Frequency of EMG abnormalities in idiopathic inflammatory myopathies under the EULAR/ACR classification criteria.

The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for idiopathic inflammatory myopathies (IIM) have been widely used in recent times. However, no studies have focused on electromyography (EMG) findings of IIM, considering the criteria. This study aimed to elucidate the frequency of EMG abnormalities, particularly fibrillation potentials and positive sharp waves (Fib/PSW), the most objective EMG findings of IIM. Clinical and EMG records of adult patients who were clinically diagnosed with polymyositis (PM), dermatomyositis (DM), amyopathic DM (ADM), or inclusion body myositis (IBM) were retrospectively reviewed and classified according to the EULAR/ACR classification criteria. The frequency of Fib/PSW in EMG was investigated in the recruited cases. Seventy-nine patients with clinically diagnosed IIM (44 with PM, 17 with DM, 7 with ADM, and 11 with IBM) were recruited. After classification using EULAR/ACR, 75 satisfied definite or probable IIM (61 and 14, respectively), and the frequency of Fib/PSW in this group was 95%. Furthermore, the remaining 4 patients with insufficient IIM probability also showed Fib/PSW. Fib/PSW may also be seen in cases with insufficient IIM probability not satisfying the criteria. EMG may help detect muscle involvement in these cases through Fib/PSW.

Palatal prolapse during nasal expiration in patients with myasthenia gravis.

INTRODUCTION/AIMS: We have encountered patients with myasthenia gravis (MG) who exhibited palatal prolapse (PP) during nasal expiration in the supine position while awake. This may be an overlooked cause of dyspnea in MG patients. This study aimed to examine and describe the characteristics of MG patients with PP. METHODS: We reviewed the medical records of 183 consecutive patients who were diagnosed with MG in our hospital from 2012 to 2021. Thirty-two patients underwent laryngoscopy because of bulbar symptoms. Eight of these patients (25%) exhibited PP on laryngoscopy. Clinical features of these eight patients were retrospectively characterized. RESULTS: Median age of the eight patients with PP was 70 years. Six were men. Median body mass index was 21.6 kg/m2 . All patients exhibited PP in the supine position but not the sitting position. Although no patient had abnormal findings on spirometry nor chest computed tomography, six reported dyspnea or difficulty with nasal expiration only in the supine position. PP improved in all four patients who underwent edrophonium testing. All eight patients eventually improved after immunotherapy. DISCUSSION: PP during nasal expiration may be a cause of dyspnea in MG patients, along with respiratory muscle impairment, lung disease, and vocal cord paralysis. Laryngoscopy in the supine position is required to confirm.

Tibial nerve SEPs in diagnosing lumbar spinal stenosis: The utility of segmental evaluation using P15 and N21.

Objective: To establish the utility of the additional evaluation of the P15 potential generated at the greater sciatic foramen in the tibial nerve somatosensory evoked potentials (SEPs) in diagnosing lumbar spinal stenosis (LSS). Methods: We retrospectively reviewed tibial nerve SEP findings in patients having MRI-confirmed LSS at the cauda equina or conus/epiconus region. P15 and N21 potentials were recorded and the following findings were defined as localizing abnormalities: 1) normal P15 latency either with prolonged P15-N21 interval or with absent N21; 2) decreased ratio of the N21 amplitude to P15 amplitude. As non-localizing abnormalities, N21 and P38 latencies were also evaluated. Tibial nerve F-wave findings were also investigated. Results: According to the entry criteria, 18 patients were included, 15 with cauda equina lesions and 3 with conus/epiconus lesions. Localizing abnormalities in SEPs were found in 67% of patients, achieving significantly higher sensitivity than delayed P38 latency (28%), and higher sensitivity than N21 abnormalities (39%), though this was not significant. Localizing abnormalities were observed even in 6 out of 11 patients lacking both sensory symptoms and signs. Tibial nerve F-wave was abnormal in 36% of 14 patients with F-wave examinations, whereas the localizing abnormalities in SEPs were found in 64% of the same patient population. P15 amplitude was depressed in 4 patients (22%), which may indicate the involvement of the dorsal root ganglion in LSS, although its latency was normal even for these patients. Conclusions: Tibial nerve SEPs with the recording of P15 and N21 potentials achieved sufficiently high sensitivity in diagnosing LSS. They have the advantage over F-wave in that they can localize the lesion at the cauda equina or conus/epiconus level. Significance: Tibial nerve SEPs are promising in evaluating LSS, especially in documenting sensory tract involvement in cases lacking sensory symptoms/signs.

