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Biomedical subjects

A E de Jager

Publications and source records attributed to A E de Jager.

At least 19 recordsLinked to original sources
PubMed2001

Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findings.

OBJECTIVE: To describe the clinical, neurophysiological, and MRI findings in 10 patients with primary lateral sclerosis (PLS). RESULTS: The course of the disease was very slowly progressive. Spasticity due to upper motor neuron dysfunction was the most prominent sign, but EMG showed slight lower motor neuron signs, such as a mixed pattern on maximal voluntary contraction and enlarged motor unit potentials. One patient had clinically mild lower motor neuron involvement. Central motor conduction times (CMCT) were more prolonged in PLS than is the case in ALS. Minor sensory signs were found on neurophysiological examination, comparable with those in ALS. In four patients serum creatine kinase activity was raised. On MRI cortical atrophy was seen, most pronounced in the precentral gyrus and expanding into the parietal-occipital region. CONCLUSIONS: PLS is a distinct clinical syndrome, part of the range of motor neuron diseases. Besides pronounced upper motor neuron symptoms, mild lower motor neuron symptoms can also be found, as well as (subclinical) sensory symptoms. PLS can be distinguished from ALS by its slow clinical course, a severely prolonged MEP, and a more extensive focal cortical atrophy.

Adult↗
PubMed2000

Comparison of serum S-100 protein levels following stroke and traumatic brain injury.

Temporal changes in serum S-100 protein levels were compared between patients with ischemic stroke, transient ischemic attack (TIA) and traumatic brain injury (TBI). In addition, S-100 levels were correlated with clinical severity and outcome. Measurements were done with a LIA-mat((R)) Sangtec((R)) 100 using an automated immunoluminometric assay. Serum S-100 was measured in 21 stroke patients, 18 TIA patients and ten TBI patients on days 1 (0-24 h), 2, 3, 4, 5 or 6 and 8 or 9. In a control group of 28 healthy volunteers one measurement was done. For the stroke and TIA patients, National Institutes of Health Stroke Scale (NIHSS) scores were obtained on admission and on day 10. For the TBI patients, Glasgow Coma Scale (GCS) scores were obtained on admission and Glasgow Outcome Scale (GOS) scores were obtained after 6 months. Changes in serum S-100 levels over the first 3 days were significantly different between stroke and TBI patients (P=0.014) and between stroke and TIA patients (P=0.006). Peak concentrations of S-100 were most often observed on day 3 or 4 after stroke and on day 1 or 2 after TBI. In the stroke patients individual S-100 peak levels correlated well with the NIHSS score on admission (r=0.58 P=0.014) and the change in NIHSS score between day 10 and day 1 (r=0.65, P=0. 005). In the TBI patients a good correlation between individual peak levels of S-100 and the GCS score on admission (r=-0.81, P=0.010) and the GOS score 6 months after the trauma was found (r=-0.87, P=0. 004). We conclude that there is a significant difference in temporal changes of S-100 levels between ischemic stroke and TBI patients. This suggests different pathophysiological mechanisms. The results of this study further confirm that peak levels of serum S-100 correlate with neurological deficit resulting from either stroke or TBI.

Adult↗
PubMed1999

Residual physical outcome and daily living 3 to 6 years after Guillain-Barré syndrome.

Three to six years after onset of Guillain-Barré syndrome, 63% of 122 patients showed one or more changes in their lifestyle, work, or leisure activities, or in the life of their partners. The changes were influenced by an impaired final functional outcome, along with loss of power and poor condition, although physically recovered patients showed these changes as well.

Activities of Daily Living↗
PubMed1999

Neurone-specific enolase and N-acetyl-aspartate as potential peripheral markers of ischaemic stroke.

BACKGROUND: After stroke, brain-specific proteins (including neurone-specific enolase) leak into the blood. The question addressed in the present study was whether N-acetyl-aspartate (amino acid derivative localized in cerebral neurones) could also serve as a peripheral marker of ischaemic damage. N-acetyl-aspartate levels were determined in the blood of stroke patients and related to clinical outcome, volume of infarction and to serum neurone-specific enolase. METHODS: Blood samples from 19 patients (seven women, 12 men, mean age of 73 years, range 56-88 years) were collected during the first 4 days after stroke and analysed for neurone-specific enolase (radioimmunoassay) and/or N-acetyl-aspartate (mass spectrometry). Clinical outcome was assessed using the Glasgow Outcome Score, and volume of infarction was calculated using computerized tomography (CT). Control values of N-acetyl-aspartate, determined in six female and nine male volunteers (mean age 47.4 years; range 28-73 years) were 0.26 +/- 0.02 mumol L-1. RESULTS: The increase in serum N-acetyl-aspartate was highly significant (P < 0.0001) within the first 24 h and at 72 h after stroke and correlated (P < 0.05) with volume of infarction only in patients with a bad prognosis (Glasgow Outcome Score < 5). Serum N-acetyl-aspartate at 24 h and neurone-specific enolase at 72 h were negatively correlated, suggesting that more N-acetyl-aspartate reaches the blood when brain tissue is less irreversibly affected. CONCLUSION: Serum N-acetyl-aspartate appears to be an early peripheral marker of ischaemically affected brain neurones, and the ratio of N-acetyl-aspartate to a protein marker, such as NSE, may serve as an index of irreversibility.

