Nerve Conduction Tests Research Articles

Effectiveness of Kabat Technique versus Mirror Book Therapy on clinical and electrophysiological parameters in patients with Bell’s palsy: a comparative interventional study

Abstract Background Bell’s palsy significantly impacts facial function, aesthetics, and quality of life. Objective To compare the effects of the Kabat Technique (KT) and Mirror Book Therapy (MBT) on clinical and electrophysiological parameters in patients with Bell’s palsy. Methods This randomized controlled trial included 31 patients divided into two groups: KT (n = 16) and MBT (n = 15). Both groups received their respective interventions (6 days/week for 3 weeks) in addition to conventional physiotherapy. Sunnybrook Facial Grading Score and Nerve Conduction Test (amplitude and latency of frontalis, nasalis, and mentalis muscles) were assessed pre- and post-intervention. Results Statistical analysis (SPSS v20) revealed significant intragroup improvements in all outcome measures (p < 0.05). Intergroup comparison showed MBT had superior improvements in amplitude of frontalis and reduced latency of nasalis. Both groups demonstrated significant improvement in the Sunnybrook grade shift. Conclusion This study suggests that KT and MBT, combined with conventional physiotherapy, positively impact clinical and electrophysiological parameters in Bell's Palsy patients. MBT showed superior results in select Nerve Conduction Test parameters.

Screening for Diabetic Peripheral Neuropathy: Subjective Versus Objective Measures.

This study compared subjective screening modalities recommended in diabetic foot screening guidelines for the detection of diabetic peripheral neuropathy (DPN) with an objective measure, the NC-Stat DPNCheck®. We assessed 63 participants (mean age 54.5 years ± 10.5) utilising subjective screening tools (Semmes-Weinstein 10-g monofilament, 128-Hz traditional tuning fork, neurothesiometer, O'Brien 128-Hz electronic tuning fork) and compared results with the objective automated sural nerve conduction test NC-Stat DPNCheck®. A significant difference was found in the number of limbs classified with DPN between all screening tools (P < .05). Therefore, this suggests that some screening modalities are more sensitive in diagnosing DPN than others, highlighting the importance of using multiple screening tools to a comprehensive understanding of the patient's neurological status. The findings also emphasize the need to incorporate objective measures in diabetic foot screening and encourage future research to establish a gold standard tool for DPN diagnosis.

Top 10 Clinical Pearls in Paraproteinemic Neuropathies.

Paraproteinemic neuropathies represent an important subset of peripheral neuropathies. Once identified, further evaluation into the paraproteinemic subtype, clinical exam pattern, and electrodiagnostic phenotype helps clarify if the paraproteinemia is coincidental or causal of the neuropathy, as not all paraproteinemias cause neuropathy. Of all paraproteinemias, immunoglobulin M (IgM)-associated peripheral neuropathy, or IgM neuropathy, is of particular importance as half of IgM neuropathies also harbor anti-myelin-associated glycoprotein antibodies, which produce a characteristic demyelinating pattern on nerve conduction testing. Immunoglobulin G and immunoglobulin A paraproteinemias are less strongly associated with peripheral neuropathy, except in the setting of multiple myeloma or osteosclerotic myeloma (POEMS syndrome), which have characteristic systemic features. In multiple myeloma, chemotherapy is more likely to result in neuropathy than the myeloma itself. Finally, the presence of systemic features (e.g., cardiomyopathy, nephropathy, recurrent carpal tunnel syndrome, and autonomic insufficiency) should raise concern for hereditary or acquired light (AL) chain amyloidosis. AL amyloidosis can occur in the setting of any light or heavy chain paraproteinemia. Central to the proper evaluation of paraproteinemic neuropathy is electrodiagnostic testing, which helps delineate axonal versus demyelinating paraproteinemic neuropathy, the latter often misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy.

The forearm elevation-compression test: a novel test for carpal tunnel syndrome.

