scholarly journals An infantile alantoaxial dislocation with patent foramen ovale managed with titanium cabling and allogenic bone grafts

2018 ◽  
Vol 10 (1) ◽  
Author(s):  
Seidu A. Richard ◽  
Zhi Gang  Lan ◽  
Xiao Yang ◽  
Siqing Huang
Keyword(s):  
Action Potentials ◽  
Patent Foramen Ovale ◽  
Early Stage ◽  
Sensory Nerve ◽  
Odontoid Process ◽  
Bone Destruction ◽  
Lower Limbs ◽  
Foramen Ovale ◽  
Compound Muscle Action Potentials ◽  
Patient Reported

Atlantoaxial dislocation is a disorder that is characterized with loss of stability of the atlas and axis (C1-C2) with consequential loss of usual articulation. Although this condition is very common, no one has reported a case as young as our patients. We present a 7-month infant with bilateral paralysis of the lower limbs for four (4) months with no history of trauma. Computer tomographic (CT-scan) imaging revealed alantoaxial dislocation with severe cervical spinal cord compression. The odontoid process is displaced outwardly with no bone destruction. Doppler echocardiogram done revealed patent foramen ovale. Thorough physical examination as well as radiological evaluation revealed no feather malformations. Electrophysio - logical studies reveal normal compound muscle action potentials (CMAP) and sensory nerve action potentials (SNAPs) in all the limbs. Electromyography (EMG) also revealed normal nerves in the limbs and the trunk. We attained a stable fusion and anatomical reduction using a posterior titanium wire and an iliac bone graft harvested from his mother. This is the youngest patient reported in literature. Infantile alantoaxial dislocation should be managed at early stage to prevent long-term neurologic disorders.

scholarly journals Mixed Upper and Lower Motor Neuron Damage in Japanese Encephalitis Virus Infection

2020 ◽  
Vol 12 (3) ◽  
pp. 482-488
Author(s):  
Ritwik Ghosh ◽  
Souvik Dubey ◽  
Subhankar Chatterjee ◽  
Biman Kanti Ray ◽  
Julián Benito-León
Keyword(s):  
Neurological Examination ◽  
Action Potentials ◽  
Sensory Nerve ◽  
Brain Magnetic Resonance Imaging ◽  
Anterior Horn ◽  
Lower Limbs ◽  
Clinical Approach ◽  
Anterior Horn Cell ◽  
Upper Limbs ◽  
Compound Muscle Action Potentials

Cerebral manifestations in Japanese B encephalitis are well known. However, there are very few studies focusing on extra-cerebral manifestations, among which focal anterior horn cell involvement is exceedingly rare. We herein report a case of Japanese B encephalitis with focal anterior horn cell involvement and unfurl how stepwise clinical approach and targeted investigations helped to solve the diagnostic conundrum. A 27-year-old female was admitted with fever, headache, altered sensorium, and convulsions. She tested positive for Japanese B encephalitis-IgM. Following conservative management, she regained consciousness after 5 days when neurological examination revealed marked cognitive impairment, medial convergence of eyeballs, upward gaze restriction, upper limbs dystonia with brisk tendon jerks, and flaccid paraparesis. A repeat neurological examination, on day 15 of admission, showed marked wasting and intermittent fasciculation in both lower limbs. Brain magnetic resonance imaging showed asymmetrical (right > left) bilateral thalamic and midbrain lesions, hyperintense on T2 and T2-fluid-attenuated inversion recovery (FLAIR)-weighted imaging with mild diffusion restriction on diffusion-weighted imaging and apparent diffusion coefficient map, suggestive of encephalitis . Nerve conduction study revealed decreased compound muscle action potentials exclusively in lower limbs with intact sensory nerve action potentials. Electromyogram showed chronic denervation potentials and presence of spontaneous activity in lower limbs, but not in upper limbs, indicative of focal anterior horn cell involvement. Prognosis of Japanese B encephalitis does not only depend on cerebral sequelae. Anterior horn cell involvement can dictate poor outcome and can easily be missed if not carefully dealt with.

