Neurogenic Lesions Research Articles

EMG phenomena of myogenic hyperexcitability

The type, distribution pattern and time course of spontaneous muscular activity are important for the diagnostics of neuromuscular diseases in the clinical practice. In neurogenic lesions with motor axonal involvement, pathologic spontaneous activity (PSA) is usually reliably detectable by needle electromyography (EMG) 2-4weeks after occurrence of the lesion. The distribution pattern correlates with the lesion location. The focus of the present work is the description of the different forms of PSA in myogenic diseases.

Malignant and Benign Tracheobronchial Neoplasms: Comprehensive Review with Radiologic, Bronchoscopic, and Pathologic Correlation.

Tracheobronchial neoplasms are much less common than lung parenchymal neoplasms but can be associated with significant morbidity and mortality. They include a broad differential of both malignant and benign entities, extending far beyond more commonly known pathologic conditions such as squamous cell carcinoma and carcinoid tumor. Airway lesions may be incidental findings at imaging or manifest with symptoms related to airway narrowing or mucosal irritation, invasion of adjacent structures, or distant metastatic disease. While there is considerable overlap in clinical manifestation, imaging features, and bronchoscopic appearances, an awareness of potential distinguishing factors may help narrow the differential diagnosis. The authors review the epidemiology, imaging characteristics, typical anatomic distributions, bronchoscopic appearances, and histopathologic findings of a wide range of neoplastic entities involving the tracheobronchial tree. Malignant neoplasms discussed include squamous cell carcinoma, malignant salivary gland tumors (adenoid cystic carcinoma and mucoepidermoid carcinoma), carcinoid tumor, sarcomas, primary tracheobronchial lymphoma, and inflammatory myofibroblastic tumor. Benign neoplasms discussed include hamartoma, chondroma, lipoma, papilloma, amyloidoma, leiomyoma, neurogenic lesions, and benign salivary gland tumors (pleomorphic adenoma and mucous gland adenoma). Familiarity with the range of potential entities and any distinguishing features should prove valuable to thoracic radiologists, pulmonologists, and cardiothoracic surgeons when encountering the myriad of tracheobronchial neoplasms in clinical practice. Attention is paid to any features that may help render a more specific diagnosis before pathologic confirmation. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

SURGICAL TREATMENT OF SEVERE TRAUMATIC PTOSIS BY CONTRALATERAL FRONTAL SUSPENSION

Ptosis, a common congenital or acquired disease, is an upper palpebral droop secondary to impaired action of the levator muscle of the upper eyelid. In its severe form, he is very unresponsive or inactive. Severe traumatic ptosis is most often the result of neurogenic, myogenic or scarring lesions. The treatment of severe ptosis is essentially surgical, with a variety of techniques giving acceptable results. Frontal suspension is the therapeutic method of choice, performed according to the Fox or Crawford procedure. But it requires an active homolateral forehead muscle, capable of raising the decient eyelid; criteria that could be missing. So what would be our alternative? In our study, we report a case of severe traumatic ptosis in a young patient, associated with a palsy of the homolateral frontal muscle, treated by contralateral frontal suspension using autologous material

S3140 Posterior Mediastinal Mass: Unraveling a Non-Traumatic Herniation of the Caudate Lobe of the Liver

