Machine learning analysis with population data for the associations of preterm birth with temporomandibular disorder and gastrointestinal diseases.

This study employs machine learning analysis with population data for the associations of preterm birth (PTB) with temporomandibular disorder (TMD) and gastrointestinal diseases. The source of the population-based retrospective cohort was Korea National Health Insurance claims for 489,893 primiparous women with delivery at the age of 25-40 in 2017. The dependent variable was PTB in 2017. Twenty-one predictors were included, i.e., demographic, socioeconomic, disease and medication information during 2002-2016. Random forest variable importance was derived for finding important predictors of PTB and evaluating its associations with the predictors including TMD and gastroesophageal reflux disease (GERD). Shapley Additive Explanation (SHAP) values were calculated to analyze the directions of these associations. The random forest with oversampling registered a much higher area under the receiver-operating-characteristic curve compared to logistic regression with oversampling, i.e., 79.3% vs. 53.1%. According to random forest variable importance values and rankings, PTB has strong associations with low socioeconomic status, GERD, age, infertility, irritable bowel syndrome, diabetes, TMD, salivary gland disease, hypertension, tricyclic antidepressant and benzodiazepine. In terms of max SHAP values, these associations were positive, e.g., low socioeconomic status (0.29), age (0.21), GERD (0.27) and TMD (0.23). The inclusion of low socioeconomic status, age, GERD or TMD into the random forest will increase the probability of PTB by 0.29, 0.21, 0.27 or 0.23. A cutting-edge approach of explainable artificial intelligence highlights the strong associations of preterm birth with temporomandibular disorder, gastrointestinal diseases and antidepressant medication. Close surveillance is needed for pregnant women regarding these multiple risks at the same time.

Immediate retrograde amnesia induced by midazolam: a prospective, nonrandomized cohort study.

BACKGROUND: Midazolam, a short-acting benzodiazepine, has sedative, anxiolytic, amnestic, and anticonvulsant effect. Given its advantages of rapid onset, short duration, and low toxicity, midazolam is optimal for any procedural sedation. Midazolam is known to cause anterograde amnesia; however, the possibility of retrograde amnesia has also been raised. This prospective cohort, nonrandomized study evaluated the presence and extent of retrograde amnesia induced by midazolam during cesarean delivery. METHODS: One-hundred parturients scheduled for elective cesarean delivery under spinal anesthesia were enrolled. As soon as giving birth, 6 picture cards were shown to the patients in one min intervals, and then midazolam (0.1 mg/kg) was given or not according to the patients' preference. This overall retrograde recall rate of six cards was the primary outcome of our study, which was asked by a blinded investigator. RESULTS: The overall retrograde card recall rate was lower in the midazolam group compared to the control group (77.0 ± 13.4 vs. 87.7 ± 3.9%, p <0.001), especially at one min before midazolam administration (58 vs. 88%, p <0.001). Decreased memory trend was observed as time progressed toward midazolam administration in the midazolam group (p = 0.035). More patients answered 'yes' to the factitious event in the midazolam group than in the control group (26% vs. 4%, p = 0.004). CONCLUSION: Intravenous midazolam could cause a brief-period retrograde amnesia in visual and event memory. Moreover, there were more spurious reports of intraoperative factitious events in the midazolam group, implying that episodic memories were also affected by midazolam.

Immediate retrograde amnesia induced by midazolam: A prospective, non-randomised cohort study.

