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https://www.readbyqxmd.com/read/27923230/skull-subsidence-due-to-periosteum-defect-following-craniotomy-in-a-child
#1
Hidetaka Arishima, Ayumi Akazawa, Ken-Ichiro Kikuta
We report a case of a 7-year-old child with a cranial deformity secondary to a craniotomy for an intracranial hematoma. He suffered from an acute epidural hematoma with a lineal fracture of the right temporal bone following a severe head injury. A large question mark-shaped skin flap with the periosteum and temporal muscle was created for a decompressive craniectomy; however, neither the acute epidural hematoma nor brain swelling was severe, and we performed a small craniotomy compared with the skin flap without a decompressive craniectomy...
December 7, 2016: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27922872/18f-fluorodeoxyglucose-uptake-in-anti-n-methyl-d-aspartate-receptor-encephalitis-associated-with-an-immature-teratoma
#2
Ryusuke Nakamoto, Yuji Nakamoto, Takayoshi Ishimori, Yasutaka Fushimi, Kaori Togashi
We report a case of anti-N-methyl-D-aspartate-receptor (anti-NMDAR) encephalitis associated with an immature teratoma developed in a 38-year-old woman. Positron emission tomography (PET) revealed focal intense uptake of F-fluorodeoxyglucose in an area of the brain corresponding to the right medial temporal lobe as well as an intrapelvic tumor. After the PET examination, the patient complained of disorientation and short-term memory loss. The ovarian tumor was resected and diagnosed as an immature teratoma. The cerebrospinal fluid analysis was positive for anti-NMDAR antibody...
December 3, 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27922286/central-pontine-myelinolysis-during-pregnancy-pathogenesis-diagnosis-and-management
#3
María Luisa Sánchez-Ferrer, María Teresa Prieto-Sánchez, Rodrigo Orozco-Fernández, Francisco Machado-Linde, Anibal Nieto-Diaz
Central pontine myelinolysis (CPM) is a rare condition usually caused by rapid sodium correction in hyponatraemia after a severe neurological syndrome. Only few cases have been reported during pregnancy, most of which were reported in patients with hyperemesis. We describe the successful management of the first case of twin pregnancy in a patient who presented with CPM after treatment for premature labour and then review the literature on CPM in pregnancy (aetiology, diagnosis and management). Our patient required emergency delivery to achieve electrolyte and fluid balance...
December 6, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/27922246/neonatal-lupus-erythematosus-report-of-a-case-with-cutaneous-hematological-and-hepatobiliary-findings
#4
Andaç Salman, Merve Hatun Sarıçam, Ayşe Deniz Yücelten, Cuyan Demirkesen, Tülin Ergun
Neonatal lupus erythematosus is an autoimmune disorder mainly affecting the heart and skin. It is the most common cause of congenital heart block. In addition, hematological, hepatobiliary and neurological involvement may occur. Herein, we report a 23-day-old infant presented with annular, erythematous, and scaly and atrophic lesions on the face and trunk. Based on the clinical, laboratory and histopathological findings, she was diagnosed as neonatal lupus erythematosus. Neonatal lupus eryhtematosus should be considered in infants presenting with annular skin lesions, and we present this case to highlight the value of high index of clinical suspicion in diagnosis...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27922168/update-of-the-swiss-guidelines-on-post-treatment-lyme-disease-syndrome
#5
Johannes Nemeth, Enos Bernasconi, Ulrich Heininger, Mohamed Abbas, David Nadal, Carol Strahm, Stefan Erb, Stefan Zimmerli, Hansjakob Furrer, Julie Delaloye, Thierry Kuntzer, Ekkehard Altpeter, Mathias Sturzenegger, Rainer Weber, For The Swiss Society For Infectious Diseases And The Swiss Society For Neurology
Lyme borreliosis is caused by Borrelia burgdorferi sensu lato infection, which responds well to antibiotic therapy in the overwhelming majority of cases. However, despite adequate antibiotic treatment some patients report persisting symptoms which are commonly summarised as post-treatment Lyme disease syndrome (PTLDS). In 2005, the Swiss Society of Infectious Diseases published a case definition for PTLDS. We aimed to review the scientific literature with a special emphasis on the last 10 years, questioning whether the definitions from 2005 are still valid in the light of current knowledge...