As the time available to spend with patients decreases, a rapid test for bedside diagnosis of carpal tunnel syndrome (CTS) could be useful. We describe the forearm elevation-compression test (FECT) in this context. The FECT was assessed in 40 patients with clinically suspected carpal tunnel syndrome and compared to Tinel's and Phalen's signs. Routine electromyography and nerve conduction tests (EMG/NCT) were performed in all cases. In addition, 85 healthy controls were examined by FECT and compared to the patient group. All three provocative tests, particularly FECT were frequently positive in suspected CTS. Neurophysiological tests were normal in 5 of 40 cases of clinically suspected CTS and the FECT was positive in all of these suggesting a positive predictive value of 87.5% if one accepts EMG/NCT as the reference. Amongst the healthy controls 18 of 85 (21.2%) were positive on the FECT suggestive of a high false positive rate or subclinical disease. It is proposed that the FECT is a useful addition to the clinical examination of suspected CTS. Although the positive rate may be falsely elevated this is offset by restricting the latency for tingling onset to 10 seconds or less (FECT2).

Ultrasound Measurements of the Ulnar Nerve at the Medial Epicondyle Correlate With Electrodiagnostic Studies.

Cubital tunnel syndrome is the second most common compressive neuropathy in the upper extremity and is commonly evaluated with electrodiagnostic studies (EDS). Ultrasound (US) has emerged as a potentially more efficient alternative to EDS. The purpose of this study is to evaluate whether measurements of the cross-sectional area (CSA) of the ulnar nerve at the elbow correlate with EDS results. This study was a prospective analysis of patients who presented with signs and symptoms consistent of cubital tunnel syndrome, who received USs of the ulnar nerve at the elbow and nerve conduction tests. Pearson correlation coefficients were used to evaluate the correlation between ulnar nerve CSA and electrodiagnostic data. t Tests were used to evaluate statistical differences between the mean ulnar nerve CSA of patients with positive or negative nerve conduction study results. Youden Index was used to calculate the optimal cut-off point for US CSA based on maximal sensitivity and specificity. Statistical significance was based on a two-sided P less than .05. The association between increasing US CSA at the medial epicondyle with slowing of the conduction velocity of the ulnar motor nerve across the elbow was statistically significant (r = -0.35, P = .02). Patients with positive EDS tests had significantly larger nerve size than those with negative tests (all Ps < .03). A cut-off point of greater than or equal to 11 mm2 had a sensitivity of 70.83% and specificity of 66.67%. Larger ulnar nerve CSAs correlate with slowing of the conduction velocity on EDSs, and those with positive EDSs have larger nerve sizes than those with negative tests.

Cubital Tunnel Syndrome Due to Intraneural Ganglion Cysts of the Ulnar Nerve With Joint-Cyst Connection at the Elbow

BACKGROUND AND OBJECTIVES: The unifying articular theory suggests that intraneural ganglion cysts in the cubital tunnel arise from the elbow joint and are connected to the ulnar nerve through an articular branch. This study aimed to report our clinical experience with these cysts and our surgical findings and outcomes. METHODS: We retrospectively analyzed 13 patients who underwent surgery for cubital tunnel syndrome caused by an intraneural ganglion cyst of the ulnar nerve. Clinical symptoms, physical examination findings, nerve conduction testing electromyography, plain radiography, and ultrasonography were evaluated before and after surgery. MRI was performed in 2 patients. Cyst decompression, disconnection of the articular branch, and ulnar nerve transposition were performed in all. RESULTS: The mean follow-up was 29 months. After surgery, local elbow pain and Tinel sign disappeared in all patients, and the average Numerical Rating Scale dropped from 3.7 (2-5) to 0 (0-0). Two-point discrimination testing was normal (&lt;5.0 mm) in 12 patients. Wartenberg sign and “claw hand” deformity became negative, and muscle function and motor nerve conduction velocity were improved in 12 patients. The Medical Research Council muscle strength grade was M4 or M5 in the flexor carpi ulnaris, flexor digitorum profundus of the 4th and 5th digits, and first dorsal interosseous muscle in 12 patients. Postoperative spontaneous potentials including fibrillation and sharp waves, and motor unit recruitment in the first dorsal interosseous muscle were obviously improved. A cystic articular branch was confirmed during surgery in all patients. No cyst recurred clinically or on follow-up ultrasonography. CONCLUSION: The unifying articular theory of intraneural ganglion cysts applies to those located in the ulnar nerve at the elbow. Surgical disconnection of the articular nerve branch from the ulnar nerve eliminated the pathway for cyst recurrence and achieved good long-term outcomes.

