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Biomedical subjects

H R Jones

Publications and source records attributed to H R Jones.

At least 55 records · Page 3Linked to original sources
PubMed1987

Intramuscular vitamin E repletion in children with chronic cholestasis.

Progressive spinocerebellar degeneration was identified in six children with chronic cholestatic liver disease and attributed to severe vitamin E deficiency. In addition to areflexia, ataxia, dysmetria, and diminished vibratory and position sense, three patients had pigmentary retinopathy. Abnormalities were present on electromyography, nerve conduction studies, and electroretinography. Because the vitamin E deficiency was not corrected by oral administration of massive doses of vitamin E, vitamin E was administered by the intramuscular route. With doses of 50 to 100 mg of vitamin E every three to seven days, over a 32-month interval (range, 15 to 44 months), vitamin E deficiency and abnormal red blood cell peroxide hemolysis were corrected. Other than discomfort and occasional edema at the site of injection, there were no side effects of parenteral vitamin E therapy. In several other studies intramuscular vitamin E therapy has produced significant neurologic improvement in patients with similar characteristics. In this study clinical progression of spinocerebellar degeneration was arrested but improvement could not be demonstrated despite adequate vitamin E replacement.

Adolescent↗
PubMed1986

Compressive neuropathy in childhood: a report of 14 cases.

During a 6-year period, 14 of 898 patients examined at a pediatric electromyographic laboratory had a compressive mononeuropathy. These included five injuries to the peroneal nerve, four to the sciatic nerve, two to the distal radial nerve, and one each to the deep palmar branch of the ulnar nerve, the musculocutaneous nerve, and the long thoracic nerve. A potentially preventable mechanism was identified in 9 of the 14 patients, including prolonged posture in three of the four sciatic and two of the peroneal neuropathies. Four other mononeuropathies were secondary to pressure from an orthopedic appliance or procedure (one injury to the musculocutaneous nerve, one to the long thoracic nerve, two to the peroneal nerve).

Adolescent↗
PubMed1986

Clinical value of electrodiagnostic studies in neuromuscular disorders.

EMG and conduction studies provide the physician with a precise means of defining the multiple diseases affecting the peripheral motor-sensory unit. These studies frequently provide clues that may be useful in arriving at the appropriate therapeutic decisions and in determining prognosis. Normal results may also support a suspected clinical diagnosis of inorganic illness, providing no evidence of central nervous system disease can be defined. Like any other test, however, results of EMG may be false-negative in bona fide neuromuscular disorders. This is particularly true early in a disease process; in neuropathies restricted primarily to small, unmyelinated nerve fibers; and in certain of the less virulent diseases of muscle and muscle energy metabolism.

Adult↗
PubMed1986

Eosinophilic fasciitis and the carpal tunnel syndrome.

Eosinophilic fasciitis is characterised by induration and inflammation of the fascia of the extremities. It is a nosologically distinct clinical entity typified by swelling, tenderness, and stiffness of the extremities associated with peripheral eosinophilia. Six of our eight patients with eosinophilic fasciitis seen in a 3 1/2 year period have had an associated carpal tunnel syndrome. The presenting complaints in three patients were those of carpal tunnel syndrome and in the other three patients were muscle aching, swelling, and tightness. Diagnosis was made by EMG and deep fascial biopsy. Although small doses of corticosteroid medications were helpful initially in five of the six patients, two patients later required surgical decompression.

Adult↗
PubMed1986

Unruptured intracranial aneurysm and transient focal cerebral ischemia: a follow-up study.

Transient focal cerebral ischemia may occasionally be due to distal embolization of a clot from an unruptured intracranial aneurysm. Follow-up data in 12 such patients revealed no ischemic strokes, subarachnoid or parenchymal hemorrhages in a mean follow-up period of 6.5 years. The aneurysms ranged in size from 2 to 12 mm in diameter, and only two were larger than 10 mm. Two patients had clip ligation of the aneurysm, five patients were given platelet antiaggregation therapy, one was given oral anticoagulants after aortic valve surgery, and four had no specific therapy. The prognosis for unruptured aneurysms presenting with transient focal ischemia was good, regardless of therapy.