Introduction: Posterior mediastinal mass is most likely due to neurogenic tumor, meningocele or thoracic spine lesions. Caudate lobe of the liver herniation presenting as posterior mediastinal mass is a rare occurrence. Diaphragmatic herniation (DH) of the caudate lobe presents in various way including dyspnea, dyspepsia or incidental finding on imaging. We present a case of diaphragmatic hernia of the caudate lobe of the liver presenting as a posterior mediastinal mass found during evaluation of dyspnea. Case Description/Methods: A 75-year-old female presented to her physician with worsening shortness of breath from her baseline of 3 days duration. She had a history of sarcoidosis, COVID pneumonia over 1 year ago, COPD, diastolic heart failure, and hypertension. She was initially evaluated for COVID re-infection, which was negative and a CT of the chest with contrast to check for sarcoidosis flare revealed posterior mediastinal mass measuring 4.5 × 6.5 × 6.4 cm. Further work up with CT chest and abdomen with contrast revealed that the posterior mediastinal mass had similar attenuation as the liver and appears continuous with the caudate lobe of the liver. This was confirmed by NM scan of liver. Review of her records from an outside organization revealed similar finding on imaging a few years ago. Patient denied any history of trauma and laboratory work up revealed normal liver functions. After pulmonologist evaluation she was started on 2 L home oxygen following six-minute walk test, and also CPAP following a positive sleep study. Pulmonary function tests were performed and inhalers were continued. Given the chronicity of her symptoms and co-morbidities with stable caudate lobe herniation, conservative management was advised with surgery warranted if symptoms persist despite treatment (Figure 1). Discussion: DH is typically found on the left side with stomach or intestine while the right side is usually guarded by the liver. Isolated herniation of part of the liver into the thoracic cavity is rarely reported and is mostly acute from traumatic or spontaneous rupture requiring immediate repair. Our patient was initially evaluated for the posterior mediastinal mass for concerns of tumor, followed by the finding of what was thought to be acute herniation of the caudate lobe of liver into the thoracic cavity. Review of records showed this to be a stable lesion, we suspect that the patient had congenital diaphragmatic defect. Chronic and stable liver herniation into thoracic cavity can be managed conservatively if uncomplicated.Figure 1.: (A) Computed Tomography of chest with contrast (axial view) showing a well-defined soft tissue density lesion in the posterior mediastinum (red arrow). (B) Computed Tomography of the chest, abdomen and pelvis with contrast (sagittal view) showing well defined soft tissue mass in the posterior mediastinum (red arrow) similar in attenuation to the adjacent liver and appears continuous with the caudate lobe of liver.

Charcot-Marie-Tooth Disease 4G Caused by Homozygous Deletion of Exon 4 in HK1 Gene due to Inbreeding: A Case Report and Literature Review

Objectives: The objective is to investigate the clinical and genotypic characteristics of Charcot-Marie-Tooth disease, caused by HK1 gene mutation. Methods: The detailed medical history of the child was collected and the clinical symptoms were summarized. The genomic DNA was extracted from the 2ml of peripheral blood of child and their parents, and the whole exome sequencing was performed, and the related literatures were reviewed. Results: A 7-year-old girl with unstable walking for 7 months, left claudication, obvious valgus of left foot, unable to squate , unable to jump on one foot, grade IV of muscle strength of lower extremity and slight limitation of dorsal extension of left foot. Electromyography showed multiple peripheral neurogenic lesions (motor and sensory nerve demyelination with axonal damage, more severe in lower limbs than in upper limbs). Whole exome sequencing and PCR verification indicated that the patient had a homozygous deletion in exon 4 of HK1 gene, and the variation site was located in the range of chr10:71048499-71048526, which had not been reported before, and the associated disease was peroneal muscular atrophy type 4G. Conclusion: This study expands the number of reported cases of CMT4G and the mutation spectrum of HK1 gene, and provides reference for prenatal diagnosis and genetic counseling.

Is paravertebral muscles edema a consequence of neurogenic changes in MuSK-positive myasthenia gravis?

Anti-MuSK myasthenia gravis (Anti-MuSK MG) is a chronic autoimmune disease caused by complement-independent dysfunction of the agrin-MuSK-Lrp4 complex, accompanied by the development of the pathological muscle fatigue and sometimes muscle atrophy. Fatty replacement of the tongue, mimic, masticatory and paravertebral muscles, revealed by muscle MRI and proton magnetic resonance spectroscopy (MRS), is considered to be a consequence of the myogenic process in anti-MuSK antibody MG in the patients with a plenty long course of the disease. However, in most experimental studies on animal models with anti-MuSK MG, complex presynaptic and postsynaptic changes are revealed, accompanied by the functional denervation of masticatory and paravertebral muscles predominantly. This study presents the MRI, nerve conduction studies (NCS), repetitive nerve stimulation (RNS) and electromyography (EMG) of neurogenic lesions of the axial muscles (m. Multifidus Th12, L3-L5; m. Erector spinae L4-L5) in two patients K. (51 years old), and P. (44 years old), both of whom were having weakness of the paravertebral muscles for 2-4 months due to anti-MuSK MG. The clinical manifestations, as well as the edematous changes in the paravertebral muscles, regressed after therapy. Thus, these clinical examples may confirm the presence of the neurogenic changes at an early stage of anti-MuSK myasthenia gravis and indicate importance of immediate initiation of therapy to avoid the development of muscle atrophy and fatty infiltration.