Contralateral Facial Innervation in Healthy Subjects and in Patients with Peripheral Facial Palsy.

Background: We aimed to investigate the extent of the response of the orbicularis oris muscle to stimulation of the contralateral facial nerve both in patients with peripheral facial palsy (PFP) and in healthy subjects. Methods: EMG was performed at 2-6 weeks after the onset of PFP in the patient group and at any time in the healthy control group. We performed nerve conduction testing, electroneurography, and surface and needle EMG. Results: A total of 276 participants (patients/healthy controls: 218/58) were analyzed. The extent of the response of the contralateral orbicularis oris muscles to facial nerve stimulation was higher in healthy controls compared to that in the affected group. The response of the contralateral orbicularis oris muscles to stimulation of the paralyzed facial nerve was more extensive in those patients to whom glucocorticoid or physical therapy had been given. Cross-facial innervation in the orbicularis oris muscle extended up to 1.5 cm in one-third of healthy controls and was higher than that in those with PFP. Glucocorticoid or physical therapy seemed to improve cross-innervation in facial palsy. Conclusions: Our findings suggest that the stimulus leading to the contralateral muscular response is mediated through crossing axons rather than muscular fibers.

Diagnosing Carpal Tunnel Syndrome in the Clinic: A Questionnaire and Physical Assessment Versus a Portable Nerve Conduction Test Device

Diagnosing Carpal Tunnel Syndrome in the Clinic: A Questionnaire and Physical Assessment Versus a Portable Nerve Conduction Test Device

Preclinical Efficacy of Peripheral Nerve Regeneration by Schwann Cell-like Cells Differentiated from Human Tonsil-Derived Mesenchymal Stem Cells in C22 Mice.

Charcot-Marie-Tooth disease (CMT) is a hereditary disease with heterogeneous phenotypes and genetic causes. CMT type 1A (CMT1A) is a type of disease affecting the peripheral nerves and is caused by the duplication of the peripheral myelin protein 22 (PMP22) gene. Human tonsil-derived mesenchymal stem cells (TMSCs) are useful for stem cell therapy in various diseases and can be differentiated into Schwann cell-like cells (TMSC-SCs). We investigated the potential of TMSC-SCs called neuronal regeneration-promoting cells (NRPCs) for peripheral nerve and muscle regeneration in C22 mice, a model for CMT1A. We transplanted NRPCs manufactured in a good manufacturing practice facility into the bilateral thigh muscles of C22 mice and performed behavior and nerve conduction tests and histological and ultrastructural analyses. Significantly, the motor function was much improved, the ratio of myelinated axons was increased, and the G-ratio was reduced by the transplantation of NRPCs. The sciatic nerve and gastrocnemius muscle regeneration of C22 mice following the transplantation of NRPCs downregulated PMP22 overexpression, which was observed in a dose-dependent manner. These results suggest that NRPCs are feasible for clinical research for the treatment of CMT1A patients. Research applying NRPCs to other peripheral nerve diseases is also needed.

Characteristics of Abnormalities in Somatosensory Submodalities Observed in Residents Exposed to Methylmercury.