2016: Swiss Medical Weekly
https://www.readbyqxmd.com/read/27920716/acute-clinical-worsening-after-steroid-administration-in-cervical-myelitis-may-reveal-a-subdural-arteriovenous-fistula
#6
Silvia Rain, Jan Udding, Daniel Broere
Subdural arteriovenous fistula (SDAVF) is a rare condition characterized by clinical manifestations ranging from mild bilateral sensory deficits to quadriplegia. The diagnosis is often delayed due to unspecific neurological symptoms, initially diagnosed as polyneuropathy or myelopathy. The diagnosis can be delayed for as long as 1-15 years. The following report describes a cervical SDAVF case initially misdiagnosed as myelitis transversa and treated with intravenous steroids. A 56-year-old male presented with sensory deficits and mild leg and right arm weakness...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27920713/late-onset-langerhans-cell-histiocytosis-with-cerebellar-ataxia-as-an-initial-symptom
#7
Jung-Min Pyun, Hyeyoung Park, Kyung Chul Moon, Beomseok Jeon
Late-onset progressive cerebellar ataxia is a diagnostic challenge because of a poor correlation between genotype and phenotype, and a broad range of secondary causes that extend beyond the neurological field. We report the case of a 45-year-old woman admitted after 2 years of slowly progressing cerebellar ataxia, dysarthria, and emotional instability. Notably, she was diagnosed with diabetes insipidus at the age of 35. As 'idiopathic cerebellar ataxia' was suspected, diagnostic tests, including genetic testing as well as serum and cerebrospinal fluid analyses, and brain magnetic resonance imaging (MRI) were performed...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27920712/hypertrophic-pachymeningitis-as-an-early-manifestation-of-relapsing-polychondritis-case-report-and-review-of-the-literature
#8
Satoru Ushiyama, Tomomi Kinoshita, Yasuhiro Shimojima, Nobuhiko Ohashi, Dai Kishida, Daigo Miyazaki, Katsuya Nakamura, Yoshiki Sekijima, Shu-Ichi Ikeda
Neurological involvement in relapsing polychondritis (RP) is relatively rare. We describe the case of an 80-year-old man who presented with hypertrophic pachymeningitis (HP) together with arthritis as the first manifestation of RP. Auricular chondritis, which subsequently determined the diagnosis of RP, occurred a few weeks after the detection of HP. The neurological symptoms, as well as arthritis, were promptly improved by treatment with corticosteroids. It is generally difficult to diagnose RP in the absence of typical cartilaginous involvement; however, the present case suggests that HP may occur as an early clinical manifestation of RP...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27920686/single-agent-carboplatin-for-a-rare-case-of-pilomyxoid-astrocytoma-of-the-spinal-cord-in-an-adult-with-neurofibromatosis-type-1
#9
Anastasie M Dunn-Pirio, Elizabeth Howell, Roger E McLendon, Katherine B Peters
INTRODUCTION: Pilomyxoid astrocytoma (PMA) is a rare and more aggressive variant of pilocytic astrocytoma, which usually affects young children and is most often located in the hypothalamic/chiasmatic region. The association of PMA with underlying genetic disorders is not well known. METHODS: We identified a 23-year-old woman with a PMA of the spinal cord who was simultaneously diagnosed with neurofibromatosis type 1. Diagnosis of neurofibromatosis type 1 was made clinically and confirmed with genetic testing that revealed a heterozygous one-amino-acid deletion (c...
September 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27920683/squamous-cell-carcinoma-in-african-children-with-xeroderma-pigmentosum-three-case-reports
#10
Mamadou Kaloga, Pauline Dioussé, Boubacar Ahy Diatta, Mariama Bammo, Sarah Kourouma, Almamy Diabate, Ndiaga Gueye, Haby Dione, Moussa Diallo, Bernard Marcel Diop
INTRODUCTION: Xeroderma pigmentosum is a rare autosomal recessive genetic disease. This disease predisposes patients to early-onset skin cancers, particularly squamous cell carcinoma. Here, we report 3 pediatric cases, including 2 deaths. OBSERVATION: The subjects included 2 boys and 1 girl with skin type VI. All subjects were from consanguineous marriages, and the average age was 7.6 years. The patients all had ulcerative budding tumor lesions in the cephalic region, and the mean disease duration was 18 months...