Management of parapharyngeal space tumors with transparotid–transcervical approach: analysis of prognostic factors related with disease-control and functional outcomes

Different therapeutic strategies have been developed to improve surgical and functional outcome of parapharyngeal space (PPS) neoplasms. The transparotid-transcervical approach (TTa) is a valid surgical option to manage most PPS tumors. Its short- and long-term disease control and cranial nerve (c.n.) function outcomes have not been discussed extensively. All patients who underwent TTa over a 10-year period at a tertiary academic center were retrospectively reviewed. Data about preoperative imaging, clinical presentation, tumor's size, location and histology, and postoperative oncological and functional results were registered and analyzed. Sixty patients matched the inclusion criteria. Most of the lesions were benign (71.7%), involved the prestyloid PPS (63.3%) and measured more than 30mm (75%). Fifty-two (86.7%) lesions were resected en-bloc. Clear margins were achieved in 91.7% of the cases, with positive margins solely associated with malignancy (p = 0.008). Post-operative c.n. function was satisfactory, with X c.n. function significantly associated with the retrostyloid location (p = .00) and neurogenic tumors (p = 0.02). Local disease-control was achieved in 96% of the cases after a median follow-up of 46 (± 19.7-82.0 IQR) months. The TTa was safe and effective, achieving a satisfactory local control rate. Nevertheless, malignancies maintain a higher rate of positive margin due to their infiltrative nature and the complex anatomy of the PPS. In such cases, multiportal approaches might be more effective. However, post-operative c.n. function remained satisfactory, irrespective of lesions' size and histopathologic behavior. A higher X c.n. deficit rate was observed in retrostyloid and neurogenic lesions.

Pituitary apoplexy without chiasm compression: A case report.

The present report presents a rare case in which a patient with pituitary apoplexy (PA) without compression of the optic chiasm experienced diagnosable visual impairment in the ensuing months. Endocrinologically, the condition was a prolactinoma followed by bleeding into the pituitary gland. Due to the unexplained functional changes in the patient, an electrophysiological examination (pattern electroretinogram and pattern visual evoked potentials) was performed, which verified a bilateral non-inflammatory neurogenic lesion. This finding was confirmed by functional magnetic resonance imaging (fMRI) examination. Structural MRI did not reveal chiasm compression in the time sequence or alteration of the optic nerves (the diameter of the optic nerve at different distances from the eye and the diameter of the optic nerve sheath at different distances from the eye). Similarly, neither the retinal nerve fiber layer (RNFL) nor the vessel density was altered. The present report suggests that changes in visual fields may be due to ischemia in the area of the chiasm and optic nerves, similar to PA.

Clinical features and genotypes of Laing distal myopathy in a group of Chinese patients, with in-frame deletions of MYH7 as common mutations

BackgroundLaing distal myopathy is a rare autosomal dominant inherited distal myopathy caused by mutations of the MYH7 gene affecting mainly the rod region. We described the clinical features, muscle MRI and pathological changes as well as genetic mutations in a group of Chinese patients with Laing distal myopathy.ResultsSix patients with the confirmed diagnoses of Laing distal myopathy were recruited. Ankle dorsiflexion and finger extension weakness, as well as neck flexion weakness were common in our patients. Myopathic as well as neurogenic lesions were suggested by electromyography in different patients. Respiratory abnormality of sleep apnea was detected in two of our patients stressing the necessity of close respiratory monitoring in this disease. Muscle MRIs showed similar features of concentric fatty infiltration of anterior thigh muscles together with early involvement of tibialis anterior and extensor hallucis longus. However, muscle pathological presentations were varied depending on the biopsied muscles and the severity of the disease. In-frame deletions of the MYH7 gene made up 3/4 of mutations in our patients, suggesting that these are common mutations of Laing distal myopathy.ConclusionsOur study further expanded the phenotypes and genotypes of Laing distal myopathy. In-frame deletions of the MYH7 gene are common causes of Laing distal myopathy.

Neurogenic vs. Myogenic Origin of Acquired Muscle Paralysis in Intensive Care Unit (ICU) Patients: Evaluation of Different Diagnostic Methods.

