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brainstem encephalitis

Ray Chang, Manuel F Mas, Prathap J Joseph, Monica Verduzco-Gutierrez
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Yuri Nakamura, Kei-Ichiro Takase, Takuya Matsushita, Satoshi Yoshimura, Ryo Yamasaki, Hiroyuki Murai, Kazufumi Kikuchi, Jun-Ichi Kira
A 34-year-old man presented with an acute onset of upbeat nystagmus, slurred speech, and limb and truncal ataxias. The patient had a history of limb ataxia and gait disturbance previously treated as brainstem encephalitis with corticosteroids 3 years previously. Brain magnetic resonance imaging showed pontine developmental venous anomaly (DVA) and hemorrhagic infarction within the drainage territory of the DVA. Three months later, the patient exhibited recurrent limb ataxia, double vision, and numbness of the left side of the body...
September 19, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Yuji Koyama, Kayako Nitta, Michi Tochikura, Takashi Kasahara, Yutaka Kametsu, Minoru Toyokura, Yoshihisa Masakado
Bickerstaff's brainstem encephalitis is an autoimmune disease with the primary lesion situated in the brainstem and three cardinal signs: ophthalmoplegia; ataxia; and impaired consciousness. A 68-year-old man was started on rehabilitation exercise 3 months after onset of Bickerstaff's brainstem encephalitis, due to remnant dysarthria and dysphagia (Functional Oral Intake Scale, level 5) after the cardinal signs of Bickerstaff's brainstem encephalitis resolved. Exercise involved using a straw in the anterior midline between the dorsal tongue and hard palate...
2016: Tokai Journal of Experimental and Clinical Medicine
Gabriela Timarova, Iveta Lisa, Peter Kukumberg
Rasmussen's encephalitis is a rare autoimmune encephalitis usually involving one brain hemisphere, presenting with refractory epileptic seizures, and neurological and cognitive decline. Only 10% of cases start later in adolescence/adulthood. The only effective treatment for refractory seizures in childhood is hemispherectomy. For late-onset cases with mild neurological deficit the hemispherectomy is usually postponed because of its severe consequences. Immunotherapy shows some temporal effect for seizure control and slowing the brain atrophy, mainly in late onset Rasmussen's encephalitis...
July 16, 2016: Neuro Endocrinology Letters
Kazuki Miyaji, Jun-Ichi Furukawa, Youichi Suzuki, Naoki Yamamoto, Yasuro Shinohara, Nobuhiro Yuki
There is a case report of a patient with overlapping Guillain-Barré syndrome and Bickerstaff brainstem encephalitis after infection with herpes simplex virus type 1 (HSV-1), who carried high titers of serum anti-GQ1b IgG antibodies. Several studies have linked viral infection to the modulation of ganglioside expression such as human T-lymphotropic virus to GD2 and simian virus 40 to GM3. Also, enhancement of the expression of GM2 on the cell membrane after cytomegalovirus infection has been reported. The objective of this study was to unveil the relationship between HSV-1 infection and the alteration of cellular ganglioside expression in neuronal and glial cell lines...
August 18, 2016: Carbohydrate Research
Gian Luca Cuneo, Irene Grazzini, Martina Guadagni, Elisabetta Venturini, Amedeo Bianchi
Bickerstaff brainstem encephalitis (BBE) is a rare neurological disease that generally has a good prognosis. We describe an atypical case of a patient with severe BBE; the presentation was uncommon because of the lack of ophthalmoplegia and because of evidence of both peripheral neuropathy and brainstem encephalitis. The article reports clinical and biochemical evaluation and focuses on magnetic resonance imaging (MRI) findings in diagnosis and management of the patient. Notably, we found a previously unreported dramatic spinal cord involvement on MRI...
October 2016: Neuroradiology Journal
Takenobu Murakami, Kenji Yoshida, Mari Segawa, Akioh Yoshihara, Akihiko Hoshi, Koichiro Nakamura, Masahiro Ichikawa, Osamu Suzuki, Yuichi Yokoyama, Yasuko Toyoshima, Yoshihiro Sugiura, Hiroshi Ito, Kiyoshi Saito, Yuko Hashimoto, Akiyoshi Kakita, Hitoshi Takahashi, Yoshikazu Ugawa
BACKGROUND: Lymphomatosis cerebri (LC) is a rare subtype of primary central nervous system malignant lymphoma. The typical features of this disease exhibited on magnetic resonance imaging (MRI) without contrast enhancement are similar to those observed with diffuse leukoencephalopathy, mimicking white matter disorders such as encephalitis. Clinical features and examination findings that are suggestive of inflammatory diseases may indeed confound the diagnosis of LC. CASE PRESENTATION: A 66-year-old woman with continuous fever over a two-month period developed left hemiparesis despite presenting in an alert state with normal cognitive function...
