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Demyelinating polyneuropathy

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https://www.readbyqxmd.com/read/27902997/chronic-inflammatory-demyelinating-polyradiculoneuropathy-cidp-clinical-features-diagnosis-and-current-treatment-strategies
#1
Jacques Reynolds, George Sachs, Kara Stavros
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits that can lead to significant neurological disability. The diagnosis is based on a combination of clinical examination findings, electrodiagnostic studies, and other supportive evidence. Recognizing CIDP and distinguishing it from other chronic polyneuropathies is important because many patients with CIDP are highly responsive to treatment with immunosuppressive or immunomodulatory therapies...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27894792/pyruvate-dehydrogenase-e1%C3%AE-deficiency-presenting-as-recurrent-acute-proximal-muscle-weakness-of-upper-and-lower-extremities-in-an-8-year-old-boy
#2
Bülent Kara, Hülya Maraş Genç, Emek Uyur-Yalçın, Ayfer Sakarya-Güneş, Uğur Topçu, Serap Mülayim, Serdar Ceylaner
The mitochondrial pyruvate dehydrogenase enzyme complex (PDHC) plays an important role in aerobic energy metabolism and acid-base equilibrium. PDHC contains of 5 enzymes, 3 catalytic (E1, E2, E3) and 2 regulatory, as well as 3 cofactors and an additional protein (E3-binding protein) encoded by nuclear genes. The clinical presentation of PDHC deficiency ranges from fatal neonatal lactic acidosis to chronic neurologic dysfunction without lactic acidosis. Paroxysmal neurologic problems such as intermittent ataxia, episodic weakness, exercise-induced dystonia and recurrent demyelination may also be seen although they are rare...
November 9, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27872470/hereditary-amyloidosis-with-recurrent-lung-infiltrates
#3
Alberto E Revelo, Crischelle Magaspi, George Maguire, Wilbert S Aronow
BACKGROUND Amyloidosis is a protein conformational disorder characterized by extracellular deposition of amyloid fibrils in extracellular tissue. Lung involvement is most commonly caused by secondary AL amyloidosis. The familial autosomal-dominant senile transthyretin (ATTR) disease manifests mainly as polyneuropathy and restrictive cardiomyopathy denoting the name familial amyloidotic polyneuropathy (FAP). Rarely, this form manifests with clinical and radiologically relevant respiratory tract symptoms and lung involvement...
November 22, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27872169/myelopathy-chameleons-and-mimics
#4
Lionel Ginsberg
The diagnosis of spinal cord disease may be delayed or missed if the presentation does not conform to the expected pattern of a symmetrical spastic paraparesis with sphincter dysfunction and a sensory level. This may occur when a myelopathy has yet to evolve fully, or is highly asymmetrical, as in Brown-Séquard syndrome. Other potential distractions include fluctuating symptoms, as may accompany spinal cord demyelination, and pseudoneuropathic features, as seen acutely in spinal shock and in the chronic setting with some high cervical cord lesions...
November 21, 2016: Practical Neurology
https://www.readbyqxmd.com/read/27861905/attenuation-of-experimental-autoimmune-neuritis-with-locally-administered-lovastatin-encapsulating-plga-nanoparticles
#5
Kelly A Langert, Bruktawit Goshu, Evan B Stubbs
Acute inflammatory demyelinating polyneuropathy (AIDP) is an aggressive antibody- and T cell-mediated variant of Guillain-Barré Syndrome (GBS), a prominent and debilitating autoimmune disorder of the peripheral nervous system. Despite advancements in clinical management, treatment of patients with AIDP/GBS and its chronic variant CIDP remains palliative and relies on the use of non-specific immune-modulating therapies. Our laboratory has previously reported that therapeutic administration of statins safely attenuates the clinical severity of experimental autoimmune neuritis (EAN), a well-characterized animal model of AIDP/GBS, by restricting the migration of autoreactive leukocytes across peripheral nerve microvascular endoneurial endothelial cells (PNMECs) that form the blood-nerve barrier...
