Critical illness neuromyopathy (CINM) is a common yet frequently overlooked complication in intensive care units (ICU). CINM often results in prolonged ventilator dependence and persistent limb weakness, significantly impacting patient recovery and long-term quality of life. CINM can be categorized into two primary subtypes: critical illness polyneuropathy (CIP) and critical illness myopathy (CIM). These conditions frequently arise in the context of severe sepsis, multiple organ failure, or as adverse effects of certain medications used in the ICU. This review aims to provide a concise overview of CINM, focusing on its pathophysiology, diagnostic approaches, and current clinical management strategies.
Leprosy is a chronic infectious disease caused by Mycobacterium leprae. It can lead to damage of the nerve. Although the incidence of leprosy is very low in South Korea, a large number of people are immigrating to South Korea from countries with a high prevalence of leprosy. We report a case of leprosy confirmed by nerve biopsy. The patient was from Nepal who presented with progressive and asymmetric sensory loss. Leprosy can be considered as a differential diagnosis in patients with progressive and asymmetric sensory loss, especially when patients are from leprosy endemic countries.
Background The younger generation in Korea easily exposed to nitrous oxide (N2O) under the name “Happy Balloon” may abuse it. N2O can irreversibly oxidize vitamin B12 and cause abnormal hematopoiesis or nervous system toxicity such as subacute combined degeneration (SCD). The objective of this study was to assist in early diagnosis of N2O-induced SCD of spinal cord by characterizing its clinical manifestations.
Methods Four patients with myelopathy after abusing N2O were enrolled. To characterize N2O-induced myelopathy, previously reported cases of N2O-induced SCD were searched through PubMed and KoreaMed. Collected cases and our four patients were analyzed.
Results A total of 30 patients with N2O-induced myelopathy (26 males and 4 females with mean age of 24 years) were analyzed. These patients took a median dose of 650 canisters for a median duration of 3.5 months. All patients presented sensory disturbances, which involved the lower extremities more frequently (100%) than the upper extremities (63.3%). Gait ataxia (76.7%), weakness in the upper (23.3%) and lower (36.7%) extremities, bladder symptoms (26.7%), Rhomberg sign (43.3%), and Lhermitte’s phenomenon (10.0%) were observed. Serum vitamin B12 levels were decreased in many (63.3%) patients and homocysteine levels were elevated in all. Of 20 patients who underwent magnetic resonance imaging (MRI) of the spine, 19 had abnormal findings. Three patients presented with contrast enhancement in lesions.
Conclusions We strongly recommend that history of N2O abuse should be asked for young patients with suspected myelopathy, especially those presenting with gait ataxia and sensory disturbances suggesting posterior column dysfunction and those presenting long-segment lesion involving the upper cervical cord on MRI.
Paraproteinemic neuropathy is a heterogeneous set of neuropathies characterized by the presence of homogeneous immunoglobulin in the serum. Most cases are associated with monoclonal gammopathy of undetermined significance. It might also occur in hematologic malignant and nonmalignant conditions. The association between neuropathy and monoclonal gammopathy requires appropriate neurological and hematological investigations. Treatment is mostly based on underlying hematologic disorders. In this review, we provide a clinically practical approach to clinical, laboratory, electrophysiological features and management of patients with paraproteinemic neuropathy.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic recurrent acquired immune-mediated disease of the peripheral nerves that presents with progressive sensory and motor deficits in all four limbs. Cranial nerve involvement is not as common as in Guillain-Barre syndrome, and central nervous system involvement including optic neuritis has rarely been reported in patients with CIDP. We recently experienced a case with classic CIDP involving bilateral facial and trigeminal nerves, right lower cranial nerves, and the right optic nerve.
Background There is inadequate information on the validation of diabetic microvascular complications in the Korean National Health Insurance Service data set. We aimed to validate the diagnostic algorithms regarding the nephropathy, neuropathy, and retinopathy of diabetes.
Methods From various secondary and tertiary medical centers, we selected 6,493 patients aged ≥ 40 years who were diagnosed with diabetic microvascular complications more than once based on codes in the 10th version of the International Classification of Diseases (ICD-10). During 2019 and 2020, we randomly selected the diagnoses of 200 patients, 100 from each of two hospitals. The positive predictive value (PPV), negative predictive value, error rate, sensitivity, and specificity were determined for each diabetic microvascular complication according to the ICD-10 codes, laboratory findings, diagnostic studies, and treatment procedure codes.
