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Biomedical subjects

H R Jones

Publications and source records attributed to H R Jones.

At least 37 records · Page 2Linked to original sources
PubMed1994

Pediatric median mononeuropathies: a clinical and electromyographic study.

Seventeen children, 6 girls and 11 boys, aged 5-17 years with pediatric median mononeuropathies (PMM) were identified among 1809 who had EMGs primarily in the electromyographic laboratory at The Children's Hospital, Boston, between 1979 and 1993. Electromyography documented the PMM to be at the wrist in 7 children, including 3 children with idiopathic carpal tunnel syndrome (CTS)--1 whose symptoms were accentuated by skiing--2 with a systemic illness (mucolipidosis III and scleroderma), and in 1 child each the distal PMM was secondary to a cast or laceration. A proximal PMM was identified in 10 children, including 8 with trauma, 1 with an osteoid osteoma, and 1 with juvenile cutaneous mucinosis. Five children (3 with CTS and 1 each with mucolipidosis III and juvenile cutaneous mucinosis) had bilateral disease. The localization (59% proximal) and cause of these PMMs differed greatly from our experience with adult median neuropathies.

Adolescent↗
PubMed1993

Pediatric peroneal mononeuropathy: a clinical and electromyographic study.

Seventeen children with pediatric peroneal mononeuropathies evaluated between 1979 and 1991 are reported. Twelve boys and 5 girls, ranging in age from 1.5 months to 17 years, were referred for footdrop in 16 children (94%) or for lower extremity pain in 1 child (6%). Causes included compression in 10 children (59%), trauma in 3 children (18%), entrapment in 3 children (18%), and indeterminate in 1 child (5%). Based on nerve conduction studies and electromyography, the level of the pediatric peroneal mononeuropathic lesion was the common peroneal nerve in 10 children (59%), the deep peroneal nerve in 2 children (12%), and the superficial peroneal nerve in 1 child (5%). In 4 other children (24%), pediatric peroneal mononeuropathy at the knee was not more precisely identified. Surgical exploration in 3 children with progressive pediatric peroneal mononeuropathy was valuable. Improvement occurred in 13 of 17 children (76%).

Action Potentials↗
PubMed1992

Proximal median neuropathies: electromyographic and clinical correlation.

Results of electrophysiologic and clinical findings in 17 patients with proximal median neuropathy were reviewed. The cause of neuropathy was trauma in 5 patients, overuse of the pronator teres in 3 patients, postinfectious in 2 patients, secondary to a congenital lesion in 1 patient, and undetermined in 6 patients. The neuropathy involved the main branch of the median nerve at or proximal to the pronator teres muscle (high median neuropathy) in 14 patients, and the anterior interosseous portion of the nerve in 3 patients. Electrophysiologic findings, especially needle electromyography (EMG), were more definitive than findings expected from clinical examinations. EMG and operative findings demonstrated that median nerve compression by the pronator teres produces denervation of this muscle as well as distal muscles. EMG cannot differentiate a median nerve lesion at the pronator teres from a more proximal lesion. Follow-up data were available in 7 of 10 nonsurgically managed patients, and in 6 of 7 patients with surgical decompression. Six patients in each group were either improved or normal.

Adult↗
PubMed1992

Guillain-Barré syndrome in children: clinical course, electrodiagnosis, and prognosis.

The electrodiagnostic features of acute childhood Guillain-Barré syndrome (GBS) have not been distinguished from those in the adult. We report nerve conduction and electromyographic data from 23 children. Sixty-one percent (14 of 23) fulfilled strict electrodiagnostic criteria for a demyelinating neuropathy, and the remainder demonstrated demyelination in at least one nerve. Reduced compound muscle action potential (CMAP) amplitude was the most common finding overall. Children less than 10 years old demonstrated significantly greater slowing of motor CV than children greater than 10 years old. Electrodiagnostic criteria associated with poor outcome (low mean CMAP and fibrillation potentials) in previous studies, primarily of adult patients, occurred in 39% (9 of 23 children). All patients on whom follow-up data were obtained recovered without residual disability. We conclude that electrodiagnostic prognostic indicators identified in general series of GBS may not apply to children.

Adolescent↗
PubMed1992

McArdle's disease with late-onset symptoms: case report and review of the literature.

McArdle's disease with late-onset symptoms is an unusual cause for muscle disease in older patients. The case of a patient with McArdle's disease whose symptoms began at 60 years of age is presented, and seven previous cases of late-onset McArdle's disease reported since 1963 are discussed. In five of the eight patients, the clinical presentation was similar to the early onset disorder with exercise intolerance, cramps, and myoglobinuria. In contrast, the remaining three patients presented with fixed proximal limb and bulbar weakness. Electromyography confirmed a myopathic process in four of four patients. Results of the forearm ischaemic exercise test were positive in seven of seven patients with complete myophosphorylase deficiency; results of muscle biopsies were diagnostic in all patients. McArdle's disease with late-onset symptoms is rare and clinically more variable than the early onset disorder.

Aged↗
PubMed1991

Extracranial cerebrovascular disease.

