| Yoon-Ho Hong | 21 Articles |
Rituximab (RTX) is a monoclonal antibody that targets CD20 on B cells and is used to reduce the relapse risk in neuromyelitis optica spectrum disorder (NMOSD). Some patients experience relapse or exacerbation shortly after RTX treatment. We report a 54-year-old female with seronegative NMOSD who relapsed soon after RTX treatment.
Background
Routine nerve conduction study (NCS) can only be used to evaluate the function of large fibers, and the results of NCS are often normal in patients with distal sensory polyneuropathy. The measurement of the current perception threshold (CPT) has been reported to represent a variety of peripheral nerve fiber functions. This study was performed to investigate the value of measuring CPT in patients with diabetic sensory polyneuropathy who have no abnormalities in routine NCS. Methods: Twenty-seven diabetic patients with sensory polyneuropathy and normal routine NCS and 18 age-matched control subjects participated in this study. The CPT was measured on the unilateral index finger and great toe of each subject at frequencies of 5 Hz, 250 Hz, and 2,000 Hz. Results: CPT values were significantly higher in the patient group than in the control group, especially with stimuli at the lowest frequency of 5 Hz (p < 0.05). There were significant correlations between the CPT values obtained at three different frequencies in the patient group, whereas the correlation was only significant in the pair of 250 Hz/5 Hz (both in the hands and feet), and in the pair of 2,000 Hz/250 Hz (in the feet) for the control group. Conclusions: Our data suggest that the CPT test, especially at a stimuli frequency of 5 Hz, may be a useful screening tool for diabetic polyneuropathy in patients who show no abnormalities in routine NCS. Citations Citations to this article as recorded by
Background: The autophagy is the major route for lysosomal degradation of misfolded protein aggregates and oxidative cell components. We hypothesized that rapamycin (autophagy enhancer) would prolong the survival of motor neuron and suppress the disease progression in amyotrophic lateral sclerosis (ALS). Methods: A total of 24 transgenic mice harboring the human G93A mutated SOD1 gene were used. The clinical status involving rotarod test and survival, and biochemical study of ALS mice model were evaluated. Results: The onset of symptoms was significantly delayed in the rapamycin administration group compared with the control group. However, after the clinical symptom developed, the rapamycin exacerbated the disease progression and shortened the survival of ALS mice model, and apoptosis signals were up-regulated compared with control group. Conclusions: Even though further detailed studies on the relevancy between autophagy and ALS will be needed, our results revealed that the rapamycin administration was not effective for being novel promising therapeutic strategy in ALS transgenic mice and exacerbated the apoptosis.
Citations Citations to this article as recorded by
Geographical differences in the incidence of amyotrophic lateral sclerosis (ALS) have been reported, and there are stillmany unresolved problems. The incidence as well as epidemiologic data of ALS is not known in Korea. Web-based multicenterregistry of ALS, the Korean ALS registry, was established at January, 2011. The aim of
Background
Although quantitative sensory test (QST) is being used with increasing frequency for measuring sensorythresholds in clinical practice and epidemiologic studies, there has been no age-matched normative data in Korean adults.The objective of this study is to evaluate the value of QST in diabetic polyneuropathy with normal range in Korean adults.Methods: The Computer Aided Sensory Examination IV 4,2 (WR Medical Electronics Co., Stillwater, Minnesota, U.S.A.),with 4,2,1 stepping algorithm was used to determine vibration and cold perception threshold in 70 normal controls and 19patients with diabetic polyneuropathy aged from 21 to 79 years. The data were used to define age-matched upper and lowernormal limits and normal range of side to side difference. We also evaluated the duration of diabetes, serum HbA1C level,and findings of nerve conduction study (NCS) and QST in patients with diabetic polyneuropathy. Results: In normal adults,sensory thresholds slightly increased with age, and a slight side-to-side difference was observed. The diagnostic sensitivityof QST was not higher than NCS in patients with diabetic polyneuropathy (36.8% vs. 42.1%, p=0.716), especially amongelderly patients. Conclusions: QST might be used as a complementary test for NCS in the diagnosis of diabeticpolyneuropathy. Although the QST is a simple method for the evaluation of peripheral nerve function, there are somelimitations. Most of all, because the QST measuring is dependent on the subjective response of patients, the degree of concentrationand cooperation of the patients can significantly affect the result. And thus, attention should be paid during theinterpretation of QST results in patients with peripheral neuropathy.