Results Among the 200 patients who visited the hospital more than once and had the diagnostic codes of diabetic microvascular complications, 142, 110, and 154 patients were confirmed to have the gold standard of diabetic nephropathy (PPV, 71.0%), diabetic neuropathy (PPV, 55.0%), and diabetic retinopathy (PPV, 77.0%), respectively. The PPV and specificity of diabetic nephropathy (PPV, 71.0–81.4%; specificity, 10.3–53.4%), diabetic neuropathy (PPV, 55.0–81.3%; specificity, 66.7–76.7%) and diabetic retinopathy (PPV, 77.0–96.6%; specificity, 2.2–89.1%) increased after combining them with the laboratory findings, diagnostic studies, and treatment procedures codes. These change trends were observed similarly for both hospitals.
Conclusions Defining diabetic microvascular complications using ICD-10 codes and their related examination codes may be a feasible method for studying diabetic complications.
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Immune checkpoint inhibitors (ICIs) have emerged as one of the most promising therapeutic options for advanced cancers. While ICIs have improved survival in multiple cancers, their increased use is restricted by various immune-related adverse events. In this report we describe a patient with renal cell carcinoma who received a combination of ICIs, nivolumab plus ipilimumab, and who developed lumbosacral polyradiculoneuropathy. Corticosteroid use was an effective treatment for this patient.
Facial diplegia (FD) rarely occurs as a regional Guillain-Barré syndrome (GBS) variant. A 70-yearold male presented with bifacial weakness that had started on the left side and extended to the right after several days. He was then treated using steroids and gradually improved. Serum antiganglioside antibody testing revealed positivity for anti-GM1 IgG antibodies. FD can be idiopathic, but it is an uncommon GBS variant. The ganglioside antibody test may increase the possibility of diagnosing isolated FD.
Small fiber neuropathy is a painful neuropathy that cannot be assessed using nerve conduction studies. A skin biopsy and quantitative sensory testing (QST) are the gold standards for small fiber neuropathy diagnosis. However, a skin biopsy is invasive and commercially unavailable in Korea. QST is a method involving a thermal threshold, but its results can be affected by cognition as well as lesions of the central nervous system. Quantitative sudomotor axon reflex test (QSART) is a quantitative method of assessing sweat glands innervated by small fibers. In this review, we assessed the utility of QSART in evaluating small fiber neuropathy.
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Nitrous oxide (N2O), an anesthetic gas, has been abused by some people for entertainment purposes. Recently, N2O (under the name of "Happy Balloon") abuse has become problematic among young people in Korea. N2O intoxication can develop a neuropathy, as well as other systemic disorders, either by inactivating vitamin B12 or by a direct neurotoxic mechanism. Here, we report a case of peripheral neuropathy with possible coexisting myelopathy following N2O abuse.
Primary hyperparathyroidism (PHP) is a disease in which excessive amounts of parathyroid hormone (PTH) are secreted and calcium levels in the blood increase. Hypercalcemia caused by PHP has a major influence on the peripheral nervous system and produces symptoms such as muscle cramps, paresthesia, and proximal muscle weakness. Here we report a rare case of sensory-dominant polyneuropathy caused by PHP, which improved after surgery.
Background Nitrous oxide (N2O) is used in surgery and dentistry for its anesthetic and analgesic effects. However, neurological and psychiatric manifestations of N2O abuse have been increasingly reported among Korean adults. The aim of this study was to demonstrate laboratory findings of N2O abuse in Korean patients.
Methods Patients diagnosed with N2O-induced neuropathy or myelopathy from August 2018 to December 2019 were enrolled. Their clinical presentations and laboratory and imaging findings were analyzed.
Results Sensory changes and limb weakness were present in nine of the enrolled patients. The laboratory findings revealed that seven patients had high homocysteine levels and five had high methylmalonic acid levels in their blood. Nerve conductions studies indicated that axonal neuropathy was present in four cases and longer F-wave and Hoffman’s-reflex latencies were present in two cases. Signal changes in cervical spine imaging occurred in five patients, while two had normal results.
Conclusions Chronic N2O abuse can cause neurological damage or psychiatric problems. Because N2O is illegal for recreational use in Korea, patients tend to hide their history of use. Even though the spinal imaging results were normal, clinicians should consider the possibility of N2O use, and further electrophysiological tests should be applied for precise evaluations.