Aged↗
PubMed1998

Ophthalmoplegic and lower cranial nerve variants merge into each other and into classical Guillain-Barré syndrome.

We delineated the place of cranial nerve variants within the concept of clinically defined Guillain-Barre syndrome (GBS). In the ophthalmoplegic variant (n = 7) the oculomotor nerves were early involved. In a lower cranial nerve variant (n = 9) the cranial nerves IX, X, and XI were early involved. During progression considerable overlap occurred between these two variants, but also with the classically ascending variant of clinically defined GBS. These findings indicate common immune mechanisms in all GBS variants.

Adolescent↗
PubMed1998

Muscle computed tomography in adult-onset acid maltase deficiency.

Nine patients with adult-onset acid maltase deficiency (Pompe's disease) were examined clinically and with computed tomography (CT). The CT scan showed early and severe involvement of the muscles of trunk and thighs, with selective sparing of the tensor fasciae latae, short head of biceps femoris, gracilis, and sartorius muscles. Shoulder and leg muscles were less affected. The disease spread over the years from trunk to extremities. Muscle strength and CT findings were positively correlated.

Adult↗
PubMed1998

Rhesus anti-D immunoglobulin in chronic autoimmune neuropathy.

OBJECTIVE: To investigate the effect of Rhesus anti-D immunoglobulin (anti-D) in patients with an autoimmune demyelinating neuropathy. MATERIAL AND METHODS: Three patients with an autoimmune mediated neuropathy received 1000 IU anti-D weekly for 2 months. RESULTS: Two patients worsened gradually during the treatment and 1 remained unchanged. CONCLUSION: Rhesus anti-D immunoglobulin has no beneficial effect in patients with autoimmune neuropathy.

Adult↗
PubMed1998

Painful Horner syndrome due to arteritis of the internal carotid artery.

A 44-year-old man presented with painful Horner syndrome: severe periorbital pain, ptosis, and miosis of his right eye, with intact facial sweating. Lymphadenitis at the right side of his neck preceded the symptoms. MRI and magnetic resonance angiography showed thickening of the right internal carotid artery, extending from the bifurcation to the cavernous sinus, without evidence for dissection. The patient was treated with corticosteroids with immediate improvement. Control MRI scanning was normal after 6 weeks. We conclude that the painful Horner syndrome was caused by a reactive arteritis of the right internal carotid artery.

Adult↗
PubMed1997

Vocational perspectives and neuromuscular disorders.

The present study analyses the actual occupational situation, vocational handicaps and past labour career of a group of about 1000 Dutch patients suffering from a neuromuscular disorder (NMD). On the basis of the likelihood of a substantial employment history and sufficient numbers of patients, four types of NMD were selected: dystrophia myotonica (DM), hereditary motor and sensory neuropathy, (HMSN), spinal muscular atrophy (SMA) and myasthenia gravis (MG). Results show that a labour career is in reach of most NMD patients, even for those with severe limitations. It is concluded that physical limitations seem not to be decisive in that respect. The loss of the quality of communication, the loss of mental abilities and the effect of the diseases on the facial expression, as with some DM patients, are also important for chances on the labour market. Though the labour participation of NMD patients tends to decrease after the age of 34, the availability of work adaptations makes it possible to prolong the labour career. Analysis of the actual work situation of NMD patients shows that both disorder-related limitations and work characteristics play an important role in the amount of physical work problems encountered. It is argued that physical labour has to be regarded as generally unsuitable for NMD patients. This has implications for the sort and level of education to be attained by NMD patients. Career counselling as a focus point for the choice of an educational programme may improve labour market opportunities as well as quality of employment of NMD patients. Allowing for and accepting the possible effects of the disorder in the work situation are considered to be important in respect to labour participation and work satisfaction of workers with NMD. Reducing time pressure demands and increasing the freedom to organize one's work, are measures to be given especial consideration.

Adult↗
PubMed1997

Residual health status after Guillain-Barré syndrome.

To study the extent to which patients experience residual problems in daily functioning several years after having Guillain-Barré syndrome (GBS) a survey of 123 patients who had had Guillain-Barré syndrome three to six years previously was performed, using the sickness impact profile (SIP) for measuring functional health status and a functional assessment scale (F score) for measuring physical condition. The patients were diagnosed according to the international criteria for Guillain-Barré syndrome and were at the time of diagnosis unable to walk more than 10 metres without support. The physical SIP score correlated positively with final physical recovery (Pearson's r = 0.79). The psychosocial SIP score indicated impairment in all patient groups compared with matched normal control values; they included the group with no, or mild, residual symptoms (P < 0.05). No relation was found between clinical variables related to the severity or duration of Guillain-Barré syndrome and residual psychosocial dysfunctioning, except for a relation with disturbance of sensation in the arms. In conclusion, in many patients with Guillain-Barré syndrome, psychosocial functioning is still seriously affected, even when they have physically recovered, or show only mild residual signs.

Adult↗
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↗