Becker Muscular Dystrophy Accompanied by Anti-HMGCR Antibody-positive Immune-mediated Necrotizing Myopathy.

Becker muscular dystrophy (BMD) is an X-linked neuromuscular disease characterized by progressive muscle weakness that currently has no cure. Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune inflammatory myopathy characterized by proximal muscle weakness that is treated with immunosuppressive therapy. We herein report a patient diagnosed with BMD complicated with IMNM by a pathological analysis.

A novel method to measure sensory nerve conduction of the posterior antebrachial cutaneous nerve.

Introduction/aims: Posterior antebrachial cutaneous (PABC) nerve conduction studies could be useful for distinguishing PABC neuropathy from C7 radiculopathy. In the conventional method using an antidromic method, the sensory nerve action potential (SNAP) is sometimes followed by a large volume-conducted motor potential. In this report we describe a reliable nerve conduction study using an orthodromic method for recording SNAPs of the PABC nerve.

Anatomical variations of the superficial branch of the radial nerve and the dorsal branch of the ulnar nerve: A detailed electrophysiological study.

Introduction: In this study we evaluated anatomic variations of the superficial branch of the radial nerve (SBRN) and the dorsal branch of the ulnar nerve (DBUN) electrophysiologically.

Methods: Antidromic nerve conduction studies (NCS) of the SBRN and DBUN were performed on healthy individuals. To identify individual responses from the distal branches of the SBRN and DBUN, sensory nerve action potentials of each finger (lateral side/medial side) were recorded.

Relationship between the Diabetic Polyneuropathy Index and the Neurological Findings of Diabetic Polyneuropathy.

Objective To achieve an accurate quantification in diabetic polyneuropathy (DPN), we developed a new electrophysiological index that we called the DPN index. The relationship between the DPN index and the neurological findings in diabetic patients was assessed. Methods The DPN index was calculated by the mean value of percentages of four parameters (tibial compound muscle action potential amplitude / F wave minimum latency, sural sensory nerve action potential amplitude / sensory nerve conduction velocity) against the mean normal values.

Influence of placement sites of the active recording electrode on CMAP configuration in the trapezius muscle.

Objective: We investigated how the active electrode placement site influences compound muscle action potential (CMAP) configuration of the upper trapezius muscle (TM).

Methods: A nerve conduction study of the accessory nerve was performed, and the CMAPs obtained with two different placement sites, i.e.

Clinical and pathological findings in familial amyloid polyneuropathy caused by a transthyretin E61K mutation.

Familial amyloid polyneuropathy (FAP) is an autosomal dominant hereditary systemic amyloidosis caused by mutation of the transthyretin (TTR) gene, and usually shows sensory-dominant polyneuropathy and autonomic neuropathy at the initial stage. The pathogenesis of this neuropathy remains unknown, although several mechanisms, including mechanical compression, vessel occlusion, TTR toxicity and Schwann cell dysfunction have been proposed. We describe a patient with late-onset FAP caused by a TTR E61K mutation.

A new technique for dorsal sural nerve conduction study with surface strip electrodes.

Objective: To obtain higher amplitude of dorsal sural sensory nerve action potentials (SNAPs), we used a new method for dorsal sural nerve conduction study with surface strip electrodes (SSEs).

Methods: Dorsal sural SNAPs were recorded orthodromically. The recording electrodes were placed behind the lateral malleolus.

Variations in the distal branches of the superficial fibular sensory nerve.

Introduction: We evaluated anatomic variations of distal branches of the superficial fibular sensory nerve electrophysiologically.

Methods: Orthodromic nerve conduction studies (NCS) of the first and third branches (M-I, M-III) of the medial dorsal cutaneous nerve and the fourth and fifth branches (I-IV, I-V) of the intermediate dorsal cutaneous nerve (IDCN) were performed. To find anomalous innervations from the dorsal sural nerve (DSN) in the IDCN territory, NCS of the fourth and fifth branches (S-IV, S-V) of the DSN were also performed.

[Sporadic Late-Onset Nemaline Myopathy Associated with MGUS].

Sporadic late-onset nemaline myopathy is an uncommon disease. Clinically, it is characterized by progressive muscle weakness that can develop in limbs or axial muscles. Asymmetrical distal weakness, facial weakness, dropped head, and dysphagia can also occur.

Whole plantar nerve conduction study with disposable strip electrodes.

Introduction: A new method to evaluate whole plantar nerve conduction with disposable strip electrodes (DSEs) is described.

Methods: Whole plantar compound nerve action potentials (CNAPs) were recorded at the ankle. DSEs were attached to the sole for simultaneous stimulation of medial and lateral plantar nerves.

A novel method to measure sensory nerve conduction of the supraclavicular nerve.

Introduction: In this report we describe a reliable method for recording sensory nerve action potentials (SNAPs) of the supraclavicular nerve.

Methods: Supraclavicular SNAPs were recorded by placing a surface active electrode at the posterior border of the sternocleidomastoid muscle at a distance of 6 cm from the sternoclavicular joint. The nerve was stimulated at the lower border of the clavicle 4.

[Acute fulminant brachial plexopathy with good recovery: electrophysiological features].

We report a case of fulminant brachial plexopathy with radicular involvement. A 25-year-old man developed acute total monoplegia in the left upper limb. Needle electromyography showed extensive acute denervation in the C5-T1 spinal segments, and peripheral sensory nerve conduction was normal, mimicking a pre-ganglionic lesion.

Coexistence of Creutzfeldt-Jakob disease, Lewy body disease, and Alzheimer's disease pathology: an autopsy case showing typical clinical features of Creutzfeldt-Jakob disease.

We report here an autopsy case of sporadic Creutzfeldt-Jakob disease (CJD) without hereditary burden and with a clinical course typical of sporadic CJD. A 77-year old man developed memory disturbance, followed by gait disturbance and myoclonus. He died of bronchopneumonia 5 months after the disease onset.