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.

Noncanonical splice-site variant in peripheral myelin protein 22 gene (PMP22) in a patient with hereditary neuropathy with liability to pressure palsies.

Aim: Hereditary neuropathy with liability to pressure palsies (HNPP) is a peripheral neuropathy with autosomal dominant inheritance. Diagnosis can be made from the characteristic abnormalities determined by nerve conduction studies (NCS), including subclinical deficits at physiological compression sites. Heterozygous deletion of the chromosome 17p11.

Weak shoulder and arm sparing signs in amyotrophic lateral sclerosis.

Introduction/aims: Various signs of selective involvement have been reported in amyotrophic lateral sclerosis (ALS). In this study, we describe two new signs, "weak shoulder" and "arm sparing" signs.

Methods: Subjects were retrospectively identified from our electrodiagnosis database.

The influence of right-left error in the placement of the Cc electrode in tibial nerve somatosensory evoked potentials (SEPs).

Objective: At our laboratory, we routinely record tibial nerve somatosensory evoked potentials (SEPs) using 5 channels including the second cervical vertebra (C2S)-contralateral central area (Cc) and Cz' (2 cm posterior to Cz)-Cc derivations. In a man with lumbar spondylotic myelopathy, symptoms improved after surgery, although the N21-P38 interval was markedly prolonged in comparison with that before surgery. We presumed that the Cc electrode was actually placed on the ipsilateral central area (Ci) at the second examination.

Aquaporin-4-antibody-positive Neuromyelitis Optica Spectrum Disorder in a Patient with Charcot-Marie-Tooth Disease Type 1A.

Charcot-Marie-Tooth disease type 1A (CMT1A) is a hereditary peripheral neuropathy, and its involvement in the central nervous system (CNS) is very rare. We herein report a 51-year-old woman with CMT1A who suffered from recurrent optic neuritis and myelopathy. Under the diagnosis of anti-aquaporin-4 (anti-AQP4) antibody positive neuromyelitis optica spectrum disorder (NMOSD), we treated her successfully with corticosteroids.

[Hemiplegia cruciata and severe facial pain due to infarction of the cervicomedullary junction: a case report].

We report the case of a 66-year-old female with hemiplegia cruciata and severe facial pain due to infarction of the cervicomedullary junction. She presented to the hospital with complaints of acute-onset left facial pain and gait disturbance. Neurological examination revealed narrow left palpebral fissure, severe left facial pain and hypothermoesthesia, weakness predominantly in the left upper and right lower extremities, decreased pain and temperature sensation in the right lower extremity, decreased vibration sensation in the left lower extremity, hyperreflexia in the left upper extremity, and mild ataxia in the left upper and lower extremities.

[Severe sensory-motor axonal neuropathy following diabetic ketoacidosis].

We report a case of severe sensory-motor axonal neuropathy on the lower extremities associated with diabetic ketoacidosis (DKA). A sixteen-year-old boy developed coma and admitted to our hospital. We diagnosed him with DKA based on remarkable hyperglycemia, severe acidosis with hyperketonemia.

Lymphovenous Anastomosis Aids Wound Healing in Lymphedema: Relationship Between Lymphedema and Delayed Wound Healing from a View of Immune Mechanisms.

Delayed wound healing in lymphedema is assumed to be caused by two reasons, pathophysiological and immunological effects of lymphedema. The aim of this review is to establish how impaired lymphatics alter wound healing pathophysiologically and immunologically, and to propose treatment modalities that can promote wound healing in lymphedema. Lymphaticovenular anastomoses (lymphovenous anastomoses [LVAs]) were performed on patients who had recurrent cellulitis several times with lymphorrhea and developed severe ulcers that were refractory to skin grafts, flaps, and conservative therapy.