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Nitrous‐oxide‐induced polyneuropathy and subacute combined degeneration of the spine: clinical and diagnostic characteristics in 70 patients, with focus on electrodiagnostic studies L. T. Hassing, F. Y. Jiang, R. Zutt, S. Arends European Journal of Neurology.2024;[Epub] CrossRef
Severe Isolated Peripheral Polyneuropathy without Myelopathy after Nitrous Oxide Abuse: A Case Report Seung-Min Baek, Seungbok Lee, Yu-Mi Kim, Eun-Sil Kim Journal of Electrodiagnosis and Neuromuscular Dise.2022; 24(2): 50. CrossRef
Critical illness neuromyopathy (CINM) is a common but frequently underdiagnosed condition in critically ill patients that contributes to ventilator weaning failure and limb weakness in intensive care unit (ICU). CINM is subdivided into critical illness polyneuropathy and critical illness myopathy, and the occurrence of these conditions in the ICU is associated with multiple organ failure due to sepsis or certain medications. CINM survivors might have persistent functional disabilities and a poor quality of life. This situation demonstrates the need for efforts to minimize or prevent CINM in critically ill patients. This article provides a current overview of CINM and the associated clinical strategies.
A 21-year-old soldier was admitted due to weakness after carrying a heavy military bag and marching for a long time. Neurophysiologic investigation revealed prominent involvement of right brachial plexus and upper cervical root with mild abnormalities of multiple nerves in the other extremities. Hereditary neuropathy with liability to pressure palsy was confirmed by gene test demonstrating deletion of PMP22 gene. This study presents backpack palsy can appear as a first manifestation of hereditary neuropathy with liability to pressure. The possibility of hereditary neuropathy with liability should be strongly considered in a young patient with non-symptomatic multiple neuropathy.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy with heterogeneous features. Appropriate treatment will produce a favorable outcome, but a poor treatment response and severe disability have also been reported. The roles of the clinical phenotypes and electrophysiological features of CIDP as well as of autoantibodies against nodal and paranodal proteins have been highlighted previously due to their association with the treatment response and long-term prognosis. This review addresses the diverse factors associated with the prognosis of CIDP.
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Electrodiagnostic tests (EDX) is essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). EDX could provide information about demyelinating pathology in the peripheral nerves. According to phenotypes, CIDP could be classified several phenotypes, which has different clinical manifestations, EDX could present a different distribution pattern of demyelinating lesions. In addition, EDX could be useful markers for predicting treatment response of prognosis of CIDP.
Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is a variant of chronic acquired demyelinating polyneuropathy. A 65-year-old women presented with upper arm weakness. A nerve conduction study showed conduction blocks over intermediate
segments with sparing of distal compound action potentials. Magnetic resonance imaging revealed asymmetric hypertrophy of the brachial plexus on the affected side. These findings
represent important electrophysiological and radiological evidence of MADSAM neuropathy. The condition of the patient began to improve after starting intravenous immunoglobulin administration.
Skin biopsy and staining the specimens with immuno-reactive markers has been proven to be a useful method to demonstrate the pathologic status of small nerve fibers. Quantification of intraepidermal nerve fiber density using anti-protein gene product 9.5 antibody is a standard method to diagnose small fiber neuropathy. Skin biopsy also makes it possible to differentiate the nerve fibers according to their function by using different markers. Quantification of dermal structures with different types of nerve fibers could reveal the pathophysiologic mechanism of the disease state.
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Paraproteinemia is caused by a proliferation of monoclonal plasma cells or B lymphocytes. Approximately 10% of idiopathic neuropathies are associated with paraproteinemia, where a certain paraprotein acts like an antibody targeted at constituents of myelin or axolemma in peripheral nerves. The relationship between paraproteinemia and peripheral neuropathy remains unclear despite this being of interest for a long time. Neurologists frequently find paraproteinemia during laboratory examinations of patients presenting with peripheral neuropathy, especially in the elderly. The possibility of a relationship with paraproteinemia should be considered in cases without an explainable cause. We review the causal association between paraproteinemia and neuropathy as well as clinical, laboratory, and electrophysiologic features, and the treatment options for paraproteinemic neuropathy.
