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Cranial neuropathy

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https://www.readbyqxmd.com/read/28069788/proteinase-3-antineutrophil-cytoplasmic-antibody-positive-ulcerative-colitis-presenting-with-abducens-neuropathy
#1
Yuki Kirito, Daisuke Yamamoto, Tsuyoshi Uchiyama
A 72-year-old man with ulcerative colitis (UC) presented with complete left abducens nerve palsy. Although MRI showed no significant changes, cerebrospinal fluid analysis revealed pleocytosis and elevated protein and interleukin (IL)-6 levels. His serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) level was also elevated to 31.1 U/mL, but granulomatosis with polyangiitis was not observed. On the basis of the diagnosis of autoimmune cranial neuropathy, he was treated with steroid therapy. While tapering steroid therapy, his serum PR3-ANCA levels; cerebrospinal fluid findings, including IL-6 levels; and symptoms improved...
January 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28009225/clinical-and-surgical-management-of-a-congenital-type-ii-split-cord-malformation-presenting-with-progressive-cranial-neuropathies-case-report
#2
Patrick R Maloney, Meghan E Murphy, Molly J Sullan, Kathryn M Van Abel, Shelagh A Cofer, John C Cheville, Nicholas M Wetjen
Split cord malformation (SCM) is a rare abnormality of notochord development. The majority of cases occur in the thoracolumbar region, with more than 30 cases of cervical SCM reported. The clinical impact of SCMs involving the cervical cord is therefore largely unknown. In addition, the concomitant finding of brainstem involvement is presumably incompatible with life in the majority of patients, resulting in a paucity of data regarding this clinical scenario. In this paper the authors present the first case, to their knowledge, of an incomplete cervical SCM involving the brainstem and discuss its clinical impact, diagnosis, and management...
December 23, 2016: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28005197/sbf1-mutations-associated-with-autosomal-recessive-axonal-neuropathy-with-cranial-nerve-involvement
#3
Andreea Manole, Alejandro Horga, Josep Gamez, Nuria Raguer, Maria Salvado, Beatriz San Millán, Carmen Navarro, Alan Pittmann, Mary M Reilly, Henry Houlden
Biallelic mutations in the SBF1 gene have been identified in one family with demyelinating Charcot-Marie-Tooth disease (CMT4B3) and two families with axonal neuropathy and additional neurological and skeletal features. Here we describe novel sequence variants in SBF1 (c.1168C>G and c.2209_2210del) as the potential causative mutations in two siblings with severe axonal neuropathy, hearing loss, facial weakness and bulbar features. Pathogenicity of these variants is supported by co-segregation and in silico analyses and evolutionary conservation...
December 22, 2016: Neurogenetics
https://www.readbyqxmd.com/read/27997917/cerebrospinal-fluid-findings-in-neurological-diseases-associated-with-sj%C3%A3-gren-s-syndrome
#4
Kaweh Pars, Refik Pul, Philipp Schwenkenbecher, Kurt-Wolfram Sühs, Ulrich Wurster, Torsten Witte, Paul Bronzlik, Martin Stangel, Thomas Skripuletz
BACKGROUND: Sjögren's syndrome is a chronic autoimmune-mediated disease that can cause a variety of neurological manifestations. METHODS: This study investigated characteristics of clinical and cerebrospinal fluid (CSF) features in patients with neurological diseases associated with Sjögren's syndrome. Eighty-two patients were examined separately according to the presence of Sjögren's syndrome alone or in combination with other autoimmune diseases. RESULTS: In the 47 patients with primary Sjögren's syndrome, peripheral neuropathy (57%) was found most frequently, followed by the involvement of the central nervous system (CNS; 17%), cranial neuropathy (15%), and myalgia (11%)...
