Utility of the Repetitive Nerve Stimulation Test and Needle EMG in the Trapezius Muscle for the Early Diagnosis of ALS.

Objectives: To document the utility of decremental responses in the repetitive nerve stimulation test (RNS) and spontaneous activities in needle electromyography (EMG) in the trapezius muscle for the diagnosis of amyotrophic lateral sclerosis.

Methods: Subjects were retrospectively identified from our EMG database. Cervical spondylosis was represented as a disease control group.

[The differential diagnosis of inflammatory and non-inflammatory myelopathy].

The differential diagnosis of inflammatory and non-inflammatory myelopathy can be challenging. Clinical information such as age, gender, speed of onset and progression, systemic symptoms, spinal cord and brain MRI, autoantibodies, and cerebrospinal fluid findings are necessary. The speed of onset is particularly important for differentiation.

Weak gluteus maximus and weak iliopsoas with normal gluteus maximus: Two complementary new signs to diagnose lower limb functional weakness.

Background And Purpose: The diagnosis of functional neurological disorder should be actively made based on the neurological signs. We described two new complementary signs to diagnose functional weakness of the lower limb, "weak gluteus maximus (weak GM)" and "weak Iliopsoas with normal gluteus maximus (weak iliopsoas with normal GM)," and tested their validity.

Methods: The tests comprised Medical Research Council (MRC) examinations of the iliopsoas and GM in the supine position.

[A case of idiopathic spinal cord herniation with incomplete Brown-Séquard syndrome].

A 50-year-old man was referred to our hospital with myelitis associated with a 10-months history of progressive muscle weakness in the left leg. Neurological examinations demonstrated diffuse muscle weakness of the left leg, touch hypoesthesia of the right leg, reduced pain sensation below the right nipple, left pyramidal sign, and urinary incontinence. On the basis of thoracic spinal MRI and thoracic CT myelography, revealing anterior displacement of the spinal cord and enlargement of the posterior subarachnoid space at the Th4 vertebral level, we diagnosed the patient as having idiopathic spinal cord herniation with incomplete Brown-Séquard syndrome.

[Nontechnical Skills for Neurology Residents].

"Nontechnical skills," such as resilience, communication, management, information literacy, and educational capability, are required to be an the effective neurologist. Neurology residents should learn nontechnical skills in their residency programs.

[Early Diagnosis of Spinal Dural Arteriovenous Fistula].

Spinal dural arteriovenous fistulas (SDAVF) are rare and most commonly affect men aged >50 years. Patients with SDAVF develop an abnormal vascular dural shunt between the dural branch of a segmental artery and a subdural radicular vein that drains the perimedullary venous system, leading to venous hypertension and secondary congestive myelopathy. Most SDAVFs are located in the thoracolumbar region, and usually patients present with slowly progressive paraparesis and urinary disturbances.

[Epiconus Syndrome and Conus Syndrome].

The most caudal part of the spinal cord shows special anatomical characteristics and it contains epiconus (L4-S2 segments), the conus medullaris (S3-S5 segments), and surrounding nerve roots. Lesions of the thoracolumbar junction cause epiconus or conus syndrome. Epiconus syndrome is characterized by segmental muscular weakness and atrophy of one or both lower extremities, often accompanied by foot drop.

[A case of proximal-type Hirayama disease associated with neck axial rotation].

Hirayama disease is characterized by juvenile onset of unilateral muscular atrophy of a distal upper extremity. The pathogenic mechanism of Hirayama disease is cervical cord compression by the posterior dura with forward displacement in the neck flexion position. A few cases of 'proximal-type Hirayama disease' have been described as showing muscular weakness and atrophy of the proximal upper extremities caused by the pathogenic mechanism similar to that of Hirayama disease.

[What is necessary to make neurology a basic specialty?: Changing medical specialty system in Japan and the mission of a basic specialty].

The Japanese Society of Neurology decided to aim to convert neurology, which is currently a subspecialty of internal medicine, to a basic specialty in the Japanese medical specialty system at the special general meeting of corporate members in January 2018. Because the details of new specialty system in Japan remain unstable, the committee to promote achievement of neurology as a basic specialty planned to hold a special symposium regarding the specialty system at the 60th annual meeting of the Japanese Society of Neurology in May 2019. This article compiles the abstracts of speakers in this symposium.

[Current Status of Clinical Ethics and Creation of Ethical Organizational Culture].

When an ethical problem arises in clinical practice, the process of discussion aimed towards a solution is important. The decision should be made by a multidisciplinary team rather than by an individual clinician. In clinical practice, highly feasible solutions are required.

Pharmacological monitoring of antiepileptic drugs in epilepsy patients on haemodialysis.