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Ulnar neuropathy at the elbow (UNE) may seem easy to diagnose when the characteristic clinical manifestations are present, and electrodiagnostic studies have high sensitivity, although they are non-localizing in some cases and unable to reveal structural lesions. Ultrasonography is noninvasive and able to find the exact location of the lesion and visualize perineural structures. We present two cases of UNE in which we found hypoechoic mass lesions near medial epicondyle with ultrasonography and discuss its usefulness in diagnosis of UNE.
Skin biopsy with investigation of small nerve fiber in human epidermis and dermis has been proven to be a useful method for demonstration of small fiber neuropathy. Quantification of intraepidermal nerve fiber density using anti-Protein Gene Product 9.5 (PGP 9.5) antibody is standardized method to diagnose the small fiber neuropathy. Skin biopsy method also makes it possible to differentiate the type of nerve fibers by using different antibodies. Quantification of dermal structures with different type of nerve fibers could be used to invest pathophysiologic mechanism of diseased state.
The paraproteinemia is a disorder in which a single clone of plasma cells (monoclonal gammopathy) is responsible for the proliferation of monoclonal proteins (M-proteins). Approximately 10% of patients with idiopathic peripheral neuropathy have monoclonal gammopathy. Some M-proteins have the properties of an antibody to the components of peripheral nerve myelin, but the pathophysiological relationship between the neuropathy and the M-protein is often obscure. The relationship between peripheral neuropathy and monoclonal gammopathy requires the appropriate neurological and hematological investigations for precise diagnosis and treatment. In this review, we provide an update on the causal associations between peripheral neuropathy and monoclonal gammopathy as well as characteristics of clinical and electrophysiologic features.
Amyloidosis is a systemic disorder associated with clonal plasma cell dyscrasia. Nephrotic syndrome, congestive heart failure, autonomic and peripheral neuropathy is often associated features in amyloidosis. Early diagnosis is most important because of different prognosis by stage. The diagnosis can be delayed since symptoms of amyloidosis may vary or nonspecific. We describe a patient of myeloma-associated amyloidosis, who showed orthostatic intolerance as the first symptom of the disease.
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Background: Early detection of neuropathy may prevent further progression of this complication in the diabetic patients. The purpose of this study was to evaluate the prevalence of early neuropathic complication in patients with newly diagnosed type 1 and type 2 diabetes. Methods: Nerve conduction studies (median, ulnar, posterior tibial, peroneal, and sural nerves) were performed for 49 type 1 (27 males, mean 14.1±7.5 years) and 40 type 2 (27 males, 42.0±14.1 years) diabetic patients at onset of diabetes. Children with age at onset under 4 years and adults over 55 years were excluded to eliminate the aging effect and the influence of obstructive arteriosclerosis. Neuropathy was defined as abnormal nerve conduction findings in two or more nerves including the sural nerve. Results: Mean HbA1c level was 12.6±3.3% for type 1 and 10.5±2.9% for type 2 diabetes. The prevalence of neuropathy was 12.2% for type 1, and 35.0% for type 2 diabetes, respectively. There were significant trends in the prevalence of neuropathy with increasing age (p<0.05). The effect of the mean level of glycosylated hemoglobin on the prevalence of polyneuropathy at onset of diabetes was borderline (p=0.0532). Neither sex of the patients nor the type of diabetes affected the neurophysiologic abnormalities at the diagnosis. Conclusions: Even in a population with diabetes at the diagnosis, the prevalence of subclinical neuropathy was not low. Neuropathy has been significantly associated with increasing age indicating the possibility of longer duration of undetected diabetes among them, especially in type 2 diabetes.
Thiamine deficiency can cause peripheral polyneuropathy and Wernicke’s encephalopathy. Wernicke’s encephalopathy is characterized by ataxia, ophthalmoplegia, nystagmus, and confusion, and typically presents acute and rapidly progressive course, whereas peripheral neuropathy associated with thiamine deficiency manifests chronic and slowly progressive one. However, acute and rapidly progressive axonal polyneuropathy combined with Wernicke’s encephalopathy is quite rare and unusual. Here, we describe a patient with Wernicke’s encephalopathy who presented with acute bilateral axonal neuropathy.
Multiple cranial and peripheral neuropathies as a delayed sequellae of ethylene glycol poisoning is a less well known clinical entity and its information about long-term electrophysiological and clinical outcomes is limited. We report a 45-year-old male who presented with acute renal failure and subsequently developed multiple cranial neuropathy, respiratory failure, and flaccid tetraparesis. Through sequential electrophysiological studies, we would like suggest that the main pathophysiology of ethylene glycol-related neuropathy is a demyelinating polyradiculoneuropathy with secondary axonal degeneration.
