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Biomedical subjects

A E de Jager

Publications and source records attributed to A E de Jager.

18 recordsLinked to original sources
PubMed1993

Anti-sulphatide antibodies in peripheral neuropathy.

A study was carried out on 135 patients with chronic idiopathic neuropathy (63), neuropathy associated with monoclonal gammopathy (51, including eight with anti-MAG antibody activity) and the Guillain-Barré syndrome (GBS) (21). Serum IgM, IgG and IgA anti-sulphatide antibody titres were compared with titres in 304 patients with other neurological or immunological diseases and in 50 normal subjects. Titres were presented a) as the highest serum dilution at which reactivity could be detected, and b) in the linear region of the optical density curve. A substantial number of patients with neurological or immunological diseases had higher titres than normal subjects. Compared with normal and disease controls, five patients with neuropathy associated with IgMk monoclonal gammopathy had raised titres of IgM anti-sulphatide antibodies and one patient with GBS had raised IgM, IgG and IgA anti-sulphatide antibodies in the acute phase of the disease. Two patients had a predominantly axonal sensory neuropathy with presenting symptoms of painful paresthesiae and minimal neurological deficit. Three patients had a predominantly demyelinating sensorimotor neuropathy associated with anti-MAG antibody activity. The patient with GBS had extensive sensory loss and antibody titres returned to normal within three weeks. Raised titres of anti-sulphatide antibodies occurred in several types of neuropathy, but all had some degree of sensory impairment and associated immunological abnormality.

Adult↗
PubMed1992

Anti-GM1 antibodies in patients with Guillain-Barré syndrome.

Anti-GM1 antibodies were measured in 22 patients with the Guillain-Barré syndrome (GBS) and compared with anti-GM1 antibody activity in patients with other neurological or immunological diseases and in normal subjects. Four out of 22 patients with GBS had raised IgM, IgG, or IgA anti-GM1 antibody activities. All four patients were tetraparetic with only minimal or no sensory deficit. Three of the patients had highly raised antibody activity and showed severe residual deficits, while of the remaining patients with GBS, only one remained severely affected. One patient had anti-GM1 antibodies specific for GM1, whereas the other three patients showed antibody activity with asialo-GM1 or GD1b. The presence of anti-GM1 antibodies may define a subgroup of patients with GBS who have a poor prognosis.

Adolescent↗
PubMed1991

Clinical signs in severe Guillain-Barré syndrome: analysis of 63 patients.

Clinical data are presented of 63 artificially ventilated Guillain-Barré patients. About half of them had an antecedent event. In 57% the disease was heralded by sensory symptoms. The mean progressive phase lasted 12 days, the plateau 12 days and the recovery phase 568 days. In all patients one or more cranial nerves were involved, most often leading to facial palsy or difficulties in swallowing. Three-quarters of the patients had sensory signs, proprioceptive more often than superficial. Autonomic disturbances were common, especially hypertension and tachycardia. Twenty-two percent of the patients were severely confused in the first weeks of the disease. Laboratory examination showed atypical lymphocytes in the blood of 37% of patients and disturbed hepatic function tests in 79%. CSF protein level was elevated in all patients, with a mean value of 1.5 g/l.

Adolescent↗
PubMed1991

Residual signs in severe Guillain-Barré syndrome: analysis of 57 patients.

The residual signs in a group of seriously paralyzed Guillain-Barré patients are reported (follow-up 2-24 years) and the social effects of the illness are discussed. Thirty-five percent of the patients had recovered completely at the time of the follow-up examination, 35% had minimal residual motor signs and 30% had a moderate or severe residual paresis. The most serious residua were found distally in the legs. Muscles innervated by nerves running along an entrapment site had the most severe deficits. Sensory signs were present in 49% of the patients. More than half of the patients considered themselves to be cured. The others were more or less handicapped by their sequelae. Of the adults, 60% resumed work, 25% found a less demanding job and 15% did not return to work. Statistically significant correlations were found between the degree of residual motor deficit and the severity of the weakness in the acute phase, the duration of the plateau phase or the duration of the artificial ventilation.

Activities of Daily Living↗
PubMed1988

Gradenigo's syndrome: a rare complication of otitis media.

A 58-year-old woman developed Gradenigo's syndrome. The clinical signs and symptoms of the otitis were rather atypical and there was a considerable delay before the correct diagnosis was made. The pathophysiology, therapy and differential diagnosis of this syndrome, which has become rare since the introduction of antibiotics, are discussed.

