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

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.

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

Pediatric Reports ◽

10.4081/pr.2018.7339 ◽

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.

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

10.21203/rs.3.rs-966814/v1 ◽

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.

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Limb-Specific Features and Asymmetry of Nerve Conduction Velocity and Nerve Trunk Size in Human

Frontiers in Physiology ◽

10.3389/fphys.2020.609006 ◽

2020 ◽

Vol 11 ◽

Author(s):

Ayaka Nobue ◽

Yoko Kunimasa ◽

Hiromu Tsuneishi ◽

Kanae Sano ◽

Hiroyuki Oda ◽

...

Keyword(s):

Conduction Velocity ◽

Nerve Conduction ◽

Action Potentials ◽

Electric Stimulation ◽

Nerve Conduction Velocity ◽

Lower Limbs ◽

Upper Limbs ◽

Cross Sectional ◽

Nerve Ultrasonography ◽

Human Nerve

This study aimed to simultaneously examine the differences of human nerve conduction velocity (NCV) and nerve cross-sectional area (nCSA) between the upper and lower limbs and between different regions of the upper and lower limbs. Thirty healthy subjects volunteered for the study. NCV and nCSA of the ulnar and tibial nerves were measured with the dominant and non-dominant arms and the supporting and reacting legs using supramaximal electric stimulation and peripheral nerve ultrasonography at three regions for ulnar and tibial nerves, respectively. Supramaximal electric stimulation was superficially applied to the ulnar and tibial nerves at each point. These action potentials were recorded from the digiti minimi and soleus muscles for the ulnar and tibial nerves, respectively. Our results clearly showed that the NCV, nCSA, and circumference of the ulnar and tibial nerves were higher and greater in the lower limbs than in the upper limbs. The greater the circumference, the greater the nCSA for both the upper and lower limbs. However, unlike the upper limbs, the supporting leg did not have higher NCV than the reacting leg despite its greater circumference. Therefore, nCSA can be related to the circumference but not necessarily function for NCV developments of the lower limbs. These various aspects between the upper and lower limbs suggest that NCV does not depend on the nCSA sizes or upper and lower limb circumference; the results indicate the existence of limb-specific NCV but not nCSA developments.

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Intraoperative compound muscle action potentials (CMAPs) amplitude changes after decompression and neurolysis of peripheral nerves in upper limbs neuropathies: electrophysiologial considerations and relation with clinical outcome

Journal of Neurosurgical Sciences ◽

10.23736/s0390-5616.18.04513-7 ◽

2021 ◽

Vol 65 (4) ◽

Author(s):

Ignazio G. VETRANO ◽

Roberto CORDELLA ◽

Irene TRAMACERE ◽

Vittoria NAZZI

Keyword(s):

Clinical Outcome ◽

Peripheral Nerves ◽

Action Potentials ◽

Upper Limbs ◽

Muscle Action ◽

Compound Muscle Action Potentials

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Poliomyelitis-Like Paralysis During Recovery From Acute Bronchial Asthma: Possible Etiology and Risk Factors

PEDIATRICS ◽

10.1542/peds.88.2.276 ◽

1991 ◽

Vol 88 (2) ◽

pp. 276-279

Author(s):

Eli M. Shahar ◽

Paul A. Hwang ◽

Charles E. Niesen ◽

E. Gordon Murphy

Keyword(s):

Bronchial Asthma ◽

Viral Disease ◽

Acute Onset ◽

Anterior Horn ◽

Lower Limbs ◽

Flaccid Paralysis ◽

Anterior Horn Cell ◽

Asthma Attack ◽

Anterior Horn Cell Disease ◽

Sensory Responses

A poliomyelitis-like paralytic disease during recovery from an attack of bronchial asthma is described in two young children. They presented at the age of 13 and 22 months, respectively, with acute flaccid paralysis of one or both lower limbs and preserved sensation. Cerebrospinal fluid examinations revealed mild protein elevation in both and pleocytosis in the second infant. Enteroviruses were isolated in a nasal swab and stools of the second patient. Acute onset of flaccid paralysis with absent motor action potential and normal sensory responses, detected by electrophysiologic studies, are highly suggestive of motor anterior horn cell disease in these infants. A multifactorial setup of immune suppression, stress, and neurotoxic drugs during an acute bronchial asthma attack triggered by a viral disease may render the patient vulnerable to viral invasion of the anterior horn cell with enteroviruses other than poliovirus. The overall experience of 22 patients with this serious complication is reviewed.

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Sensory Nerve Conduction Studies

Clinical Neurophysiology ◽

10.1093/med/9780190259631.003.0018 ◽

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.

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Lambert-Eaton syndrome without an identified neoplasm

10.5327/1516-3180.504 ◽

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.

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