Some patients with leprosy may present with atypical features, such as isolated peripheral neuropathy without skin lesions, or marked proprioceptive dysfunction. We report a 56-year-old female who presented with predominant proprioceptive loss without skin lesion, but was finally confirmed as leprous neuropathy by sural nerve biopsy. It is postulated that large myelinated fibers were affected by chronic immunological reactions triggered by inactive bacterial particles, producing a peripheral neuropathy presenting as predominant proprioceptive sensory loss without typical skin lesions.
It was sometimes difficult to differentiate between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and subacute inflammatory demyelinating polyneuropathy (SIDP). The CNS involvement of these polyneuropathies has rarely reported in the literature. We present the case of a 42-year-old man who developed rapidly developing inflammatory demyelinating polyneuropathy followed by right optic neuritis. This case showed progressive motor weakness and sensory dysfunction with time to nadir at 8 weeks, demyelination in nerve conduction study, no other etiology of neuropathy, no relapse during follow-up of 18 months, good response to steroid and complete recovery which favor SIDP more than A-CIDP. We experienced the case of SIDP associated with optic neuritis.
Ischemic monomelic neuropathy and myopathy are rare complications of peripheral arterial occlusive disease. We reporta case of ischemic monomelic neuropathy of the right sural, common peroneal and posterior tibial nerves and ischemic myopathyof the right tibialis anterior resulting from the occlusion of the right common femoral arteries despite successfulrevascularization. Ischemic monomelic neuropathy and myopathy can occur as a result of occlusion of the specific peripheralartery.
Anorexia nervosa(AN) is a disorder characterized by disturbance of body image, fear of gaining weight, severe weight loss and, in female, amenorrhea. Compared with normal persons, patients with AN have neuropathic symptoms more frequently. But electrophysiologic abnormalities have rarely been reported. We experienced a case with recurrent neuropathic symptoms after severe weight loss. Further evaluation revealed AN. Electrophysiologic study showed sensorimotor polyneuropathy and focal neuropathy with conduction block. As far as we know, this feature of neuropathy in AN has not been described. We describe unusual feature of neuropathy in our patient with literature review.
Acute pandysautonomic neuropathy(APN) is an uncommon clinical entity involvement vasomotor, sudomotor, pupilomotor, secretomotor and other autonomic systems. Both sympathetic or parasympathetic fibers are involved with relative preservation of somatic sensory and motor function. Although APN shares several clinical features with GBS, it is not clear whether APN is a subvariety of GBS. We reported two young patients with APN. Patient I1 was a 18-year-old girl with recurrent fainting spells. Patients 2 was a 23-year-old man suffering from unexplained nausea and vomiting. Both had a history of previous upper respiratory infection. They presented with gastroparesis, anhydrosis and orthstatic hypotension. Mild numbness and tingling sense was present, but motor power was infact. Neurologic examination showed bilateral tonic pupil, decreased pain and vibration sense, and absent tendon reflexes. Nerve conduction study indicated diffuse sensorymotor polyneuropathy. Nerve biopsy in patient 2 revealed axonal degeneration. After conservative management, gastrointestinal symptoms were improved in patient 2, however, dysautonomic symptoms in young patient may indicate the diagnosis of APN. Although the natural course is generally benign, accurate diagnosis and proper management may be mandatory for the better clinical outcome.
Lateral antabrachial cutaneous neuropathy(LACN) was diagnosed in a 42-year-old woman who developed pain and paresthesia in the forearm after several days of heavy labor. The symptoms were resolved with conservative treatment, including cessation of heavy labor and a brief course of oral corticosteroids. But the symptoms recurred after 9 months. Those were also resolved with same treatment as the first attack. LACN is important to recognize because the symptoms may mimic the pathology of a cervical root, the brachial plexus, the radial and median nerves at the level of the elbow, and a focal idiopathic inflammatory neuritis.