BACKGROUND: Midazolam, a short-acting benzodiazepine, has sedative, anxiolytic, amnestic and anticonvulsant effects. Given its advantages of rapid onset, short duration and low toxicity, midazolam is optimal for any procedural sedation. Midazolam is known to cause anterograde amnesia; however, the possibility of retrograde amnesia has also been raised. This prospective cohort, non-randomised study evaluated the presence and extent of retrograde amnesia induced by midazolam during caesarean delivery. METHODS: One hundred parturients scheduled for elective caesarean delivery under spinal anaesthesia were enrolled. As soon as giving birth, six picture cards were shown to the patients in 1-min intervals, and then midazolam (0.1 mg/kg) was given or not according to the patients' preference. This overall retrograde recall rate of six cards was the primary outcome of our study, which was asked by a blinded investigator. RESULTS: The overall retrograde card recall rate was lower in the midazolam group compared with the control group (77.0 ± 13.4 vs. 87.7 ± 3.9%, P < .001), especially at 1 minute before midazolam administration (58% vs. 88%, P < .001). Decreased memory trend was observed as time progressed towards midazolam administration in the midazolam group (P = .035). More patients answered 'yes' to the factitious event in the midazolam group than in the control group (26% vs. 4%, P = .004). CONCLUSION: Intravenous midazolam could cause a brief-period retrograde amnesia in visual and event memory. Moreover, there were more spurious reports of intraoperative factitious events in the midazolam group, implying that episodic memories were also affected by midazolam.

Association of neuromuscular reversal by sugammadex and neostigmine with 90-day mortality after non-cardiac surgery.

BACKGROUND: Reversing a neuromuscular blockade agent with sugammadex is known to lessen postoperative complications by reducing postoperative residual curarization. However, its effects on 90-day mortality are unknown. Therefore, this study aimed to compare the effects of sugammadex and neostigmine in terms of 90-day mortality after non-cardiac surgery. METHODS: This retrospective cohort study analyzed the medical records of adult patients aged 18 years or older who underwent non-cardiac surgery at a single tertiary care hospital between 2011 and 2016. Propensity score matching and Cox regression analysis were used to investigate the effectiveness of sugammadex and neostigmine in lowering 90-day mortality after non-cardiac surgery. RESULTS: A total of 65,702 patients were included in the analysis (mean age: 52.3 years, standard deviation: 15.7), and 23,532 of these patients (35.8%) received general surgery. After propensity score matching, 14,179 patients (3906 patients from the sugammadex group and 10,273 patients from the neostigmine group) were included in the final analysis. Cox regression analysis in the propensity score-matched cohort showed that the risk of 90-day mortality was 40% lower in the sugammadex group than in the neostigmine group (hazard ratio: 0.60, 95% confidence interval: 0.37, 0.98; P = 0.042). These results were similar in the multivariable Cox regression analysis of the entire cohort (hazard ratio: 0.62, 95% confidence interval: 0.39, 0.96; P = 0.036). CONCLUSIONS: This retrospective cohort study suggested that reversing rocuronium with sugammadex might be associated with lower 90-day mortality after non-cardiac surgery compared to neostigmine. However, since this study did not evaluate quantitative neuromuscular function in the postoperative period due to its retrospective design, the results should be interpreted carefully. Future prospective studies with quantitative neuromuscular monitoring in the postoperative period should be performed to confirm these results.

Effects of deep neuromuscular blockade on the stress response during laparoscopic gastrectomy Randomized controlled trials.

Maintaining deep neuromuscular block during surgery improves surgical space conditions. However, its effects on patient outcomes have not been well documented. We examined whether maintaining deep neuromuscular blockade during surgery could decrease the stress response compared to moderate neuromuscular blockade. Patients undergoing laparoscopic gastrectomy were randomly allocated to either the moderate (train-of-four counts of 1-2) or deep (post-tetanic counts of 1-2) neuromuscular blockade group. The primary outcome variable was the postoperative blood level of interleukin-6, and the secondary outcome variables were intraoperative or postoperative blood levels of tumor necrosis factor-α, interleukin-1ß, interleukin-8, and C-reactive protein. A total of 96 patients were recruited and 88 (44 in each group) were included in the analyses. The levels of tumor necrosis factor-α and interleukin-1ß measured at the end of surgery, interleukin-6 and interleukin-8 measured at 2 h postoperatively, and C-reactive protein measured at 48 h postoperatively were all significantly increased compared to the preoperative values, but there were no differences between the moderate and deep neuromuscular block groups. We found no differences in surgical stress response measured using determining levels of interleukin-6 and other mediators released between the moderate and deep neuromuscular blockade groups in patients undergoing laparoscopic gastrectomy.

