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Neurologic amyotrophy

Brenda C M De Winter, Dennis A Hesselink, Nassim Kamar
Hepatitis E virus (HEV) is the most common cause of viral hepatitis worldwide. Genotypes 1 and 2 (GT1 and GT2) are mainly present in developing countries, while GT3 and GT4 are prevalent in developed and high-income countries. In the majority of cases, HEV causes a self-limiting hepatitis. GT3 and GT4 can be responsible for a chronic hepatitis that can lead to cirrhosis in immunocompromized patients, i.e. solid-organ- and stem-cell-transplant-patients, human immunodeficiency virus-infected patients, and patients receiving chemotherapy or immunotherapy...
February 27, 2018: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
Thais Lampert Monte, Estela da Rosa Reckziegel, Marina Coutinho Augustin, Lucas D Locks-Coelho, Amanda Senna P Santos, Gabriel Vasata Furtado, Eduardo Preusser de Mattos, José Luiz Pedroso, Orlando Póvoas Barsottini, Fernando Regla Vargas, Maria-Luiza Saraiva-Pereira, Suzi Alves Camey, Vanessa Bielefeldt Leotti, Laura Bannach Jardim
BACKGROUND: Spinocerebellar ataxia type 2 (SCA2) affects several neurological structures, giving rise to multiple symptoms. However, only the natural history of ataxia is well known, as measured during the study duration. We aimed to describe the progression rate of ataxia, by the Scale for the Assessment and Rating of Ataxia (SARA), as well as the progression rate of the overall neurological picture, by the Neurological Examination Score for Spinocerebellar Ataxias (NESSCA), and not only during the study duration but also in a disease duration model...
January 25, 2018: Orphanet Journal of Rare Diseases
M Jiang, X Yan, L R Yan, Y B Zhan, H T Hu
Objective: To investigate the value of split hand in the differential diagnosis of amyotrophic lateral sclerosis(ALS) and cervical spondylotic amyotrophy (CSA). Methods: A total of 62 ALS patients, 57 CSA patients and 65 normal controls who visited the Neurology and Spine Department of Beijing Jishuitan Hospital from May 2013 to June 2017 were enrolled into this study.The amplitudes of compound muscle action potentials (CMAP) were recorded from abductor digiti minimi (ADM) and abductor pollicis brevis (APB)...
December 19, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Shangming Zhang, Lucy Q Zhang, Megan Wright, David R Gater
Neuralgic amyotrophy (NA) is a neurological syndrome of unknown etiology primarily affecting the brachial plexus. We are reporting an unusual case of acute bilateral NA that was possibly secondary to Lyme disease. The patient demonstrated significant functional gains and was discharged home after 2 weeks of inpatient rehabilitation, supporting the role of inpatient rehabilitation in acute NA. In this report, we discussed the diagnosis, electrodiagnostic progression, pain management, goals for inpatient rehabilitation and overall prognosis of NA...
December 20, 2017: PM & R: the Journal of Injury, Function, and Rehabilitation
Juan Du, Ya-Cen Hu, Bei-Sha Tang, Hong Jiang, Lu Shen
AIM OF THE STUDY: To investigate the mutation frequency of SPG11, SPG15, SPG5 and SPG7 in China. MATERIALS AND METHODS: We have scanned the whole exons of KIAA1840, ZFYVE26, SPG7 and CYP7B1 genes in a group of 36 unrelated Chinese ARHSP families. RESULTS: SPG11 mutations were found in 33.33% (12/36) of ARHSP patients in our study, and no mutation was identified in SPG15, SPG5 or SPG7 genes. Among the SPG11 mutations detected, c.1755_1758delAGCA/p...
