The severity of muscle atrophy in the distribution of a single nerve, with almost complete loss of the sternocleidomastoid and trapezius muscles, in retrospect was atypical of an acute neuralgic amyotrophy. The muscles of the affected area then become weaker and appear wasted, and the patient becomes unable to move the muscles. Jeroen jj van eijk1,3 md, jan t groothuis2 md phd, nens van alfen3 md phd 1 department of neurology, jeroen bosch hospital, shertogenbosch, the netherlands 2 department of rehabilitation, donders centre for neuroscience, radboud university medical. Van alfens takehome points na is not a rare disease and occurs in roughly one per 1,000 in general practice. Neuralgic amyotrophy parsonageturner syndrome is an idiopathic brachial plexus neuritis which commonly affects the upper trunks 1.
Neuralgic amyotrophy na is a distinct clinical entity. Apr 23, 2018 brachial neuritis bn, also known as neuralgic amyotrophy, is a rare syndrome of unknown etiology affecting mainly the lower motor neurons of the brachial plexus andor individual nerves or nerve branches. Rapid resolution of severe neuralgic amyotrophy after treatment with. The cause of reiters disease is also unknown but there is some evidence that it could be dueto a virus. Neuralgic amyotrophy na, parsonageturner syndrome, and brachial plexus neuritis are different names for the same condition. Abstract this study looks at disease diversity, location of lesions, and progression of neuralgic amyotrophy na.
Modeling of lung function recovery in neuralgic amyotrophy. The etiology is unknown, and there is a multitude of alternative clinical presentations as well as secondary triggers, which make the diagnosis challenging. The most common form of dpn is a symmetrical, predominantly sensory, polyneuropathy with distal onset and slow proximal progression. Neuralgic amyotrophy also called parsonage turner syndrome or brachial. Neuralgic amyotrophy of the lumbar area springerlink.
Fourteen patients with neuralgic amyotrophy were examined using high resolution ultrasound. While not much is known about this disorder, it has been characterized to be similar to parsonageturner syndrome in prognosis. Many patients experience difficulties in performing activities of daily life and are unable to resume work. The clinical manifestations and outcomes of neuralgic amyotrophy 1jung soo lee md phd, 2yoon tae kim md phd, 3joon sung kim md phd, 3bo young hong md phd, 3lee chan jo md, 3seong hoon lim md phd 1department of rehabilitation medicine, uijeongbu st. Pdf cranial nerve involvement in neuralgic amyotrophy.
Neuralgic amyotrophy na, also called idiopathic brachial plexus. The nerve itself is small in diameter and fragile and is vulnerable at many sites, particularly within the scalenus medius. Surgical and postpartum hereditary brachial plexus attacks and prophylactic immunotherapy. Mar 01, 2020 neuralgic amyotrophy is also known as brachial neuritis,or parsonageturner syndrome. While recognizing the danger of making a diagnosis without having examined the patient, i would like to suggest that this man was probably suffering from neuralgic amyotrophy, or the parsonageturner syndrome lancet1. Hereditary neuralgic amyotrophy is a disorder characterized by episodes of severe pain and muscle wasting amyotrophy in one or both shoulders and arms. Pdf the bpl is a part of the peripheral nervous system. Neuralgic amyotrophy parsonageturner syndrome or brachial plexus neuritis is an uncommon syndrome whose cause is unknown. Neuralgic amyotrophy definition of neuralgic amyotrophy. The prevalence, magnitude, and time course of diaphragm recovery are uncertain. Parsonage turner syndrome is a very painful and debilitating disorder of the peripheral nerves of the shoulder and arm.
Neuralgic amyotrophy of the lumbar plexus similar conditions involving lumbosacral plexus rather than brachial plexus have been described. Neuralgic pain is felt along the path of one or more nerves and often has no obvious physical cause. The suprascapular and axillary nerves and corresponding muscles are affected most frequently. At first you attribute this to some strain a day or so before, but after a few hours the pain has become so severe that you go to your family practitioner or emergency department in a slight panic. Neuralgic amyotrophy is also known as brachial neuritis,or parsonageturner syndrome. Neuralgic amyotrophy na is a syndrome characterized by the sudden onset of pain. Neuralgic amyotrophy is more common in males and on the right side. The doctor looks at your shoulder and arm but sees nothing amiss, apart from the fact that you are. The brachial plexus is a network of nerve fusions and divisions that originate from cervical and upper thoracic nerve roots and terminate as named nerves that innervate muscles and skin of the shoulder and arm. Neuralgic amyotrophy is considered a rare peripheral nervous system. Neuralgic amyotrophy definition of neuralgic amyotrophy by. Signs and symptoms usually begin around 20 years of age and may include episodes of severe pain and muscle loss in one or both shoulders and arms. Idiopathic neuralgic amyotrophy ina also called parsonageturner syndrome or brachial plexus neuritis is a distinct clinical syndrome which presents with acute severe pain in the upper extremity, shoulder or arm, lasting for several days or weeks, followed by.