Fiberoptic laryngoscopic neurological examination of amyotrophic lateral sclerosis patients with bulbar symptoms.

OBJECTIVE: Bulbar symptoms in amyotrophic lateral sclerosis (ALS) are variable, reflecting bulbar and pseudobulbar palsy. The current study sought to characterize the pharyngeal findings in ALS using a fiberoptic laryngoscope and compare them with the findings of general neurological examination. METHODS: We enrolled ALS patients with bulbar symptoms who were admitted between 2014 and 2020. All participants were evaluated on salivary status, velopharyngeal movement during speech and swallowing, pharyngeal constriction, and vocal cord movement using fiberoptic laryngoscopy. The laryngoscopic findings were compared with general neurological examination results. RESULTS: A total of 50 patients (31 men; median age: 69 years) were enrolled. Salivary residue in the hypopharynx was the most common abnormal finding on laryngoscopy (40 patients; 80%). Twenty-three patients (46%) exhibited velopharyngeal insufficiency, 18 of which exhibited good velopharyngeal closure in swallowing and poor velopharyngeal closure in speech. Thus, these patients presented speech-swallow dissociation (SSD) in velopharyngeal closure. Five patients (10%) exhibited unilateral weakness of the pharyngeal constrictor muscles in phonation. Compared with general neurological examinations, emotional incontinence was more frequent in patients who presented with SSD in velopharyngeal movement, compared with those who did not. None of the five patients with unilateral weakness of pharyngeal constriction showed curtain movement on examination via the mouth. CONCLUSION: The specific findings of laryngoscopy in ALS patients, such as SSD in velopharyngeal closure and laterality in pharyngeal constriction, could not be evaluated by general neurological examination via the mouth.

[The odor stick identification test for Japanese (OSIT-J) in a case of coronavirus disease 2019 (COVID-19) without pneumonia].

Anosmia is a frequently experienced symptom in coronavirus disease 2019 (COVID-19). Previous studies have suggested the potential use of olfactometry to identify infected individuals. We performed a sequential olfactometry using the odor stick identification test for Japanese (OSIT-J) in a COVID-19 patient without pneumonia. The test uses 12 odorants that are familiar to the Japanese population. Our patient was a 56-year-old man diagnosed with COVID-19 who was admitted to our hospital following the development of anosmia. He had no respiratory symptoms except pharyngeal pain. Chest CT findings did not reveal the presence of pneumonia. The patient underwent OSIT-J on the 1st hospital day, and his score was 1 out of 12. Following the olfactometry, ciclesonide was administered. The patient did not develop any new symptoms during hospitalization, and his anosmia was gradually improved. The OSIT-J scores were 9 and 11 on the 7th and 16th hospital day, respectively. The patient was discharged on the 25th hospital day after two negative PCR test results. In our case, OSIT-J could identify anosmia in a COVID-19 patient. Some COVID-19 patients are asymptomatic, expect for olfactory disturbances, and OSIT-J may help identify such patients in the Japanese population.

Visualization of Motor Cortex Involvement by 18F-THK5351 PET Potentially Strengthens Diagnosis of Amyotrophic Lateral Sclerosis.

ABSTRACT: Amyotrophic lateral sclerosis (ALS) involves both upper motor neurons (UMNs) and lower motor neurons. The detection of UMN involvement, a core component of ALS criteria, is primarily dependent on neurological examination because of a lack of definitive biomarkers. We present the 18F-THK5351 PET images of a 76-year-old man diagnosed with ALS comorbid with Alzheimer disease, demonstrating marked accumulation of 18F-THK5351 in the bilateral precentral gyri. Because 18F-THK5351 binds to monoamine oxidase B highly expressed in astrocytes, where the neurodegenerative process is ongoing, our case highlights that 18F-THK5351 tracer should be a useful marker for detecting UMN neurodegeneration in ALS.

Polyradiculoneuropathy induced by immune checkpoint inhibitors: a case series and review of the literature.

