We report two cases of numbness and pain of neuropathy due to ANCA (antineutrophil cytoplasmic antibody)-associated vasculitis successfully treated with Ogikeishigomotsuto. The first case was a 57-year-old female who complained of high fever, painful skin eruptions of the lower limbs, and proteinuria. Although the severe pain was reduced with steroid therapy, neuropathy-related numbness and pain remained widespread in her lower limbs. After we prescribed Ogikeishigomotsuto, most of her pain disappeared while her degree of numbness diminished by almost half in two weeks. In addition, her steroid therapy could be tapered off without adverse effect. The second case was an 82-year-old female with fever and myalgia. Although myalgia of the upper arm disappeared with steroid treatment, walking became difficult due to numbness from the lower legs, to the soles of her feet. With Ogikeishigomotsuto administration, she became better able to walk because her legs had warmed up, and the range of her numbness had decreased. These cases suggest that Ogikeishigomotsuto may be effective against numbness and neuropathic pain due to ANCA-associated vasculitis.
Numbness ; Pain ; Peripheral neuropathy ; Antineutrophil cytoplasmic antibody measurement ; Vasculitis

Hereditary motor and sensory neuropathy (HMSN), or Charcot-Marie-Tooth (CMT) disease, was described by Charcot and Marie in France and, independently, by Tooth in England in 1886. CMT is the most common form of inherited motor and sensory neuropathy, and is a genetically heterogeneous group of disorders in the peripheral nervous system. Traditionally, CMT has been subclassified into autosomal dominant inherited demyelinating (CMT1) and axonal (CMT2) neuropathies, X-linked neuropathy (CMTX), and autosomal recessive inherited neuropathy (CMT4). There are several related peripheral neuropathies, such as Dejerine-Sottas neuropathy (DSN), congenital hypomyelination neuropathy (CHN), hereditary neuropathy with liability to pressure palsies (HNPP), and giant axonal neuropathy (GAN). A large amount of new information on the genetic causes of CMT has become available, and mutations causing the disease have been associated with more than 20 different genes and 40 chromosomal loci. Advances in our understanding of the molecular basis of CMT have revealed an enormous diversity in genetic mechanisms, despite the clinical entity that is relatively uniform at presentation. Recent studies have shown therapeutic effects of certain chemicals in animal models of CMT1A, which suggests potential therapies for the most common form of CMT, CMT1A. This review focuses on the subgroups of inherited motor and sensory neuropathy on which there has been an explosion of new molecular genetic information over the past decade.
Axons ; Charcot-Marie-Tooth Disease* ; England ; Explosions ; France ; Giant Axonal Neuropathy ; Hereditary Sensory and Motor Neuropathy ; Models, Animal ; Molecular Biology ; Paralysis ; Peripheral Nervous System ; Peripheral Nervous System Diseases ; Tooth

The reported cases of upper limb nerve injury followed by needle procedure such as intramuscular injection or routine venipuncture are rare. However, it should not be overlooked, because neurological injury may cause not only minor transient pain but also severe sensory disturbance, hand deformity and motor dysfunction with poor recovery. Recognizing competent level of anatomy and adept skill of needle placement are crucial in order to prevent this complication. If a patient notices any experience of abnormal pain or paresthesia during the needle procedures, an administrator should be alert to the possibility of nerve injury and should withdraw the needle immediately. Careful monitoring of the injection site for hours is required for early detection of nerve injury.
Administrative Personnel ; Catheterization, Peripheral ; Hand Deformities ; Humans ; Injections, Intramuscular* ; Median Neuropathy ; Needles ; Paresthesia ; Peripheral Nerve Injuries ; Phlebotomy* ; Radial Neuropathy ; Ulnar Neuropathies ; Upper Extremity*

Charcot-Marie-Tooth (CMT) disease is the most common form of inherited motor and sensory neuropathy. CMT is a genetically heterogeneous disorder of the peripheral nervous system; thus, many genes have been identified as CMTcausative genes. Traditionally, subclassification of CMT has been divided into autosomal dominant inherited demyelinating (CMT1) and axonal (CMT2) neuropathies, X-linked neuropathy (CMTX), and autosomal recessive inherited neuropathy (CMT4). Recently, intermediate type (CMT-Int) with NCVs between CMT1 and CMT2 is considered as a CMT type. There are several related peripheral neuropathies, such as Dejerine- Sottas neuropathy (DSN), congenital hypomyelination (CH), hereditary neuropathy with pressure palsies (HNPP), and giant axonal neuropathy (GAN). Great advances have been made in understanding the molecular basis of CMT, and 17 distinct genetic causes of CMT have been identified. The number of newly discovered mutations and identified genetic loci is rapidly increasing, and this expanding list has proved challenging for physicians trying to keep up with the field. In addition, the encouraging studies have been published on rational potential therapies for the CMT1A.
Axons ; Charcot-Marie-Tooth Disease* ; Genetic Loci ; Giant Axonal Neuropathy ; Paralysis ; Peripheral Nervous System ; Peripheral Nervous System Diseases