scholarly journals Clinical Features of Familial Amyloidotic Polyneuropathy With Transthyretin p.Ala117Ser Mutation in Mainland China

2021 ◽  
Author(s):  
Yeli Zhu ◽  
Xiying Zhu ◽  
Wei Li ◽  
Zhaoyong Zhang ◽  
Hui Zheng ◽  
...  
Keyword(s):  
Clinical Features ◽  
Action Potentials ◽  
Laboratory Data ◽  
Mainland China ◽  
Sensory Nerve ◽  
Lower Limbs ◽  
Familial Amyloidotic Polyneuropathy ◽  
Initial Symptom ◽  
Female Ratio ◽  
Compound Muscle Action Potentials

Abstract Objective: Our study aimed to report the clinical features of ATTR-PN with TTR p.Ala117Ser mutation in mainland China. Methods: Thirteen patients from 13 different families diagnosed with p.Ala117Ser ATTR-PN were identified from three centres. Clinical and laboratory data were retrospectively retrieved for analysis. Results: The male/female ratio was 11:2. All patients showed late onset, with the age of onset at 57.8 ± 5.8 years. The initial symptom was numbness of the lower or upper extremities in 9 patients (69.2%). Paraesthesia was present in all patients. Eleven patients (84.6%) had autonomic dysfunction. Cardiac, renal, hepatic, and ocular dysfunctions were noted in 8 (61.5%), 1 (7.7%), 2 (15.4%), and 3 (23.1%) patients, respectively. Nerve conduction studies have shown axonal-type sensorimotor polyneuropathy. The decline in sensory nerve action potentials was more noticeable than in compound muscle action potentials. The nerve damage present in the lower limbs was more severe than that in the upper limbs. Nerve biopsy revealed positive Congo red staining in 7/10 patients (70%). Conclusion: Our study is the largest population report on this rare p.Ala117Ser mutation in mainland China.

Sensory Nerve Conduction Studies

2016 ◽  
pp. 292-311
Author(s):  
Eric J. Sorenson
Keyword(s):  
Nerve Conduction ◽  
Action Potentials ◽  
Sensory Nerve ◽  
Nerve Conduction Studies ◽  
Nerve Action ◽  
Compound Muscle Action Potentials ◽  
Sensory Nerve Action ◽  
Electrical Generator ◽  
Nerve Dysfunction ◽  
Potential Waveform

Sensory nerve action potentials (SNAPs) are an important component of an electrodiagnostic test of peripheral nerves and can localize lesions either proximal or distal to the dorsal root ganglia. They are among the most sensitive measures of peripheral nerve dysfunction, and can provide information about the pathophysiology of the neuropathy, suggesting demyelination or axonal loss in some cases. Because SNAPs are much smaller than compound muscle action potentials, they are technically more difficult to study. Temperature will have a greater impact on SNAPs than motor potentials, with cooler limb temperatures leading to longer latencies, slower conduction velocities, and larger amplitudes. Responses are commonly averaged 3 to 5 times to minimize the effect of the background noise on the waveforms. Distance from the electrical generator and inter-electrode distance will also impact the appearance and size of the nerve potential waveform. This chapter reviews the concepts and applications of sensory nerve action potentials and sensory nerve conduction studies.

scholarly journals Lambert-Eaton syndrome without an identified neoplasm

2021 ◽  
Author(s):  
Ana Cláudia Pires Carvalho ◽  
Stella Angelis Trivellato ◽  
Guilherme Jardini Drumond Anastacio ◽  
Fernanda Rezende Dias ◽  
Luisa Crevelin Costa ◽  
...  
Keyword(s):  
Neuromuscular Junction ◽  
Action Potentials ◽  
Low Frequency ◽  
Diagnostic Methods ◽  
Lower Limbs ◽  
High Frequency Stimulation ◽  
Voltage Gated Calcium Channels ◽  
Low Frequency Stimulation ◽  
Compound Muscle Action Potentials ◽  
Frequency Stimulation