OBJECTIVE: The purpose of the present study is to report the clinical characteristics of polyradiculoneuropathy induced by immune checkpoint inhibitors (ICIs). METHODS: We retrospectively reviewed lists of all inpatients with neurological immune-related adverse events (irAEs) treated at the neurology departments of three hospitals in January 2017 and December 2019. We also performed a review of the previous case reports with polyradiculoneuropathy induced by ICI therapy. RESULTS: We had 4 patients with polyradiculoneuropathy following ICI therapy. We comprehensively reviewed our 4 patients and 32 previous case reports. There were 28 men and 8 women with a mean onset age of 61 years. ICI monotherapy was performed in 27 patients, whereas the combination of ICIs was administered in 9 patients. All patients except 2 showed limb weakness, which was observed symmetrically and predominantly in the legs rather than the arms. Bulbar involvement was observed in 7 patients. The laboratory findings were demyelination in electrophysiological studies and elevated protein with lymphocytes in the cerebrospinal fluid. Disease severity was ranked on the Hughes functional scale; 17 patients were grade 4 or greater. The treatment responses to corticosteroid and intravenous methylprednisolone were favorable. Intravenous immunoglobulin was also used in combination with steroids. Seven patients died, including 4 who on mechanical ventilation. CONCLUSION: Polyradiculoneuropathy induced by ICIs has a distinct subset of neurological irAEs and requires early recognition.

[Acute myeloid leukemia diagnosed by dysphagia due to bilateral vagus nerve palsy: a case report].

This is the rare case report that bilateral vagus nerve paralysis was emerged as the initial symptom of acute myelogenous leukemia (AML). An 83-year-old man admitted to our hospital because of dysphagia. His dysphagia progressed two months prior to admission. Although physical examination revealed no abnormality, videoendoscopy and videofluorography examination clearly revealed bilateral vagus nerve palsy. Brain MRI showed hypointense signals at the bilateral clivus on T1 weighted images, suggesting tumor infiltration to bilateral petroclivus. He was diagnosed as AML by blood samples and bone marrow biopsy. After initiation of the treatment including radiation therapy, dysphagia shows mild improvement. Although bilateral cranial nerve palsy due to malignant tumor involving at the clivus is very uncommon, we should pay attention to the symptom.

The NAG scale can screen for hematoma expansion in acute intracerebral hemorrhage-a multi-institutional validation.

BACKGROUND: Early hematoma expansion (HE) is seen in approximately 30% of patients with intracerebral hemorrhage (ICH), but detecting patients with a high HE risk is challenging. AIMS: The NAG scale is a simple predictive scale for HE in acute ICH patients. Multi-institutional validation of the usefulness of this scale was the aim of this study. METHODS: We retrospectively reviewed 142 consecutive primary ICH patients admitted to our hospital between September 2016 and December 2018. The NAG scale consists of three factors: National Institutes of Health Stroke Scale (NIHSS) score ≥ 10, anticoagulant use, and glucose ≥133 mg/dl (1 point each). Patients underwent non-contrast computed tomography (CT) within 24 h of symptom onset and follow-up CT 6 h, 24 h, and 7 days after admission. We defined HE as increased hemorrhage volume > 33% or an absolute increase of >6 mL on follow-up CT. Poor prognosis was defined as a modified Rankin scale score of 4-6 at discharge. We performed logistic regression analysis and created receiver operating characteristic curves to determine the discrimination ability of the NAG score. RESULTS: Patients constituted 96 men and 46 women (median age: 64 years; median NIHSS: 11), and HE was observed in 38/142 patients (27%). Higher NAG sores were associated with HE (P < .001), poor prognosis (P < .001), and in-hospital death (P < .001). The C statistic was 0.72 (95% confidence interval [CI]: 0.63-0.82) for HE, 0.67 (95% CI: 0.58-0.76) for poor prognosis, and 0.85 (95% CI: 0.74-0.95) for in-hospital death. Multivariate logistic regression analysis with known risk factors showed that NAG scale score was an independent risk factor for HE (odds ratio: 2.95; 95% CI: 1.57-5.52; P = .001). CONCLUSION: The NAG scale showed good discrimination in our multi-institutional validation.

Riluzole-induced interstitial lung disease is a rare and potentially life-threatening adverse event successfully treated with high-dose steroid therapy: Case reports and review of the literature.