February 2018: International Journal of Neuroscience
Alexander Katzer, Wolf-Peter Niedermauntel, Jörg Rump
Neuralgic amyotrophy of the shoulder (Parsonage-Turner syndrome) is a rare condition of unknown aetiology which manifests as acute neuropathy of the brachial plexus. Diagnosis is based on typical symptoms and physical examination. In addition, magnetic resonance imaging of the affected shoulder and the cervical spine is advisable, in order to distinguish this syndrome from other conditions with similar symptoms. There is no specific treatment for neuralgic amyotrophy, but in about 50 - 67% of cases complete recovery occurs within two to three years, depending on the severity of the symptoms when they first appear...
September 19, 2017: Zeitschrift Für Orthopädie und Unfallchirurgie
Harry R Dalton, Jeroen J J van Eijk, Pascal Cintas, Richie G Madden, Catherine Jones, Glynn W Webb, Benjamin Norton, Julie Pique, Suzanne Lutgens, Nikki Devooght-Johnson, Kathy Woolson, John Baker, Maria Saunders, Liz Househam, James Griffiths, Florence Abravanel, Jacques Izopet, Nassim Kamar, Nens van Alfen, Baziel G M van Engelen, Jeremy G Hunter, Annemiek A van der Eijk, Richard P Bendall, Brendan N Mclean, Bart C Jacobs
BACKGROUND & AIMS: Hepatitis E virus (HEV) has been associated with a number of neurological syndromes, but causality has not yet been established. The aim of this study was to explore the relationship between HEV and neurological illness by prospective HEV testing of patients presenting with acute non-traumatic neurological injury. METHODS: Four hundred and sixty-four consecutive patients presenting to hospital with acute non-traumatic neurological illnesses were tested for HEV by serology and PCR from four centres in the UK, France and the Netherlands...
November 2017: Journal of Hepatology
Thais Lampert Monte, Fernanda Santos Pereira, Estela da Rosa Reckziegel, Marina Coutinho Augustin, Lucas Dorídio Locks-Coelho, Amanda Senna P Santos, José Luiz Pedroso, Orlando Barsottini, Fernando Regla Vargas, Maria-Luiza Saraiva-Pereira, Laura Bannach Jardim
BACKGROUND: Spinocerebellar ataxia type 2 (SCA2) is due to a CAG expansion (CAGexp) at ATXN2. SCA2 presents great clinical variability, alongside characteristic ataxia with saccadic slowness. AIMS: To study parkinsonism, dementia, dystonia, and amyotrophy as subphenotypes of SCA2, and to explore the effect of CAG repeats at different loci and of mitochondrial polymorphism A10398G as modifiers of phenotype. METHODS: Symptomatic subjects were classified by presence/absence of neurological signs mentioned above; SARA and NESSCA scores were obtained...
September 2017: Parkinsonism & related Disorders
Brendan N Mclean, James Gulliver, Harry R Dalton
Hepatitis E is the most common cause of hepatitis worldwide. While originally considered a disease of developing countries, it is increasingly recognised in developed countries, probably related to contaminated pork meat, and where infection is often asymptomatic. However, several non-liver manifestations have become apparent, the most important of which are neurological, including Guillain-Barré syndrome (acute inflammatory demyelinating polyradiculoneuropathy (AIDP)), neuralgic amyotrophy and meningoencephalitis...
August 2017: Practical Neurology
Halil Ay
Hirayama disease (HD) is a rare motor neuron disorder that involves a single upper extremity. It is clinically characterized by weakness and atrophy of the muscles of the hand and forearm. This article presents a 19-year-old woman who visited the orthopedics outpatient clinic with weakness and atrophy in her right hand and was clinically diagnosed with advanced stage carpal tunnel syndrome and scheduled for surgical intervention; she was later diagnosed with HD by an electrophysiological study. As a result, it has been found that a careful electrophysiological study and neurological examination can be used to diagnose HD...
2017: Neuropsychiatric Disease and Treatment
Michael P Collins, Robert D Hadden
Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy...
April 27, 2017: Nature Reviews. Neurology
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...
April 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 de 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
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