Intraoperative Nefopam Reduces Acute Postoperative Pain after Laparoscopic Gastrectomy: a Prospective, Randomized Study.

BACKGROUND: We assessed whether intraoperative nefopam would reduce opioid consumption and relieve postoperative pain in patients undergoing laparoscopic gastrectomy. METHODS: The 60 enrolled patients were randomly assigned to the control (n = 32) or nefopam (n = 28) group. All patients were blinded to their group assignment. We administered 100 ml of normal saline only (control group) or 20 mg of nefopam mixed in 100 ml normal saline (nefopam group) after anesthesia induction and at the end of surgery. The cumulative amount of fentanyl via intravenous patient-controlled analgesia (PCA), incidence of rescue analgesic medication, and numerical rating scale (NRS) for postoperative pain were evaluated along with the total remifentanil consumption. RESULTS: The mean infusion rate of remifentanil was significantly lower in the nefopam group (0.08 ± 0.05 µg/kg/min) than in the control group (0.13 ± 0.06 µg/kg/min) (P < 0.001). Patients in the nefopam group required less fentanyl via intravenous PCA than those in the control group during the first 6 h after surgery (323.8 ± 119.3 µg vs. 421.2 ± 151.6 µg, P = 0.009). Additionally, fewer patients in the nefopam group than in the control group received a rescue analgesic during the initial 6 h postoperatively (78.6 vs. 96.9%, P = 0.028). The NRS measured while patients were in the post-anesthetic care unit was significantly lower in the nefopam group than in the control group (3.8 ± 1.1 vs. 4.8 ± 1.4, P = 0.012). The subsequent NRS obtained after patients had been transferred to the general ward was comparable between the two groups during the following postoperative period. CONCLUSIONS: Intraoperative nefopam decreased postoperative pain and opioid consumption in the acute postoperative period after laparoscopic gastrectomy. Hence, nefopam may be considered as a component of multimodal analgesia after laparoscopic gastrectomy.

Characteristics of South Korean Patients with Hereditary Transthyretin Amyloidosis

BACKGROUND AND PURPOSE: This retrospective cross-sectional study included 18 patients from unrelated families harboring mutations of the transthyretin gene (TTR), and analyzed their characteristics and geographical distribution in South Korea. METHODS: The included patients had a diagnosis of systemic amyloidosis, clinical symptoms, such as amyloid neuropathy or cardiomyopathy, and confirmation of a TTR gene mutation using genetic analysis recorded between April 1995 and November 2014. RESULTS: The mean age at disease onset was 49.6 years, and the mean disease duration from symptom onset to diagnosis was 3.67 years. Fifteen of the 18 patients were classified as mixed phenotype, 2 as the neurological phenotype, and only 1 patient as the cardiac phenotype. The most-common mutation pattern in South Korea was Asp38Ala, which was detected in eight patients. Thirteen patients reported their family hometowns, and five of the eight harboring the Asp38Ala mutation were from the Gyeongsang province in southeast Korea. The other eight patients exhibited a widespread geographical distribution. A particularly noteworthy finding was that the valine at position 30 (Val30Met) mutation, which was previously reported as the most-common TTR mutation worldwide and also the most common in the Japanese population, was not detected in the present South Korean patients. CONCLUSIONS: South Korean patients with hereditary TTR amyloidosis exhibited heterogeneous TTR genotypes and clinical phenotypes. The findings of this study suggest that the distribution of TTR amyloidosis in South Korea is due to de novo mutations and/or related to the other countries in East Asia.

Magnesium sulphate improves pulmonary function after video-assisted thoracoscopic surgery: A randomised double-blind placebo-controlled study.