2016: BMC Neurology
G Ortega Suero, N Sola-Valls, D Escudero, A Saiz, F Graus
OBJECTIVE: Analyse the clinical profile, associated tumour types, and response to treatment of paraneoplastic neurological syndromes associated with antibodies against Ma proteins. METHODS: A retrospective study of patients with antibodies against Ma proteins identified in a neuroimmunology laboratory of reference. RESULTS: Of the 32 patients identified, 20 showed reactivity against Ma2 only (anti-Ma2 antibodies), 11 against Ma1 and Ma2 (anti-Ma antibodies), and 1 with reactivity against Ma1 only (anti-Ma1 antibodies)...
July 23, 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
Linda J Locht, Morten Blaabjerg
Bickerstaff brainstem encephalitis (BE) is a very rare neurological condition with subacute onset of ophthalmoplegia, ataxia and altered sensorium, often postinfectious. The condition is associated with the anti-GQ1b antibody syndrome and is part of the spectrum of diseases including Miller Fisher syndrome and Guillain-Barré syndrome. In this case, we report the history, workup, treatment and follow-up of a 48-year-old woman with probable BE without anti-GQ1b antibodies in relation to the international diagnostic criteria...
July 4, 2016: Ugeskrift for Laeger
Scott A McLeod, Wallace Wee, Francois D Jacob, Isabelle Chapados, Francois V Bolduc
Introduction. Acute complete external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem. Ophthalmoplegia has been reported with acute ataxia in Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE). Up to 95% of these cases are associated with anti-GQ1b antibodies. Only a small number of cases of anti-GQ1b negative MFS have been documented in pediatric patients. This is the first case reporting a recurrence of ocular symptoms in an anti-GQ1b antibody negative patient with BBE...
2016: Case Reports in Neurological Medicine
Hiroshi Ureshino, Takero Shindo, Hiroyoshi Nishikawa, Nobukazu Watanabe, Eri Watanabe, Natsuko Satoh, Kazutaka Kitaura, Hiroaki Kitamura, Kazuko Doi, Kotaro Nagase, Hiromi Kimura, Makoto Samukawa, Susumu Kusunoki, Masaharu Miyahara, Tadasu Shin-I, Ryuji Suzuki, Shimon Sakaguchi, Shinya Kimura
The regulatory T cells (Treg) with the most potent immunosuppressive activity are the effector Tregs (eTreg) with a CD45RA(-)Foxp3(++)CCR4(+) phenotype. Adult T-cell leukemia (ATL) cells often share the Treg phenotype and also express CCR4. Although mogamulizumab, a monoclonal antibody to CCR4, shows marked antitumor effects against ATL and peripheral T-cell lymphoma, concerns have been raised that it may induce severe autoimmune immunopathology by depleting eTregs. Here, we present case reports for two patients with ATL who responded to mogamulizumab but developed a severe skin rash and autoimmune brainstem encephalitis...
August 2016: Cancer Immunology Research
Kamini Gupta, Avik Banerjee, Kavita Saggar, Archana Ahluwalia, Karan Saggar
BACKGROUND: Infections of the central nervous system (CNS) are common and routinely encountered. Our aim was to evaluate the neuroimaging features of the various infections of the CNS so as to differentiate them from tumoral, vascular, and other entities that warrant a different line of therapy. AIMS: Our aim was to analyze the biochemical and magnetic resonance imaging (MRI) features in CNS infections. SETTINGS AND DESIGN: This was a longitudinal, prospective study over a period of 1½ years...
January 2016: Journal of Pediatric Neurosciences
Valerio Brunetti, Elisa Testani, Raffaele Iorio, Giovanni Frisullo, Marco Luigetti, Daniela Di Giuda, Giacomo Della Marca
We describe a 70-year-old man who, after a viral encephalitis associated with pneumonia, progressively developed a parkinsonism associated with lethargy. Encephalitis manifested with persistent hiccups, seizures and impairment of consciousness. After 2 weeks, the initial neurologic symptoms subsided and the patient progressively developed movement disorders (rigidity and bradykinesia, resistant to L-DOPA), lethargy and behavioral hypersomnia. Magnetic resonance imaging showed thalamic and hippocampal signal abnormalities, immunohistochemistry on a mouse brain substrate revealed serum autoantibodies binding to the brainstem neuropil...
