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Neurologic amyotrophy

Tom McEnery, Ronan Walsh, Conor Burke, Aisling McGowan, John Faul, Liam Cormican
Neuralgic Amyotrophy (NA) or Parsonage-Turner syndrome is an idiopathic neuropathy commonly affecting the brachial plexus. Associated phrenic nerve involvement, though recognised, is thought to be very rare. We present a case series of four patients (all male, mean age 53) presenting with dyspnoea preceded by severe self-limiting upper limb and shoulder pain, with an elevated hemi-diaphragm on clinical examination and chest X-ray. Neurological examination of the upper limb at the time of presentation was normal...
January 30, 2017: Lung
Atsushi Mizuma, Chikage Kijima, Eiichiro Nagata, Shunya Takizawa
Metastasis of breast cancer is often detected through a long-term course and difficult to diagnose. We report a case of brachial plexopathy suspected to be the initial lesion of breast cancer metastasis, which was only detected by magnetic resonance (MR) neurography. A 61-year-old woman was admitted to our hospital within 2 years after operation for breast cancer because of progressive dysesthesia and motor weakness initially in the upper limb on the affected side and subsequently on the contralateral side...
May 2016: Case Reports in Oncology
Valerio Vitale, Ferdinando Caranci, Chiara Pisciotta, Fiore Manganelli, Francesco Briganti, Lucio Santoro, Arturo Brunetti
BACKGROUND: Hirayama's disease (HD), is a benign, self-limited, motor neuron disease, characterized by asymmetric weakness and atrophy of one or both distal upper extremities. In the present study we report the clinical, electrophysiological and MRI features of a group of Italian patients, with review of the literature. Moreover we propose an optimized MRI protocol for patients with suspected or diagnosed HD in order to make an early diagnosis and a standardized follow up. METHODS: Eight patients with clinical suspicion of Hirayama disease underwent evaluation between January 2007 and November 2013...
August 2016: Quantitative Imaging in Medicine and Surgery
Sven Pischke, Ulrike Ryll, Andres De Weerth, Friederike Ufer, Mathias Gelderblom
History and initial findings | A patient with bilateral pain in his shoulders is presented at the emergency room. Investigations | There were strongly increased transaminases. Diagnosis | Serology and PCR led to the diagnosis of hepatitis E. Neurological examination revealed a neuralgic amyotrophy Treatment and course | The pain in the shoulders disappeared spontaneously after vanishing of HEV viremia. Conclusion | Patients with shoulder pain of unknown origin and elevated transaminases should be tested for hepatitis E...
August 2016: Deutsche Medizinische Wochenschrift
J van der Wardt, T B G Olde Dubbelink, H F Visée, P M Schneeberger, S P M Lutgens, J J J van Eijk
BACKGROUND: Infection with hepatitis E virus genotype 3 (HEV3) is an emerging zoonosis in the industrialized world. The infection usually proceeds asymptomatically. Extrahepatic sequelae including neurological symptoms have been described. CASE DESCRIPTION: A 52-year-old man presented at the emergency department with pain, muscle weakness and sensory disorders in both shoulders and arms. He was found to have bilateral neuralgic amyotrophy accompanying an HEV3 infection...
2016: Nederlands Tijdschrift Voor Geneeskunde
Ciaran John Clarke, Emma Torrance, Jerome McIntosh, Lennard Funk
BACKGROUND: Neuralgic amyotrophy (NA) was first described in 1948. Traditional literature describes a painful attack with sudden onset, followed by paresis, with varied outcomes. Recent studies have suggested NA is currently underdiagnosed. However, a large number of studies detailing NA originate from a small group of sources. Our study compared the onset, diagnosis, investigation, and treatment of all neurologic shoulder conditions to provide comparable data for these studies. METHODS: Data were collected from 60 patients (81...
December 2016: Journal of Shoulder and Elbow Surgery
Harry R Dalton, Glynn W Webb, Ben C Norton, Kathy L Woolson
Until recently, hepatitis E virus (HEV) was thought not to occur in developed countries. It is now clear that locally acquired HEV is common in such settings. HEV infection acquired in these areas differs from that in developing countries in a number of important aspects: it is caused by genotype 3 (and 4 in China and Japan), it mainly affects middle-aged/elderly males and it is zoonotic with a porcine primary host. Pig herds worldwide are infected with HEV genotype 3 and HEV has been found in the human food chain in a number of developed countries...