To retrospectively evaluate the pharmacological profiles of antiepileptic drugs (AEDs) in epilepsy patients during haemodialysis using therapeutic drug monitoring data. The serum concentration of AEDs was collected before and after haemodialysis, and the clearance rate and concentration-to-dose ratio were calculated as pharmacological parameters. Thirty-six patients were enrolled in the study (25 males, 11 females; age: 65.

Autopsy case of MV2K-type sporadic Creutzfeldt-Jakob disease with spongiform changes of the cerebral cortex.

Comprehensive analysis is required for the accurate diagnosis of MV2-type sporadic Creutzfeldt-Jakob disease (sCJD) because it shows a wide clinicopathological spectrum. Here, we describe the clinical findings and neuropathologic observations of an autopsy-confirmed MV2K-type sCJD case with extensive spongiform changes of the cerebral cortex. In the early disease stages, the patient exhibited gait disturbance with ataxia and gradually showed cognitive dysfunction.

Long-read sequencing identifies GGC repeat expansions in NOTCH2NLC associated with neuronal intranuclear inclusion disease.

Neuronal intranuclear inclusion disease (NIID) is a progressive neurodegenerative disease that is characterized by eosinophilic hyaline intranuclear inclusions in neuronal and somatic cells. The wide range of clinical manifestations in NIID makes ante-mortem diagnosis difficult, but skin biopsy enables its ante-mortem diagnosis. The average onset age is 59.

Ultrastructure and three-dimensional architecture of the anterior cruciate ligament in the knee joints of young and old monkeys.

We examined the ultrastructure of the anterior cruciate ligament and assessed age-related changes by comparing the ligaments of young and old monkeys. Ultrathin sections of the anterior cruciate ligament were observed by transmission electron microscopy. The three-dimensional architecture of collagen fibers in the ligament was examined by scanning electron microscopy after tissue specimens were treated with 2 N NaOH to digest the extracellular matrix.

Autopsied case of sporadic Creutzfeldt-Jakob disease classified as MM1+2C-type.

We encountered an autopsy case of sporadic Creutzfeldt-Jakob disease (CJD) pathologically classified as MM1+2C-type, where Western blot analysis of prion protein (PrP) mainly showed type-1 scrapie PrP (PrP ) but also, partially, mixed type-2 PrP . A Japanese woman complained of visual disorder at the age of 86 years and then showed disorientation and memory disturbances. Magnetic resonance imaging (MRI) showed cerebral cortical hyperintensity on diffusion-weighted images.

[Management of Cervical Spondylosis by Neurologists].

Neurologists have to manage patients with cervical spondylosis, a common neurological disease in individuals over the age of 50 years. The diagnosis of cervical spondylosis requires a detailed neurological examination that takes into account cervical levels. To prevent misdiagnosis, it is important to integrate the levels of the lesions revealed by imaging with the clinical findings.

Autopsy case of V180I genetic Creutzfeldt-Jakob disease presenting with early disease pathology.

The patient was a Japanese woman who experienced a decrease in activity and gait disturbance as the initial symptoms at the age of 86, followed by disorientation and memory dysfunction. Magnetic resonance imaging showed extensive cortical regions with hyperintensity in diffusion-weighted images, and these regions showed swelling in T2-weighted and fluid-attenuated inversion recovery (FLAIR) images. The medial occipital cortex and striatum showed no apparent hyperintensity on diffusion-weighted imaging (DWI).

Phosphate Binders Derived from Natural Ores Contain Many Kinds of Metallic Elements Besides Their Active Ingredient Metals.

Most patients undergoing dialysis are required to take many phosphate binder pills to control hyperphosphatemia. Phosphate binders prescribed in Japan are classified into two types: metal-based binders (Ca carbonate, lanthanum carbonate, ferric citrate hydrate, and sucroferric oxyhydroxide) and chemically synthesized polymers (sevelamer hydrochloride and bixalomer). The raw materials of metal-based phosphate binders are natural ores; thus, such binders may contain several other metallic elements.

Availability of right femoral vein as a route for tunneled hemodialysis catheterization.

Purpose: This study was performed to investigate the primary patency rate and catheter-related problems associated with use of the femoral vein as a route for tunneled hemodialysis catheterization compared with those of the right internal jugular vein as the first-choice route in patients undergoing maintenance hemodialysis.

Patients And Methods: Twenty-two patients underwent placement of indwelling tunneled hemodialysis catheters in the right internal jugular vein as the first option for maintenance hemodialysis, and 20 patients underwent placement in the right femoral vein as the second option. The primary patency rate of the catheters and catheter-related problems at 1, 3, 6, and 12 months after placement were investigated.

Acute hepatitis E in a renal transplantation recipient: a case report.

Hepatitis E is caused by infection with the hepatitis E virus (HEV). HEV is transmitted orally via HEV-contaminated food or drink. Hepatitis E usually shows mild symptoms and is self-limiting in the general population; however, it may progress to chronic hepatitis in immunosuppressed patients such as recipients of organ transplantation.