April 25, 2016: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
Junko Ishii, Nobuhiro Yuki, Michi Kawamoto, Hajime Yoshimura, Susumu Kusunoki, Nobuo Kohara
OBJECTIVE: Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), and Bickerstaff brainstem encephalitis (BBE) are usually monophasic, but some patients experience recurrences after long asymptomatic intervals. We aimed to investigate clinical features of recurrent GBS, MFS, and BBE at a single hospital. METHODS: Records from 97 consecutive patients with GBS, MFS or BBE who were admitted to a tertiary hospital between 2001 and 2013 were reviewed. Clinical and laboratory features of patients with recurrent GBS, MFS, or BBE were investigated...
May 15, 2016: Journal of the Neurological Sciences
Kyung Yeon Lee, Myoung Sook Lee, Dong Bin Kim
Enterovirus 71 frequently involves the central nervous system and may present with a variety of neurologic manifestations. Here, we aimed to describe the clinical features, magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) profiles of patients presenting with neurologic complications of enterovirus 71 infection. We retrospectively reviewed the records of 31 pediatric patients hospitalized with acute neurologic manifestations accompanied by confirmed enterovirus 71 infection at Ulsan University Hospital between 2010 and 2014...
April 2016: Journal of Korean Medical Science
Federico A Filippín, Alejandra Alfonso, Héctor López-Presas
INTRODUCTION: The celiac disease is an autoimmune bowel disease trigger by gliadine proteins, that can has systemic involvement with varied neurological manifestation since polineuropathy, ataxia, epilepsy, migraine to encephalitis. The aim of the current work is to report a case of possible refractory encephalitis due to celiac disease. CASE REPORT: A 45 years-old man with histopathologic diagnosis of celiac disease but negative celiac antibodies developed encephalic, brainstem and periphery nervous system manifestations: cognitive declaim and seizures, diplopia and ataxia, polyneuropathy and polyradiculopathy respectively; inflammatory cebrospinal fluid and cortico-subcortical and multiple brainstem lesions with mild contrast enhancement; the neurologic deficits progress in spite of gluten a free diet and immuno-suppressive treatment...
April 1, 2016: Revista de Neurologia
Y Sekiguchi, M Mori, S Misawa, S Sawai, N Yuki, M Beppu, S Kuwabara
BACKGROUND AND PURPOSE: Fisher syndrome (FS) may overlap with Guillain-Barré syndrome (GBS), in particular the pharyngeal-cervical-brachial variant form (PCB-GBS), or Bickerstaff brainstem encephalitis (BBE). Our aim was to elucidate the frequency of this overlap and the patterns of clinical progression in patients with FS. METHODS: Sixty consecutive patients with FS were studied. FS/PCB-GBS was diagnosed when the patients developed pharyngeal, cervical and/or brachial weakness...
June 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Yuri Mizuno, Hiroshi Shigeto, Takeshi Yamada, Norihisa Maeda, Satoshi O Suzuki, Jun-ichi Kira
We report a case of primary central nervous system vasculitis (PCNSV) diagnosed by second brain biopsy. A 53-year-old man initially presented with left lateral gaze diplopia. Brain MRI revealed multiple enhanced lesions in the bilateral frontal lobe, bilateral basal ganglia, left cerebellum and brainstem. An initial brain biopsy of the right frontal lobe suggested immune-related encephalitis with angiocentric accumulation of chronic inflammatory cells, while malignant lymphoma could not be completely ruled out...
2016: Rinshō Shinkeigaku, Clinical Neurology
Melanie Ramberger, Markus Reindl
No abstract text is available yet for this article.
August 2016: Developmental Medicine and Child Neurology
Dushyanth Babu Jasti, S V Naveen Prasad, Thota Naveen, Bhuma Vengamma
Central nervous system involvement in Kikuchi-Fujimoto disease is a very rare clinical manifestation. We report a 15-year-old girl who presented to us with fever, drowsiness, neck swellings, and involuntary closure of both eyelids of 2 days duration. Magnetic resonance imaging brain showed T2-weighted and fluid-attenuated inversion recovery hyperintensities in dorsal midbrain and pons. Cervical lymph node fine-needle aspiration cytology was suggestive of Kikuchi-Fujimoto disease. Blepharospasm secondary to infectious etiology is rare...
January 2016: Journal of Neurosciences in Rural Practice
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