Riluzole (RZ)-induced interstitial lung disease (RZ-ILD) is a rare and potentially life-threatening adverse event in amyotrophic lateral sclerosis (ALS) patients, which is rarely reported. Therefore, the optimal treatment for RZ-ILD is unclear. We describe herein three Japanese cases of ALS complicated with RZ-ILD, of which two were successfully treated with high-dose steroid therapy. In our all ALS cases with RZ-ILD, the duration of RZ exposure until RZ-ILD onset was within 2 months. All three cases showed respiratory symptoms, dorsal predominant ground-glass opacities by imaging analysis, and abnormal laboratory findings associated with interstitial lung diseases, such as Krebs von den Lungen-6 and surfactant protein-D. Intravenous high-dose steroid therapy together with the discontinuation of RZ in two cases with respiratory symptoms markedly ameliorated their symptoms and abnormal findings of RZ-ILD. One case showed mild respiratory symptoms compared with the others and recovered after the withdrawal of RZ only. According to previous case reports and our cases, RZ-ILD may develop 2 months after initiating RZ and exacerbate respiratory symptoms rapidly in ALS patients with severe respiratory muscle involvement or complicating aspiration pneumonia. Transient high-dose steroid therapy in addition to discontinuation of RZ might be a good therapeutic option for RZ-ILD.

Endovascular Therapy for Concurrent Cardio-Cerebral Infarction in a Patient With Trousseau Syndrome.

Only a few patients have been reported to undergo endovascular therapy for Trousseau syndrome. This is the first report of a patient with Trousseau syndrome who developed synchronous cardiocerebral infarction and underwent endovascular therapy for both. A 55-year-old woman with Trousseau syndrome arising from stage IV ovarian cancer presented with consciousness disturbance, aphasia, and right hemiparesis. Magnetic resonance imaging showed acute cerebral infarction limited to the left basal ganglia and occlusion of the left middle cerebral artery (MCA). Electrocardiography showed ST elevation in leads II, III, and aVF with reciprocal change. Mild elevation of myocardial enzymes was observed in laboratory data. She was diagnosed with synchronous cardiocerebral infarction. Both infarctions were considered as appropriately indicated for endovascular therapy. Since her vital signs were stable, a decision was made to treat the cerebral infarction first. Thrombectomy with a stent retriever was performed, which achieved complete recanalization of the left MCA. Percutaneous coronary intervention successfully recanalized the occluded right coronary artery. She suffered no recurrence of stroke or acute coronary syndrome upon heparin administration. Cardiocerebral infarction caused by Trousseau syndrome is rare and demands optimal planning of endovascular therapy.

Irreversible Vasculopathy Proceeds Rapidly in POEMS Syndrome.

A 48-year-old woman with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin change (POEMS) syndrome suddenly presented with numbness of her right upper limb. Magnetic resonance imaging showed multiple acute infarctions in her left cerebrum, and magnetic resonance angiography (MRA) showed multiple intra-cranial vascular lesions, which contrasted with previously normal MRA results obtained eight months prior to the stroke. After completing successful treatment for POEMS syndrome, there were no recurrent stroke episodes. A six-month follow-up scan showed that although the vascular lesions did not progress, they did not improve much either. POEMS syndrome is associated with the rapid extension of large blood vessels-vasculopathy-resulting in nearly irreversible brain lesions.

[A case of Creutzfeldt-Jakob disease with E200K mutation presenting with hearing loss and central hypoventilation].

We report the case of a 43-year-old female patient who presented with symptoms of abnormal behavior, hearing loss, ataxic gait, central hyperventilation which had appeared over the course of one month. Brain MRI showed no abnormal findings in DWI and EEG did not indicate periodic synchronous discharge (PSD). Over the course of the same month, she also presented with central apnea that intermittently showed spontaneous improvement and reappearance. Cerebrospinal fluid 14-3-3 protein tested negative and there was no family history, but an abnormal prion protein was detected in the cerebrospinal fluid by the RT-QUIC assay. We diagnosed her with familial Creutzfeldt-Jakob disease (CJD) with an E200K mutation after genetic examination. Both high cortical signals on MRI and PSD on EEG were not recognized even in the advanced stage. Central apnea was presumed to be caused by disorders of the respiratory center of the brainstem. Hearing loss was also considered to be an obstacle at the brainstem level from the latency delay after the III wave in auditory brainstem response (ABR). The possibility of brain stem symptoms occurring in the early stages of CJD should be considered.