November 8, 2016: Journal of Neurochemistry
https://www.readbyqxmd.com/read/27852440/igg4-anti-neurofascin155-antibodies-in-chronic-inflammatory-demyelinating-polyradiculoneuropathy-clinical-significance-and-diagnostic-utility-of-a-conventional-assay
#6
Masato Kadoya, Kenichi Kaida, Haruki Koike, Hiroshi Takazaki, Hidenori Ogata, Kota Moriguchi, Jun Shimizu, Eiichiro Nagata, Shunya Takizawa, Atsuro Chiba, Ryo Yamasaki, Jun-Ichi Kira, Gen Sobue, Katsunori Ikewaki
We aimed to validate the diagnostic utility of enzyme-linked immunosorbent assay (ELISA) for the detection of anti-neurofascin (NF) 155 antibody in 191 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Human NF155-based ELISA clearly distinguished between anti-NF155 antibody-positive and -negative sera. Fifteen CIDP patients (8%) were IgG4 anti-human NF155 antibody-positive, which were confirmed by western blot, cell-based assay and immunohistochemical study. None of disease controls or healthy subjects had positive results...
November 9, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27821089/posterior-reversible-encephalopathy-as-the-first-manifestation-of-bickerstaff-s-brainstem-encephalitis
#7
Pei-Ru Chen, Shih-Pin Chen
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) has been associated with Guillain-Barre syndrome in rare cases. Here we report a patient in whom PRES was the presenting manifestation of Bickerstaff's brainstem encephalitis. CASE PRESENTATION: A 75-year-old woman presented with acute onset of hypertension, headache, blurred vision, and left eyelid drooping. Magnetic resonance imaging of the brain showed characteristic PRES lesions involving the parietal and occipital lobes bilaterally...
November 8, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27819421/early-electrodiagnostic-features-of-upper-extremity-sensory-nerves-can-differentiate-axonal-guillain-barr%C3%A3-syndrome-from-acute-inflammatory-demyelinating-polyneuropathy
#8
Yong Seo Koo, Ha Young Shin, Jong Kuk Kim, Tai Seung Nam, Kyong Jin Shin, Jong Seok Bae, Bum Chun Suh, Jeeyoung Oh, Byeol A Yoon, Byung Jo Kim
BACKGROUND AND PURPOSE: Serial nerve conduction studies (NCSs) are recommended for differentiating axonal and demyelinating Guillain-Barré syndrome (GBS), but this approach is not suitable for early diagnoses. This study was designed to identify possible NCS parameters for differentiating GBS subtypes. METHODS: We retrospectively reviewed the medical records of 70 patients with GBS who underwent NCS within 10 days of symptom onset. Patients with axonal GBS and acute inflammatory demyelinating polyneuropathy (AIDP) were selected based on clinical characteristics and serial NCSs...
October 2016: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/27805350/combined-central-and-peripheral-demyelination
#9
Pranav B Joshi, Sudhir V Shah, Mayank A Patel, Shailesh Shailesh
Demyelinating disorders are very common, but remains isolated to the part of nervous system they involve. However, infrequently, combined involvement of central and peripheral nervous system with demyelinating process have been described. We report one such rare case, with possible theories of common etiological basis. We present a middle aged male patient with Chronic Inflammatory Demyelinating Polyneuropathy(CIDP), who responded to immuno-modulation. Subsequently, he developed Acute Transverse Myelitis (ATM)...
November 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27793469/challenges-in-pediatric-chronic-inflammatory-demyelinating-polyneuropathy
#10
Göknur Haliloğlu, Deniz Yüksel, Cağri Mesut Temoçin, Haluk Topaloğlu
Chronic inflammatory demyelinating neuropathy, a treatable immune-mediated disease of the peripheral nervous system is less common in childhood compared to adults. Despite different sets of diagnostic criteria, lack of a reliable biologic marker leads to challenges in diagnosis, follow-up and treatment. Our first aim was to review clinical presentation, course, response to treatment, and prognosis in our childhood patients. We also aimed to document diagnostic and therapeutic pitfalls and challenges at the bedside...
