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Demyelinating polyneuropathy

Kalliopi Pitarokoili, Moritz Kronlage, Philip Bäumer, Daniel Schwarz, Ralf Gold, Martin Bendszus, Min-Suk Yoon
Background: We present a clinical, electrophysiological, sonographical and magnetic resonance neurography (MRN) study examining the complementary role of two neuroimaging methods of the peripheral nervous system for patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Furthermore, we explore the significance of cross-sectional area (CSA) increase through correlations with MRN markers of nerve integrity. Methods: A total of 108 nerve segments on the median, ulnar, radial, tibial and fibular nerve, as well as the lumbar and cervical plexus of 18 CIDP patients were examined with high-resonance nerve ultrasound (HRUS) and MRN additionally to the nerve conduction studies...
2018: Therapeutic Advances in Neurological Disorders
Christoph Niemietz, Christoph Röcken, Matthias Schilling, Jörg Stypman, Constantin E Uhlig, Hartmut H-J Schmidt
Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated...
March 2018: Deutsche Medizinische Wochenschrift
Amey Dilip Sonavane, Sanjiv Saigal, Abhishek Kathuria, Narendra S Choudhary, Neeraj Saraf
Extrahepatic syndromes are uncommon manifestations of acute and chronic hepatitis B. The pathogenesis likely involves an aberrant immunologic response to extrahepatic viral proteins. Antiviral therapy reduces the availability of these viral protein antigens and thus halts immune activation. Approximately 1% of all cases of acute inflammatory demyelinating polyneuropathy are associated with hepatitis B. Guillain-Barre syndrome (GBS) is a remarkably clinically diverse disorder with distinctive variants characterised by an immune-mediated attack to components of the peripheral nervous system...
March 14, 2018: Clinical Journal of Gastroenterology
Jeffrey A Allen, Kenneth C Gorson, Deborah Gelinas
Introduction: We explored adherence to the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines for the diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) by reviewing data from a specialty pharmacy database. Materials and Methods: Clinical and electrophysiologic data were reviewed for 65 consecutive patients treated with intravenous immunoglobulin (IVIG) for CIDP. Three neuromuscular neurologists independently classified cases according to EFNS/PNS criteria as (1) fulfilling CIDP criteria; (2) non-CIDP (neither clinical nor electrophysiologic criteria met); or (3) unknown (insufficient information)...
March 2018: Brain and Behavior
Yasutaka Tajima, Mariko Matsumura, Hiroaki Yaguchi, Yasunori Mito
A 27-year-old woman with optic neuritis and cervical myelitis developed hypertrophic demyelinating polyneuropathy. It was hypothesized that the diagnosis was combined central and peripheral demyelination. A hypertrophic nerve was observed subcutaneously, and magnetic resonance imaging demonstrated marked hypertrophy of the nerve roots. The patient was negative for anti-aquaporin 4 antibodies. Her anti-neurofascin 155 antibody levels was slightly elevated, but it was not definitely positive. Pulsed steroid therapy and the administration of immunoglobulin ameliorated her symptoms...
2018: Internal Medicine
Broes Martens, Michel De Pauw, Jan L De Bleecker
Familial amyloid polyneuropathy (FAP) is a most often length-dependent axonal neuropathy, often part of a multisystem disorder also affecting other organs, such as cardiac, gastrointestinal, genitourinary, renal, meningeal and eye tissue. It is most frequently the result of a mutation in the TTR gene, most commonly a p.Val50Met mutation. TTR-FAP is a rare autosomal dominant heritable disabling, heterogeneous disease in which early diagnosis is of pivotal importance when attempting treatment. This paper discusses the course of four Belgian FAP patients with different TTR mutations (p...
March 9, 2018: Acta Neurologica Belgica
Bo Hu, Megan Mccollum, Vignesh Ravi, Sezgi Arpag, Daniel Moiseev, Ryan Castoro, Bret C Mobley, Bryan W Burnette, Carly Siskind, John W Day, Robin Yawn, Shawna Feely, Yuebing Li, Qing Yan, Michael E Shy, Jun Li
OBJECTIVE: Charcot-Marie-Tooth type 4J (CMT4J) is a rare autosomal recessive neuropathy caused by mutations in FIG4 that result in loss of FIG4 protein. This study investigates the natural history and mechanisms of segmental demyelination in CMT4J. METHODS: Over the past 9 years, we have enrolled and studied a cohort of 12 CMT4J patients, including 6 novel FIG4 mutations. We evaluated these patients and related mouse models using morphological, electrophysiological and biochemical approaches...