2016: Digestive Diseases
Nassim Kamar, Olivier Marion, Florence Abravanel, Jacques Izopet, Harry R Dalton
Hepatitis E virus can cause acute, fulminant and chronic hepatitis and has been associated with a range of extrahepatic manifestations. Guillain-Barré syndrome, neuralgic amyotrophy and encephalitis are the main neurological manifestations associated with acute and chronic hepatitis E virus infection. Renal injuries have been also reported, including membranoproliferative glomerulonephritis with or without cryoglobulinemia and membranous glomerulonephritis. Acute pancreatitis, haematological disorders and other autoimmune extrahepatic manifestations of hepatitis E virus, such as myocarditis and thyroiditis, have been also reported...
April 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Eman Al-Ghawi, Talal Al-Harbi, Adnan Al-Sarawi, Mohamed Binfalah
BACKGROUND: Monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. Proximal involvement of the arm and shoulder muscles is an unusual presentation that has been rarely reported in the literature. CASE PRESENTATION: A 28-year-old white man presented with insidious-onset, slowly progressive, unilateral weakness and atrophy of his left shoulder girdle and deltoid muscles. A neurological examination revealed weakness and atrophy in his left deltoid, infraspinatus and supraspinatus muscles...
March 17, 2016: Journal of Medical Case Reports
Mauro Silva, Benoît Wicki, Pinelopi Tsouni, Sophie Cunningham, Christopher Doerig, Giorgio Zanetti, Vincent Aubert, Roland Sahli, Darius Moradpour, Thierry Kuntzer
INTRODUCTION: We describe a patient who developed neuralgic amyotrophy (NA) related to hepatitis E virus (HEV) infection. METHODS: The patient underwent neurological and electrodiagnostic examinations, high-resolution analysis of serological changes, and HEV load profile, and was treated with intravenous immunoglobulin. RESULTS: There was evidence of bilateral, asymmetric acute inflammatory cervical polyradiculopathy and possible brachial plexopathy...
August 2016: Muscle & Nerve
S A Drave, Y Debing, S Walter, D Todt, M Engelmann, M Friesland, H Wedemeyer, J Neyts, P Behrendt, E Steinmann
Hepatitis E virus (HEV) is the causative agent of hepatitis E in humans and a member of the genus Orthohepevirus in the family Hepeviridae. Infection usually leads to acute hepatitis that can become fulminant, particularly among pregnant women and in patients with preexisting liver disease, or may evolve to a chronic state, especially in immunosuppressed individuals. HEV has been shown to produce a range of extra-hepatic manifestations including aplastic anaemia, acute thyroiditis, glomerulonephritis as well as neurological disorders such as Guillain-Barré syndrome, neuralgic amyotrophy and encephalitis...
July 2016: Journal of Viral Hepatitis
Harry R Dalton, Nassim Kamar, Jeroen J J van Eijk, Brendan N Mclean, Pascal Cintas, Richard P Bendall, Bart C Jacobs
Hepatitis E is hyperendemic in many developing countries in Asia and Africa, and is caused by hepatitis E virus (HEV) genotypes 1 and 2, which are spread via the faecal-oral route by contaminated water. Recent data show that HEV infection is also endemic in developed countries. In such geographical settings, hepatitis E is caused by HEV genotypes 3 and 4, and is mainly a porcine zoonosis. In a minority of cases, HEV causes acute and chronic hepatitis, but infection is commonly asymptomatic or unrecognized. HEV infection is associated with a number of extrahepatic manifestations, including a range of neurological injuries...
February 2016: Nature Reviews. Neurology
Arjit Agarwal, Shruti Chandak, Pawan Joon
Hirayama disease is a rare type of neurological disease commonly manifesting as brachial monomelic amyotrophy in young males of Asian origin, easily understood as juvenile non-progressive cervical amyotrophy. The first case was reported by Hirayama in 1959. The pathogenesis is attributed towards chronic compression of cervical spinal cord during flexion movements of neck in cases where there is detachment of posterior dura mater. This chronic event, invariably leads to features of cord atrophy along with other MRI features...