September 22, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27790622/an-occult-malignancy-behind-a-demyelinating-disease-poems-syndrome
#11
Saberio Lo Presti, Prashanth Kanagarajah, Daniela Pirela, Diana Morlote, Mike Cusnir
We report a case of a 38-year-old man presenting with bilateral lower extremity weakness and paresthesias that progressed during a 4-month period to severe polyneuropathy forcing the patient to be bed bound. Throughout his multiple hospitalizations, he was treated erroneously for chronic inflammatory demyelinating polyneuropathy, without significant improvement in his symptoms. In addition, he developed hepatosplenomegaly (organomegaly); endocrinopathies such as diabetes mellitus, central hypogonadism, and hypothyroidism; monoclonal spike evidenced in the serum electrophoresis; and hyperpigmentation of skin, altogether consistent with POEMS syndrome...
October 2016: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/27790558/intrathecal-dexmedetomidine-for-anaesthetic-management-of-a-patient-with-chronic-inflammatory-demyelinating-polyneuropathy
#12
S Bala Bhaskar, D Srinivasalu
Chronic demyelinating disorders have multifactorial origin but common important physiologic and anaesthetic considerations. Choice of anaesthesia technique and the drugs used, undertanding the pros and cons of using central neuraxial blocks will help in successful management of such patients. We describe the anaesthetic management of a 34-year-old male with chronic inflammatory demyelinating polyneuropathy posted for cystolithotripsy.
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27761272/clinical-heterogeneity-of-guillain-barr%C3%A3-syndrome-in-the-emergency-department-impact-on-clinical-outcome
#13
Athanasios Papathanasiou, Ioannis Markakis
Guillain-Barré syndrome (GBS) is mainly classified into acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Although diagnosis of GBS requires progressive weakness and universal areflexia or hyporeflexia, cases of GBS with preserved or increased deep tendon reflexes (DTRs) have been increasingly recognized. We report three cases of GBS, presenting at a single unit in six months. Our first case presented with pure sensory symptoms. The second case had nonspecific generalized weakness, while the third presented with typical ascending weakness...
2016: Case Reports in Emergency Medicine
https://www.readbyqxmd.com/read/27756115/neurological-nuance-hodgkin-lymphoma-presenting-with-guillain-barr%C3%A3-syndrome
#14
Dustin Anderson, Grayson Beecher, Trevor A Steve, Ho Jen, Richard Camicioli, Douglas W Zochodne
INTRODUCTION: Hodgkin lymphoma (HL) is a common lymphoid malignancy rarely associated with Guillain-Barré syndrome (GBS). In most cases, GBS does not precede HL. METHODS: We describe a patient with acute inflammatory demyelinating polyneuropathy who fulfilled criteria for GBS that heralded undiagnosed HL. RESULTS: Cerebrospinal fluid (CSF) studies revealed albuminocytologic dissociation with significant protein elevation (250 mg/dl). The patient worsened during intravenous immunoglobulin (IVIg) therapy...
October 18, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27750406/optimizing-electrodiagnosis-for-guillain-barr%C3%A3-syndrome-clues-from-clinical-practice
#15
Yusuf A Rajabally, Fu Liong Hiew
INTRODUCTION: The most efficient method of performing electrophysiology for Guillain-Barré syndrome (GBS) is unknown. METHODS: We retrospectively analyzed electrophysiological data of 97 consecutive GBS patients from Birmingham, U.K. (2001-12) studied ≤3 weeks post-onset. RESULTS: The sensitivity of electrophysiology for each GBS subtype was dependent on the tested upper and lower limb nerves. In acute inflammatory demyelinating polyneuropathy (AIDP), abnormalities were predominant in the arms, whereas leg abnormalities predominated in axonal GBS...