Introduction. The acquired muscle paralysis associated with modern critical care can be of neurogenic or myogenic origin, yet the distinction between these origins is hampered by the precision of current diagnostic methods. This has resulted in the pooling of all acquired muscle paralyses, independent of their origin, into the term Intensive Care Unit Acquired Muscle Weakness (ICUAW). This is unfortunate since the acquired neuropathy (critical illness polyneuropathy, CIP) has a slower recovery than the myopathy (critical illness myopathy, CIM); therapies need to target underlying mechanisms and every patient deserves as accurate a diagnosis as possible. This study aims at evaluating different diagnostic methods in the diagnosis of CIP and CIM in critically ill, immobilized and mechanically ventilated intensive care unit (ICU) patients. Methods. ICU patients with acquired quadriplegia in response to critical care were included in the study. A total of 142 patients were examined with routine electrophysiological methods, together with biochemical analyses of myosin:actin (M:A) ratios of muscle biopsies. In addition, comparisons of evoked electromyographic (EMG) responses in direct vs. indirect muscle stimulation and histopathological analyses of muscle biopsies were performed in a subset of the patients. Results. ICU patients with quadriplegia were stratified into five groups based on the hallmark of CIM, i.e., preferential myosin loss (myosin:actin ratio, M:A) and classified as severe (M:A < 0.5; n = 12), moderate (0.5 ≤ M:A < 1; n = 40), mildly moderate (1 ≤ M:A < 1.5; n = 49), mild (1.5 ≤ M:A < 1.7; n = 24) and normal (1.7 ≤ M:A; n = 19). Identical M:A ratios were obtained in the small (4–15 mg) muscle samples, using a disposable semiautomatic microbiopsy needle instrument, and the larger (>80 mg) samples, obtained with a conchotome instrument. Compound muscle action potential (CMAP) duration was increased and amplitude decreased in patients with preferential myosin loss, but deviations from this relationship were observed in numerous patients, resulting in only weak correlations between CMAP properties and M:A. Advanced electrophysiological methods measuring refractoriness and comparing CMAP amplitude after indirect nerve vs. direct muscle stimulation are time consuming and did not increase precision compared with conventional electrophysiological measurements in the diagnosis of CIM. Low CMAP amplitude upon indirect vs. direct stimulation strongly suggest a neurogenic lesion, i.e., CIP, but this was rarely observed among the patients in this study. Histopathological diagnosis of CIM/CIP based on enzyme histochemical mATPase stainings were hampered by poor quantitative precision of myosin loss and the impact of pathological findings unrelated to acute quadriplegia. Conclusion. Conventional electrophysiological methods are valuable in identifying the peripheral origin of quadriplegia in ICU patients, but do not reliably separate between neurogenic vs. myogenic origins of paralysis. The hallmark of CIM, preferential myosin loss, can be reliably evaluated in the small samples obtained with the microbiopsy instrument. The major advantage of this method is that it is less invasive than conventional muscle biopsies, reducing the risk of bleeding in ICU patients, who are frequently receiving anticoagulant treatment, and it can be repeated multiple times during follow up for monitoring purposes.

Clinical, neuroelectrophysiological and muscular pathological analysis of chronic progressive external ophthalmoplegia.

The aim of the present study was to explore the clinical, neuroelectrophysiological and muscular pathological characteristics of chronic progressive external ophthalmoplegia (CPEO) and to improve the understanding of CPEO. Clinical manifestations, neuroelectrophysiology and pathological features of muscle biopsies from 12 patients with CPEO were retrospectively analyzed. The average age of onset for the 12 patients (6 males and 6 females) was 17.2 years. All patients had different degrees of blepharoptosis. A total of 11 patients experienced ocular dyskinesia, but diplopia was rare. Electrophysiological testing in 12 patients revealed abnormal changes in 6 patients, including 4 patients with a myogenic lesion, 1 patient with a neurogenic lesion, and 1 patient with mixed myogenic/neurogenic lesions. Two patients had slow sensory nerve conduction velocity. Muscle biopsies in 12 patients demonstrated ragged-red, irregular and broken fibers in 11 patients through Gomori trichrome and hematoxylin and eosin (H&E) staining, increased lipid levels in some muscle fibers in 4 patients through Οil Ρed O staining and abnormal distribution of type I and II muscle fibers in 3 patients through ATPase staining. Electron microscopy in 5 patients showed an increased number of mitochondria and abnormal mitochondrial aggregation between submucosa and myofibrils in 4 patients. These findings suggest that the possibility of CPEO should be considered if patients present with obvious extraocular muscle paralysis without diplopia. Furthermore, the identification of ragged-red fibers by Gomori trichrome and H&E staining of muscle biopsies from patients is an important basis for the diagnosis of CPEO.