Cases of hevna were compared with na cases without evidence of hev. Pdf neuralgic amyotrophy associated with temporary vocal. In most cases, pain may persist for a few hours to a few weeks and is followed by wasting and weakness of the muscles amyotrophy in the affected areas. Neuralgic amyotrophy na, also known as parsonageturner syndrome, is an idiopathic disorder characterized by rapidonset unilateral upper. The clinical manifestations and outcomes of neuralgic amyotrophy. Diagnosis of na is clinical and has a series of inclusion and compatibility criteria established by. Hereditary neuralgic amyotrophy genetics home reference nih. A typical patient suffering from the condition first experiences severe pain in one shoulder. Parsonageturner syndrome brachial neuritis patchwork amyotrophy localised neuritis of the shoulder girdle seratus magnus.
Methods this observational cohort study recruited adult patients with neuralgic amyotrophy and symptoms of. Neuralgic amyotrophy na is a syndrome of unknown etiology associated with pain in the necl and shoulder girdle followed by wasting and weakness in muscles supplied by the brachial plexus. On admission to hospital three months after the start of his symptoms he had also developed splenomegaly and the murmur of aortic regurgitation. Diagnosis and management of diabetic amyotrophy about 50% of patients with diabetes mellitus worldwide suffer from diabetic peripheral neuropathy dpn. Clinical phenotype and outcome of hepatitis e virus. This paper reports an unusual clinical manifestation associated with vocal fold paralysis. Neuralgic amyotrophy na, also known as immunemediated brachial plexopathy or brachial. A properly trained electromyographer can combine sensory and motor nerve conduc. Heredofamilial branchial plexus neuropathy hereditary neuralgic amyotrophy with branchial predilection in childhood. Neuralgic amyotrophy na is a distinct peripheral neurological. Departmentofmedicine, walsgrave hospital, coventry cv22dx summary arare neurological condition, neuralgic amyotrophy, in a diabetic is reported. The muscles of the affected area then become weaker and appear wasted, and the. Electrodiagnosis of brachial plexopathies and proximal. This illness typically starts with pain across the shoulders, sometimes with radiation into the neck or down the arm.
Diabetic amyotrophy is a nerve disorder which is a complication of diabetes mellitus. Imagine yourself waking up early one morning with a severe pain in your shoulder and upper arm. Neuralgic amyotrophy is an uncommon condition affecting the shoulder and upper arm. In this case neuralgic amyotrophy appears to have been the presenting feature of infective endocarditis. Objective to describe the clinical phenotype and recovery of diaphragm dysfunction caused by neuralgic amyotrophy in a large cohort of patients, to improve accurate awareness of this entity, and to encourage adoption of a standardized approach for diagnosis and treatment. It is also called by several other names, including proximal diabetic neuropathy, lumbosacral radiculoplexus neurophagy and femoral neurophagy. The condition manifests as a rare set of symptoms most likely resulting from autoimmune inflammation. It usually starts with acute onset of excruciating pain in the shoulder region, that, when it subsides often evolves into severe paresis that.
Core features are the extreme neuropathic pain at onset and the rapid development of multifocal pareses and atrophy, usually in the upper extremity, as well as the slow recovery in months to years. Neuralgic amyotrophy na is characterized by neuropathic pain, subsequent patchy paresis and possible sensory loss in the upper extremity. Request pdf on aug 1, 2011, nicholas e johnson and others published rapid resolution of severe neuralgic amyotrophy after treatment with corticosteroids. Neuralgic amyotrophy, or parsonageturner syndrome, is an idiopathic. The trouble with neuralgic amyotrophy practical neurology. Neuralgic amyotrophy journal of neurology, neurosurgery. Comment is made on striking similarities between the clinical presenta. Pdf hosted at the radboud repository of the radboud university nijmegen the following full text is a publishers version. Pdf hosted at the radboud repository of the radboud. Neuralgic amyotrophy also know as parsonageturner syndrome or brachial plexus neuritis is a distinct peripheral nervous system disorder characterised by episodes attacks of extreme neuropathic pain and rapid multifocal weakness and atrophy in the upper limbs. Ultrasonographic identification of nerve pathology in neuralgic amyotrophy. Jul 12, 2017 hereditary neuralgic amyotrophy is a type of nervous system disease that affects the group of nerves that control movement in the arms and shoulders called the brachial plexus.