October 17, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27743887/severe-leukoencephalopathy-with-cortical-involvement-and-peripheral-neuropathy-due-to-folr1-deficiency
#16
Yu Kobayashi, Jun Tohyama, Tomoyuki Akiyama, Shinichi Magara, Hideshi Kawashima, Noriyuki Akasaka, Mitsuko Nakashima, Hirotomo Saitsu, Naomichi Matsumoto
Cerebral folate deficiency due to folate receptor 1 gene (FOLR1) mutations is an autosomal recessive disorder resulting from a brain-specific folate transport defect. It is characterized by late infantile onset, severe psychomotor regression, epilepsy, and leukodystrophy. We describe a consanguineous girl exhibiting severe developmental regression, intractable epilepsy, polyneuropathy, and profound hypomyelination with cortical involvement. Magnetic resonance imaging showed cortical disturbances in addition to profound hypomyelination and cerebellar atrophy...
October 12, 2016: Brain & Development
https://www.readbyqxmd.com/read/27727849/chronic-inflammatory-demyelinating-polyneuropathy-variant-lewis-sumner-syndrome-in-hiv
#17
Nilesh Machhindra Jagdale
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27705091/guillain-barr%C3%A3-syndrome-associated-with-zika-virus-infection-in-colombia
#18
Beatriz Parra, Jairo Lizarazo, Jorge A Jiménez-Arango, Andrés F Zea-Vera, Guillermo González-Manrique, José Vargas, Jorge A Angarita, Gonzalo Zuñiga, Reydmar Lopez-Gonzalez, Cindy L Beltran, Karen H Rizcala, Maria T Morales, Oscar Pacheco, Martha L Ospina, Anupama Kumar, David R Cornblath, Laura S Muñoz, Lyda Osorio, Paula Barreras, Carlos A Pardo
Background Zika virus (ZIKV) infection has been linked to the Guillain-Barré syndrome. From November 2015 through March 2016, clusters of cases of the Guillain-Barré syndrome were observed during the outbreak of ZIKV infection in Colombia. We characterized the clinical features of cases of Guillain-Barré syndrome in the context of this ZIKV infection outbreak and investigated their relationship with ZIKV infection. Methods A total of 68 patients with the Guillain-Barré syndrome at six Colombian hospitals were evaluated clinically, and virologic studies were completed for 42 of the patients...
October 5, 2016: New England Journal of Medicine
https://www.readbyqxmd.com/read/27680225/a-case-of-intravenous-immunoglobulin-dependent-chronic-inflammatory-demyelinating-polyneuropathy-of-pure-motor-form-responsive-to-mycophenolate-mofetil-therapy
#19
Motomi Arai
This report concerns a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with frequent treatment-dependent relapses. A 75-year-old male presented with a 2-month history of progressive weakness of the limbs with no sensory symptoms. Neurological examination revealed normal cranial nerves, MRC grade 4 power in the proximal and distal muscles of the limbs, and generalized areflexia. However, the sensory examination results, serum immunoelectrophoresis, anti-HIV antibody, and vitamins B1 and B12 levels were normal...
October 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27680223/a-case-of-chronic-inflammatory-demyelinating-polyradiculoneuropathy-after-treatment-with-pegylated-interferon-%C3%AE-2a-in-a-patient-with-chronic-hepatitis-b-virus-infection
#20
Hiroyuki Naito, Ikuko Takeda, Akiko Segawa, Masataka Tsuge, Hirofumi Maruyama, Masayasu Matsumoto
We report the case of a 42-year-old man with chronic hepatitis B virus infection who developed weakness and paresthesia in the extremities 2 months after administration of pegylated interferon (Peg-IFN)α-2a. Nerve conduction studies conducted 6 months after onset showed abnormal temporal dispersions in both tibial nerves. We diagnosed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) resulting from treatment with Peg-IFNα-2a. Neurological symptoms were prolonged despite suspension of the treatment...
September 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
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