Hundreds of thousands of people living along the Yatsushiro Sea coast have been exposed to methylmercury from the contaminated water of the Chisso factory in Minamata. The most common neurological disorder caused by methylmercury is somatosensory disturbance, but very few studies have been conducted in the world to determine its pathophysiology and origin, including the Japanese cases, which have produced numerous intoxicated individuals. We have already shown in previous studies the body part where the disorder occurs and that its cause is not peripheral nerve damage but damage to the parietal lobes of the cerebrum. We reanalyzed the results of subjective symptoms, neurological findings, and quantitative sensory measurements in 197 residents (63.2 ± 10.7 years old) from contaminated areas exposed to methylmercury from seafood and 130 residents (63.7 ± 9.3 years old) from control areas, the same subjects as in previous studies, to determine the characteristics of somatosensory disturbance in detail. The most commonly affected sensory modalities were superficial peripheral touch and pain in the extremities, followed by two-point discrimination and deep senses, and in the most severe cases, full-body sensory dysfunction and impairment of all sensory submodalities. The severity of sensory submodalities correlated with each other but not with peripheral nerve conduction test indices, further confirming the correctness of our assertion about the responsible foci of sensory disturbance. The health effects of chronic methylmercury toxicosis can be elucidated by a detailed examination of sensory deficits.

Long-Term Evaluation of Ulnar Nerve Function in Patients with Distal Humerus Fractures Treated with Open Reduction and Internal Fixation: A Nerve Conduction Study.

To investigate the long-term evaluation with electromyography of ulnar nerve function in patients with distal humerus fractures (DHFs) treated with open reduction and internal fixation. Retrospective cohort study. Level 1 trauma center. Fifty-two patients (20 men and 32 women) with DHFs. All patients underwent open reduction and internal fixation between 2002 and 2017 with a minimum five years' follow-up. The nerve conduction test was done for evaluation ulnar nerve function. Secondary outcomes were modified McGowan grading system for symptoms of ulnar neuropathy. The mean follow-up time was 112.7 ± 39 months after surgery. 28.8% (15) of the patients scored in Grade I, 30 (57.6%) in Grade II, and 7 (13.6%) in Grade III on the affected side according to McGowan grading scale. According to EMG results, 40.1% (21/52) of patients had abnormal results. There was significant difference between fracture and unaffected side regarding ulnar nerve motor wrist CMAP amplitude, motor below elbow CMAP amplitude, above elbow CMAP amplitude, above elbow MNCV, and sensory wrist amplitude. Ulnar nerve motor wrist-ADM latency (p = 0.01; r = 0.446) and ulnar nerve sensory conduction velocity (p < 0.001, r = -0.504) were significant correlation with McGowan grading scale. There were significant difference ulnar nerve motor amplitude and sensory wrist amplitude between fracture and unaffected side. A mean decrease in sensory amplitude of fracture elbow was found 25% compared to the unaffected side in our result. Diagnostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Systemic biomarkers of microvascular alterations in type 1 diabetes associated neuropathy and nephropathy - A prospective long-term follow-up study

IntroductionThis study aimed to investigate circulating biomarkers associated with the risk of developing diabetic peripheral neuropathy (DPN) and nephropathy in type 1 diabetes (T1D). Materials and methodsPatients with childhood-onset T1D (n = 49, age 38.3 ± 3.8 yrs.) followed prospectively were evaluated after 30 years of diabetes duration. DPN was defined as an abnormality in nerve conduction tests. Matrix metalloproteinase-9 (MMP-9) and its tissue inhibitor TIMP-1, neutrophil gelatinase-associated lipocalin-2 (NGAL), soluble P-selectin (sP-selectin), estimated GFR (eGFR), micro/macroalbuminuria and routine biochemistry were assessed. For comparison, control subjects were included (n = 30, age 37.9 ± 5.5 yrs.). ResultsIn all, twenty-five patients (51 %) were diagnosed with DPN, and nine patients (18 %) had nephropathy (five microalbuminuria and four macroalbuminuria). Patients with DPN had higher levels of TIMP-1 (p = 0.036) and sP-selectin (p = 0.005) than controls. Patients with DPN also displayed higher levels of TIMP-1 compared to patients without DPN (p = 0.035). Patients with macroalbuminuria had kidney disease stage 3 with lower eGFR, higher levels of TIMP-1 (p = 0.038), and NGAL (p = 0.002). In all patients, we found only weak negative correlations between eGFR and TIMP-1 (rho = −0.304, p = 0.040) and NGAL (rho = −0.277, p = 0.062, ns), respectively. MMP-9 was higher in patients with microalbuminuria (p = 0.021) compared with normoalbuminuric patients. ConclusionsOur findings indicate that TIMP-1 and MMP-9, as well as sP-selectin and NGAL, are involved in microvascular complications in T1D. Monitoring and targeting these biomarkers may be a potential strategy for treating diabetic nephropathy and neuropathy.