March 8, 2018: Annals of Neurology
Stefan Wietek
AIM: To present data from three studies of a Post-Authorization Safety Surveillance (PASS) program for the subset of patients receiving octagam® 5% or 10% for chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Data on patients with CIDP treated with octagam were analyzed to assess its safety and tolerability. RESULTS: Of 2314 patients included in the studies, 58 patients (mean age: 64.6 years) received octagam for CIDP, mean dose of which was 0...
March 8, 2018: Neurodegenerative Disease Management
Masayoshi Yamamoto, Yoko Shibata, Nobuyuki Oka, Manabu Inoue, Naoko Tachibana, Toshiaki Hamano
The patient was a 58-year-old man, who was hospitalized with progressive bilateral leg weakness, and sensory impairment in all four extremities. Chronic inflammatory demyelinating polyneuropathy (CIDP) was suspected based on an electrophysiological examination, and intravenous immunoglobulin therapy (IVIg) was initiated. However, his symptoms progressed. The serum and cerebrospinal fluid were positive for human T-lymphotropic virus type 1 (HTLV-1) antibodies; hence the patient was diagnosed as having HTLV-1 associated neuropathy...
February 28, 2018: Rinshō Shinkeigaku, Clinical Neurology
Haruki Koike, Ryoji Nishi, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Naoki Atsuta, Tomohiko Nakamura, Masaaki Hirayama, Hidenori Ogata, Ryo Yamasaki, Jun-Ichi Kira, Masahisa Katsuno, Gen Sobue
We herein report a woman with chronic inflammatory demyelinating polyneuropathy (CIDP) in whom positivity for anti-neurofascin 155 antibodies was revealed 23 years after the onset of neuropathy. The patient initially reported numbness in the face at 50 years of age and subsequently manifested features compatible to typical CIDP. Steroid administration initiated at 54 years of age ameliorated her neuropathic symptoms. Although the nerve conduction indices at 59 years of age deteriorated, those at 68, 72, and 73 years of age showed a gradual recovery...
February 28, 2018: Internal Medicine
Cameron Green, Tess Baker, Ashwin Subramaniam
OBJECTIVE: To systematically review the literature regarding the ability of clinical features to predict respiratory failure in patients with Guillain-Barré syndrome (GBS). DATA SOURCES: We searched the PubMed and Ovid MEDLINE databases with the search terms "guillain barre syndrome" OR "acute inflammatory demyelinating polyneuropathy" OR "acute motor axonal neuropathy" OR "acute motor sensory axonal neuropathy" AND "respiratory failure" OR "mechanical ventilation"...
March 5, 2018: Medical Journal of Australia
Oliver Findling, Rens van der Logt, Krassen Nedeltchev, Lutz Achtnichts, John H J Allum
INTRODUCTION: We compared changes in balance control due to chronic inflammatory demyelinating polyneuropathy (CIDP) and non-inflammatory (non-inf) polyneuropathy (PNP) to each other and with respect to healthy controls (HCs). Differences in patients' subjective impressions of balance capabilities were also compared. METHODS: Balance control of 11 CIDP patients (mean age 61.1±(sd) 11, 8 male) and 10 non-inf PNP patients (mean age 68.5±11.7, all male) was examined and compared to that of 18 age- and gender-matched healthy controls...
2018: PloS One
Sumit Verma, Parul Goyal, Lokesh Guglani, Charlotte Peinhardt, Diane Pelzek, Paul E Barkhaus
OBJECTIVES: COL6A and LAMA2 are subtypes of congenital muscular dystrophy. METHODS: Retrospective chart review of clinical findings, spirometry, muscle histology, muscle ultrasound, neuroimaging, and Electromyography (EMG)/Nerve Conduction Study data in genetically confirmed COL6A and LAMA2 subjects. RESULTS: We identified 8 COL6A and 6 LAMA2 subjects: the female-to-male ratio was 1.3:1 and the mean age was 11.9 ± 3.6 years. Gross motor delays since birth, proximal muscle weakness, and contractures were noted in both groups...
