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Demyelinating polyneuropathy

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
Dustin Anderson, Grayson Beecher, Trevor 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 (AIDP) who fulfilled criteria for GBS that heralded undiagnosed HL. RESULTS: Cerebrospinal fluid (CSF) studies revealed albuminocytologic dissociation with significant protein elevation (250 mg/dL)...
October 18, 2016: Muscle & Nerve
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
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
Nilesh Machhindra Jagdale
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
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
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...
September 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
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
Abhijeet R Joshi, Laura Holtmann, Ilja Bobylev, Christian Schneider, Christian Ritter, Joachim Weis, Helmar C Lehmann
BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is often associated with chronic disability, which can be accounted to incomplete regeneration of injured axons. We hypothesized that Schwann cell support for regenerating axons may be altered in CIDP, which may account for the poor clinical recovery seen in many patients. METHODS: We exposed human and rodent Schwann cells to sera from CIDP patients and controls. In a model of chronic nerve denervation, we transplanted these conditioned Schwann cells intraneurally and assessed their capacity to support axonal regeneration by electrophysiology and morphometry...
September 27, 2016: Journal of Neuroinflammation
Clément Provost, Giulia Piccinini, Sophie Tasseel-Ponche, Pierre Lozeron, Bertrand Arnulf, Alain Yelnik
OBJECTIVE: As proposed after stroke, the study of reactions to a sensory stimulation allows to a better understanding of postural strategies. Below, we provide the analysis in chronic acquired demyelinating polyneuropathies (CADP). MATERIAL/PATIENTS AND METHODS: Prospective study of 25 healthy subjects (57±12years, 8M, 17F) and 25 CADP (66±14years, 20M, 5F) on stabilometric platform (Techno-Concept(®), France). Proprioception was disturbed by tendon vibration on Triceps Surae (TS) and Tibialis Anterior (TA)...
September 2016: Annals of Physical and Rehabilitation Medicine
John W Hawkins
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Brenton C Bohlig, Steven Papuchis, Catherine Schuster
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Anand M Navarasala, Vasilios Stambolis
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
John W Hawkins, Tara S Swim
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Jessalynn Adam, Kristin Gustafson
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Rakhi J Vyas, Wesley Chay
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Totka Koutzeva, John S Georgy, Francis J Lopez
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Ingrid Vasconcellos Faber, Joana Rosa Marques Prota, Alberto Rolim Muro Martinez, Anamarli Nucci, Iscia Lopes-Cendes, Marcondes Cavalcante França Júnior
INTRODUCTION: Chediak-Higashi syndrome (CHS) is a very rare autosomal recessive disorder (gene CHS1/LYST) characterized by partial albinism, recurrent infections, and easy bruising. Survivors develop a constellation of slowly progressive neurological manifestations. METHODS: We describe clinical, laboratory, electrophysiological, and genetic findings of a patient who developed an immune-mediated demyelinating neuropathy as the main clinical feature of CHS. RESULTS: The patient presented with subacute flaccid paraparesis, absent reflexes, and reduced vibration sense...
September 26, 2016: Muscle & Nerve
Semiha Kurt, Betul Cevik, Durdane Aksoy, E Irmak Sahbaz, Aslı Gundogdu Eken, A Nazli Basak
Here, we describe the clinical features of several members of the same family diagnosed with Friedreich ataxia (FRDA) and cerebral lesions, demyelinating neuropathy, and late-age onset without a significant cardiac involvement and presenting with similar symptoms, although genetic testing was negative for the GAA repeat expansion in one patient of the family. The GAA repeat expansion in the frataxin gene was shown in all of the family members except in a young female patient. MRI revealed arachnoid cysts in two patients; MRI was consistent with both cavum septum pellucidum-cavum vergae and nodular signal intensity increase in one patient...
2016: Case Reports in Neurological Medicine
Vikram A Londhey
SLE (systemic lupus erythematosus) is a multisystem autoimmune disorder of unknown aetiology which can present with myriad clinical presentation. The neurological manifestations of SLE consist of central nervous system (CNS) and peripheral nervous system manifestations (PNS). The CNS manifestations are aseptic meningitis, cerebrovascular accidents (stroke), demyelinating disorders, headache, involuntary movements like chorea, myelopathy, acute confusional states, cognitive dysfunction, mood disorder, seizures, psychosis and cranial nerve palsies...
December 2015: Journal of the Association of Physicians of India
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