Morphogenetic Variability as Potential Biomarker of Neurogenic Lesion Degree in Children with Spina Bifida.

Aims. In this study we analyzed the degree of genetic homozygosity among spina bifida patients with different degrees of neurogenic lesion (N = 82), as well as their clinical and neurological characteristics, compared to healthy control individuals (N = 100). Methods. According to clinical and electromyographic findings, we separately assessed the type of neurogenic lesion (paresis or paralysis). Regarding the degree of neurogenic lesion, patients were classified into three groups: mild, moderate and severe. We analyzed six muscles. For assessing the degree of individual genetic homozygosity, we tested the presence and distribution of 15 homozygous recessive characteristics (HRC). Results. The predominant type of neurogenic lesion was paresis. Every third evaluated muscle was affected in the group with mild neurogenic lesion, while more than half were affected in the group with severe neurogenic lesion. The average values of HRCs among different groups of patients and the control showed the population-genetic differences that exist among them (control HRC/15 = 3.0 ± 0.2; mild HRC/15 = 3.6 ± 0.2; moderate HRC/15 = 4.8 ± 0.3; severe neurogenic lesion HRC/15 = 5.0 ± 0.3). Conclusions. Spina bifida patients have a significant increase of recessive homozygosity and a decreased variability compared to the control group. As neurogenic lesions are more severe, more affected muscles are present, as well as the increase of individual recessive homozygosity.

A Pathogenic Missense Variant (c.1617G&gt;A, p.Met539Ile) in UBA1 Causing Infantile X-Linked Spinal Muscular Atrophy (SMAX2)

Background: Infantile X-linked spinal muscular atrophy (SMAX2) is a rare type of spinal muscular atrophy associated with UBA1 variants.Methods: Clinical imaging and neurophysiological tests were performed on a Chinese patient with SMAX2. Further, focused panel sequencing of UBA1 was carried out on samples of both the proband and his maternal relatives.Results: The proband, a 4-year-old boy with the SMAX2 phenotype, suffered from reduced exercise capacity since infancy. His other symptoms included speech difficulties, severe nasal tone, reduced distal muscle strength, areflexia, and inadequate sucking ability. The brain MRI of the proband's showed normal results but the electromyography results showed multiple peripheral neurogenic lesions. Five male members of the proband's family were affected with the SMAX2 phenotype. They presented similar symptoms and had experienced a long and autonomous life. Molecular analysis revealed a novel missense variant (c.1617G>A, p.Met539Ile) in the exon 15 of UBA1. The proband's mother, as well as grandmother, carried the heterozygous missense UBA1 variant; whereas, the male patients from the family carried the hemizygotic variant.Conclusions: The affected members in this Chinese family showed unique features such as extended life span, no fractures, and cramps as compared with previously reported SMAX2 cases. The novel missense variant (c.1617G>A (p.Met539Ile) in UBA1 highlights the critical role of this gene in causing SMAX2 phenotype.

Electrophysiological Study of Nerve Involvement in Rheumatoid Arthritis

Background and Aim: Rheumatoid arthritis is a chronic systemic inflammatory disease of undetermined etiology. Rheumatoid arthritis is primarily considered a disease of joint but abnormal systemic immune response produce a variety of extra-articular manifestation. Rheumatoid Arthritis (RA) is associated with various neurological manifestations including nerve compression by synovial proliferation, sensory or sensorimotor neuropathies causing neurologic symptom. This study aims to assess peripheral nerve involvement on conduction in rheumatoid arthritis. Methods: 100 patients and healthy controls were included in this study. Rheumatoid arthritis patients diagnosed at the Department of Medicine in VIMSAR during the time period of November 2016-May 2017 were included in the study. Nerve conduction of median, ulnar (motor), posterior tibial and common personal nerves and surely nerves were studied for all healthy controls and patients. Results: In this study, peripheral neuropathy was detected in 54 patients; Mononeuritis simplex was the commonest lesion and detected in 36 out of the 54 patients. Mon neuritis multiplex was detected in 11 and symmetrical polyneuropathy found in 7 patients. The entrapment neuropathy was found in 25 patients, affecting the median in 13, posterior tibial 8 and ulnar 4. So, the axonopathy (mainly due to vasculitis) and local demyelination (mainly due to entrapment) were the common types of nerve injury seen in rheumatoid arthritis. Conclusion: Neurogenic lesions were detected in patients with rheumatoid arthritis without neurological symptoms. So, nerve conduction study are recommended in routine examination to diagnose early neuropathy in RA patients.

