Distal symmetric polyneuropathy (DSPN) is the most common form of diabetic neuropathy and a major cause of morbidity in patients with diabetes. Despite extensive research, diagnostic advances have been incremental rather than transformative, and accurate diagnoses in clinical practice still rely on careful clinical assessments. This review focuses on diagnostic approaches for DSPN that can be applied in routine neurological practice. We summarize the evolving definition and diagnostic criteria of DSPN, including the graded classification proposed by the Toronto consensus panel. Core diagnostic elements such as detailed history-taking, bedside neurological examinations, and validated clinical scoring systems are reviewed. We also discuss the role and limitations of nerve conduction studies, skin biopsy, corneal confocal microscopy, and tests of small-fiber function. Laboratory evaluations for differential diagnoses and current American Diabetes Association screening recommendations are also discussed.
Since 2020, updates in the diagnostic criteria for various neuromuscular diseases have emerged by advancements in electrophysiological studies, biomarker research, and imaging modalities. These developments reflect an effort to improve diagnostic accuracy and allow for earlier intervention. This review summarizes the most recent revisions in the diagnostic criteria for amyotrophic lateral sclerosis, primary lateral sclerosis, Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, and myasthenia gravis.
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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The effect of sarpogrelate compared to aspirin in high- or very-high-risk diabetes for primary prevention Soo Hyun Kang, Kilyoon Pack, Jung Ho Kim, Youngwoo Jang Scientific Reports.2025;[Epub] CrossRef
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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The prevalence, risks, and detection of driving under the influence of nitrous oxide Frederick R. J. Vinckenbosch, Dinesh Durán Jiménez, Hendrik Helmerhorst, Albert Dahan, Leon Aarts, Floris Bikker, Eef Theunissen, Johannes G. Ramaekers WIREs Forensic Science.2024;[Epub] CrossRef
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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Neurological aspects of anhidrosis: differential diagnoses and diagnostic tools Kee Hong Park, Ki-Jong Park Annals of Clinical Neurophysiology.2019; 21(1): 1. CrossRef
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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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A Practical Review of Paraproteinemic Neuropathy Joong-Yang Cho Korean Journal of Neuromuscular Disorders.2022; 14(2): 23. CrossRef
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.
Swept-Source OCT Visualization of Macular Hole Closure in Gas-Filled Eyes Daniel Q. Li, Netan Choudhry Ophthalmic Surgery, Lasers and Imaging Retina.2017; 48(5): 392. CrossRef
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.
We present a case with stepwise weakness and sensory involvement of both hands for more than 2 months. His nerve conduction study findings revealed prolonged terminal latencies, decreased motor and sensory conduction velocities and conduction blocks of both ulnar nerves, more severely on left side. And there were other abnormalities manifested with mononeuropathy multiplex. Increased cerebrospinal fluid protein was found. We diagnosed him as Lewis-Sumner syndrome and tried high dose oral steroid therapy for 2 months. He showed improvement of motor functioning with persistent conduction block.
Critical illness polyneuropathy (CIP) and critical illness myopathy (CIM) occur commonly in the patients who havebeen on mechanical ventilation for more than 1 week. Even in some patients diagnosed with CIP, an underlyingmyopathy may be the primary cause of the muscle weakness. The cormorbid status of CIP and CIM is called as criticalillness polyneuropathy and critical illness myopathy (CIPNM). We describe a 56-year-old man with acute quadriparesisand areflexia after systemic inflammatory response syndrome. The diagnosis of CIPNM is important to avoid unnecessaryinvestigations and unreasonably pessimistic prognosis. Electrophysiologic studies are essential for the diagnosis and forplanning further clinical management.