March 2018: Journal of Clinical Neuromuscular Disease
Choong Yi Fong, Hnin Wint Wint Aung, Arie Khairani, Chin Seng Gan, Nortina Shahrizaila, Khean Jin Goh
Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP)...
February 16, 2018: Brain & Development
Junliang Yuan, Juan Zhang, Bingwei Zhang, Wenli Hu
OBJECTIVE: To evaluate all the coincidence cases of Guillain-Barre syndrome (GBS) and myasthenia gravis (MG). METHODS: We performed web-based research of the overlapping incidence of GBS and MG in studies occurring from 1982 to 2016 and restricted to the English language. RESULTS: Among 15 cases, an elevated CSF protein level without pleocytosis was found in 10 cases (66.7%); reduced nerve conduction was found in 13 cases (86.6%); a positive repetitive nerve stimulation test occurred in 11 cases (73...
January 2018: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
Hillary Elizabeth Lockemer, Kathryn Maria Sumpter, Sandy Cope-Yokoyama, Abhimanyu Garg
BACKGROUND: Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient. CASE PRESENTATION: A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. The patient underwent tumor debulking and cranial irradiation for the craniopharyngioma, and received monthly intravenous immunoglobulin for the CIDP...
February 19, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
Nilesh H Pawar, Ealing Loke, Derrick C Aw
Spinal cord infarction (SCI) is a rare type of stroke. The initial magnetic resonance imaging (MRI) is usually normal and can mimic the presentation of the acute transverse myelitis (ATM), acute inflammatory demyelinating polyneuropathy, and compressive myelopathies from neoplasm, epidural or subdural hematoma, or abscess. The aim of this report is to describe and discuss the case of a patient with SCI presenting as a diagnostic confusion with acute transverse myelitis. A 64-year-old male with a medical history of hypertension presented with an acute onset of urinary retention with lower limb weakness...
December 6, 2017: Curēus
Haruki Koike
Recent studies revealed an association between Zika virus infection and a variety of neurological disorders, including microcephaly, meningoencephalitis, myelitis, and Guillain-Barré syndrome (GBS). Following the first report of a patient diagnosed with GBS after Zika virus infection in December 2013, the number of GBS patients significantly increased in endemic countries, such as French-Polynesia and Latin American countries. Electrophysiological studies suggested that patients suffering from GBS associated with Zika virus infection manifest acute inflammatory demyelinating polyneuropathy (AIDP), rather than acute motor axonal neuropathy (AMAN)...
February 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
David von der Ahe, Petra Huehnchen, Mustafa Balkaya, Sarah Peruzzaro, Matthias Endres, Wolfgang Boehmerle
Suramin is a trypan blue analogon originally developed to treat protozoan infections, which was found to have diverse antitumor effects. One of the most severe side effects in clinical trials was the development of a peripheral sensory-motor polyneuropathy. In this study, we aimed to investigate suramin-induced neuropathy with a focus on calcium (Ca2+) homeostasis as a potential pathomechanism. Adult C57Bl/6 mice treated with a single injection of 250 mg/kg bodyweight suramin developed locomotor and sensory deficits, which were confirmed by electrophysiological measurements showing a predominantly sensory axonal-demyelinating polyneuropathy...
February 7, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
Xiaolei Liu, Roi Treister, Magdalena Lang, Anne Louise Oaklander
Objectives: Small-fiber polyneuropathy (SFPN) has various underlying causes, including associations with systemic autoimmune conditions. We have proposed a new cause; small-fiber-targeting autoimmune diseases akin to Guillain-Barré and chronic inflammatory demyelinating polyneuropathy (CIDP). There are no treatment studies yet for this 'apparently autoimmune SFPN' (aaSFPN), but intravenous immunoglobulin (IVIg), first-line for Guillain-Barré and CIDP, is prescribed off-label for aaSFPN despite very high cost...
2018: Therapeutic Advances in Neurological Disorders
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