Newborn Screening for Spinal Muscular Atrophy in China Using DNA Mass Spectrometry.

Background: Spinal muscular atrophy (SMA) is the most common neurodegenerative disorder and the leading genetic cause of infant mortality. Early detection of SMA through newborn screening (NBS) is essential to selecting pre-symptomatic treatment and ensuring optimal outcome, as well as, prompting the urgent need for effective screening methods. This study aimed to determine the feasibility of applying an Agena iPLEX SMA assay in NBS for SMA in China. Methods: We developed an Agena iPLEX SMA assay based on the matrix-assisted laser desorption/ionization time-of-flight mass spectrometry, and evaluated the performance of this assay through assessment of 167 previously-genotyped samples. Then we conducted a pilot study to apply this assay for SMA NBS. The SMN1 and SMN2 copy number of screen-positive patients were determined by multiplex ligation-dependent probe amplification analysis. Results: The sensitivity and specificity of the Agena iPLEX SMA assay were both 100%. Three patients with homozygous SMN1 deletion were successfully identified and conformed by multiplex ligation-dependent probe amplification analysis. Two patients had two SMN2 copies, which was correlated with severe SMA type I phenotype; both of them exhibited neurogenic lesion and with decreased muscle power. Another patient with four SMN2 copies, whose genotype correlated with milder SMA type III or IV phenotype, had normal growth and development without clinical symptoms. Conclusions: The Agena iPLEX SMA assay is an effective and reliable approach for population-based SMA NBS. The first large-scale pilot study using this assay in the Mainland of China showed that large-scale implementation of population-based NBS for SMA is feasible.

Broadening the phenotype of the TWNK gene associated Perrault syndrome

BackgroundPerrault syndrome is a genetically heterogenous, very rare disease, characterized clinically by sensorineural hearing loss, ovarian dysfunction and neurological symptoms. We present the case of a 33 years old female patient with TWNK-associated Perrault syndrome. The TWNK gene is coding the mitochondrial protein Twinkle and currently there are only two reports characterizing the phenotype of TWNK-associated Perrault syndrome. None of these publications reported about special brain MRI alterations and neuropathological changes in the muscle and peripheral nerves.Case presentationOur patients with TWNK-dependent Perrault syndrome had severe bilateral hypoacusis, severe ataxia, polyneuropathy, lower limb spastic paraparesis with pyramidal signs, and gonadal dysgenesis. Psychiatric symptoms such as depression and paranoia were present as well. Brain MRI observed progressive cerebellar hyperintensive signs associated with cerebellar, medulla oblongata and cervical spinal cord atrophy. Light microscopy of the muscle biopsy detected severe neurogenic lesions. COX staining was centrally reduced in many muscle fibers. Both muscle and sural nerve electron microscopy detected slightly enlarged mitochondria with abnormal cristae surrounded by lipid vacuoles. In the sural nerve, dystrophic axons had focally uncompacted myelin lamellae present. Genetic investigation revealed multiple mtDNA deletion and compound heterozygous mutations of the TWNK gene (c.1196 A > G, c.1358 G > A).ConclusionThis study demonstrates that TWNK associated Perrault syndrome has a much broader phenotype as originally published. The coexistence of severe hypoacusis, spastic limb weakness, ataxia, polyneuropathy, gonadal dysgensia, hyperintense signals in the cerebellum and the presence of the mtDNA multiple deletion could indicate the impairment of the TWNK gene. This is the first report about pyramidal tract involvement and cerebellar MRI alteration associated with TWNK-related Perrault syndrome.