Meralgia paresthetica (MP) is a benign entrapment neuropathy which is characterized by sensory impairment and paresthesia in the cutaneous distribution of the lateral femoral cutaneous nerve. A 79-year-old woman presented with intermittent right inguinal burning sensation. The sensory nerve conduction study (NCS) showed decreased right sidesensory nerve action potential (SNAP) on lateral femoral cutaneous nerve compared to the contralateral one. Abdomino-pelvic CT showed bilateral huge renal cysts (The size of largest one on right side: about 6.2
Analysis of intraepidermal nerve fibers using skin biopsy is a recently developed technique, providing diagnosticinformation on small fiber neuropathies. The specimens are obtained by 3 mm punch biopsy, which is safe andminimally invasive. Immunohistochemical staining by Protein gene product (PGP) 9.5 demonstrate not onlyintraepidermal nerve fibers but dermal structures, such as sweat gland and erector papillae. Up to now, many studiesagree that intraepidermal nerve fiber density is dramatically reduced in various sensory neuropathies. The utility ofdensity measure was confirmed with high sensitivity in the diagnosis of sensory neuropathy, comparable to sural nervebiopsy or quantitative sensory testing. Besides quantitative methods, morphological changes like axonal swelling andfragmentation can be used as predegenerative markers. This article reviews the technique of skin biopsy and clinical andexperimental usefulness of skin biopsy in diagnosing and monitoring peripheral neuropathies.
A 33-year-old women developed weakness in all limbs 3 days prior to admission. Motor examination showed decreased strength in all limbs, but sensory examination was normal. Deep tendon reflexes were areflexia. Electrophysiological examination showed conduction blocks with nearly normal conduction velocities and terminal latencies in motor nerves and normal amplitudes and velocities in sensory nerves. Her serum was positive for IgG antibodies to gangliosides GM1, GD1b, and galactocerebroside. Acute motor conduction block neuropathy may be another variant of Guillain-Barre
We report a 31-year-old man with cerebral adrenomyeloneuronopathy variant, who presented as progressive gait disturbance. He had spastic paraparesis, hyperreflexia without Babinski
Diabetic polyneuropathy (DPN) is the most frequently encountered form of neuropathy in diabetic patients, and it either relentlessly progresses or remains relatively stable for many years, not showing any trend towards improvement. From this point of view, early detection of DPN is very important to prevent the irreversible change of the peripheral nerve from diabetic insults. Although a number of clinical symptoms and/or deficit scales have been developed for clinical or research purposes, nerve conduction study (NCS) has been known one of the most objective and sensitive tools to detect peripheral nerve dysfunctions in diabetic patients. NCS, however, also have several shortcomings. The next two consecutive articles will focus on debates about diagnostic usefulness of NCS and on recent updates of other diagnostic tests including quantitative sensory testings and skin biopsy in the field of diabetic polyneuropathy.
Diabetic polyneuropathy (DPN) is the most common form of diabetic neuropathy, and causes a significant morbidity with an impact on the quality of life in the patients with diabetes. Since DPN frequently induces foot deformity and ulceration, which finally leads to foot amputation, the early detection and treatment is very important for the prevention of a permanent structural change. In the early stage of DPN, the diagnostic methods which can evaluate the function or structure of small nerve fibers should be employed because small nerve fibers are first involved in the course of DPN. However, the nerve conduction study cannot reflect the function of the small nerve fibers, and thus, has a definite limitation in the early diagnosis of DPN. For the early detection of DPN, electrodiagnostic data should be interpreted on a clinical context, along with the careful evaluation of the small nerve fiber functions using the tests such as the analysis of intraepidermal nerve fiber density.
Acute autonomic neuropathy is a rare disease. Since the first case was reported by Young et.al., in 1969, a number ofsimilar cases have been described, with some variation of the accompanied neurologic deficits. Acute autonomic andsensory neuropathy(AASN) is characterized by the acute onset of autonomic dysfunction and sensory disturbances. A16-year-old girl experienced high fever(40
A 32-year-old man who had worked at aluminum processing plant for 4 months visited us. He complained of numbnessand paresthesia of both foot and hands and weakness of all extremities. Electrophysiologic study showed motorsensorypolyneuropathy of diffuse axonal type with focal conduction block. And we discovered higher concentration ofn-Hexane in his workplace. On sural nerve biopsy, bubbly enlarged nerve fibers in light microscope and thick myelinsheath and axonal degeneration on electron microscope were found. We diagnosed it as n-Hexane induced neuropathy.
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.
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