have been performed in many studies but failed to become standard of care. The purpose of this article is to review the history of intraarterial drug delivery and osmotic blood-brain barrier disruption, identify the challenges for clinical translation, and identify future directions for these approaches., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Cerebral angiography including internal and external carotid artery injections is crucial in young patients with a spontaneous subdural hematoma., Case Description: We present the first reported case of an accessory meningeal artery aneurysm in a 46-year-old male with a history of hypertension that led to a spontaneous nontraumatic acute subdural hematoma. A PubMed review of the literature was performed using a keyword search to identify cases examining nontraumatic spontaneous intracranial hematomas related to meningeal artery aneurysms. The literature review summarizes all published reports of middle meningeal artery aneurysms resulting in nontraumatic acute intracranial bleeds. The patient underwent successful coiling of the accessory meningeal artery., Conclusion: We propose endovascular treatment for accessory meningeal artery aneurysms and emphasize the utility of angiography of internal and external carotid arteries in a patient with an unexplained intracranial hematoma., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)

Background: Progressive and/or unresectable pilocytic astrocytomas (PAs) carry a poor prognosis compared to typical PA. Early radiotherapy (RT) may have severe long-term neurocognitive side effects in this patient population. Intra-arterial (IA) chemotherapy is a viable alternative or addition to intravenous (IV) chemotherapy, which may be beneficial in avoidance of early RT., Objective: To evaluate the safety and efficacy of IA chemotherapy in this subset of patients., Methods: This is a retrospective review of medical records of PA patients who are treated with IA chemotherapy at Oregon Health & Science University from 1997 until 2019. Response to treatment was categorized as complete response (CR), partial response (PR), stable disease (SD), or progressive disease (PD). Progression free survival (PFS) and overall survival (OS) are also reported., Results: Twelve patients were identified. All patients experienced progression prior to initiation of IA chemotherapy. The most common grade 3 or 4 toxicities related to chemotherapy were thrombocytopenia (66%), neutropenia (66%), leukopenia (50%), anemia (33%), and lymphopenia (16%). Responses achieved were CR in 1, PR in 3, SD in 7, and PD in 1. Median PFS and median OS were 16.5 and 83.5 mo, respectively. A total of 112 procedures (IA injections) were performed and 250 arteries were catheterized. There were 3 minor and no major complications attributable to procedures., Conclusion: This study demonstrates that IA chemotherapy can be safely used in patients with unresectable or progressive PA., (© Congress of Neurological Surgeons 2021.)

Microvascular decompression (MVD) is a common surgical technique used for treatment of trigeminal neuralgia (TN) caused by direct vascular compression of the nerve at the brainstem entry zone (BEZ). Here we report a case of a patient (status postcraniotomy for microsurgical clip obliteration of a ruptured mid-basilar artery aneurysm) who developed right-sided TN 6 years after the procedure. During MVD surgery the clip head was found to be compressing the trigeminal nerve at the BEZ, causing Type 1 TN in V3 distribution. This is consistent with the commonly held theory that a pulsatile stimulus is needed to cause TN. To our knowledge there are no previous reports in the literature of an instrument causing TN.

Background: The aim of this study was to establish reference jitter values for the voluntary activated sternocleidomastoid (SCM) muscle using a concentric needle electrode (CNE)., Methods: The study included 39 healthy participants (20 female and 19 male) aged 18-77 y. Jitter was expressed as the mean consecutive difference (MCD) of 80-100 consecutive discharges. Filters were set at 1 and 10 kHz. The mean MCDs for all participants were pooled, and the mean value +2.5 SD was accepted as the upper limit for the mean MCD. The upper limit for individual MCD was calculated using +2.5 SD of the upper 10th percentile MCD for individual participants., Results: Mean age of the participants was 45 ± 14.5 y. Mean MCD was 16.20 ± 2.23 μs (range: 12-21 μs), and the upper limit of normal for mean MCD was 21.8 μs. The mean value for 823 individual jitters was 23.3 ± 4.61 μs (range: 6.6-36.9 μs), and the upper limit of normal for each individual jitter was 34.6 μs., Conclusions: The present findings indicate that upper normal limit for mean MCD is 22 μs and for individual data it is 35 μs., (© 2020 Wiley Periodicals LLC.)