Ultrasonic measurement for the diagnosis of cubital tunnel syndrome: A study in the Hong Kong Chinese population

Introduction: Ulnar nerve enlargement is observed in ultrasound (USG) in patients with cubital tunnel syndrome (CuTS). This study aims to compare the ultrasound size of the ulnar nerve between CuTS patients and control subjects, to find the cut off size for diagnosis, and to validate the use of USG as an adjunct in CuTS diagnosis. Materials and Methods: There were 23 elbows with clinical and nerve conduction test (NCT) confirmed CuTS, and 42 elbows in the control group. Cases with elbow deformities, old ulnar nerve injuries and postoperative cases were excluded. The ulnar nerve cross sectional area (CSA) was measured at 6 different levels and positions: over the medial epicondyle (ME) in elbow flexion/extension, 2 cm and 5 cm distal to the ME, 2 cm and 5 cm proximal to the ME. A cut off CSA value for CuTS diagnosis was derived. Correlation between ulnar nerve CSAs and NCT was analysed. Results: The age and gender distribution were similar in both groups (61.2 vs 56.6; M &gt; F, p &gt; 0.05). The mean CSA of the CuTS group vs control group was 19.2 mm2 vs 7.0 mm2, 19.5 mm2 vs 7.1 mm2, 20.8 mm2 vs 8.1 mm2 at ME flexion, ME extension and maximal CSA respectively. The derived CSA cut off value for CuTS at ME flexion, ME extension and maximal CSA were 10.5 mm2, 11.5 mm2, and 15 mm2 respectively. The CSA difference at different levels between the 2 groups were significant except at 5 cm proximal to ME. A strong negative correlation was seen between the CSA and the across elbow nerve conduction velocity, with correlation coefficient of −0.748 at ME flexion, −0.654 at ME extension and -0.676 at maximal CSA. Conclusion: USG can be used as an adjunct for the diagnosis of CuTS with high accuracy and patient safety. It can also be used to delineate possible anatomical etiologies at the cubital tunnel.

Temporal serum metabolomic and lipidomic analyses distinguish patients with access-related hand disability following arteriovenous fistula creation

For end-stage kidney disease (ESKD) patients, hemodialysis requires durable vascular access which is often surgically created using an arteriovenous fistula (AVF). However, some ESKD patients that undergo AVF placement develop access-related hand dysfunction (ARHD) through unknown mechanisms. In this study, we sought to determine if changes in the serum metabolome could distinguish ESKD patients that develop ARHD from those that have normal hand function following AVF creation. Forty-five ESKD patients that underwent first-time AVF creation were included in this study. Blood samples were obtained pre-operatively and 6-weeks post-operatively and metabolites were extracted and analyzed using nuclear magnetic resonance spectroscopy. Patients underwent thorough examination of hand function at both timepoints using the following assessments: grip strength manometry, dexterity, sensation, motor and sensory nerve conduction testing, hemodynamics, and the Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire. Nineteen of the forty-five patients displayed overt weakness using grip strength manometry (P < 0.0001). Unfortunately, the serum metabolome was indistinguishable between patients with and without weakness following AVF surgery. However, a significant correlation was found between the change in tryptophan levels and the change in grip strength suggesting a possible role of tryptophan-derived uremic metabolites in post-AVF hand-associated weakness. Compared to grip strength, changes in dexterity and sensation were smaller than those observed in grip strength, however, post-operative decreases in phenylalanine, glycine, and alanine were unique to patients that developed signs of motor or sensory disability following AVF creation.