Visual symptoms are frequently encountered in the patient with cerebrovascular disease(CVD), whereas these complaints are frequently neglected by the clinician if it occurs in a single eye. Among the visual symptoms following CVD, ischemic optic neuropathy(ION) implies visual disturbances associated with inadequate blood supply of the optic nerve. Vascular insufficiencies involving the optic nerve have three common mechanisms as the CVD, thrombotic, embolic, and hypoperfusion. Monoocular blindness is one of the most frequent complaint of ION, which commonly arises from the opthalmic artery insufficiency. Acute or transient monocular blindness is an ominous sign of following cerebral ischemia, hence early diagnosis and vigorous treatment has an immense significances. Central retinal artery occlusions(CRAO) is usually embolic in nature, and results poor consequencies. Early and accurate diagnosis of ION is important in the treatment and prevention of following CVD.
The occurrence of muscle weakness in patients with sepsis or multiple organ failure managed in the intensive care unit has been recognized with increasing frequency in the last two decades. The difficulty in examining critically ill patients may explain why this complication has been only recently recognized. This weakness is due to an axonal polyneuropathy which is called critical illness polyneuropathy(CIP). It must be differentiated from myopathy or neuromuscular junction disturbance that can also occur in the intensive care setting. Neither the cause nor the exact mechanism of CIP has been elucidated. Electrophysiological studies demonstrated an acute axonal damage of the peripheral nerves. Before the recognition of CIP, these cases were usually misdiagnosed as Guillain-Barr?syndrome. Clinical recovery from the neuropathy is rapid and nearly complete in those patients who survive. Thus, neuropathy acquired during critical illness, although causing a delayed in weaning from ventilatory support and hosptial discharge, does not worsen long-term prognosis.
Nerve compression of the suprascapular nerve by a suprascapular notch occasionally occurs, but comperssion by a ganglion is very rare. We had experienced a case of compression of the suprascapular nerve by ganglionic cyst at the suprascapular notch, which confirmed by electromyographic studies after the diagnosis was suspected. MRI scan showed multiobulated cyst at the right suprascapular notch. The patient was treated by excision of the ganglion and had excellent result.
Multifocal motor neuropathy (MMN) is a chronic immune-mediated peripheral myelinopathy. The major clinical features include slowly progressive, painless, and asymmetric weakness, usually of distal limb muscle. Early in the course of the disease, weakness is not necessarily associated with muscle trophy, owing to the initial primary involvement of peripheral myelin. Chronic progressive weakness is often associated with some degree of concurrent axonal loss and subsequent muscle atrophy. Sensory symptoms are usually mild or absent, and involvement of cranial and respiratory muscles in rare. The findings of multifocal motor conduction block, abnormal temporal dispersion, and focal conduction slowing at segments not at risk for common entrapment or compression injury, associated with normal sensory conduction studies along the same segments, are the hallmark electrophysiologic features of MMN. The slow progression and absence of upper motor neuron signs are the major clinical points that separate MMN from amyotrophic lateral sclerosis. The role of GMI antibodies, found in high titers in 22~84% of MMN patients, remains uncertain. The contention that MMN is an autoimmune disorders is largely based on the often dramatic improvement in symptoms following the administration of intravenous of immunoglobulin or cyclophosphamide.
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.
Progressive systemic sclerosis (PSS) is a multi-systemic disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. But it rarely affects the peripheral nervous system. We report a 36-year-old man of painful trigeminal neuropathy as a complication of PSS. He was referred from Rheumatology for the evaluation of abruptly developed bilateral facial pain. He had facial hyperesthesia and paresthesia on neurologic examinations. In the blink reflex, ipsilateral and contralateral R1 and R2 responses were not detected during bilateral supraorbital stimulation. But normal latency and CMAP amplitude of facial NCV were found. Under the impression of trigeminal neuropathy caused by PSS, steroid therapy was tried, and his clinical symptoms and electrophysiologic findings were improved. PSS could be the cause of the painful trigeminal neuropathy.
Small-fiber neuropathy (SFN) is a common clinical problems. The disorder is a generalized peripheral polyneuropathy that selectively involves small-diameter myelinated and unmyelinated nerve fibers. It is often idiopathic and typically presents with painful feet in patients over the age of 60. And autoimmune mechanisms are often suspected, but rarely identified. The clinical features consisted of painful dysesthesias and postganglion sympathetic dysfunction, as well as reduced pinprick and temperature sensation. Although affected patients complain of neuropathic pain, this conditionis often difficult to diagnose because of the few objective physical signs and normal nerve conduction studies. Diagnosis of SFN is made on the basis of the clinical features, normal nerve conduction studies, and abnormal specialized tests of small fiber function. These specialized studies include assessment of epidermal nerve fiber density as well as sudomotor, quantitative sensory, and cardiovagal testing. Unless an underlying disease is identified, treatment is usually directed toward alleviation of neuropathic pain.