Background: Gross total resection of arteriovenous malformations (AVMs) of the central nervous system confirmed by formal angiography is accepted as a cure for patients. In some cases, this may not be possible. Even though in these cases other treatment modalities such as endovascular embolization and radiotherapy can be used, long-term follow-up is lacking in the literature., Case Description: Here we report a case of a 57-year-old woman with history of a right-sided parieto-occipital/periatrial AVM, initially treated with a combination of endovascular embolization and radiotherapy., Conclusions: The patient subsequently presented (12 years later) with a symptomatic, enlarging, contrast-enhancing mass at the same location that was angiographically occult but ultimately proven to be an AVM on a background of reactive changes on pathology., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: In contemporary microneurosurgery reducing retraction-induced injury to the brain is essential. Self-retaining retractor systems are commonly used to improve visualization and decrease the repetitive microtrauma, but sometimes self-retaining retractor systems can be cumbersome and the force applied can cause focal ischemia or contusions. This may increase the morbidity and mortality. Here, we describe a technique of retraction using 10-0 sutures in the arachnoid., Objective: To evaluate the imaging and clinical results in patients where 10-0 suture retraction was used to aid the surgical procedure., Methods: Adjacent cortex was retracted by placing 10-0 nylon suture in the arachnoid of the bank or banks of the sulcus. The suture was secured to the adjacent dural edge by using aneurysm clips, allowing for easy adjustability of the amount of retraction. We retrospectively analyzed the neurological outcome, signal changes in postoperative imaging, and ease of performing surgery in 31 patients with various intracranial lesions including intracranial aneurysms, intra- and extra-axial tumors, and cerebral ischemia requiring arterial bypass., Results: Clinically, there were no injuries, vascular events, or neurological deficits referable to the relevant cortex. Postoperative imaging did not show changes consistent with ischemia or contusion due to the retraction. This technique improved the visualization and illumination of the surgical field in all cases., Conclusion: Retraction of the arachnoid can be used safely in cases where trans-sulcal dissection is required. This technique may improve initial visualization and decrease the need for dynamic or static retraction.

Introduction: An increased response to painful stimuli without spontaneous pain suggests a role of central hyperexcitability of pain pathways in the pathogenesis of myofascial pain syndrome (MPS). In this study we aimed to test the hypothesis that spinal pain pathways are affected in MPS. We used cutaneous silent period (CSP) parameters to demonstrate the hyperexcitability of spinal pain pathways in MPS., Methods: Twenty-nine patients diagnosed with MPS and 30 healthy volunteers were included in the study. The CSP recordings were performed in the right upper and left lower extremities., Results: In both upper and lower extremities, patients had prolonged CSP latencies (P = 0.034 and P = 0.049 respectively) and shortened CSP durations (P = 0.009 and P = 0.008, respectively)., Discussion: Delayed and shortened CSP in MPS patients implies dysfunction in the inhibitory mechanism of the spinal/supraspinal pain pathways, suggesting central sensitization in the pathogenesis of MPS and supporting our research hypothesis. Muscle Nerve 57: E24-E28, 2018., (© 2017 Wiley Periodicals, Inc.)

Purpose: To evaluate the usefulness of somatosensory evoked potential as a screening tool for spinal pathologies in patients with treatment refractory overactive bladder., Methods: This prospective study was performed between January 2011 and January 2014. Children >5 years old with treatment refractory overactive bladder were enrolled after exclusion of anatomical and neurological causes of incontinence. All patients underwent urodynamic studies, spinal MRI, and somatosensory evoked potential (SEP). Sensitivity, specificity, PPV, and NPV were calculated for SEP., Results: Thirty-one children (average age 8.3 ± 2.9 years) were included in the study. SEP was abnormal in 13 (41.9%), and MRI was abnormal in 8 (25.8%) patients. SEP was found to have a sensitivity of 87.5%, a specificity of 73.9%, positive predictive value of 53.85%, and negative predictive value (NPV) of 94.4%., Conclusion: In patients with treatment refractory OAB, SEP is an important tool for the screening of tethered cord/spinal pathologies. Our results suggest that a child with a normal SEP study in this group of patients may not require further investigation with MRI.

