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Cranial neuropathy

Ibrahim Hussain, Theodore H Schwartz, Jeffrey P Greenfield
Basilar invagination is defined as abnormal upward and/or posterior displacement of the odontoid leading to ventral compression of the cervicomedullary junction. This condition leads to lower cranial neuropathies, sensorimotor deficits, and myelopathy. These symptoms can persist even after posterior decompression, which is an indication for ventral decompression. Transoral approaches to the upper cervical spine carry significant morbidity, limiting their utility. The endonasal approach to the upper cervical spine presents an alternative for patients with amenable anatomy...
March 13, 2018: Clinical Spine Surgery
Marc Vecchi, Ricardo Pereira Mestre, S Luciamma Thiekalamuriyil, Roberto Cartolari
In the context of painful cranial neuropathies, a very rare cause is represented by the irritation of the glossopharyngeal nerve due to various aetiologic factors. Here, we present a case of neuralgia of the ninth right cranial nerve due to a compression of its nerve root upon the kinking of the homolateral vertebral artery, resulting in a disabling clinical overview for the patient. Our objective was to focus the reader's attention on the clinical manifestation, which alone could lead to an immediate diagnosis...
September 2017: Case Reports in Neurology
K J Low, K Stals, R Caswell, M Wakeling, J Clayton-Smith, A Donaldson, N Foulds, A Norman, M Splitt, K Urankar, K Vijayakumar, A Majumdar, Ddd Study, S Ellard, S F Smithson
CHN is genetically heterogeneous and its genetic basis is difficult to determine on features alone. CNTNAP1 encodes CASPR, integral in the paranodal junction high molecular mass complex. Nineteen individuals with biallelic variants have been described in association with severe congenital hypomyelinating neuropathy, respiratory compromise, profound intellectual disability and death within the first year. We report 7 additional patients ascertained through exome sequencing. We identified 9 novel CNTNAP1 variants in 6 families: three missense variants, four nonsense variants, one frameshift variant and one splice site variant...
March 6, 2018: European Journal of Human Genetics: EJHG
Bernardo Cacho-Díaz, Nydia A Lorenzana-Mendoza, Gervith Reyes-Soto, Allan Hernández-Estrada, Alejandro Monroy-Sosa, Paola Guraieb-Chahin, David Cantu-de-León
This study aimed to establish the prognostic utility of lactate dehydrogenase (LDH) levels in the cerebrospinal fluid (CSF) of patients with neoplastic meningitis (NM). Patients with a confirmed diagnosis of NM at a cancer referral center were included. Data on demographic and oncological background, clinical symptoms, diagnostic tests, treatment, and survival were analyzed. In total, 119 patients were included, 74% of whom were females. The mean age was 44.2 years at the time of cancer diagnosis and 46.6 years at the time between NM diagnosis...
February 23, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Margaret L Pfeiffer, Helen A Merritt, Lucy A Bailey, Karina Richani, Margaret E Phillips
Purpose: To describe a case of orbital apex syndrome as a result of isolated bacterial sinusitis. Observations: A 63-year-old woman presented with an orbital apex syndrome from isolated bacterial sinusitis with rapidly declining visual acuity to no light perception. We compared our case with 6 similar cases of severe vision loss from isolated bacterial sinusitis. In contrast to previously published cases, our patient presented with good vision yet deteriorated to no light perception despite appropriate treatment...
June 2018: American Journal of Ophthalmology Case Reports
Brandon J Baartman, Chrysavgi Adamopoulou
A 40-year-old African American man with recently diagnosed Human Immunodeficiency Virus (HIV) presented with isolated left abducens palsy. Initial neuro-imaging and laboratory evaluation, including cerebrospinal fluid (CSF) analysis, were unremarkable. Continued search for causative aetiology revealed systemic lymphoma diagnosed ultimately by bone marrow biopsy. Systemic lymphoma is commonly encountered in the HIV patient population, but presentation can be unusual and has been seen, albeit rarely, in the setting of isolated cranial neuropathy...
February 2018: Neuro-ophthalmology
Yun Wang, Qian Dong, Shu-Juan Li, Wen-Li Hu
Hand knob infarction (HKI) is a rare clinical condition which is often misdiagnosed as peripheral neuropathy. This study aimed to identify the clinical characteristics and risk factors of HKI. Nine HKI patients admitted between January 2013 and March 2016 were confirmed by magnetic resonance imaging. Their medical records were collected and analyzed. The modified Rankin Scale was used to assess clinical outcomes. Routine laboratory tests, electrocardiogram, echocardiography, cranial magnetic resonance imaging, magnetic resonance angiography, computed tomography angiography, and Doppler ultrasonography examinations were performed...