BACKGROUND: Reduced lung volumes are unavoidable after lung resection surgery. Magnesium sulphate (MgSO4) administration has been reported to reduce the requirement for neuromuscular blocking drugs and postoperative analgesics in surgical patients. OBJECTIVE: To investigate the effect of MgSO4 on pulmonary function after video-assisted thoracoscopic surgery (VATS). DESIGN: A randomised, double-blind, placebo-controlled trial. SETTING: A university tertiary care centre. PATIENTS: Sixty-six patients scheduled for pulmonary lobectomy or segmentectomy via VATS. INTERVENTION: Patients were allocated to one of two groups: the Mg (MgSO4 50 mg kg intravenously for 10 min, followed by a continuous infusion of 15 mg kg h during surgery) or the control (same volume of 0.9% saline). MAIN OUTCOME MEASURES: Pulmonary function tests [forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC) and peak expiratory flow rate] were measured before surgery and at three time points after surgery (2, 24 and 48 h postoperatively) using a portable spirometer. Twelve months after surgery, pulmonary function test values were repeated at a regular outpatient follow-up visit. RESULTS: FEV1 at 24 (1.7 ±â€Š0.6 vs. 1.3 ±â€Š0.5 l, P = 0.033) and 48 h (1.7 ±â€Š0.6 vs. 1.4 ±â€Š0.5 l, P = 0.021) and FVC at 24 (2.0 ±â€Š0.8 vs. 1.6 ±â€Š0.6 l, P = 0.038) and 48 h (2.2 ±â€Š0.8 vs. 1.7 ±â€Š0.7 l, P = 0.008) after surgery were significantly greater in the Mg group. Patients in the Mg group required less rocuronium than those in the control group (64.2 ±â€Š19.9 vs. 74.9 ±â€Š20.3 mg, respectively; P = 0.041). Consumption of postoperative patient-controlled analgesia was also significantly less at 24 and 48 h after surgery in the Mg group (P = 0.022 and 0.015, respectively), although pain scores and rescue analgesics were comparable. Five patients in the control group were diagnosed with postoperative pneumonia using clinical and radiological criteria before discharge. FEV1 and FVC at 12 months after surgery were not different between the two groups. CONCLUSION: Intraoperative administration of MgSO4 improved pulmonary function and reduced the need for rocuronium and postoperative analgesics in patients who underwent VATS. TRIAL REGISTRATION: cris.nih.go.kr identifier: KCT0001410.

The impact of irrigating fluid absorption on blood coagulation in patients undergoing transurethral resection of the prostate: A prospective observational study using rotational thromboelastometry.

Although endoscopic transurethral resection of the prostate (TURP) is a well-established procedure as a treatment for benign prostatic hyperplasia, its complications remain a concern. Among these, coagulopathy may be caused by the absorption of irrigating fluid. This study aimed to evaluate such phenomenon using a rotational thromboelastometry (ROTEM).A total of 20 patients undergoing TURP participated in this study. A mixture of 2.7% sorbitol-0.54% mannitol solution and 1% ethanol was used as an irrigating fluid, and fluid absorption was measured via the ethanol concentration in expired breath. The effects on coagulation were assessed by pre- and postoperative laboratory blood tests, including hemoglobin, hematocrit, platelet count, international normalized ratio of prothrombin time (PT-INR), activated partial thromboplastin time, electrolyte, and ROTEM.INTEM-clotting time (INTEM-CT) was significantly lengthened by 14% (P = 0.001). INTEM-α-angle was significantly decreased by 3% (P = 0.011). EXTEM-clot formation time was significantly prolonged by 18% (P = 0.008), and EXTEM-maximum clot firmness (EXTEM-MCF) was significantly decreased by 4% (P = 0.010). FIBTEM-MCF was also significantly decreased by 13% (P = 0.015). Moreover, hemoglobin (P < 0.001), hematocrit (P < 0.001), platelet counts (P < 0.001), potassium (P = 0.024), and ionized calcium (P = 0.004) were significantly decreased, while PT-INR (P = 0.001) was significantly increased after surgery. The amount of irrigating fluid absorbed was significantly associated with the weight of resected prostatic tissue (P = 0.001) and change of INTEM-CT (P < 0.001).As shown by the ROTEM analysis, the irrigating fluid absorbed during TURP impaired the blood coagulation cascade by creating a disruption in the coagulation factor activity or by lowering the coagulation factor concentration via dilution.

