• Annals of Clinical Neurophysiology
• /
• 제14권1호
• /
• pp.29-35
• /
• 2012

Background: Peripheral neuropathy is the most frequent neurological complication in human immunodeficiency virus (HIV) infection, related with diverse etiologies including inflammation, opportunistic infection and side effects of medications. The purpose of the present study was to evaluate characteristics of HIV associated neuropathy according to the stage of HIV infection. Methods: In reviewing the medical records of HIV patients who underwent electrodiagnostic studies between 1997 and 2011, total 11 patients (all males; median age, 47 years; range, 28-71 years) with comorbid neuropathy were enrolled. Stage of HIV infection was categorized according to the Centers for Disease Control and Prevention (CDC) criteria. Classification of peripheral neuropathy was based on clinical and electrophysiological features. Results: Distal symmetric polyneuropathy was observed in 8 patients (72.7%), inflammatory demyelinating polyneuropathy in 2 patients (18.1%), and polyradiculopathy in 1 patient (9.1%). Median CD4+ T cell count was $123/mm^3$ (range, $8-540/mm^3$) and 7 patients (60%) had the most advanced HIV disease stage (CDC-C3). There was no neuropathy caused by CMV infection. Conclusions: Distal symmetric polyneuropathy was the most common type of neuropathy in HIV infection, but various forms of neuropathy such as inflammatory demyelinating polyneuropathy and polyradiculopathy were also present. HIV associated neuropathy is more frequently associated with advancing immunosuppression, although it can occur in all stages of HIV infection.

• Annals of Clinical Neurophysiology
• /
• 제6권1호
• /
• pp.20-25
• /
• 2004

Background and Objectives: The proximal and distal nerve segments are preferentially involved in acquired demyelinating polyneuropathies (ADP). This study was undertaken in order to assess the usefulness of motor evoked potential (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 the cervical/lumbar spinal roots. SSEP were elicited by stimulating the median and posterior tibial nerves. Latency from cortex and cervical/lumbar roots, central motor conduction time (CMCT), EN1-CN2 interpeak latency were measured for comparison. Results: MEP was recorded in 24 limbs (12 upper and 12 lower limbs) and SSEP in 24 limbs (12 median nerve, 12 posterior tibial nerve). F-wave latency was prolonged in 25 motor nerves (25/34, 73.5%). Prolonged CML and PML were found in 41.7% (10/24) and 45.8% (11/24), respectively. Interside difference (ISD) of CMCT was abnormally increased in the upper extremity, 66.7% (4/6 pairs) in case of CML-PML. EN1-CN2 interpeak latency was abnormally prolonged 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. MEP and SSEP is supplemental examination as well as complementary to conventional NCS.

• Journal of Korean Neurosurgical Society
• /
• 제55권6호
• /
• pp.370-374
• /
• 2014

To present a case of cauda equina syndrome (CES) caused by chronic inflammatory demyelinating polyneuropathy (CIDP) which seemed clinically similar to Charcot-Marie-Tooth disease type1 (CMT1). CIDP is an immune-mediated polyneuropathy, either progressive or relapsing-remitting. It is a non-hereditary disorder characterized by symmetrical motor and sensory deficits. Rarely, spinal nerve roots can be involved, leading to CES by hypertrophic cauda equina. A 34-year-old man presented with low back pain, radicular pain, bilateral lower-extremity weakness, urinary incontinence, and constipation. He had had musculoskeletal deformities, such as hammertoes and pes cavus, since age 10. Lumbar spine magnetic resonance imaging showed diffuse thickening of the cauda equina. Electrophysiological testing showed increased distal latency, conduction blocks, temporal dispersion, and severe nerve conduction velocity slowing (3 m/s). We were not able to find genetic mutations at the PMP 22, MPZ, PRX, and EGR2 genes. The pathologic findings of the sural nerve biopsy revealed thinly myelinated nerve fibers with Schwann cells proliferation. We performed a decompressive laminectomy, intravenous IgG (IV-IgG) and oral steroid. At 1 week after surgery, most of his symptoms showed marked improvements except foot deformities. There was no relapse or aggravation of disease for 3 years. We diagnosed the case as an early-onset CIDP with cauda equine syndrome, whose initial clinical findings were similar to those of CMT1, and successfully managed with decompressive laminectomy, IV-IgG and oral steroid.