February 17, 2018: Neurological Sciences
Francis Renault, Roberto Flores-Guevara, Bernard Sergent, Jean Jacques Baudon, Jessie Aouizerate, Marie-Paule Vazquez, Cyril Gitiaux
INTRODUCTION: We designed a retrospective study of 59 patients with congenital sporadic non-progressive bilateral facial and abducens palsies. METHODS: Examinations included needle EMG of facial and oral muscles, facial nerve motor latency and conduction velocity (FNCV), and blink responses (BRs). RESULTS: Neurogenic EMG changes were found in one or more muscles in 55/59 patients, with no abnormal spontaneous activity. EMG changes were homogeneously neurogenic in 17 patients, homogeneously myopathic in one, and heterogeneous in 41/59 patients...
February 9, 2018: Muscle & Nerve
Stephen W English, Theodore J Passe, E Paul Lindell, James P Klaas
Cervical artery dissection is an underrecognized cause of lower cranial neuropathies and diagnosis can remain elusive if not properly investigated. We present a case of an internal carotid artery dissection that was initially missed in a 48-year-old man who presented with subacute-onset of dysarthria, dysphagia, and unilateral tongue weakness. Knowledge of the most common presenting symptoms, relevant neuroanatomy, and neuroimaging techniques is essential to avoid misdiagnosis. Pseudoaneurysm formation from subadventitial carotid artery dissection may result in compressive neuropathies of cranial nerves IX, X, XI, and XII without associated cerebral ischemia...
February 5, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Thien Nguyen, Courtney Duong, John P Sheppard, Seung Jin Lee, Amar U Kishan, Percy Lee, Stephen Tenn, Robert Chin, Tania B Kaprealian, Isaac Yang
Vestibular schwannomas (VS) are benign tumors stemming from the eighth cranial nerve. Treatment options for VS include conservative management, microsurgery, stereotactic radiosurgery, and fractionated radiotherapy. Though microsurgery has been the standard of care for larger lesions, hypo-fractionated stereotactic radiotherapy (hypo-FSRT) is an emerging modality. However, its clinical efficacy and safety have yet to be established. We conducted a systematic review and meta-analysis of manuscripts indexed in PubMed, Scopus, Web of Science, Embase, and Cochrane databases reporting outcomes of VS cases treated with hypo-FSRT...
March 2018: Clinical Neurology and Neurosurgery
Asuka Asanome, Kohei Kano, Kae Takahashi, Tsukasa Saito, Jun Sawada, Takayuki Katayama
A 58-year-old female was admitted to our hospital because of recurrent multiple cranial neuropathy (right facial palsy followed by involvement of the left trigeminal, facial, acoustic, pharyngeal, and vagal nerves and the right abducens nerve). Brain MRI showed gadolinium enhancement of the right abducens, bilateral facial/acoustic, and left pharyngeal/vagal nerves, and 18F-Fluorodeoxyglucose (FDG)-positron emission tomography revealed abnormal FDG uptake in the right facial, acoustic, pharyngeal, and vagal nerves and the left cervical lymph nodes...
January 31, 2018: Rinshō Shinkeigaku, Clinical Neurology
Ru-Wen Zheng, Di Liu, Tay E Eric, Yan-Zhe Ning, Lu-Lu Chen, Hui Hu, Yi Ren
RATIONALE: Ramsay Hunt syndrome in conjunction with cranial polyneuritis is not extensively documented, and is very easily misdiagnosed. PATIENT CONCERNS: A case of a 53-year-old male with Ramsay Hunt syndrome in conjunction with cranial polyneuritis is presented with early symptoms of vertigo, cephalalgia, and facial palsy, followed by zoster oticus 10 days later. DIAGNOSES: Diagnosis was challenging as this condition presents with multiple neuropathies, and attempting to diagnose based on clinical symptoms was often misleading...
November 2017: Medicine (Baltimore)
Kunwarpal Singh, Kamlesh Gupta, Sukhdeep Kaur
Aim of the study: High-resolution ultrasonography of the tibial nerve is a fast and non invasive tool for diagnosis of diabetic peripheral neuropathy. Our study was aimed at finding out the correlation of the cross sectional area and maximum thickness of nerve fascicles of the tibial nerve with the presence and severity of diabetic peripheral neuropathy. Material and methods: 75 patients with type 2 diabetes mellitus clinically diagnosed with diabetic peripheral neuropathy were analysed, and the severity of neuropathy was determined using the Toronto Clinical Neuropathy Score...