Aneurysms of the distal anterior cerebral artery (dACA) are rare, consisting only 2-6% of all intracranial aneurysms. dACA aneurysms are often associated with anomalies such as azygous, bihemispheric and triplication of ACA. Among these anomalies accessory anterior cerebral artery (accACA) is an anatomical variant found in 3.3% to 15% of the population. Here we report a case of a 60-year-old female who presented with a Hunt and Hess grade II, Fisher grade III subarachnoid hemorrhage secondary to a ruptured saccular aneurysm originating from the pericallosal-callosomarginal artery bifurcation at the A3 segment of the accessory ACA and underwent an uncomplicated microsurgical clipping.

BACKGROUND The purpose of this study was to present the clinical results of our retrospective series of carpal tunnel release (CTR) operations. For these operations we used a unique type of incision, for the first time, for treatment of carpal tunnel syndrome (CTS) consisting of a 1-cm semi-vertical (SV) incision made into the wrist crease for macroscopic open CTR. MATERIAL AND METHODS This retrospective study included 114 patients (101 females and 13 males) with CTR who were operated upon in our neurosurgery clinic between December 2010 and June 2015. Patient ages ranged from 35 to 83 years (mean 55.05±12.04 years). In total, 127 hands (73 right and 54 left) were operated upon using the SV skin incision technique. After an average follow-up of 18 months (ranging from 6 to 30 months), clinical and electrophysiological (EP) evaluations were performed. RESULTS A review of the English language literature published since 1957, when Phalen first popularised the diagnosis and treatment of this disease, determined that no previous reports of the mini-open incision technique as described in our study have been published. In our retrospective patient case review, we found that after operations using the SV incision technique, statistically significant differences were detected in electromyography (EMG) improvements (p<0.01). In addition, patients who showed improvement in EMG studies (n=90) were satisfied with the result of their surgery. CONCLUSIONS Our study demonstrated that 1-cm skin SV incision was a cosmetically satisfying, fast, and safe approach to CTR that was not only clinically effective but also electrophysiologically effective.

Purpose of the Study: We proposed a new electrophysiological parameter medial plantar (MP)-to-radial amplitude ratio (MPRAR), similar to sural-to-radial amplitude ratio (SRAR), in the diagnosis of distal sensory polyneuropathy (DSP), based on the concept that distal nerves are affected more and earlier than proximal nerves in axonal neuropathies. We aimed to investigate the diagnostic sensitivity of this parameter in diabetic DSP, together with sensitivities of SRAR and MP nerve action potential (NAP) amplitude., Materials and Methods: In 124 healthy controls and 87 diabetic patients with clinically defined DSP and normal sural responses, we prospectively performed sensory nerve conduction studies (NCS), and evaluated the MP NAP amplitude, MPRAR and SRAR values. We determined the lower limits of normal (LLN) of these parameters in the healthy controls and calculated their sensitivities and specificities in detecting DSP in diabetic patients., Results: MP nerve amplitude and MPRAR values were significantly lower in the patient group, compared to controls. However, SRAR values did not differ significantly between the two groups. The LLN of MP NAP amplitude was found to be 4.1 μV. The cutoff values for SRAR and MPRAR were determined as 0.24 and 0.16, respectively. MPRAR was abnormal in 21.8% of patients. However, the most sensitive parameter in detection of DSP was MP NAP amplitude, which showed a sensitivity of 31% and a specificity of 100%., Conclusions: Although MPRAR is more sensitive than SRAR in detecting DSP, it does not provide additional diagnostic yield to the assessment of MP NCS alone in diabetic DSP patients with normal sural responses.

Tumors of the lateral and third ventricles are cradled on all sides by vital vascular and eloquent neural structures. Microsurgical resection, which always requires attentive planning, plays a critical role in the contemporary management of these lesions. This article provides an overview of the open microsurgical approaches to the region highlighting key clinical perspectives., Competing Interests: Dr. Aaron Cohen–Gadol has a consulting agreement with Zeiss Meditec, the rest of the authors declare that they have no conflict of interest. Informed consent Informed consent was obtained from all individual participants included in the study.