Short report histology of hereditary neuralgic amyotrophy. Neuralgic definition of neuralgic by the free dictionary. Ultrasonographic identification of nerve pathology in. Parsonageturner syndrome, also known as acute brachial neuropathy and neuralgic amyotrophy, is a syndrome of unknown cause. Pdf mr imaging findings in brachial plexopathy with. Neuralgic amyotrophy parsonage turner syndrome or brachial plexus neuritis is an uncommon syndrome whose cause is unknown. In about half of the cases it is associated with a mutation of the sept9 gene 17q25. There are two clinically similar, yet etiologically distinct forms of neuralgic amyotrophy na. Sharp, severe paroxysmal pain extending along a nerve or group of nerves. Neuralgic amyotrophy with cervical root and cranial nerves. A 36yearold male presented with hoarseness and progressive weakness. Hereditary neuralgic amyotrophy is a type of nervous system disease that affects the group of nerves that control movement in the arms and shoulders called the brachial plexus.
Neuralgic amyotrophy na, also called parsonageturner syndrome, or brachial plexus neuritis, is an acute and painful neuropathy that involves mainly the upper brachial plexus. Hereditary neuralgic amyotrophy hna is a rare genetic disorder characterized by recurrent episodes of severe pain in the shoulder and arm. Brachial neuritis parsonageturner syndrome shoulder. A scapular winging with manual muscle testing of the right serratus anterior. To characterize the ultrasonographic findings on nerves in neuralgic amyotrophy. Cureus neuralgic amyotrophy presenting with multifocal. The exact pathophysiological mechanism in na is not known, but it is thought to be complex and multifactorial. An uncommon disorder characterized by severe shoulder pain followed by patchy muscle paralysis and sensory loss involving the shoulder girdle and upper extremity two clinical types. Additionally, the trial will combine clinical measures with measures derived to. Chronic brachial plexus neuritis that developed into typical. The disorder exhibits a broad range of clinical manifestations, and patients frequently present to physicians of different subspecialties.
Neuroimaging of classic neuralgic amyotrophy doris liebasamal, md,1 suren jengojan, md, 2gregor kasprian, md, christian wober, md, 1 and gerd bodner, md2 1department of neurology, medical university of vienna, wahringer g urtel 1820 1090 vienna, austria 2department of radiology and imageguided therapy, medical university of vienna, vienna, austria. Investigations confirmed the diagnoses of infective endocarditis and neuralgic amyotrophy. Bn usually is characterized by the acute onset of excruciating unilateral shoulder pain, followed by flaccid paralysis of shoulder and pa. Neuralgic amyotrophy is characterized by recurrent, painful, unilateral neuropathy involving mainly the upper brachial. Postpartum neuralgic amyotrophy with involvement of the. Abstractneuralgic amyotrophy consists of severe pain around the shoulder and arm followed by weakness in one or several muscles of the same area. Strong evidence for a causal relationship is suggested. Forty patients 28 male and 12 female, age range 15 to 70 years were clinically examined. Cases of na were identified in 11 centers from 7 european countries, with retrospective analysis of demographics, clinicallaboratory findings, and treatment and outcome. Neuralgic amyotrophy na is characterized by the clinical triad of pain, muscle weaknessatrophy, and sensory symptoms. Electrodiagnosis of brachial plexopathies and proximal upper. Neuralgic amyotrophy as a presenting feature of infective.
To determine the clinical phenotype and outcome in hepatitis e virusassociated neuralgic amyotrophy hevna. Hereditary neuralgic amyotrophy genetics home reference. We investigated the symptoms, course and prognosis of neuralgic amyotrophy na in a large group of patients with idiopathic neuralgic amyotrophy ina, n 199 and hereditary neuralgic amyotrophy hna, n 47 to gain more insight into the broad clinical spectrum of the disorder. Since the first description during world war ii 2,3 there have been multiple reports highlighting the manifestations of this syndrome 47. For additional information about this publication click this link. Phenotypic spectrum of hereditary neuralgic amyotrophy. The exact cause is not known, therefore it is known by numerous different names such as. It affects the thighs, hips, buttocks and legs, causing pain and muscle wasting. Its a clinical diagnosis first and foremost, and not just by exclusion. After each course his manual muscle test mmt results. Neuralgic amyotrophy can be distinguished from stretch and compression nerve injuries such as intraoperative positioning injuries because the neuralgic amyotrophy tends to develop weakness over time whereas stretch and compression injuries manifest with immediate onset of neurologic deficit. Department of neurology and clinical neurophysiology, donders centre for neuroscience, radboud university medical center, nijmegen, the netherlands.