Surgical treatment of benign tumors of parapharyngeal space

Background: Neoplasms of the oropharynx and parapharyngeal space are quite rare, characterized by nonspecific development of the disease, and the involvement of important anatomical structures in the pathological process. Problems of diagnosis and choice of tactics of surgical treatment are relevant today. Aim: To evaluate the effectiveness of transoral approach when removing benign tumors of the parapharyngeal space. Materials and methods: In State Institution “O.S. Kolomiychenko Institute of Otolaryngology of National Academy of Medical Sciences of Ukraine” in recent years, 92 patients underwent surgical treatment with benign tumors of the parapharyngeal space. Men – 39 (42.4%), women – 53 (57.6%). All patients underwent total removal of the tumor in the parapharyngeal space by transoral approach. Results: In patients evaluated the postoperative period, the type of postoperative wound healing, the presence of complications and the quality of life. The following postoperative complications were observed: in 13 (14.1%) patients lockjaw was observed, which regressed on average for 2-3 days, bleeding from the postoperative site in the early postoperative period – 4 (4.3%) and acute cerebrovascular accident in the early postoperative period in 1 patient (1.1%). Conclusions: Given the good blood supply to the parapharyngeal space, bleeding is one of the most common complications. Vascular complications are more common when neurogenic or vascular tumors are removed. Angiography is useful in evaluating post-styloid lesions in order to demonstrate their connection with large vessels and differentiate neurogenic and vascular lesions. The advantages of transoral removal of the tumors of the parapharyngeal space are the absence of cosmetic defects, the rapid healing of postoperative wounds and the rapid restoration of the patient’s working capacity.

P72-F Electromyography in the course of amyotrophic lateral sclerosis

Background Amyotrophic lateral sclerosis is a progressive, incurable neurodegenerative disease resulting in neurogenic muscle damage. Material and methods The study covered 20 patients – 5 women and 15 men aged 43–85; 65.5 on average. The group included patients with clinically proven or probable ALS diagnosis established based on the modified El Escorial criteria. The study was performed three times with three-month long intervals. Muscles from three layers of the central nervous system were subject to analysis: bulbs (muscle of the tongue), cervical (left biceps brachii – BB and left first dorsal interosseous – FDI), sacrolumbar (right vastus lateralis – VL and right tibialis anterior – TA). Resting activity and MUP parameters (amplitude, area, duration, size index – SI) were evaluated. Results Incorrect results most frequently concerned the amplitude, area, SI MUP – BB (100%), FDI (100% – SI; 95% – the MUP amplitude; 90% – the MUP area), VL (90% – the MUP amplitude and area; 80% – SI), TA (95% – SI; 85% – the MUP amplitude and area) and duration – BB (85%), FDI (90%). Moreover, at rest, the majority of patients had, in all the muscles subject to the study, their active denervation potentials recorded, i.e. fibrillation or positive slow waves. Significant differences were observed within the course of a six-month period as regards the measurements concerning the duration of MUP of the muscles subject to the study (prolonged duration of MUP). Conclusion Neurogenic lesions are already present at the time of ALS diagnosis.Relationship was observed between motor neurons loss and the gradually increasing duration of MUP of particular muscles in the course of ALS.

Electrodiagnostic Case Study of Diabetic Amyotrophy: Variant of Diabetic Neuropathy

The neuropathies are very common in diabetes and one of the most important presentations is proximal neuropathy which is called as diabetic amyotrophy. Diabetic amyotrophy is a condition presented as a proximal motor neuropathy which is asymmetric and with weakening of the proximal muscles supplied by the femoral and obturator nerves and sometimes there may be distal polyneuropathy. The diabetic amyotrophy includes various elements of lumbosacral plexus and electrodiagnostic study often gives the diagnosis of neurogenic lesion affecting at the level of root called as lumbosacral radiculopathy, or involving plexus as plexopathy, or proximal neuropathy. Thus, the disorder is called lumbosacral radiculoplexus neuropathy. This article is a case study related with neurophysiological findings of diabetic amyotrophy which shows asymmetric proximal motor neuropathy.

Histopathological study of mediastinal lesion in a medical college-3 years study

Background: It is very important to know about Histological pattern, incidence in relation to age, sex and location of mediastinal lesions for better outcome of patients. This will help the clinicians in both treating the patient and determining the outcome of the disease process. Material and method: The material for the study was collected from the in patients being admitted in N.H.L. Medical College, V.S. Hospital Ahmedabad affiliated with Gujarat University. Data was collected in pretested proforma. Results: Total 35 cases of mediastinal lesions were studied. The data according to age incidence, sex incidence, location, nature of the lesion and clinical symptoms was prepared and analyzed. Most common lesion was thymic follicular hyperplasia (26%) followed by Mailgant thymoma (23%). Maximum number of cases were found in 1st & 5th decade (23% in each decade) which were followed by fourth decade. Anterior mediastinum was the commonest to be involved in present study (comprising of 66% of lesions). Conclusion: Most common lesions are thymic lesion (including benign/malignant thymoma, thymic hyperplasia, Cystic lesion, and neurogenic lesion and Germ cell tumor. Benign lesions were more common (74%) than malignant lesion and have better prognosis.