December 21, 2016: European Neurology
https://www.readbyqxmd.com/read/27982499/hereditary-gelsolin-amyloidosis-hga-a-neglected-cause-of-bilateral-progressive-or-recurrent-facial-palsy
#5
Anna Sagnelli, Giuseppe Piscosquito, Daniela Di Bella, Laura Fadda, Lisa Melzi, Antonio Morico, Claudia Ciano, Franco Taroni, Dante Facchetti, Ettore Salsano, Davide Pareyson
We report the first Italian family affected by hereditary gelsolin amyloidosis (HGA), a rare autosomal dominant disease characterized by adult-onset slowly progressive cranial neuropathy, lattice corneal dystrophy, and cutis laxa. The index case was a 39-year-old male with a 9-year history of progressive bilateral facial nerve palsy. His mother had two episodes of acute facial palsy, and his maternal aunt and grandfather were also affected. Electrophysiological studies confirmed bilateral facial nerve involvement, without signs of peripheral polyneuropathy, and ophthalmological examination showed bilateral lattice corneal dystrophy, in both the index case and his mother...
December 16, 2016: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27957617/neuromyotonia-as-an-unusual-neurological-complication-of-primary-sj%C3%A3-gren-s-syndrome-case-report-and-literature-review
#6
REVIEW
Fei Xiao
Primary Sjögren's syndrome (PSS) is a systemic autoimmune disorder characterized by chronic inflammation of exocrine glands such as the lachrymal and salivary glands, leading to xerophthalmia and xerostomia. Neurological manifestations are sometimes found in patients with PSS. A variety of neurological complications has been reported in patients with PSS, and both the central nervous system (CNS) and peripheral nervous system (PNS) can be involved in PSS. Several forms of neuropathy, including polyneuropathy, cranial neuropathy, and multiple mononeuropathy, are often seen in PSS patients...
December 12, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27898604/long-term-symptom-specific-outcomes-for-patients-with-petrous-apex-cholesterol-granulomas-surgery-versus-observation
#7
Shawn M Stevens, Amy Manning, Myles L Pensak, Ravi N Samy
OBJECTIVE: Review long-term symptom-specific outcomes for petrous apex cholesterol granulomas (PACG). STUDY DESIGN: Retrospective review. SETTING: Tertiary center. PATIENTS: Adults with PACG were assessed from 1998 to 2015. INTERVENTION(S): Symptomatic patients were stratified into surgical and observation subgroups. MAIN OUTCOME MEASURE(S): Resolution rates of individual symptoms and chief complaints were assessed as was the impact of surgical approach and stent usage on symptom-specific outcomes...
November 24, 2016: Otology & Neurotology
https://www.readbyqxmd.com/read/27891417/a-commonly-missed-well-known-entity-acute-intermittent-porphyria-a-case-report
#8
Smilu Mohanlal, Radha Gulati Ghildiyal, Alpana Kondekar, Poonam Wade, Richa Sinha
Acute Intermittent Porphyria (AIP) usually presents with abdominal pain, peripheral neuropathy and psychiatric manifestations. Incidence of AIP being 5 in 1,00,000. We present a case of an 11-year-old male child with multiple cranial nerve involvement, quadriparesis, focal convulsions, hypertension, hyponatremia with history of recurrent abdominal pain. His complete haemogram, ultrasonography (USG) abdomen, renal function tests were normal, he was also evaluated for tuberculosis which was negative. On further evaluation Electroencephalography (EEG) was suggestive of a generalised seizure disorder, MRI Brain suggestive of Posterior Reversible Encephalopathy Syndrome (PRES), Electromyography revealed a sensory motor axonal polyneuropathy and urine UV fluoresence test was positive for porphobilinogen which clinched the diagnosis of AIP...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27885483/neuroborreliosis
#9
John J Halperin
Appropriate, critical application of evidence-based diagnostic criteria enables both a clear definition of what constitutes neuroborreliosis-nervous system infection with Borrelia burgdorferi sensu stricto in the US, B garinii and less commonly B. afzelii and other species in Europe-and recognition that this disorder is quite similar in Europe and the US. Most commonly evidenced by lymphocytic meningitis and/or multifocal inflammation of the peripheral (common; cranial neuropathy, radiculopathy, mononeuropathy multiplex) or central (rare) nervous system, it is readily diagnosed and highly antibiotic responsive...