Background s and Objectives : In the length-dependent axonal polyneuropathy like diabetic polyneuropathy (DPN), the distal part of the longer axons are affected earlier. In cases of minimal distal axonal changes, nerve conduction studies (NCS) are frequently normal. If sural nerve is affected in the early stage of DPN, supportive parameters to detect the early axonal degeneration may be helpful. We investigated whether the sural/lunar SNAP amplitude ratio (SUAR) may be a more sensitive indicator than sural amplitude alone in the diagnosis of early diabetic polyneuropathy. Methods: We analyzed medical records and electrophysiological studies of 141 patients with DM and 30 healthy subject. The patients with early stage of DPN were defined as those having symptoms of neuropathy and normal NCS findings among the patients with DM. We compared SUAR between 57 patients with early stage of DPN and 71 age-matched control subjects. Results: Fifty seven patients had an average SUAR of 0.8, compared to that of 1,1 in the 71 normal controls. The SUAR of less than 0.9 was supplementary predictor of axonal polynerupathy, with the best balance of sensitivity and specificity (70%). The SUAR did not vary significantly with age, height or duration of DM. Conclusions: We conclude that the SUAR is a useful electrodiagnostic indicator to detect early stage of DPN.
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.
B a c k g r o u n d: Somatosensory evoked potential (SSEP) is valuable for the evaluation of the central pathway.However, peripheral neuropathy sometimes renders the test useless by preventing the conduction from reaching theCNS. We postulated that the peripheral conduction problems could be overcome by proximal stimulation in SSEP andwanted to verify this in the study.Methods: Twenty patients with diabetic sensorimotor polyneuropathy were included. SSEP was elicited by stimulatingthe median and posterior tibial nerves. We compared the effect of distal and proximal stimulations in each SSEP inthe aspect of presence/absence and various latencies of resultant waves.Results: Among the 40 cases, proximal stimulation caused reappearance of subsided waves in 10 cases (25%). In themedian nerve SSEP, proximal stimulation made EN1 and CN2 visible which were not evident when distally stimulated.In the posterior tibial nerve SSEP, there was also improvement of forming waves when proximally stimulated.Conclusions: In the diabetic polyneuropathy, proximal stimulation of SSEP is more effective than the conventionaldistal stimulation in evaluating central pathway.
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.
The occurrence of polyneuropathy in association with monoclonal gammopathy of undetermined significance(MGUS) is quite common. However, reports of MGUS associated cranial neuropathies are rare. A 63 year-old womenwas presented with diplopia and swallowing difficulty. Neurological examination showed limitation of abduction ofright eye, right peripheral facial palsy, decreased hearing and gag reflex, left side deviation of uvula, and decreasedDTR. Sensorimotor polyneuropathy were observed with elctrophysiological studies. Protein and immunoelectrophoresisrevealed IgG
Acute motor axonal neuropathy (AMAN) is a subtype of Guillain-Barre syndrome and characterized by selectiveinvolvement of motor fibers. Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of central nervoussystem. The coincidence of central and peripheral nervous system involvement has been reported rarely. Wedescribed a 37-year-old male patient presented with fever and altered consciousness. The examination of cerebrospinalfluid and brain magnetic resonance imaging was compatible with acute disseminated encephalomyelitis. Several daysafter admissionb his mentality was improved but quadriparesis, multiple cranial neuropathies, and areflexia were detected.Electrophysiologic studies suggested axonal form of motor dominant polyneuropathy. We report a case of acutemotor axonal neuropathy combined with ADEM. We consider that this case is an example of simultaneous immunologicprocess to the common pathogenic epitope of central nervous system and peripheral nervous system.
Trigeminal sensory neuropathy is a clinical diagnosis in which the main feature is facial numbness limited to territory of one or more sensory branches of the trigeminal nerve. We describe a 46-year-old woman who presented with left facial numbness in the territories of maxillary nerve and mandibular nerve. MRI disclosed a lesion in left trigeminal nerve root entry zone. In Blink test stimulating infraorbital foramen, ipsilateral R1 was delayed compared with contralateral R1. Lesion in pons or medulla can present as trigeminal sensory neuropathy.
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