Repetitive Nerve Stimulation in MuSK-Antibody-Positive Myasthenia Gravis

BACKGROUND AND PURPOSE: Responses to repetitive nerve stimulation (RNS) in patients with muscle-specific tyrosine kinase (MuSK) antibody (Ab)-positive myasthenia gravis (MG) vary depending on the muscles tested. We analyzed the RNS responses of limb and facial muscles in MuSK-Ab-positive and acetylcholine receptor (AChR)-Ab-negative MG (MuSK MG) and MuSK-Ab-negative and AChR-Ab-negative [double-seronegative (DSN)] MG patients. METHODS: We retrospectively compared RNS responses between 45 MuSK MG and 29 DSN MG. RNS was applied to the abductor digiti minimi, flexor carpi ulnaris, trapezius, orbicularis oculi, and nasalis muscles. RESULTS: Abnormal RNS responses in limb muscles were observed in 22.2 and 58.6% of MuSK MG and DSN MG patients, respectively, with abnormal facial responses observed in 77.8 and 65.5%, and abnormal responses observed in any of the five muscles in 86.7 and 72.4%. Abnormal RNS responses in the abductor digiti minimi or flexor carpi ulnaris were less frequent in MuSK MG (8.9 and 15.6%, respectively) than in DSN MG (37.9 and 55.2%), whereas the findings for other muscles were not significantly different between the groups. Abnormal facial responses but normal limb responses were independently associated with MuSK MG (odds ratio=5.224, 95% confidence interval=1.300–20.990). CONCLUSIONS: Abnormal RNS responses primarily in facial muscles without involvement of limb muscles were more pronounced in MuSK MG than in DSN MG. RNS of both facial and limb muscles in AChR-Ab-negative MG can increase the test sensitivity and aid in early suspicion of MuSK MG.

Decremental Responses to Repetitive Nerve Stimulation in X-Linked Bulbospinal Muscular Atrophy

BACKGROUND AND PURPOSE: X-linked bulbospinal muscular atrophy (X-BSMA) is characterized by bulbar and spinal muscular weakness and fasciculations. Although X-BSMA is a motor neuronopathy, there are several reports of myasthenic symptoms or decremental responses to repetitive nerve stimulation (RNS). We report the results of applying the RNS test to 15 patients among 41 with genetically confirmed X-BSMA; these 15 patients complained of fatigue, ease of becoming tired, or early muscular exhaustion. METHODS: The 3-Hz RNS test was performed on the trapezius, nasalis, orbicularis oculi, flexor carpi ulnaris, and abductor digiti quinti muscles. A decrement greater than 10% was considered abnormal. Additionally, a pharmacologic response to neostigmine was identified in three patients. RESULTS: A significant decrement was observed in 67% of patients, and was most common in the trapezius muscle (nine cases). The decrement of the trapezius muscle response ranged from 15.9% to 36.9%. The decrement was inversely correlated with the amplitude of compound muscle action potentials at rest. Neostigmine injection markedly improved the decrement in three patients, who showed noticeable decremental responses to 3-Hz RNS. CONCLUSIONS: This study shows that myasthenic symptoms and abnormal decremental responses to low-rate RNS are common in X-BSMA.

Erratum: Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.