September 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/27920102/modified-lambrinudi-arthrodesis-for-the-acute-treatment-of-neurogenic-clubfoot-a-case-report
#11
Eric So, Lee M Hlad
: Neurogenic contracture often results in spastic, nonreducible equinovarus deformity. Rigid contracture leads to pain, instability, and bracing difficulties. This case report details the utilization of the modified Lambrinudi triple arthrodesis intended to create a plantigrade, functional limb that is amenable to an extremity brace in a case of an acquired neurologic clubfoot. LEVELS OF EVIDENCE: Therapeutic, Level IV: Case report.
December 4, 2016: Foot & Ankle Specialist
https://www.readbyqxmd.com/read/27919794/diagnostic-utility-of-contrast-enhanced-3d-t1-weighted-imaging-in-acute-cerebral-infarction-associated-with-graves-disease
#12
Yasufumi Gon, Manabu Sakaguchi, Naoki Oyama, Hideki Mochizuki
Graves disease is rarely complicated with cerebrovascular steno-occlusive diseases. Previous studies have suggested several hypotheses for this occurrence, including excess thyroid hormone, which stimulates the sympathetic nervous system, which in turn causes an abnormal hemodynamic response with consequent atherosclerotic changes, and antithyroid antibodies cause local vascular inflammation in patients with Graves disease. However, radiological findings of vasculitis in patients with Graves disease and cerebral infarction remain less known...
December 2, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/27919547/granuloma-formation-in-a-patient-with-gne-myopathy-a-case-report
#13
Keiko Nakamura, Tsuyoshi Hamaguchi, Kenji Sakai, Daisuke Noto, Kenjiro Ono, Yukiko Hayashi, Ichizo Nishino, Masahito Yamada
We report a patient with GNE myopathy with a homozygous mutation (c.1505-4G>A) in GNE gene. The patient recognized progressive weakness of extremities at age 60. Neurological examination at age 65 revealed severe weakness and atrophy in the tibialis anterior muscles and distal predominant moderate weakness in the extremities. Muscle biopsy performed at age 65 showed myopathic changes with rimmed vacuoles, and the noteworthy finding was non-caseating epithelioid cell granuloma formation surrounded by numerous inflammatory cells...
November 18, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27919464/smart-syndrome-classic-transient-symptoms-leading-to-an-unusual-unfavorable-outcome
#14
F Bompaire, L Zinchenko, M Lahutte, K Mokhtari, D Psimaras, C Gaultier, A Monjour, J-Y Delattre, D Ricard
BACKGROUND: Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare complication of cerebral radiation therapy that usually presents>10 years after treatment as reversible paroxysmal episodes of neurological dysfunction associated with headaches. CASES: We report here on two cases of SMART syndrome in long-term survivors of high-grade glioma for whom neuropathological data were available. The course of the disease was unfavorable. Although the clinico-radiological picture of SMART syndrome clearly differs from classic cerebral radionecrosis, the gross neuropathological lesions observed in our two patients appeared to be similar to those described in focal radionecrosis...
December 2, 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27919270/delayed-hyperbaric-oxygen-therapy-for-air-emboli-after-open-heart-surgery-case-report-and-review-of-a-success-story
#15
Eva Niyibizi, Guillaume Elyes Kembi, Claude Lae, Rodrigue Pignel, Tornike Sologashvili
BACKGROUND: The current case describes a rare diagnosis of iatrogenic air emboli after elective cardiopulmonary bypass that was successfully treated with delayed hyperbaric oxygen therapy, with good clinical evolution in spite of rare complications. CASE PRESENTATION: A 35 years old male was admitted to the intensive care unit (ICU) for post-operative management after being placed on cardiopulmonary bypass (CPB) for an elective ventricular septal defect closure and aortic valvuloplasty...
December 5, 2016: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/27919237/a-novel-ano3-variant-identified-in-a-53-year-old-woman-presenting-with-hyperkinetic-dysarthria-blepharospasm-hyperkinesias-and-complex-motor%C3%A2-tics
#16
Patrick R Blackburn, Michael T Zimmermann, Jennifer M Gass, Kimberly G Harris, Margot A Cousin, Nicole J Boczek, Owen A Ross, Eric W Klee, Paul W Brazis, Jay A Van Gerpen, Paldeep S Atwal
BACKGROUND: Cervical dystonias have a variable presentation and underlying etiology, but collectively represent the most common form of focal dystonia. There are a number of known genetic forms of dystonia (DYT1-27); however the heterogeneity of disease presentation does not always make it easy to categorize the disease by phenotype-genotype comparison. CASE PRESENTATION: In this report, we describe a 53-year-old female who presented initially with hand tremor following a total hip arthroplasty...