November 24, 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27879149/increasing-amount-of-amyloid-are-associated-with-the-severity-of-clinical-features-in-hereditary-gelsolin-agel-amyloidosis
#10
Tiia Pihlamaa, Sinikka Suominen, Sari Kiuru-Enari, Maarit Tanskanen
BACKGROUND: Patients with hereditary gelsolin (AGel) amyloidosis (HGA) present with hanging skin (cutis laxa) and bilateral cranial neuropathy, and require symptomatic plastic surgery. Our clinical observation of tissue fragility prompted us to design a prospective study. METHODS: Twenty-nine patients with HGA undergoing surgery were interviewed and clinically examined. The height and thickness of skin folds in standard anatomical localizations were measured. The presence and distribution of amyloid in skin samples were analyzed using Congo red staining and immunohistochemistry using antibodies against gelsolin amyloid (AGel) subunit...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27861223/gastrointestinal-and-urologic-sphincter-dysfunction-in-stiff-person-syndrome
#11
Oana M Dumitrascu, Evgeny I Tsimerinov, Richard A Lewis
OBJECTIVES: Stiff person syndrome is a neurologic disorder characterized by axial rigidity leading to progressive disability, with broad clinical spectrum. METHODS: We report 2 cases with unique clinical presentation. RESULTS: Two young men suffered progressive urinary retention requiring bladder catheterization, anorectal spasms and constipation, complicated subsequently with lower extremity trigger-induced spasms, and gait instability. Associated symptoms revealed brainstem involvement (vertigo, diplopia, and cranial neuropathies) and dysautonomia (abnormal sweating and orthostatic hypotension)...
December 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27847569/retrospective-analysis-of-cerebrospinal-fluid-profiles-in-228-patients-with-leptomeningeal-carcinomatosis-differences-according-to-the-sampling-site-symptoms-and-systemic-factors
#12
Youngbo Shim, Ho-Shin Gwak, Sohee Kim, Jungnam Joo, Sang-Hoon Shin, Heon Yoo
OBJECTIVE: Elevated cell counts and protein levels in cerebrospinal fluid (CSF) result from disease activity in patients with leptomeningeal carcinomatosis (LMC). Previous studies evaluated the use of CSF profiles to monitor a treatment response or predict prognosis. CSF profiles vary, however, according to the sampling site and the patient's systemic condition. We compared lumbar and ventricular CSF profiles collected before intraventricular chemotherapy for LMC and evaluated the association of these profiles with patients' systemic factors and LMC disease activity...
November 2016: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/27846819/clinical-features-treatment-and-outcome-in-neurosarcoidosis-systematic-review-and-meta-analysis
#13
REVIEW
Daan Fritz, Diederik van de Beek, Matthijs C Brouwer
BACKGROUND: Neurosarcoidosis is a rare variant of sarcoidosis and is only described in small cohort studies. We define clinical features, treatment and outcome of patients with neurosarcoidosis over the last 35 years. METHODS: We performed a systematic review and meta-analysis of studies on neurosarcoidosis published between 1980 and 2016. Studies were included if they reported at least 5 cases. Studies describing one specific neurological presentation were excluded...
November 15, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27819416/neurological-manifestations-of-acute-posterior-multifocal-placoid-pigment-epitheliopathy
#14
Hussein Algahtani, Ashjan Alkhotani, Bader Shirah
BACKGROUND AND PURPOSE: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before. METHODS: A retrospective database review of all patients with a diagnosis of APMPPE was performed...
October 2016: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/27818809/neurobrucellosis-a-case-report-from-himachal-pradesh-india-and-review-of-the-literature
#15
Sujeet Raina, Ashish Sharma, Rajesh Sharma, Amit Bhardwaj
Human brucellosis is a multisystem disease that commonly presents as a febrile illness along with variable spectrum of clinical manifestations. Neurological complications include encephalitis, meningoencephalitis, radiculitis, myelitis, peripheral and cranial neuropathies, subarachnoid hemorrhage, and psychiatric manifestations. We report a case diagnosed as neurobrucellosis who presented with fever and bilateral upper motor neuron symptoms and signs along with bilateral sensorineural deafness. Diagnosis was confirmed by Rose Bengal Test (RBT) and standard tube agglutination test (SAT)...