A Case of Lambert-Eaton Myasthenic Syndrome with Small-Cell Lung Cancer and Transient Increase in Anti-Acetylcholine-Receptor-Binding Antibody Titer

BACKGROUND: Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic neuromuscular junction disorder that is most frequently associated with small-cell lung cancer (SCLC). The titers of antibodies against voltage-gated calcium channels are frequently increased in LEMS, but only rarely is titer of anti-acetylcholine-receptor-binding antibodies (AChR-abs) increased. CASE REPORT: A 57-year-old male was admitted to our hospital due to dry mouth and eyes and progressive proximal limb weakness of 2 months duration. The results of a repetitive nerve stimulation test disclosed all criteria for the electrophysiological LEMS pattern, and the patient's AChR-abs titer was 0.587 nmol/L. At a follow-up performed 5 years after successful treatment of SCLC and LEMS, his AChR-abs titer had decreased to 0.001 nmol/L. CONCLUSIONS: We suggest that this was a case of transient pseudopositivity of AChR-abs in SCLC with LEMS.

Heterogeneous Characteristics of Korean Patients with Dysferlinopathy

Dysferlinopathy is caused by mutations in the DYSF gene. To characterize the clinical spectrum, we investigated the characteristics of 31 Korean dysferlinopathy patients confirmed by immunohistochemistry. The mean age of symptom onset was 22.23 +/- 7.34 yr. The serum creatine kinase (CK) was highly increased (4- to 101-fold above normal). The pathological findings of muscle specimens showed nonspecific dystrophic features and frequent inflammatory cell infiltration. Muscle imaging studies showed fatty atrophic changes dominantly in the posterolateral muscles of the lower limb. The patients with dysferlinopathy were classified by initial muscle weakness: fifteen patients with Miyoshi myopathy phenotype (MM), thirteen patients with limb girdle muscular dystrophy 2B phenotype (LGMD2B), two patients with proximodistal phenotype, and one asymptomatic patient. There were no differences between LGMD2B and MM groups in terms of onset age, serum CK levels and pathological findings. Dysferlinopathy patients usually have young adult onset and high serum CK levels. However, heterogeneity of clinical presentations and pathologic findings upon routine staining makes it difficult to diagnose dysferlinopathy. These limitations make immunohistochemistry currently the most important method for the diagnosis of dysferlinopathy.

Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.

Sequential Involvement of the Nervous System in Subacute Combined Degeneration

PURPOSE: Subacute combined degeneration (SCD) involves progressive degeneration of the spinal cord, optic nerve, and peripheral nerves. Vitamin B12 (VB12) is a co-factor in myelin synthesis. Because each cell that constitutes the myelin component in the central nervous system and peripheral nervous system is different, it is improbable that these cells undergo simultaneous degeneration. However, the sequence of degeneration in SCD has not been established. MATERIALS AND METHODS: In this study, we analysed medical records and electrophysiological data of patients who showed neurological symptoms and whose serum VB12 levels were lower than 200 pg/mL. RESULTS: We enrolled 49 patients in this study. Their mean VB12 level was 68.3 pg/mL. Somatosensory evoked potential (SEP) study showed abnormal findings in 38 patients. Of the 40 patients who underwent visual evoked potential (VEP) study, 14 showed abnormal responses. Eighteen patients showed abnormal findings on a nerve conduction study (NCS). In this study, abnormal posterior tibial nerve SEPs only were seen in 16 patients, median nerve SEPs only were seen in 3 patients, abnormal VEPs only in two, and abnormal NCS responses in one patient. No patient complained of cognitive symptoms. CONCLUSION: In SCD, degeneration appears to progress in the following order: lower spinal cord, cervical spinal cord, peripheral nerve/optic nerve, and finally, the brain.

A Case of Acute Polyneuropathy with Nephrotic Syndrome Showing Transient Proximal Sensory Conduction Defects

Acute sensorimotor polyneuropathy that resembles Guillain-Barre syndrome (GBS) is rarely accompanied with nephrotic syndrome, and its underlying immunological mechanisms are unclear. A 56-year-old man presented with simultaneous acute progressive symmetric sensorimotor polyneuropathy and proteinuria. A kidney biopsy revealed focal segmental glomerulosclerosis. Serial electrophysiologic studies showed only a transient proximal conduction block in the median nerve, stimulated somatosensory evoked potential and prolonged terminal latencies of the median and peroneal nerves. The patient's neurologic deficits and kidney dysfunction recovered with corticosteroid treatment. Our case showed that somatosensory evoked potential study can be an important objective tool in the diagnosis of acute polyneuropathy with normal distal nerve conduction and that corticosteroids should be considered in the initial treatment of GBS-resembling polyneuropathy associated with nephrotic syndrome.