Adult↗
PubMed1985

Ataxic Creutzfeldt-Jakob disease: diagnostic techniques and neuropathologic observations in early disease.

We studied two cases of ataxic Creutzfeldt-Jakob disease. EEG, CT, evoked responses, and CSF were normal in one purely ataxic patient. Diagnosis was established by cerebellar biopsy. Autopsy demonstrated devastating spongiform changes in the cerebellum, basal ganglia, and thalamus with rare focal changes in cerebral cortex. In the second patient, late generalized changes developed with dementia. Diagnostic studies included abnormal visual evoked responses, CSF with abnormal oligoclonal bands and IgG, and subacute spongiform encephalopathy in frontal lobe biopsy. Early diagnosis is best established by biopsy of brain areas most likely to be involved on the basis of clinical neurologic findings.

Ataxia↗
PubMed1983

Cerebral emboli of paradoxical origin.

A diagnosis of paradoxical cerebral embolus (PCE) was made in five patients aged 31 to 62 years who sustained eight cerebral ischemic events. No patient had evidence of primary carotid system or left heart disease. A probe-patent foramen ovale was the presumed mechanism in four patients, and an unsuspected congenital atrial septal defect was found in the fifth patient. Clinically apparent pulmonary emboli or venous thrombosis preceded the cerebral event in only one instance. Review of the literature reveals a high mortality with PCE. However, careful clinical search for this lesion may be rewarding: four of our five patients survived. One should consider PCE in any patient with cerebral embolus in whom there is no demonstrable left-sided circulatory source. This principle applies particularly if there is concomitant venous thrombosis, pulmonary embolism, or enhanced potential for venous thrombosis due to, for example, morbid obesity, use of hormonal birth control pills, prolonged bed rest (especially postoperatively), or systemic carcinoma.

Adult↗
PubMed1983

Idiopathic hemochromatosis (IHC): dementia and ataxia as presenting signs.

Idiopathic hemochromatosis (IHC) is a genetically determined impairment in control of iron absorption that results in excessive parenchymal iron deposition, particularly in the liver. Of patients with IHC, 50% have little or no chemical evidence of liver dysfunction. Cirrhosis may be clinically occult, but still cause a syndrome of chronic hepatocerebral degeneration. Two patients are reported with IHC and a syndrome of ataxia, rigidity, myoclonic jerks, and dementia. Other associated symptoms may include diminished libido, decreased hearing, peripheral neuropathy, and large joint disease. Because symptoms of IHC can be reversed by phlebotomy, appropriate laboratory studies should be considered to exclude IHC in any patient with unexplained dementia, encephalopathy, and gait ataxia.

Aged↗
PubMed1982

Primary cerebellopontine progressive multifocal leukoencephalopathy diagnosed premortem by cerebellar biopsy.

A subacute progressive cerebellar brainstem syndrome developed in a patient with systemic lupus erythematosus in remission. Cerebellar biopsy documented the diagnosis of progressive multifocal leukoencephalopathy (PML). Data from this patient and 10 others in the literature emphasize the need to consider this diagnosis when ataxia develops in any patient with underlying malignancy, chronic infection, or other disease that involves immunological incompetence. Although the ataxic form of PML is not of nosological relevance, early diagnosis may eventually have therapeutic importance.

Biopsy↗
PubMed1979

Disease of the vertebral basilar system.

Patients with acute bilateral, crossed, or multisystem neuroanatomic symptoms associated with cranial nerve palsies may have vertebral basilar system disease. Surgically correctable lesions are usually not found in these patients, and medical therapy with short-term anticoagulation or antiplatelet agglutination may be prescribed in those patients with transient ischemic attacks or progressing stroke.

Aged↗