2016: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/27811127/collet-sicard-syndrome-a-rare-but-important-presentation-of-internal-jugular-vein-thrombosis
#16
Shermyn Neo, Kim En Lee
We describe a rare neurological presentation of internal jugular vein thrombosis induced by central venous catheter placement in a patient with cancer. A 71-year-old man gave a 3-week history of dysphagia and dysarthria with left-sided neck pain and headache. He was receiving chemotherapy for appendiceal adenocarcinoma. On examination, he had left 9th-12th cranial neuropathies, manifesting as voice hoarseness, decreased palatal movement, absent gag reflex, weakness of scapular elevation and left-sided tongue wasting...
November 3, 2016: Practical Neurology
https://www.readbyqxmd.com/read/27806892/necrotizing-otitis-externa-diagnosis-treatment-and-outcome-in-a-case-series
#17
Eran Glikson, Doron Sagiv, Michael Wolf, Yisgav Shapira
We reviewed 25 cases of patients diagnosed with necrotizing otitis externa in our tertiary university-affiliated medical center between 2009 and 2015. Mean overall hospitalization duration was 14.52days, 95% of the patients showed specific seasonal incidence. Mean duration of symptoms prior to hospitalization was 6weeks and the duration correlated with outcome. Only 8% of the patients presented with cranial neuropathies; however, this presentation correlated with adverse outcome. Pseudomonas aeruginosa was the main causative organism (50%), with a 30% multidrug-resistance rate...
January 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/27770404/side-locked-headaches-an-algorithm-based-approach
#18
REVIEW
Sanjay Prakash, Chaturbhuj Rathore
The differential diagnosis of strictly unilateral hemicranial pain includes a large number of primary and secondary headaches and cranial neuropathies. It may arise from both intracranial and extracranial structures such as cranium, neck, vessels, eyes, ears, nose, sinuses, teeth, mouth, and the other facial or cervical structure. Available data suggest that about two-third patients with side-locked headache visiting neurology or headache clinics have primary headaches. Other one-third will have either secondary headaches or neuralgias...
December 2016: Journal of Headache and Pain
https://www.readbyqxmd.com/read/27758985/role-of-pulmonary-evaluation-in-diagnosis-of-neurosarcoidosis
#19
Pascal Kingah, Muhammad Alam, Karan Chugh, John Kamholz, Lobelia Samavati
BACKGROUND: Neurosarcoidosis is a serious extra pulmonary manifestation of sarcoidosis. Its presentation ranges from peripheral or cranial neuropathy to central nervous system dysfunction. It can mimic stroke or multiple sclerosis. Due to the variation in clinical presentation, diagnosis is difficult and often delayed. OBJECTIVE: Determine the proportion of patients with neurosarcoidosis who have positive findings on chest CT, lung biopsy or lymph node biopsy. METHODS: Retrospective study at the Sarcoidosis and Interstitial Lung Disease Center at Wayne State University-Detroit Medical Center in Detroit, MI...
October 7, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/27742375/combined-isolated-trigeminal-and-facial-neuropathies-from-perineural-invasion-by-squamous-cell-carcinoma-a-case-series-and-review-of-the-literature
#20
REVIEW
Pierre R Bourque, Gabrielle Bourque, William Miller, John Woulfe, Jodi Warman Chardon
Perineural invasion is a targeted cellular proliferation guided by neurotrophins, rather than a simple diffusion of tumor in a path of least resistance. Invasion of cranial nerves by squamous cell carcinoma can represent an important diagnostic dilemma. It commonly presents as a distinct clinical neurological syndrome of combined isolated trigeminal and facial neuropathies. The focal cancer source may have been overlooked or remain occult. This case series illustrates diverse clinical presentations and neuroimaging challenges in four patients with squamous cell carcinoma of the cranial nerves...
January 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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