Abducens Nerve↗
PubMed1984

Capillary gas chromatographic determination of cholestanol/cholesterol ratio in biological fluids. Its potential usefulness for the follow-up of some liver diseases and its lack of specificity in diagnosing CTX (cerebrotendinous xanthomatosis).

The concentration ratios of cholestanol/cholesterol in biological materials (serum, cerebrospinal fluid and tendon biopsy) were determined using a capillary gas chromatographic method. The method was validated by gas chromatography-mass spectrometry. The ratio was determined in several groups of patients: (a) patients with cerebrotendinous xanthomatosis (in serum, cerebrospinal fluid and tendon biopsy), before and during chenodeoxycholic acid therapy, (b) patients receiving cholestyramine therapy (in serum), (c) patients suffering from various liver diseases (in serum) and (d) one patient before and after liver transplantation (in serum). It can be concluded that the cholestanol/cholesterol concentration ratio is a potentially useful parameter for monitoring liver diseases but is not specific for establishing the diagnosis of cerebrotendinous xanthomatosis.

Brain Diseases, Metabolic↗
PubMed1983

Diagnosis of cerebrotendinous xanthomatosis (CTX) and effect of chenodeoxycholic acid therapy by analysis of urine using capillary gas chromatography.

By means of capillary gas chromatography urine samples of patients with cerebrotendinous xanthomatosis (CTX) were investigated before and during treatment by oral administration of chenodeoxycholic acid. The occurrence of various conjugated bile alcohols, presumably glucuronides, was demonstrated, the major compound being 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 23 xi, 25-pentol. In the bile acid fraction norcholic acid and hydroxycholic acid were shown to be present in considerable amounts. In this way the presence of CTX can be demonstrated conclusively. After chenodeoxycholic acid therapy the excretion of both abnormal bile acids as well as of bile alcohols rapidly decreased within a few weeks, showing the effectiveness of the treatment. By early discovery and subsequent therapy it may be possible to prevent the onset of the detrimental symptoms such as mental deficiency, caused by the accumulation of cholestanol and cholesterol in CTX patients.

Bile Acids and Salts↗
PubMed1983

Acid maltase deficiency: treatment of respiratory insufficiency with cuirass respirator.

Three patients with the late-onset form of acid maltase deficiency showed a gradual weakening of proximal limb and trunk muscles leading to severe respiratory insufficiency. Considerable deterioration of pulmonary function occurred owing to a vicious cycle of hypoventilation and exhaustion. Treatment by nocturnal cuirass ventilation with tailor-made shells was successful. The muscle weakness still progressed very slowly but there were no more respiratory problems.

Adult↗
PubMed1983

Polyneuropathy and benign monoclonal gammopathy.

Recently the rather frequent occurrence of benign monoclonal gammopathy (BMG) has been reported in peripheral neuropathy. Sometimes this syndrome is part of a multisystemic disorder in which organomegaly, endocrine disturbances, skin changes and focal bone lesions may also occur. The clinical picture and the cerebro-spinal fluid findings resemble the chronic relapsing Guillain-Barré syndrome. The polyneuropathy seems to be of the primarily demyelinating type. The pathogenetic relationship with the gammopathy is as yet not clear, but treatment of the plasma cell dyscrasia has a favourable effect on the polyneuropathy. We report our experiences with 5 patients with polyneuropathy and BMG and compare our clinical, laboratory and histological data with the literature.

Adult↗
PubMed1981

Polyneuropathy after massive exposure to parathion.

Many organophosphorus compounds, including the organophosphate insecticides, may cause polyneuropathy of delayed onset. An exception is parathion, which has been considered the prototype of nonneurotoxic cholinesterase inhibitors. Nevertheless, we describe a patient with delayed polyneuropathy after suicidal ingestion of parathion.

Adult↗
PubMed1981

Plasma exchange in five patients with acute Guillain Barré syndrome.

During the last 3 years plasma exchanges were undertaken in 5 patients with acute Guillain Barré syndrome (G.B.S.). All the patients were admitted in the intensive respiratory care unit and had received six plasma exchange procedures over two weeks (each procedure consists of 2-3 L exchange). The first patient improved dramatically after the second exchange. Moderate success was obtained in two patients. One patient did not show any effect. The fifth patient received plasma exchange one day after her recovery phase had begun but the course of recovery remained uneffected. The effect of plasma exchange was analysed as the patients' response to motor activity, and compared with a historical control group consisting of 50 acute G.B.S. patients admitted in the intensive respiratory care area over the last 25 years. Plasma exchange does not seem to have exerted any significant effect although at any given time the plasma exchange group had higher motor activity than that of the control group. A controlled clinical trial especially in the early phase of the disease is emphasized.

Adult↗