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Case Reports in Neurology

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https://www.readbyqxmd.com/read/27920716/acute-clinical-worsening-after-steroid-administration-in-cervical-myelitis-may-reveal-a-subdural-arteriovenous-fistula
#1
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/27920715/intra-arterial-mechanical-thrombectomy-an-effective-treatment-for-ischemic-stroke-caused-by-endocarditis
#2
Olafur Sveinsson, Lars Herrman, Staffan Holmin
Patients with stroke secondary to infectious endocarditis have a high in-hospital morbidity and mortality, with only one-third becoming functionally independent. Infective endocarditis is usually considered a relative contraindication to thrombolytic therapy. We describe 3 consecutive cases of acute middle cerebral artery occlusion due to infective endocarditis, who were all successfully treated with intra-arterial mechanical thrombectomy using the Solitaire device. From this limited experience, mechanical thrombectomy could be used as an effective acute treatment for ischemic stroke in patients with infective endocarditis...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27920714/idarucizumab-as-antidote-to-intracerebral-hemorrhage-under-treatment-with-dabigatran
#3
Valentin Held, Philipp Eisele, Christoph C Eschenfelder, Kristina Szabo
BACKGROUND AND PURPOSE: Non-vitamin K anticoagulants (NOAC) such as dabigatran have become important therapeutic options for the prevention of stroke. Until recently, there were only nonspecific agents to reverse their anticoagulant effects in a case of emergency. Idarucizumab, an antibody fragment targeting dabigatran, is the first specific antidote for a NOAC to be approved, but real-world experience is limited. METHODS: We report two cases of patients on dabigatran with acute intracerebral hemorrhage who received idarucizumab...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27920713/late-onset-langerhans-cell-histiocytosis-with-cerebellar-ataxia-as-an-initial-symptom
#4
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
#5
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/27790126/hemiplegic-migraine-presenting-with-prolonged-somnolence-a-case-report
#6
Christian Saleh, Geneviève Pierquin, Stefan Beyenburg
Hemiplegic migraine is a rare and complex disease, characterized by migraine with a reversible motor aura. Hemiplegic migraine can be easily misdiagnosed at its first presentation with an atypical severe form of migraine, a stroke, multiple sclerosis, metabolic disorders, conversion disorder or an epilepsy. We present the case of a young 24-year-old male patient, who since the age of 4 years had been having multiple episodes of migraine associated with hemiparesis, paraesthesia, prolonged somnolence, aphasia and confusion...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27790125/a-case-of-turner-syndrome-with-multiple-embolic-infarcts
#7
Cindy W Yoon, Eungseok Lee, Byung-Nam Yoon, Hee-Kwon Park, Joung-Ho Rha
Only a few cases of Turner syndrome (TS) with ischemic stroke have been reported. Various arteriopathies of the cerebral arteries, including fibromuscular dysplasia, congenital hypoplasia, moyamoya syndrome, and premature atherosclerosis have been assumed to be the cause of ischemic stroke in TS. There has been no case report of a TS patient presenting with an embolic stroke pattern without any cerebral arteriopathy. A 28-year-old woman with TS was referred to our hospital because of abnormal brain magnetic resonance imaging (MRI) findings...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27790124/cerebral-cavernous-malformation-a-portuguese-family-with-a-novel-ccm1-mutation
#8
João Pedro Marto, Inês Gil, Sofia Calado, Miguel Viana-Baptista
INTRODUCTION: Cerebral cavernous malformation (CCM) is a vascular disorder characterized by the presence of central nervous system cavernomas. In familial forms, mutations in three genes (CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10) were identified. We describe a Portuguese family harboring a novel CCM1 mutation. CASE PRESENTATION: The proband is a woman who at the age of 55 years started to have complex partial seizures and episodic headache. Although nothing was found during her neurological examination, brain MRI showed bilateral, supra- and infratentorial cavernomas...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27790123/sodium-valproate-exacerbating-an-underlying-disorder-of-fatty-acid-metabolism
#9
Jamie Bellinge, Sanjaya Herath, Dharmesh Sonigra
We describe a 29-year-old female who presented with rhabdomyolysis shortly after starting a course of sodium valproate. A thorough investigation revealed a likely mitochondrial origin inducing this susceptibility. An underlying mitochondrial disorder should be considered in all patients who present with undifferentiated disease whilst taking sodium valproate.