Introduction: Lambert-Eaton syndrome occurs due to the attack of autoantibodies to voltage-gated calcium channels in the presynaptic terminal of the neuromuscular junction and is usually paraneoplastic. Objectives: Describe the case of a patient with weakness which was investigated for neoplasm. Design and setting: Case report Methods: Analysis of medical record, photographic record of the diagnostic methods and literature review. Case description: Woman, 60 years old, diabetic, hypertensive and ex-smoker, with proximal weakness in the lower limbs for 4 months with paresthesia in the extremities. In 2 months she needed a cane due to frequent falls, followed by proximal weakness of the upper limbs. She lost 8 kg in 4 months. Neurological examination showed hypotrophy in thighs, proximal tetraparesis predominantly in lower limbs and global hyporeflexia. Electroneuromyography showed decreases to repetitive low-frequency stimulation, but significant increases with repetitive high-frequency stimulation and increased amplitude of compound muscle action potentials after effort, suggesting impairment of the neuromuscular junction in the presynaptic topography. She was diagnosed with LambertEaton syndrome. An investigation of paraneoplastic syndrome was carried out, with tumor markers, tomography of the chest, abdomen and pelvis, thyroid ultrasound, mammography and oncotic colposcopy, all without findings of neoplasia. It was proposed a treatment with human immunoglobulin and followup with physiotherapy, occupational therapy and psychology. She showed a significant improvement in strength after starting treatment. Conclusion: Patients with Lambert-Eaton syndrome should be investigated for an underlying neoplasm and followed up periodically, considering the possibility of cancer diagnosis even months or years after the neurological syndrome.

Percutaneous Treatment of Congenital Defects of the Inter-atrial Septum in Adults – Recent Advances and Persistent Pitfalls

2009 ◽  
Vol 4 (1) ◽  
pp. 76
Author(s):  
James Slater ◽  
Mark Fisch ◽  
◽  
Keyword(s):  
Atrial Septal Defect ◽  
Adult Patient ◽  
Patent Foramen Ovale ◽  
Septal Defect ◽  
Percutaneous Treatment ◽  
Atrial Septum ◽  
Congenital Defects ◽  
Foramen Ovale ◽  
Anatomy And Physiology ◽  
Specific Tissue

William Harvey was the first scientist to describe the heart as consisting of separate right- and left-sided circulations. Our understanding of the heart’s anatomy and physiology has grown significantly since this landmark discovery in 1628. Today, we recognise not only the importance of these separate systems, but also the specific tissue that divides them. Our growing understanding of the inter-atrial septum has allowed us to identify defects within this structure and develop effective percutaneous devices for closure of these defects in the adult patient. This article discusses the formation of a patent foramen ovale (PFO) and atrial septal defect (ASD). In addition, we describe the medical illnesses caused by these defects and summarise the indications and risks related to percutaneous closure of these defects. We also report the most up-to-date transcatheter therapeutic options for closure of these common congenital defects in the adult patient.

Stroke Prophylaxis by Percutaneous Closure of Patent Foramen Ovale and Left Atrial Appendage

2011 ◽  
Vol 6 (1) ◽  
pp. 67
Author(s):  
Antonio L Bartorelli ◽  
Claudio Tondo ◽  
◽  
Keyword(s):  
Left Atrial ◽  
Patent Foramen Ovale ◽  
Stroke Risk ◽  
Left Atrial Appendage ◽  
Cryptogenic Stroke ◽  
Atrial Appendage ◽  
Foramen Ovale ◽  
Percutaneous Procedures ◽  
Cerebrovascular Events

Innovative percutaneous procedures for stroke prevention have emerged in the last two decades. Transcatheter closure of the patent foramen ovale (PFO) is performed in patients who suffered a cryptogenic stroke or a transient ischaemic attach (TIA) in order to prevent recurrence of thromboembolic events. Percutaneous occlusion of the left atrial appendage (LAA) has been introduced to reduce stroke risk in patients with atrial fibrillation (AF). The role of PFO and LAA in the occurrence of cerebrovascular events and the interventional device-based therapies to occlude the PFO and LAA are discussed.

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