Autoimmune myasthenia gravis (MG) frequently involves the extrinsic ocular muscles (EOM) and the disease clinically restricted to the EOM is referred to as ocular myasthenia (OMG). It has been reported that approximately 50% of patients presenting with OMG subsequently progress to develop generalized disease, usually within the first one to two years. Several clinical and laboratory factors, such as older age at onset, presence of anti-acetylcholine receptor (AChR) antibody, high antibody titers, and the presence of thymoma, have been claimed to predict the risk for secondary generalization. As for the treatment of OMG, early immunosuppressive therapy, specifically corticosteroids, has been suggested to prevent or delay the progression into generalized disease, although the cost-to-benefit ratio of this treatment remains controversial with no randomized controlled study conducted so far. There is mounting epidemilogical evidence of ethnic difference in clinical manifestation of MG, particularly between Caucasians and Asians. Asians appears to have an earlier childhood onset, higher prevalence of OMG, and lower titers of AChR antibody. This article should be focused on OMG, particularly regarding its definition, natural course, diagnostic and therapeutic challenges. Also it will be covered why EOM is susceptible to MG.
Many neurologists, particularly whose subspecialty is in peripheral neurology, may agree that patients with peripheralneuropathy often complain of the
Colchicine is a drug used for the treatment of acute gouty arthritis or various autoimmune diseases. Gastrointestinaladverse effects such as abdominal pain and vomiting are the common side effects of the drug, but rarely myopathy hasbeen reported to occur particularly in renal recipients who were treated concomitantly with immunosuppressants. Herein,we report a case who presented with acute myopathy after treated with colchicine for acute gouty arthritis.
Autoimmune myasthenia gravis (MG) is the neuromuscular junction disorder mostly caused by antibody against the acetylcholine receptor (AChR antibody) at the muscle endplate. The goal of treatment is to induce and maintain remission, i.e., absence of symptoms, with the least cost-to-benefit ratio. Although corticosteroids are effective in inducing remission in most patients, they have numerous potentially serious adverse effects with their long-term use. In addition, some patients do not respond or are intolerant to the conventional treatment. In this article, we discuss the difficulties encountered in long-term immunosuppressive treatment of MG, and review useful tips for the use of corticosteroids. Long-term immunosuppressive agents that can be used in steroid-refractory or -dependent patients will be reviewed with their safety profiles and efficacy in MG.
Nemaline myopathy is one of the congenital myopathy, which is characterized by histological findings of nemaline bodies (rods) and type 1 fiber hypotrophy and predominance. It can be classified into three forms according to the age of onset and clinical features: congenital form, childhood-onset form, and adult-onset form. We report an 18-year-old patient with generalized muscle weakness and dysmorphic features who was diagnosed as typical childhood-onset nemaline myopathy.
Background
There is no currently effective treatment for amyotrophic lateral sclerosis (ALS), although this disorder is a progressive neurodegenerative disease resulting in death within several years. Because recent evidence suggests that homocysteine (HC) is highly related to neurodegenerative disorders with aging, we tried to elucidate the effects of multi-vitamin therapy on G93A SOD1 transgenic mice.Methods: We treated this murine model of ALS with multi-vitamin (folic acid 1.97 mg/day, pyridoxine 0.98 mg/day, cyanocobalamin 0.1 mg/day) from 45 days of age, per oral. We performed the rotarod test from postnatal 10th week,weekly.Results: We found that multi-vitamin reinforcement significantly prolonged average lifespan and delayed disease onset with improvement of motor performance. However, it did not significantly slow disease progression and statistical differences of weight loss were not observed between in transgenic mice and controls.Conclusions: These results suggest that multi-vitamin can be a potent therapeutic strategy for familial forms of ALS.
Background
s and objectives: POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes)syndrome is the rare cause of polyneuropathy. Although the polyneuropathy is essential for the diagnosis of the disease,the pattern of electrodiagnostic abnormalities has not been characterized in detail. The purpose of this study was to elucidatethe features of nerve conduction abnormalities in POEMS syndrome. Methods: We reviewed the medical recordsand nerve conduction studies (NCS) of 12 consecutive patients with POEMS. Results: A total of 68 motor and 46 sensorynerves were examined. Compound muscle action potentials (CMAPs) and sensory nerve action potentials wereabnormally attenuated or not elicited in majority of motor and sensory nerves (80.88% in motor, and 82.6% in sensorynerves). Frequency of the nerves with no potential was significantly higher in lower limbs than in upper limbs (p<0.01in both motor and sensory nerves), and CMAP amplitude was more reduced in lower limbs than in upper limbs(p<0.01). Conduction slowing was very frequently observed with 95% and 76% of motor and sensory nerves, respectively,having the abnormally reduced values of conduction velocity. Distal motor latencies were abnormally prolongedin 75% of motor nerves, and terminal latency indices were significantly higher in patients than in normal controls (p <0.05). Conduction block was observed only in 5% of motor nerves. Conclusions: NCS in POEMS syndrome showedcharacteristic patterns, in which conduction abnormalities were more frequently and severely affected in the lowerlimbs, and more predominantly in the intermediate nerve segments than in the distal portions. The recognition of thesecharacteristic patterns may be helpful in early diagnosis of polyneuropathy in POEMS syndrome.