• Korean Journal of Radiology
• /
• 제21권4호
• /
• pp.483-493
• /
• 2020

Objective: To evaluate the distribution and characteristics of peripheral nerve abnormalities in chronic inflammatory demyelinating polyneuropathy (CIDP) using magnetic resonance neurography (MRN) and to examine the diagnostic efficiency. Materials and Methods: Thirty-one CIDP patients and 21 controls underwent MR scans. Three-dimensional sampling perfections with application-optimized contrasts using different flip-angle evolutions and T1-/T2- weighted turbo spin-echo sequences were performed for neurography of the brachial and lumbosacral (LS) plexus and cauda equina, respectively. Clinical data and scores of the inflammatory Rasch-built overall disability scale (I-RODS) in CIDP were obtained. Results: The bilateral extracranial vagus (n = 11), trigeminal (n = 12), and intercostal nerves (n = 10) were hypertrophic. Plexus hypertrophies were observed in the brachial plexus of 19 patients (61.3%) and in the LS plexus of 25 patients (80.6%). Patterns of hypertrophy included uniform hypertrophy (17 [54.8%] brachial plexuses and 21 [67.7%] LS plexuses), and multifocal fusiform hypertrophy (2 [6.5%] brachial plexuses and 4 [12.9%] LS plexuses) was present. Enlarged and/or contrast-enhanced cauda equina was found in 3 (9.7%) and 13 (41.9%) patients, respectively. Diameters of the brachial and LS nerve roots were significantly larger in CIDP than in controls (p < 0.001). The largest AUC was obtained for the L5 nerve. There were no significant differences in the course duration, I-RODS score, or diameter between patients with and without hypertrophy. Conclusion: MRN is useful for the assessment of distribution and characteristics of the peripheral nerves in CIDP. Compared to other regions, LS plexus neurography is more sensitive for CIDP.

• 대한수의학회지
• /
• 제33권2호
• /
• pp.295-300
• /
• 1993

홍역이환개의 중추신경계에서 발생한 수초탈락을 수반하는 뇌척수염을 병리조직학적으로 관찰하고 조직반응과 수축탈락의 과정을 알아보기 위하여 중추신경계 조직을 파라핀 포매, 절편을 만들고 병소가 빈발하는 소뇌와 시신경로는 수초의 주요 구성단백인 MBP와 MAG 그리고 별아교세포의 Marker인 GFAP의 항혈청을 반응시켜 면역세포학적으로 관찰하였다. 조직학적으로는 대뇌에서 신경세포의 일부괴사, 신경아교세포의 결절형성, 소뇌에서는 4뇌실에 인접하여 백질부에서 수초의 공포변성, 수초탈락과 염증세포의 침윤이 현저하였고 피질부의 과립층에 인접한 부위와 시신경로에서도 수초탈락이 인정되었다. MBP와 MAG를 면역반응시킨 결과 수초의 공포화와 수초탈락이 현저한 부위에서는 MBP와 MAG가 동시에 소실되었고 부위에 따라서는 MBP또는 MAG가 먼저 소실되기도 하였다. 동시에 별아교세포의 반응은 수초탈락 초기에는 GFAP양성의 섬유와 세포가 크게 증가한 반면 수초탈락이 심하게 진행된 부위에서는 오히려 소실되는 경향이었다. 따라서 홍역이환 개에서 발생되는 수초탈락은 일차적으로 수초가 공격을 받아 파괴됨을 알 수 있었고 동시에 부위에 따라 다른 소견을 요인별로 비교하였다.