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27721782/role-of-therapeutic-plasma-exchange-in-treatment-of-tumefactive-multiple-sclerosis-associated-low-cd4-and-cd8-levels
#10
Kristen Lew, Nishith Mewada, Sahana Ramanujam, Bahareh Hassanzadeh, John E Donahue, Leema Reddy Peddareddygari, Robert Moser, Charles Kososky, Raji P Grewal
We report a 35-year-old healthy male who developed central nervous system inflammatory demyelinating disease consistent with tumefactive multiple sclerosis. About 2 weeks after onset of symptoms and prior to initiation of therapy, the patient had lymphopenia and low CD4 and CD8 levels. His lymphocyte count was 400 cells/µl (850-3,900 cells/µl), CD4 was 193 cells/µl (490-1,740 cells/µl) and CD8 was 103 cells/µl (180-1,170 cells/µl). He was treated with intravenous methylprednisolone followed by therapeutic plasma exchange, the levels of CD4 and CD8 normalized, and ultimately, he recovered completely...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27721781/pudendal-neuralgia-as-the-initial-manifestation-of-infiltrative-sacrococcygeal-chordoma
#11
Sira Carrasco García de León, José Manuel Flores Barragán, Natalia Villasanti Rivas
Sacrococcygeal chordoma is a malignant tumour originating from remnants of the notochord. Chordomas are slow-growing tumours whose symptoms develop insidiously. We present the case of a 72-year-old woman with a 6-month history of genital pain radiating to the perianal area and exacerbating when she was in a sitting position. MRI and PET studies revealed a large mass in the sacrococcygeal region causing bone destruction and invasion of neurovascular structures. The immunohistochemical study of the surgical specimen determined it to be chordoma...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27721780/the-need-for-a-rational-approach-to-vasoconstrictive-syndromes-transcranial-doppler-and-calcium-channel-blockade-in-reversible-cerebral-vasoconstriction-syndrome
#12
Elisabeth B Marsh, Wendy C Ziai, Rafael H Llinas
INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) typically affects young patients and left untreated can result in hemorrhage or ischemic stroke. Though the disorder has been well characterized in the literature, the most appropriate way to diagnose, treat, and evaluate therapeutic response remains unclear. In previous studies, transcranial Doppler ultrasound (TCD) has shown elevated velocities indicative of vasospasm. This imaging modality is noninvasive and inexpensive; an attractive option for diagnosis and therapeutic monitoring if it is sensitive enough to detect changes in the acute setting given that RCVS often affects the distal vessels early in the course of disease...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27721779/anti-cyclic-citrullinated-peptide-antibody-positive-meningoencephalitis-in-the-preclinical-period-of-rheumatoid-arthritis
#13
Tomoya Shibahara, Tomonaga Matsushita, Ryu Matsuo, Yoshihisa Fukushima, Kenji Fukuda, Hiroshi Sugimori, Masahiro Kamouchi, Takanari Kitazono, Tetsuro Ago
Rheumatoid meningoencephalitis (RM) is a rare complication of rheumatoid arthritis (RA). This report describes a 63-year-old man with complaints of high-grade fever, headache, and vomiting for several days before admission. Both his serum and cerebrospinal fluid were positive for anti-cyclic citrullinated peptide (CCP) antibody and rheumatoid factor, and contrast-enhanced fluid-attenuated inversion recovery magnetic resonance imaging (MRI) showed abnormal gadolinium enhancement of the meninges and high-intensity lesions in the subarachnoid spaces...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27504092/brown-heroin-associated-candida-albicans-ventriculitis-and-endophthalmitis-treated-with-voriconazole
#14
Nora Elfiky, Kelly Baldwin
Chronic meningitis and ventriculitis are defined as inflammatory pleocytoses in the cerebrospinal fluid (CSF) and ependyma that persists for at least 1 month without spontaneous resolution. Because the CSF communicates directly with the posterior compartments of the eye, fungal infections in the brain often cause secondary ophthalmologic complications. We report a 23-year-old male who presented to the emergency room with progressive severe headaches associated with insidious monocular vision loss. After extensive workup and a multidisciplinary team effort, the patient was diagnosed with ventriculitis and endogenous endopthalmitis...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27504091/a-young-woman-with-ischemic-stroke-should-we-pay-more-attention-to-varicella-zoster-infection
#15
Cláudia Borbinha, João Pedro Marto, Sofia Calado, Miguel Viana-Baptista