December 2017: Journal of Ultrasonography
Prashanth Prabhu, Pratyasha Jamuar
Introduction  Vestibular symptoms and damage to the vestibular branch of the eighth cranial nerve is reported in individuals with auditory neuropathy spectrum disorder (ANSD). However, the real life handicap caused by these vestibular problems in individuals with ANSD is not studied. Objective  The present study attempted to evaluate the dizziness-related handicap in adolescents and adults with ANSD. Method  The dizziness handicap inventory (DHI) was administered to 40 adolescents and adults diagnosed with ANSD...
January 2018: International Archives of Otorhinolaryngology
Derek Ebner, Kelsey Smith, Daniel DeSimone, Muhammad Rizwan Sohail
A 61-year-old man presented to the emergency department in the summer with a right seventh cranial nerve lower motor neuron palsy and worsening paraesthesias for 6 weeks. He had debilitating pain at the scalp and spine. Prior work up was unrevealing. The patient resided in the upper Midwest region of the USA and worked outdoors, optimising the landscape for white tailed deer. Repeat cerebrospinal fluid testing revealed a lymphocytic pleocytosis and positive IgM Lyme serology. Brain MRI demonstrated enhancement of multiple cranial nerves bilaterally...
January 23, 2018: BMJ Case Reports
Mark Peter Maskery, Paul N Cooper, Adrian Pace
Sarcoidosis is an idiopathic multisystem granulomatous disorder of unknown cause. Nervous system involvement (central and/or peripheral) is uncommon, developing in 5%-10%. The presenting symptoms are variable, reflecting the level of involvement, and frequently fluctuate and progress. Diagnosing neurosarcoidosis in people with previously confirmed systemic disease may be relatively straightforward, but diagnosing primary neurosarcoidosis is challenging. Managing neurosarcoidosis is primarily consensus based; corticosteroid is its mainstay, alongside corticosteroid-sparing agents and emerging novel therapies...
January 23, 2018: Practical Neurology
Heather E Moss, Peter W MacIntosh, Konstantin V Slavin, John M Collins
An 86-year old man developed sequential dysfunction of trigeminal (V1), facial, abducens, trigeminal (v2), oculomotor, and hypoglossal cranial nerves on the right over 20 months. Magnetic resonance imaging (MRI) showed a lesion in the right cavernous sinus. Although there was clinical suspicion that this was related to perineural spread of an extracranial tumour, a primary lesion was not discovered. Stereotactic biopsies of the intracranial lesion were non-diagnostic, and the patient succumbed to his tumour following a period of rapid growth...
August 2017: Neuro-ophthalmology
Divya Arya, Tushar Bajaj, Jose Gonzalez, Rene Elkin
BACKGROUND Ramsay Hunt syndrome is a rare otologic complication resulting from varicella zoster virus reactivation that can present with a myriad of clinical presentations. Most common being triad of ear pain, vesicles at auricle, and ear canal with same side facial palsy. CASE REPORT We report a case of a 29-year-old male with a human immunodeficiency virus (HIV) infection who presented with left facial palsy, vesicles, pain in the left ear, dysphagia, dizziness, and headache resulting from multiple cranial nerves involvement such as cranial nerve V, VII, VIII, IX, and X...
January 18, 2018: American Journal of Case Reports
Amro M Stino, Benn E Smith
Multiple techniques have been developed for the electrodiagnostic evaluation of cranial nerves XI and XII. Each of these carries both benefits and limitations, with more techniques and data being available in the literature for spinal accessory than hypoglossal nerve evaluation. Spinal accessory and hypoglossal neuropathy are relatively uncommon cranial mononeuropathies that may be evaluated in the outpatient electrodiagnostic laboratory setting. A review of available literature using PubMed was conducted regarding electrodiagnostic technique in the evaluation of spinal accessory and hypoglossal nerves searching for both routine nerve conduction studies and repetitive nerve conduction studies...
January 2018: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
Alan D Legatt
The electrocochleogram and brainstem auditory evoked potentials (BAEPs) are electrophysiologic signals used to assess the auditory nerve. The electrocohleogram includes the cochlear microphonic, the cochlear summating potential, and the eighth nerve compound action potential. It is used predominantly for hearing assessment and for diagnosis of Ménière disease and auditory neuropathy. Brainstem auditory evoked potentials are used for hearing assessment, diagnosis of dysfunction within the cochlea, the auditory nerve, and the brainstem auditory pathways up to the level of the mesencephalon, and intraoperative monitoring of these structures...
January 2018: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
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