Repetitive Nerve Stimulation Test in Amyotrophic Lateral Sclerosis with Predominant Oropharyngeal Manifestations

BACKGROUND AND PURPOSE: Amyotrophic lateral sclerosis (ALS) patients display easy fatigability and abnormal decrements on repetitive nerve stimulation (RNS) test of clinically involved limb muscles, which can result in ALS being misdiagnosed as myasthenia gravis. We retrospectively analyzed the RNS tests of ten ALS patients with only or predominant oropharyngeal symptoms without ocular or facial weakness. METHODS: RNS tests were performed on the abductor digiti quinti, flexor carpi ulnaris, orbicularis oculi (OO), nasalis and trapezius muscles at low-rate stimulation frequencies of 3 and 5-Hz. Decrements greater than 10% of the compound muscle action potential amplitude on the fifth stimulation compared to the first was regarded as abnormal. RESULTS: Six patients complained of muscular fatigue or diurnal fluctuation. Among the ten patients, three exhibited abnormal decrements during low-rate stimulation in the facial muscles but not in the limb muscles, two exhibited abnormal decrements in the OO and nasalis muscles, and one exhibited abnormal decrements in the OO muscle. CONCLUSIONS: These findings show that the facial muscles may be involved in some early oropharyngeal forms of ALS, although facial weakness may not be clinically evident. We confirm herein that abnormal decrement of facial muscles to RNS test cannot make a definite diagnose for myasthenia gravis.

Myopathy With Anti Signal Recognition Particle Antibody

Myopathies associated with anti-signal-recognition particle (SRP) antibodies usually present with severe muscle weakness and exhibit necrotizing myopathy with little inflammation pathologically. Here we report a case of a 61-year-old man who presented with subacute progressive proximal muscle weakness, dysarthria, and dysphagia. Although polymyositis was expected clinically, muscle biopsy revealed myopathic changes with degenerating fibers without definite inflammation. Further laboratory study revealed that the patient was positive for anti-SRP antibodies.

Clinical Heterogeneity in Korean Patients with Nemaline Myopathy

PURPOSE: Nemaline myopathy (NM) is a clinical heterogeneous congenital myopathy characterized by the presence of subsarcolemmal or cytoplasmic rod-like structures that call nemaline bodies in the muscle fibers. The purpose of this study was to investigate the clinical diversity and pathological features of Korean patients with NM. MATERIALS AND METHODS: Eight patients underwent analyses of clinical manifestations by a structured protocol. Diagnoses were established by a muscle biopsy. RESULTS: Two patients had the typical congenital type, which exhibited neonatal hypotonia and delayed motor milestone, and five patients had the childhood onset type, which exhibited mild gait disturbance as a first symptom. One patient had the adult onset type, which showed acute respiratory failure. Limb weakness was proximal-dominant occurred in six patients. Hyporeflexia was observed in most patients. Elongated faces and high arched palates and feet were also observed. On light microscopy, the nemaline bodies were observed in type 1 and 2 fibers. All patients showed type 1 predominance and atrophy. In the two cases in which ultrastructural studies were performed, typical nemaline rods and disorganized myofibrillar apparatus were detected. CONCLUSION: In conclusion, the eight Korean patients in this study with NM shared common clinical expressions such as proximal limb weakness, reduced deep tendon reflex, and dysmorphic features. This study, however, showed that clinical heterogeneity ranged from typical congenital, mildly affected childhood to the adult onset form with acute respiratory failure. The pathological findings in this study were in accordance with those of other previous reports.