BACKGROUND: Mutations in the gene encoding periaxin (PRX) are known to cause autosomal recessive Dejerine-Sottas neuropathy (DSN) or Charcot-Marie-Tooth disease type 4F. However, there have been no reports describing Korean patients with these mutations. CASE REPORT: We examined a Korean DSN patient with an early-onset, slowly progressive, demyelinating neuropathy with prominent sensory involvement. Whole-exome sequencing and subsequent capillary sequencing revealed novel compound heterozygous nonsense mutations (p.R392X and p.R679X) in PRX. One mutation was transmitted from each of the patient's parents. No unaffected family member had both mutations, and the mutations were not found in healthy controls. CONCLUSIONS: We believe that these novel compound heterozygous nonsense mutations are the underlying cause of DSN. The clinical, electrophysiologic, and pathologic phenotypes in this family were similar to those described previously for patients with PRX mutations. We have identified the first PRX mutation in a Korean patient with DSN.
Capillaries ; Charcot-Marie-Tooth Disease ; Codon, Nonsense ; Hereditary Sensory and Motor Neuropathy* ; Humans ; Parents ; Peripheral Nerves ; Phenotype

Hereditary motor and sensory neuropathy is an unusual disease which is characterized by deformity of phe foot, acral sensory loss, decreare of deep tendon reflexes, enlargement of peripheral nerve and diminished motor conduction velocity. We report a case of hereditary motor and sensory neuropathy type I.
Congenital Abnormalities ; Foot ; Hereditary Sensory and Motor Neuropathy ; Peripheral Nerves ; Reflex, Stretch

OBJECTIVE: To investigate waveform changes of compound muscle action potentials (CMAPs) related to voluntary muscle contraction and alteration of muscle length and to evaluate the effect of peripheral neuropathy on temporal and spatial summations of CMAPs. METHOD: The influence of voluntary muscle contraction and alteration of muscle length on CMAP was studied in 37 median nerves of 21 patients with median neuropathy. RESULTS: In patients with no apparent axonopathy, temporal summation was partially disturbed without significant change of spatial summation. Shortening of muscle length or voluntary contraction produced a physiologic improvement of spatial and temporal summations. There was a decrease in temporal and spatial summations, more prominent in temporal summation, with lengthening of the muscle. In axonopathy, spatial summation was markedly deteriorated with partial reduction of temporal summation. Spatial summation was not affected by the change of muscle length or voluntary contraction. Temporal summation was improved by muscle shortening or voluntary contraction and was decreased by muscle lengthening. CONCLUSION: Peripheral neuropathy has an effects on physiological spatial and temporal summations of CMAPs. Temporal summation is preferentially decreased in cases without axonopathy. When axonopathy is apparent, spatial summation is profoundly disturbed with partial reduction of temporal summation.
Action Potentials* ; Humans ; Median Nerve ; Median Neuropathy ; Muscle Contraction ; Muscle, Skeletal* ; Peripheral Nervous System Diseases*

A case of hypertrophic interstitial neuropathy in a 32 years old man developed in the median nerve is reported. This is a rare disease characterized by thickened peripheral nerves which may be palpable and visible. This case was treated by excision of transverse carpal ligament and external and internal neurolysis.
Hereditary Sensory and Motor Neuropathy ; Ligaments ; Median Nerve ; Peripheral Nerves ; Rare Diseases

Humans ; Sciatic Neuropathy/etiology* ; Arthroplasty, Replacement, Hip/adverse effects* ; Peripheral Nerve Injuries/etiology* ; Sciatic Nerve/injuries*

Carbon monoxide (CO) intoxication is a leading cause of the variable neuropsychiatric impairment. Despite of widely known central nerve system complications after CO intoxication, peripheral neuropathy due to CO poisoning is rare and has been under-recognized. We report interesting case of a 29-year-old male who suffered from motor weakness and sensory abnormalities in his lower extremity following acute CO intoxication. The patient revealed direct and indirect signs of peripheral neuropathy of the left inferior gluteal and sciatic nerve on magnetic resonance imaging.
Adult ; Carbon Monoxide* ; Carbon* ; Humans ; Lower Extremity ; Magnetic Resonance Imaging ; Male ; Peripheral Nervous System Diseases* ; Poisoning ; Sciatic Nerve ; Sciatic Neuropathy