The prevention, management, and prognosis of patients with transient ischemic attack or stroke caused by extracranial cerebrovascular disease depend on the underlying pathophysiologic mechanisms involved. Atherosclerotic vascular disease is the predominant pathophysiologic mechanism. Management of this entity remains controversial and should be individualized. Recent data have clearly confirmed that carotid endarterectomy is better than medical therapy. Patients with a critically tight carotid stenosis appropriate in location to their symptoms are considered for carotid endarterectomy. This procedure should be performed only in the setting of excellent neuroradiologic support and surgical expertise, however, with a cumulative complication risk of less than 3%. Intimal dissection appears to be associated with a good prognosis with or without treatment. However, patients with symptoms should receive short-term therapy with antiplatelet or anticoagulant agents to prevent distal embolization. Corticosteroids are the drugs of choice for treatment of patients with extracranial arteritis. The presence of fibromuscular dysplasia in the cerebrovascular system has not proved to be a definitive risk for stroke or transient ischemic attack.

Aged↗
PubMed1990

EMG evaluation of the floppy infant: differential diagnosis and technical aspects.

Electromyographic examination of the newborn and young infant provides a relatively uncommon challenge to most electromyographers. The usual reason for referral for electromyographic studies in the newborn and young infant is to evaluate a floppy baby. The electromyographer must not only be aware of important differences in normal physiologic parameters but must also be familiar with a spectrum of diseases that are not typically encountered in the adult. The results of electromyography must also be correlated with the normal maturation of neuromuscular function. Although the most common pathophysiologic mechanisms affecting the peripheral motor unit are infantile motor neuron disease and the congenital myopathies, a large number of other disease entities warrant careful consideration.

Diagnosis, Differential↗
PubMed1989

Neurological manifestations of infective endocarditis. Review of clinical and therapeutic challenges.

The nervous system is frequently involved in patients with infective endocarditis. When a careful review of presenting complaints is undertaken, neurological symptoms have been found in as high as 29% of patients. Because these manifestations may be so protean in nature, for example, stroke or transient ischaemic attack (the most common), toxic encephalopathy, meningitis, brain abscess, visual loss, seizures, headache, backache, or acute mononeuropathy, the neurologist needs to consider infective endocarditis as a possible diagnosis in many patients. During the past two decades, infective endocarditis has occurred in an ever widening clinical setting. It may often be found in persons unknown to have predisposing cardiac disease. This is particularly true in certain subsets of the population, including the elderly, patients subjected to various invasive procedures leading to nosocomial infection, and drug abusers. New diagnostic studies, including refined bacteriological culture techniques, echocardiography, computed tomography, magnetic resonance imaging, and greater availability of skillful cerebral angiography, make earlier diagnosis of infective endocarditis possible. Despite this, patients with neurological complications continue to have an uncertain prognosis.

Acute Disease↗
PubMed1988

Acute fulminating fatal leukoencephalopathy as the only manifestation of human immunodeficiency virus infection.

A case of acute human immunodeficiency virus (HIV) infection manifested by a rapidly fulminating, necrotizing, demyelinating encephalopathy that led to brain death in 5 days is reported. Autopsy demonstrated predominant white matter lesions, acute neuronal damage, and scanty cellular response. Cultures of cerebrospinal fluid were positive for HIV, suggesting an acute infection.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Spontaneous reinnervation of a free muscle flap.

A latissimus dorsi muscle flap was used to repair a severe traumatic avulsion defect of the dorsum of the foot in a 3-year-old girl. The severed peroneal nerve apparently regenerated across a large gap and spontaneously reinnervated the denervated muscle flap. This resulted in a functional flap as demonstrated clinically and electromyographically. Surgical methods of muscle reinnervation and the influence of neurotrophic factors are discussed.

Child, Preschool↗
PubMed1988

Bilateral hypoglossal nerve injury after bilateral carotid endarterectomy.

A case of severe bilateral injury to the hypoglossal nerves after two-stage carotid endarterectomy is described. Injury to the hypoglossal nerve occurs in up to 20% of patients undergoing carotid endarterectomy and may result in mild or unnoticed deficits. These injuries must be carefully searched for in patients who will undergo a similar procedure on the opposite side since a bilateral deficit of the hypoglossal nerve is poorly tolerated, causing potentially serious impairment of speech and risk of aspiration.

Aged↗
PubMed1988

Sciatic neuropathies in childhood: a report of ten cases and review of the literature.

Primary lesions affecting the sciatic nerve are uncommon, especially in children. Isolated sciatic nerve involvement was found in ten patients during an 8 1/2-year period at a metropolitan children's hospital. Etiologic mechanisms included three with compression and one each with stretch injury after operation using the lithotomy position, stretch injury after closed reduction of hip dislocation, puncture wound, lymphoma, hypersensitivity vasculitis associated with hypereosinophilia, indeterminate lesion associated with transverse myelitis, and idiopathic progressive lesion with negative findings on exploration. Neonatal injuries associated with breech delivery or intragluteal injections were not causative factors in this series. Children with sciatic neuropathies have a variable prognosis depending on the etiology. Compression was the only potentially preventable pathophysiologic mechanism.

Adolescent↗