OA18 Small vessel and large vessel vasculitis due to rheumatoid vasculitis: a case of mononeuritis multiplex with pulseless foot leading to amputation

Abstract Introduction Rheumatoid vasculitis occurs in patients with longstanding severe seropositive rheumatoid arthritis. The incidence has reduced drastically with availability of more effective medication. Rheumatoid vasculitis primarily affects small and medium vessels, and rarely large vessels as well. Case description This 77-year-old lady with deforming seropositive rheumatoid arthritis was stable on methotrexate under the care of multiple Trusts for the last 30 years. Methotrexate had been stopped a year ago due to pancytopenia and she was started on prednisolone and hydroxychloroquine. While on these medications she flared with arthralgia and had difficulty in walking attributed to ankle synovitis. She was started on adalimumab, after 3 doses of which she developed widespread skin psoriasis leading to discontinuation. Subsequently, she was admitted to our Trust with left foot gangrene and was referred to rheumatology. Further questioning revealed that numbness in both lower limbs, inability to walk and frequent falls had started following the cessation of methotrexate. On examination, there was bilateral foot drop, worse on the right side. The left foot was gangrenous with diminished pulses in dorsalis pedis and posterior tibial arteries with preserved all other pulses. There was no vasculitic rash or nail fold infarcts. Psoriatic rash had resolved and there was no active synovitis in the joints. Nerve conduction test revealed severe ongoing axonal degenerative type sensory and motor polyneuropathy in the lower limbs without any upper limb involvement. CT angiogram showed multifocal stenoses, more so in the left common iliac, external iliac and superficial femoral artery. There were no conventional vascular risk factors such as hypertension, diabetes, dyslipidaemia or smoking, and there was no past history of cardiovascular disease. PET-CT scan did not reveal any features of vasculitis or aortitis. The vasculitis screening was negative including cryoglobulins. Initially she was on IV antibiotics for left foot cellulitis, hence she was managed with steroids without any potent immunosuppressive agents. Subsequently, she underwent left sided below knee amputation. After the wound had healed, she was started on IV rituximab with low-dose methotrexate and steroid taper. Discussion Although rare, rheumatoid vasculitis can cause significant morbidity and complications. Rheumatoid vasculitis tends to occur in patients with so-called ‘burnt-out’ RA and strongly associated with seropositivity. While it can affect many organ systems, skin and the peripheral nervous system are most frequently affected. Cutaneous vasculitis occurs in around 90% of patients and is the most common clinical manifestation. This includes parable purpura, leg ulcers and nailfold infarcts. It may lead to digital ischaemia, necrosis and gangrene if large vessels are involved. Another common manifestation is a vasculitic neuropathy due to involvement of the vaso nervorum. Around 40% have a sensory neuropathy and 20% have a mixed sensory motor neuropathy like the index patient. Ocular manifestations such as scleritis have been described and may be the first indication of rheumatoid vasculitis. Aortitis in rheumatoid vasculitis may present with aortic insufficiency and aneurysm formation. Pericarditis has also been described as a possible disease manifestation in patients with rheumatoid vasculitis. Our patient did not have any of these clinical features and the PET-CT scan was unremarkable, which could have been due to the steroids she was on prior to the scan. Clinically, there was pulseless gangrene of the left foot with CT angiogram showing multiple stenosis suggesting large vessel involvement in the absence of conventional vascular risk factors. Renal involvement in rheumatoid vasculitis is very rare. PAN like renal involvement and glomerulonephritis has been described. Our patient did not have any haematuria or any significant proteinuria, and imaging did not reveal any renovascular aneurysms. Isolated nailfold infarcts do not require any systemic treatment. Systemic involvement with rheumatoid vasculitis requires aggressive treatment with high-dose steroids with cyclophosphamide or rituximab. Our patient had neuropathy and gangrene of the left foot, hence she was started on rituximab accordingly. Key learning points Rheumatoid vasculitis is a rare but serious complication which can present with a vasculitic neuropathy/mononeuritis multiplex. Rarely, it can cause ischemia and gangrene. If there is systemic involvement, aggressive therapy with steroids, cyclophosphamide or rituximab is needed.