Ischemic and hemorrhagic stroke are recognized complications of Varicella zoster virus (VZV) infections, although uncommon and poorly documented. The authors report the case of a 31-year-old woman admitted with acute ischemic stroke of the right posterior cerebral artery and a history of a thoracic rash 1 month before. Aspirin and simvastatin were prescribed, but the patient suffered a stepwise deterioration the following days, with new areas of infarction on brain imaging. Despite no evidence of cardiac or large vessel embolic sources, anticoagulation was started empirically 6 days after stroke onset...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27482196/intravenous-thrombolysis-after-reversal-of-dabigatran-by-idarucizumab-a-case-report
#16
Waldemar Kafke, Peter Kraft
We describe a 75-year-old female patient with nonvalvular atrial fibrillation who presented with acute ischemic stroke during treatment with dabigatran 2 × 110 mg per day. After informed consent, we reversed the anticoagulant effects of dabigatran using idarucizumab and applied an intravenous thrombolysis (IVT) with recombinant tissue plasminogen activator (off-label use). An intracerebral hemorrhage was excluded after systemic thrombolysis. Despite the IVT, the patient's clinical condition deteriorated and she developed an ischemic lesion in the right pons, the right thalamus and right cerebellum...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27482195/confocal-cornea-microscopy-detects-involvement-of-corneal-nerve-fibers-in-a-patient-with-light-chain-amyloid-neuropathy-caused-by-multiple-myeloma-a-case-report
#17
Dietrich Sturm, Tobias Schmidt-Wilcke, Tineke Greiner, Christoph Maier, Marc Schargus, Martin Tegenthoff, Matthias Vorgerd
Changes in the subbasal corneal plexus detected by confocal cornea microscopy (CCM) have been described for various types of neuropathy. An involvement of these nerves within light-chain (AL) amyloid neuropathy (a rare cause of polyneuropathy) has never been shown. Here, we report on a case of a patient suffering from neuropathy caused by AL amyloidosis and underlying multiple myeloma. Small-fiber damage was detected by CCM.
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27462243/new-onset-refractory-status-epilepticus-more-investigations-more-questions
#18
Philippe Dillien, Susana Ferrao Santos, Vincent van Pesch, Vanessa Suin, Sophie Lamoral, Philippe Hantson
A 27-year-old previously healthy woman was admitted to the hospital with recurrent seizures. Status epilepticus developed that became refractory to third-line therapy with propofol and barbiturates. The patient had a very extensive diagnostic workup including autoimmune, viral and genetic investigations. A tentative immune therapy was proposed with high doses of steroids and plasma exchanges. Our patient had an inherited heterozygous single nucleotide variant in the sequence c.1280A>G [p.Lys427Arg] of the SMC3 gene that was insufficient to explain the seizures...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27462242/factors-affecting-phenotype-variability-in-a-family-with-cmt2b-gender-and-lrsam1-genotype
#19
Leema Reddy Peddareddygari, Kinsi Oberoi, Jaasrini Reddy Vellore, Raji P Grewal
Charcot-Marie-Tooth disease type 2 (CMT2) is an autosomal dominant axonal neuropathy caused by mutations in various genes. The subtype CMT2B results from missense mutations in RAB7A, member RAS oncogene family gene, whereas missense mutations in the Leucine-rich repeat and sterile alpha motif-containing protein 1 (LRSAM1) gene cause CMT2P. We describe the genotype/phenotype analysis of a family in which a previously described mutation in the RAB7A gene and a novel mutation in the LRSAM1 gene were identified...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27462241/reversible-valproate-induced-pisa-syndrome-and-parkinsonism-in-a-neuro-oncology-patient-with-depression-and-epilepsy
#20
Andrea Botturi, Antonio Silvani, Gabriella Pravettoni, Riccardo Augusto Paoli, Claudio Lucchiari
Neurological and psychiatric conditions frequently overlap in neuro-oncology. This overlapping negatively affects patients' quality of life and decreases the ability of providers to manage specific symptoms by therapy modulation, especially when psychopharmacotherapy needs to be prescribed. We describe here a patient with recurrent brain tumor, symptomatic epilepsy and depression who developed Pisa syndrome and parkinsonism after several months of valproic acid use. An accurate recognition of symptoms and treatment side effect allowed an appropriate clinical approach so as to rapidly improve both movement disorder and depression without increasing the risk of developing seizure...
May 2016: Case Reports in Neurology
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