Efficacy of a combined physical and occupational therapy. Althoughthe causeis unknown, hughes 1944 has suggested that a virus may be responsible for some cases. Neuralgic amyotrophy is a polyneuropathy that classically involves the brachial plexus. Although the clinical features and evolution are very similar, pelvis belt neuralgic amyotrophy is excluded from parsonageturner syndrome 23. Because of a tendency to heal by itself, the disease has a good prognosis.
Residual complaints after neuralgic amyotrophy archives of. We developed a combined physical and occupational therapy program for patients recovering from na. Idiopathic brachial plexus neuritis or neuralgic amyotrophy is a. When the nerves located outside of the brachial plexus are involved, the term neuralgic amyotrophy extended ane is used. Hereditary neuralgic amyotrophy genetic and rare diseases. Hereditary neuralgic amyotrophy hna, also known as hereditary brachial plexus neuropathy, is a rare autosomal dominant disorder involving recurrent episodes of painful brachial plexus neuropathies.
Neuralgic amyotrophy has both an idiopathic and hereditary form, with similar clinical symptoms but generally an earlier age. Hereditary neuralgic amyotrophy hna is a neuralgic disorder that is characterized by nerve damage and muscle atrophy, preceded by severe pain. The disorder has several phenotypic variations, with a classic form in 70% of the patients. We describe four patients with distal neuralgic amyotrophy in whom acute, severe, and transient pain around the shoulder or arm was followed by weakness of the forearm and hand muscles only. It was first reported after antitetanic serotherapy, by dyke, in 1918. Electromyographic studies typically show evidence of denervation in affected muscles. Neuralgic amyotrophy is a common although underappreciated disorder that frequently affects individual upper extremity nerves or the brachial plexus.
Incidence of neuralgic amyotrophy parsonage turner syndrome. Parsonageturner syndrome brachial neuritis patchwork amyotrophy localised neuritis of the shoulder girdle seratus magnus paralysis. This study modeled the course of recovery of lung function in 16 subjects with diaphragm impairment from neuralgic amyotrophy. Two cases of a rare peripheral neurological disease neuralgic amyotrophy of the lower extremity are presented, with clinical symptoms severe sciatic pain with motor paresis that pose differential diagnostic difficulties against a hernia of a lumbar disk. Neuralgic amyotrophy na, also known as brachial neuritis and previously known as parsonageturner syndrome, has an unknown etiology. Neuralgic amyotrophy na is a rare disturb of the peripheral nervous system that can include extreme pain, multifocal paresis and atrophy of the muscles of the upper limbs. Neuralgic amyotrophy knowledge for medical students and. Several findings from earlier smallerscale studies were tested, and for the first time the potential. Hereditary neuralgic amyotrophy nord national organization. Diagnosing neuralgic amyotrophy parsonageturner syndrome an understanding of the basic science, clinical presentation, and treatment options for neuralgic amyotrophy is necessary to facilitate timely diagnosis and treatment.
Neuralgic amyotrophy also referred to as brachial neuritis or parsonageturner syndrome is a selflimiting inflammatory disorder of the brachial plexus, that mainly affects males between 2030 years of age. The condition is known by various names, including parsonageturner syndrome, brachial plexitis, idiopathic brachial plexopathy, and brachial amyotrophy. Neuralgic amyotrophy is an uncommon condition characterised by the acute onset of severe pain in the shoulder and arm, followed by weakness and atrophy of the affected muscles, and sensory loss as. Electrodiagnosis of brachial plexopathies and proximal upper extremity neuropathies zachary simmons, md introduction. Neuralgic amyotrophy na, also known as parsonageturner syndrome, is an idiopathic disorder characterized by rapidonset unilateral upper extremity pain, paralysis, and sensory disturbance in the distribution of the brachial plexus. Although known for over a century, it is underrecognized in daily practice. Neuralgic amyotrophy is an inflammatory peripheral nerve disorder in which phrenic nerve involvement can lead to diaphragm paralysis. Transverse myelitis and neuralgic amyotrophy allan belzberga, glendaliz bosquesb and kelly phamc adepartment of neurosurgery, the johns hopkins hospital, baltimore, md, usa bchildrens memorial. The network of nerves involved in hereditary neuralgic amyotrophy, called the brachial plexus, controls movement and sensation in the. Neuralgic amyotrophy is frequently precipitated byafebrile illness andappears to be morecommon in youngmenthanin women. Hereditary neuralgic amyotrophy hna, or hereditary brachial plexus neuropathy, is a rare autosomal dominant disorder omim 162100 linked to chromosome 17q25 in a number of families. Phrenic neuropathy and diaphragm dysfunction in neuralgic. An update on diagnosis, pathophysiology, and treatment.