Case Report: Lower Extremity Paresthesia and Pain with Diabetic Polyneuropathy Combated with Complex Korean Medical Treatment

Background: Diabetic polyneuropathy is the most common complication in diabetics, occurring in 50% of all cases. About 10-20% of all diabetics are accompanied by neurological pain, showing a tendency to increase with age. Clinical aspects are very diverse, from mild abnormalities on nerve conduction tests to severe abnormalities in all sensory, motor, and autonomic nerves; however, sensory symptoms usually precede motor symptoms. Patients typically express sensory symptoms, such as positive and negative symptoms, which decrease the quality of life and have marked clinical implications, such as increased morbidity and mortality. Although Western medical drugs, such as tricyclic antidepressants, anticonvulsants, and narcotic analgesics, are used for diabetic polyneuropathy, a standard treatment has not been established.Case report: A 65-year-old male with paresthesia and pain due to diabetic polyneuropathy was treated with &lt;i&gt;Uchashinki-hwan&lt;/i&gt;, acupuncture, electroacupuncture, moxibustion, and Jungsongouhyul pharmacopuncture for 10 days. We used the Toronto Clinical Neuropathy Scoring System, EuroQol-5 Dimension, and Visual Analog Scale to evaluate symptoms. Subsequently, the Neuropathy Scoring System, EuroQol-5 Dimension, and subjective discomfort improved.Conclusion: The present case report suggests that combined Korean medicine treatment might be an effective treatment for paresthesia and pain with diabetic polyneuropathy. Several follow-up studies should be conducted to clarify the effectiveness of the treatment.

Anti-Tumor Effect and Neurotoxicity of Ethanol Adjuvant Therapy after Surgery of a Soft Tissue Sarcoma.

Wide resection is the main treatment for sarcomas; however, when they are located near major nerves, their sacrifices might affect limb function. The efficacy of ethanol adjuvant therapy for sarcomas has not been established. In this study, the anti-tumor effect of ethanol, as well as its neurotoxicity, were assessed. In vitro anti-tumor effect of ethanol as evaluated using MTT, wound healing, and invasion assays on a synovial sarcoma cell line (HS-SY-II). In vivo, an assessment was conducted in nude mice (implanted with subcutaneous HS-SY-II) treated with different ethanol concentrations after surgery with a close margin. Sciatic nerve neurotoxicity was assessed with electrophysiological and histological examination. In vitro, ethanol concentrations at 30% and higher showed cytotoxic effects in MTT assay and markedly reduced migration and invasive ability of HS-SY-II. In vivo, both 30% and 99.5% ethanol concentrations, compared to 0% concentration, significantly reduced the local recurrence. However, in the group treated with 99.5% ethanol, nerve conduction tests showed prolonged latency and decreased amplitude, and morphological changes suggestive of nerve degeneration were observed in the sciatic nerve, while the 30% ethanol did not cause neurological damage. In conclusion, 30% is the optimal concentration for ethanol adjuvant therapy after close-margin surgery for sarcoma.

Association between brachial-ankle pulse wave velocity and microvascular complications in type 2 diabetes mellitus