Background
and Objectives: The proximal and distal nerve segments are preferentially involved in acquireddemyelinating polyneuropathies (ADP). This study was undertaken in order to assess the usefulness of motor evokedpotential (MEP) and somatosensory evoked potential (SSEP) in the detection of the proximal nerve lesion in ADP.Methods: MEP, SSEP and conventional NCS were performed in 6 consecutive patients with ADP (3 AIDP, 3 CIDP).MEP was recorded from abductor pollicis brevis and abductor hallucis using magnetic stimulation of the cortex and thecervical/lumbar spinal roots. SSEP were elicited by stimulating the median and posterior tibial nerves. Latency fromcortex and cervical/lumbar roots, central motor conduction time (CMCT), EN1-CN2 interpeak latency were measuredfor comparison.Results: MEP was recorded in 24 limbs (12 upper and 12 lower limbs) and SSEP in 24 limbs (12 median nerve, 12posterior tibial nerve). F-wave latency was prolonged in 25 motor nerves (25/34, 73.5%). Prolonged CML and PMLwere found in 41.7% (10/24) and 45.8% (11/24), respectively. Interside difference (ISD) of CMCT was abnormallyincreased in the upper extremity, 66.7% (4/6 pairs) in case of CML-PML. EN1-CN2 interpeak latency was abnormallyprolonged in one median nerve (1/10) and LN1-P1 interpeak latency was normal in all posterior tibial nerves.Conclusions: MEP and SSEP may provide useful information for the proximal nerve and root lesion in ADP. MEPand SSEP is supplemental examination as well as complementary to conventional NCS.
Sarcoidosis is multisystemic granulomatous disease of unknown etiology ogenesis, and most frequently presented with bilateral hilar lymphadenopathy, pulmonary infiltration, skin and eye lesion. However, neurological involvement including peripheral neruopathy is relatively rare. We report a patient who had sensorimotor polyneuropathy without other systemic or organ involvements frequently reported in sarcoidosis. Laboratory investigation suggestive of sarcoidosis lead to sural nerve biopsy for confirmation, which demonstrated noncaseating granulomatous changes. Sarcoidosis should be included in the differential diagnosis in subacute polyneuropathy even if there is no usual symptoms or signs suggestive of the systemic disease.
Background
: Guillain-Barre syndrome(GBS) is a neurologically emergent condition, leading to respiratory insufficiency without an early and appropriate treatment. Thus, the treatment of GBS requires early diagnosis but it is difficult due to the low sensitivity of laboratory tools in the initial stage. Hoffman reflex (H-reflex) and its facilitation by Jendrassik maneuver (JM) are sensitive tools evaluating the central circuit of motor system on the spinal cord level. The aim of this study is to test whether the change of H-reflex and F-wave under the JM is able to detect the early stage of GBS and whether GBS involves the central nervous system (CNS). Material and Methods : All 7 GBS patients who showed normal or nearly normal nerve conduction study were included. The facilitation of H-reflex and changes of F-wave were calculated by measuring the percent difference f H-reflex or F-wave amplitude under JM compared to basal H-reflex of F-wave amplitude. The changes of F-wave and H-reflex in the GBS patients were compared with them of 8 healthy controls. Results : The F-wave amplitudes of both healthy controls and GBS patients did not changed under the influence of JM (102.4
Objectives
: Although the diagnosis of hereditary neuropathy with liability to pressure palsies(HNPP) in important for correct prognostic evaluation and genetic counseling, the diagnosis is frequently missed or delayed. Our main aim on undertaking this study was to characterized\ the electrodiagnostic features of HNPP. Material and Methods : Clinical, electrophysiologic and molecular studies were performed on Korean HNPP patients with 17P11.2 deletion. The results of eletrophysologic studies were compared with those of Charcot-Marie-Tooth disease type 1A (CMT1A) patients carrying 17p11.2 duplication. Results : Eight HNPP (50 motor, 39 sensory nerves) and six CMTIA (28 motor, 16 sensory nerves) patients were included. The slowing of sensory conduction in nearly all nerves and the distal accentuation of motor conduction abnormalities are the main features of background polyneuropathy in HNPP. In contrast to CMTIA, where severity of nerve conduction slowing was not different among nerve groups, HNPP sensory nerve conduction was more slowed in the median and ulnar nerves than in the sural nerve (p<0.01), and DML was more prolonged in the median nerve than in the other motor nerves (p<0.01). TLIs were significantly lower in HNPP than in the normal control and CMTIA patients for the median and ulnar nerves (p<0.01), and were also significantly reduced for the peroneal nerve (p<0.05) compared with those of the normal controls. Conclusion : The distribution and severity of the background electrophysiologic abnormalities are closely related to the topography of common entrapment or compression sites, which suggests the possible pathogenetic role of subclinical pressure injury at these sites in the development of the distinct background polyneuropathy in HNPP.
|
|