August 2015: Journal of Clinical and Diagnostic Research: JCDR
Cecilia Quarracino, Florencia Aguirre, Carlos A Rugilo, Luciana De Negri, Andrés M Villa
Hirayama disease is a rare neurological disorder characterized by an insidious progressive subacute unilateral or bilateral weakness of the hands and forearm muscles leading to a painless amyotrophy. The disease primarily affects young men in the second to third decades of life. It has always been described as a second motor neuron disease, thus sparing the pyramidal and sensitive pathways. It usually has a slow progression course of 3 to 5 years followed by stabilization. Since its initial description by Keyzo Hirayama in 1959, most cases have been reported in Asia, particularly Japan and India, although the disease reportedly has worldwide distribution...
October 2015: Asian Spine Journal
Fateh Bazerbachi, Samir Haffar, Sushil K Garg, John R Lake
BACKGROUND AND AIMS: Hepatitis E virus (HEV) infection is a significant public health problem that afflicts almost 20 million individuals annually and causes acute liver injury in 3.5 million, with approximately 56 000 deaths. As with other viral hepatitides, extra-hepatic manifestations could represent an important aspect of this infection. The spectrum of these manifestations is still emerging. Acute pancreatitis and neurological, musculoskeletal, hematological, renal, and other immune-mediated manifestations have been described...
February 2016: Gastroenterology Report
Ravindra Kumar Garg, Hardeep Singh Malhotra, Amita Jain, Kiran Preet Malhotra
Dengue is associated with many neurological dysfunctions. Up to 4% of dengue patients may develop neuromuscular complications. Muscle involvement can manifest with myalgias, myositis, rhabdomyolysis and hypokalemic paralysis. Diffuse myalgia is the most characteristic neurological symptom of dengue fever. Dengue-associated myositis can be of varying severity ranging from self-limiting muscle involvement to severe dengue myositis. Dengue-associated hypokalemic paralysis often has a rapidly evolving course; benign nature; excellent response to potassium; and, often leads to diagnostic confusion with other dengue-associated neuromuscular disorders...
July 2015: Neurology India
Anaïs Dartevel, Barbara Colombe, Annick Bosseray, Sylvie Larrat, Françoise Sarrot-Reynauld, Aude Belbezier, Emmeline Lagrange, Laurence Bouillet
Hepatitis E virus infection - mainly genotype 3 - is increasingly common in industrialized countries. Infection is usually asymptomatic, but cases of central or peripheral neurological symptoms with hepatitis E have been described. The most frequent is Guillain-Barre but somes cases of neuralgic amyotrophy have been described. In our center, since 2010, we have identified five cases of neuralgic amyotrophy associated with acute hepatitis E in immunocompetent patients. For all these patients, neuralgic amyotrophy was diagnosed with electromyogram and positive IgM for hepatitis E, and detectable HEV RNA in 4 of the cases...
August 2015: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
Nens van Alfen, Jeroen J J van Eijk, Tessa Ennik, Sean O Flynn, Inge E G Nobacht, Jan T Groothuis, Sigrid Pillen, Floris A van de Laar
OBJECTIVE: Neuralgic amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic amyotrophy in a primary care setting. METHODS: In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118...
2015: PloS One
Naotoshi Iwahara, Shin Hisahara, Takashi Hayashi, Jun Kawamata, Shun Shimohama
BACKGROUND: Mutations of the lamin A/C gene have been associated with several diseases such as Emery-Dreifuss muscular dystrophy, dilated cardiomyopathy and Charcot-Marie-Tooth disease, referred to as laminopathies. Only one report of spinal muscular atrophy and cardiomyopathy phenotype with lamin A/C gene mutations has been published. The concept that lamin A/C gene mutations cause spinal muscular atrophy has not been established. CASE PRESENTATION: We report a man aged 65 years who presented with amyotrophy of lower limbs, arrhythmia and cardiac hypofunction...
2015: BMC Neurology
Naotoshi Iwahara, Shin Hisahara, Takashi Hayashi, Jun Kawamata, Shun Shimohama
BACKGROUND: Mutations of the lamin A/C gene have been associated with several diseases such as Emery-Dreifuss muscular dystrophy, dilated cardiomyopathy and Charcot-Marie-Tooth disease, referred to as laminopathies. Only one report of spinal muscular atrophy and cardiomyopathy phenotype with lamin A/C gene mutations has been published. The concept that lamin A/C gene mutations cause spinal muscular atrophy has not been established. CASE PRESENTATION: We report a man aged 65 years who presented with amyotrophy of lower limbs, arrhythmia and cardiac hypofunction...
December 2015: BMC Neurology
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