• Annals of Clinical Neurophysiology
• /
• 제12권2호
• /
• pp.76-78
• /
• 2010

• Clinical and Experimental Pediatrics
• /
• 제58권5호
• /
• pp.194-198
• /
• 2015

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-$Barr{\acute{e}}$ syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

• Annals of Clinical Neurophysiology
• /
• 제1권2호
• /
• pp.91-98
• /
• 1999

Background : The pathogenesis of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), Guillain Barre syndrome (GBS) is not clear, but it has been known that the immune mechanisms play an important role. Authors performed this study to establish an animal model of experimental allergic neuritis (EAN) by immunizing the myelin components of peripheral nerves and to understand the electrophysiological and histopathological features as well as the ${CD_5}^+$ B-lymphocyte changes in peripheral bloods in the EAN models. Methods : Lewis rats weighing 150-200 gm were injected subcutaneously in soles two times with total myelin, P0, P1, or P2 proteins purified from the bovine cauda eguina. The EAN induction was assessed by evaluating clinical manifestations. The electrophysiological and histopathological features were studied as routine methods. The ${CD_5}^+$ Blymphocytes were double stained using monoclonal FITC conjugated anti-rat CD45RA and R-PE conjugated anti-rat ${CD_5}^+$ antibodies and calculated using a fluorescence activated cell sorter (FACS). Results : The EAN animal models were established. In two out of five, in one out of two, in none out of three, and in none out of one Lewis rats injected with purified total myelin, P0, P1, P2 proteins respectively, They showed slow spontaneous motor activity and weak resistance against pulling back by tails. The typical electrophysiological and histologic findings in total protein and P0 induced EAN animal models were the decreased conduction velocity, the decreased compound muscle action potential (CMAP) amplitude and the dispersion phenomenon. The perivascular infiltrates of lymphocytes with focal demyelinating process were found in light microscopy. The ${CD_5}^+$ B-lymphocyte expression in three EANs were 2.38%, 3.50% 2.50%, which were not significantly increased, compared with those in normal controls. Conclusion : The EAN animal models were successfully established by injecting the total myelin and P0 myelin and they showed electrophysiological and histological features typical of demyelinating process. However they did not show an increased expression of ${CD_5}^+$ B-lymphocyte in peripheral bloods which could be indirect evidence of humoral autoimmunity.

• 대한임상검사과학회지
• /
• 제53권2호
• /
• pp.151-157
• /
• 2021

선천성 유전질환인 CMT와 후천성 면역 매개 질환인 CIDP는 임상적 증상이 유사하므로 두 질환의 감별진단을 위해서는 말초신경의 전기생리학적 특징을 비교하는 것이 도움이 될 수 있다. 본 연구는 CIDP와 CMT 1형으로 확진된 환자의 신경전도검사 결과 중 감각신경전도검사의 주요 지표별 결과를 후향적으로 정량분석하여 두 질환군의 전기생리학적 특징을 규명하고자 하였다. CIDP (N=35)와 CMT1 (N=30)로 확진된 환자의 dCNAP와 SNCV를 이용하여 두 질환군의 중증도 분석, 유의성 검정, 비정상 범위별 비율분석 및 상관분석을 실시하여 통계적 차이를 기반으로 특징을 비교하였다. 두 질환 모두 전신성 말초신경다발신경병증의 특징이 관찰되었고, 장딴지신경의 손상이 가장 심한 것으로 확인되었다. CMT1군은 탈수초성 및 축삭성 신경병증을 동반하는 전신성 신경병증이고, CIDP보다 더 중증의 신경병증임이 확인되었다. 또한, 상관계수 및 산점도 분석에서 CMT1은 신경 손상 범위가 전체 신경에서 균등한 전기생리학적 특징이 관찰되었다. 감각신경전도검사의 결과를 기반으로한 본 연구결과가 CIDP와 CMT 1형의 감별진단 및 연구에 도움이 될 것으로 사료된다.

• Annals of Clinical Neurophysiology
• /
• 제8권2호
• /
• pp.179-181
• /
• 2006

Multiple sclerosis is an autoimmune demyelinating disorder of the nervous system. The ocular manifestation includes optic neuritis, internuclear opthalmoplegia and nystagmus. Upbeat nystagmus is a rare manifestation of multiple sclerosis. We report a patient with relapsing multiple sclerosis who presented with upbeat nystagmus from a circumscribed lesion in the caudal medulla.