December 5, 2016: BMC Medical Genetics
https://www.readbyqxmd.com/read/27919116/stylohyoid-complex-eagle-syndrome-starting-in-a-9-year-old-boy
#17
Maite Gárriz-Luis, Pablo Irimia, Juan M Alcalde, Pablo Domínguez, Juan Narbona
Background There are only four previous pediatric reports of the glossopharyngeal neuralgic form of the stylohyoid complex syndrome. Stylohyoid complex has merely been described as cases of glossopharyngeal neuralgia in children. Case Report A 12-year-old boy came to our hospital because of recurrent episodes of severe cranial pain (9/10) lasting for 5 to 15 minutes. Pain affected the right tonsillar fossa, ear, and mastoid region. Since the start at the age of 9 years, the frequency of painful episodes has progressively increased: when admitted to our clinics 3 years later, the child was having up to five episodes daily in spite of analgesic, antiepileptic, and antidepressant drugs; he had abandoned school and leisure...
December 5, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/27918309/intraoperative-presentation-of-malignant-hyperthermia-confirmed-by-ryr1-gene-mutation-c-7522c-t-p-r2508c-leads-to-diagnosis-of-king-denborough-syndrome-in-a-child-with-hypotonia-and-dysmorphic-features
#18
Mark R Joseph, Mary C Theroux, James J Mooney, Shawn Falitz, Barbara W Brandom, Debra L Byler
We present the novel case report of a child with hypotonia and dysmorphic features who developed malignant hyperthermia (MH) intraoperatively. Neurology workup revealed the presence of a known causative ryanodine receptor (RYR1) mutation for MH, c.7522C>T; p.R2508C. Furthermore, the neurology workup diagnosed the child with King-Denborough syndrome (KDS). This particular mutation has never been documented in a patient with KDS. Atypical presentation of MH is more likely in patients with RYR1-related myopathy...
December 1, 2016: A & A Case Reports
https://www.readbyqxmd.com/read/27918268/severe-paediatric-conditions-linked-with-ev-a71-and-ev-d68-france-may-to-october-2016
#19
Denise Antona, Manoëlle Kossorotoff, Isabelle Schuffenecker, Audrey Mirand, Marianne Leruez-Ville, Clément Bassi, Mélodie Aubart, Florence Moulin, Daniel Lévy-Bruhl, Cécile Henquell, Bruno Lina, Isabelle Desguerre
We report 59 cases of severe paediatric conditions linked with enterovirus (EV)-A71 and EV-D68 in France between May and October 2016. Fifty-two children had severe neurological symptoms. EV sequence-based typing for 42 cases revealed EV-A71 in 21 (18 subgenotype C1, detected for the first time in France) and EV-D68 in eight. Clinicians should be encouraged to obtain stool and respiratory specimens from patients presenting with severe neurological disorders for EV detection and characterisation.
November 17, 2016: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
https://www.readbyqxmd.com/read/27918255/outbreak-of-enterovirus-d68-of-the-new-b3-lineage-in-stockholm-sweden-august-to-september-2016
#20
Robert Dyrdak, Malin Grabbe, Berit Hammas, Jonas Ekwall, Karin E Hansson, Joachim Luthander, Pontus Naucler, Henrik Reinius, Maria Rotzén-Östlund, Jan Albert
We report an enterovirus D68 (EV-D68) outbreak in Stockholm Sweden in 2016. Between 22 August and 25 September EV-D68 was detected in 74/495 respiratory samples analysed at the Karolinska University Hospital. During the peak week, 30/91 (33%) samples were EV-D68 positive. Viral protein (VP)P4/VP2 sequencing revealed that cases were caused by B3 lineage strains. Forty-four (59%) EV-D68-positive patients were children aged ≤ 5 years. Ten patients had severe respiratory or neurological symptoms and one died...
November 17, 2016: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
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