Purpose/AimTo investigate the relationship between brachial-ankle pulse wave velocity (baPWV) and microvascular complications in type 2 diabetes mellitus (T2DM).Materials and methodsFrom 2019 to 2021, our hospital enrolled 322 patients with T2DM. Clinical information and biochemical indicators of patients were collected from the inpatient electronic medical record system and analyzed retrospectively. Fundus photography, nerve conduction testing, and sensory threshold measurement were all conducted on the subjects. We measured the pulse wave velocity on both sides of the arm and ankle, then averaged the results. The enrolled cases were divided into two groups based on the baPWV: Group 1 (<the reference cutoff value, n = 160) and Group 2 (≥ the reference cutoff value, n = 162). The association between associated factors and baPWV abnormalities was investigated using a logistic regression model.ResultsGroup 2 had higher Systolic Blood Pressure(SBP), Diastolic Blood Pressure (DBP), duration of diabetes, Fasting Plasma Glucose (FPG), Blood Urea Nitrogen (BUN) and Serum Uric Acid (SUA) than group 1 (all p < 0.05). The prevalence of diabetic retinopathy, peripheral neuropathy and nephropathy in group 2 was higher compared to group 1 (p < 0.05). After classifying subjects according to the presence or absence of diabetic complications, we found that the baPWV of the Diabetic Peripheral Neuropathy (DPN) group and Diabetic Nephropathy (DN) group was noticeably higher than that of non-DPN group and non-DN group, respectively (both p < 0.05). The baPWV increased with the aggravation of Diabetic Retinopathy (DR) (p < 0.05). Multivariate logistic regression analysis showed that DBP (OR 1.039, 95%CI 1.010–1.068 p = 0.008), duration of diabetes (OR 1.059, 95%CI 1.017–1.103 p = 0.006), FPG (OR 1.104, 95%CI 1.025–1.188 p = 0.009) and BUN (OR 1.128, 95%CI 1.001–1.270 p = 0.048) were all independently and positively associated with baPWV.ConclusionsThe baPWV is strongly associated with microvascular complications of T2DM. The DBP, duration of diabetes, FPG, and BUN were all independent associated factors of baPWV.

Unusual case of mononeuropathy in child: Intraneural perineurioma of sciatic nerve

Intraneural perineurioma is a benign nerve sheath tumor of the perineurium characterized by focal perineural cell proliferation. It is primarily seen in young adults. Early childhood presentation is uncommon. We present this interesting case in a child with overall clinical assessment, nerve conduction tests, and magnetic resonance findings in keeping with intraneural perineurioma of the sciatic nerve. It is essential to recognize this potentially treatable disease entity in the early childhood and exclude other causes of nerve palsy.

Novel Homozygous Variant in COQ7 in Siblings With Hereditary Motor Neuropathy.

Coenzyme Q10 (CoQ10) is an important electron carrier and antioxidant. The COQ7 enzyme catalyzes the hydroxylation of 5-demethoxyubiquinone-10 (DMQ10), the second-to-last step in the CoQ10 biosynthesis pathway. We report a consanguineous family presenting with a hereditary motor neuropathy associated with a homozygous c.1A > G p.? variant of COQ7 with abnormal CoQ10 biosynthesis. Affected family members underwent clinical assessments that included nerve conduction testing, histologic analysis, and MRI. Pathogenicity of the COQ7 variant was assessed in cultured fibroblasts and skeletal muscle using a combination of immunoblots, respirometry, and quinone analysis. Three affected siblings, ranging from 12 to 24 years of age, presented with a severe length-dependent motor neuropathy with marked symmetric distal weakness and atrophy with normal sensation. Muscle biopsy of the quadriceps revealed chronic denervation pattern. An MRI examination identified moderate to severe fat infiltration in distal muscles. Exome sequencing demonstrated the homozygous COQ7 c.1A > G p.? variant that is expected to bypass the first 38 amino acid residues at the n-terminus, initiating instead with methionine at position 39. This is predicted to cause the loss of the cleavable mitochondrial targeting sequence and 2 additional amino acids, thereby preventing the incorporation and subsequent folding of COQ7 into the inner mitochondrial membrane. Pathogenicity of the COQ7 variant was demonstrated by diminished COQ7 and CoQ10 levels in muscle and fibroblast samples of affected siblings but not in the father, unaffected sibling, or unrelated controls. In addition, fibroblasts from affected siblings had substantial accumulation of DMQ10, and maximal mitochondrial respiration was impaired in both fibroblasts and muscle. This report describes a new neurologic phenotype of COQ7-related primary CoQ10 deficiency. Novel aspects of the phenotype presented by this family include pure distal motor neuropathy involvement, as well as the lack of upper motor neuron features, cognitive delay, or sensory involvement in comparison with cases of COQ7-related CoQ10 deficiency previously reported in the literature.