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Biomedical subjects

H J Oosterhuis

Publications and source records attributed to H J Oosterhuis.

At least 19 recordsLinked to original sources

[Physical diagnosis--signs of lumbosacral irritation].

Lasègue's test is a clinical test to demonstrate lumbosacral radicular irritation. Published evidence gives a high sensitivity (0.80-0.97) for a low lumbar disc protrusion but a low specificity (about 0.4) since this test is also positive in ischialgia due to other causes. A crossed Lasègue test had a high specificity (> or = 0.90) for presence of a (ruptured) disc prolapse. There is little or no literature about the reversed Lasègue and provocation by intra-abdominal pressure. The reproducibility of the Lasègue test is moderate (kappa about 0.55).

Female↗

[A series of articles on the usefulness and uselessness of physical diagnostics; comment].

The sensitivity, specificity and reproducibility (especially the interassessor variation) of many physical diagnostic tests taught in the medical curricula appear not to have been established. Nevertheless, many of these tests are mentioned as end terms in the General plan 1994 medical education. A series of articles in this journal will be concerned with tests from physical diagnostics, with reference to usefulness and uselessness: sensitivity, specificity and reproducibility.

Curriculum↗

[Favorable results of plasmapheresis in severe myasthenia gravis].

OBJECTIVE: Evaluation of the additional effect of plasma exchange in treatment with steroids of severe myasthenia gravis. DESIGN: Retrospective study. SETTING: Department of Neurology, University Hospital of Groningen, the Netherlands. PATIENTS AND METHODS: The clinical course was analysed in 24 patients with a total of 28 plasma exchange treatments. Prednisone was introduced or its maintenance dose was increased in 19 cases. Another 13 patients with severe myasthenia gravis were included to study the effect of prednisone monotherapy. RESULTS: Of the patients treated with plasma exchange and steroids 71% improved within the first week of treatment as against only 15% of the patients treated with prednisone monotherapy. After two weeks of plasma exchange plus prednisone or prednisone monotherapy, 88% and 69%, respectively were ameliorated. None of the patients receiving plasma exchange deteriorated or experienced adverse effects or complications due to plasma exchange. Of the patients receiving prednisone monotherapy 31% deteriorated in the first week of treatment. CONCLUSION: Patients with severe myasthenia gravis may safely be treated with plasma exchange in combination with prednisone. In our patient group, a state of rapid aggravation was cut short more quickly by prednisone plus plasma exchange than by prednisone monotherapy.

Adolescent↗

Myasthenia gravis: diagnosis and follow-up of 100 consecutive patients.

One hundred consecutive patients with myasthenia gravis (MG) referred between 1985 and 1989 were analysed for epidemiological characteristics, evolution of early signs, delay in diagnosis, yield of diagnostic tests and effects of treatment. The female to male ratio was 1.6:1.0. Sixteen patients had a thymoma. Ocular MG occurred in 14. Associated autoimmune diseases were found in 15 patients. In 34% of the women and 10% of the men the diagnosis was delayed for more than 2 years. In the first 3 months progression was more rapid in men than in women. Anti-acetylcholine receptor antibodies were found in 94% of the patients with generalized MG and in 29% of the ocular patients. The neostigmine or the edrophonium test was positive in 84% of the generalized and in 60% of the ocular patients. Electromyography was diagnostic in 71% of the generalized and in 42% of the ocular patients tested. Thymectomy was performed in 56 patients (12 with thymomas). Fifty-one per cent were treated with one or more immunosuppressive drugs, at any time. After a mean follow-up of 9.6 years after onset remissions had occurred in 43%, considerable improvement in 25%, moderate improvement in 20% and 12% remained unchanged. There were no deaths due to MG. Thirty-six per cent remained dependent on immunosuppressive drugs. Medication-free remission was most frequent (35%) in the early-onset (< 50 years) group. Side-effects of pyridostigmine were noted in 34% of 99 patients, of prednisone in 65% of 49 patients, and of azathioprine in 54% of 28 patients, but these necessitated stopping the drug in only 1%, 10% and 14% respectively.

Age of Onset↗

[Quantification and evaluation of 5 neurological equilibrium tests in test subjects and patients].

OBJECTIVE: Quantification and evaluation of five neurological balance tests as part of the routine neurological examination in order to obtain objective test results for healthy individuals and several neurological patient groups. SETTING: Department of neurology, University Hospital Groningen, the Netherlands. DESIGN: Prospective, controlled study. METHODS: 220 subjects (113 healthy individuals and 107 neurological patients) participated in a balance test investigation comprising gang board, Romberg's test, tandem gait, one-leg-stance, hopping. Tests were performed on a hopscotch (3 meters long, divided in 6 rectangles of 20 by 50 cm) and using a stopwatch to register times and faults. RESULTS: All five quantified tests were feasible for healthy individuals. Up to the age of 70 results gradually declined, above 70 a strong decline was observed. Patients with gait and balance disorders performed less well in all tests except the Romberg's test. CONCLUSIONS: Taking into account a strict standardisation of these five existing tests standard values were obtained for a fast and simple quantification of balance in the routine neurological examination, which can also be used to monitor progress. Especially the results obtained in tandem gait, one-leg-stance and hopping differ significantly between healthy individuals and patients. The gang board appears to be useful for quantifying serious balance disorders. Romberg's test is a specific test for the perceptual sensibility and does not belong in the routine neurological examination of balance.

Adult↗

Acquired blepharoptosis.

A review is given of the aetiology and possible treatment of acquired (non-congenital), blepharoptosis, which is a common but not specific sign of neurological disease. The diagnostic categories of upper eyelid drooping are scheduled as (a) pseudo-ptosis due to a local process or overactivity of eye closure, including blepharospasm, and (b) true ptosis due to a paresis of the eyelid levators (m. tarsalis superior or m. levator palpebrae) or to a disinsertion of the m. levator palpebrae (aponeurotic ptosis). A paresis of the m. tarsalis is due to a lesion in the central, intermediate or peripheral neuron of the sympathetic chain and constitutes one of the components of Horner's syndrome. A paresis of the m. levator palpebrae may be due to a failure in central innervation, in oculomotor (n.III) function, in neuromuscular transmission or to a lesion in the muscle itself.

Blepharoptosis↗

The clinical spectrum of limb girdle muscular dystrophy. A survey in The Netherlands.

A cross-sectional study was performed in the Netherlands to define the clinical characteristics of the various subtypes within the broad and heterogeneous entity of limb girdle muscular dystrophy (LGMD). An attempt was made to include all known cases of LGMD in the Netherlands. Out of the reported 200 patients, 105 who fulfilled strictly defined criteria were included. Forty-nine patients, mostly suffering from dystrophinopathies and facioscapulohumeral muscular dystrophy, appeared to be misdiagnosed. Thirty-four cases were sporadic, 42 patients came from autosomal recessive and 29 from autosomal dominant families. The estimated prevalence of LGMD in the Netherlands was at least 8.1 x 10(-6). The clinical features of the autosomal recessive and sporadic cases were indistinguishable from those of the autosomal dominant patients, although calf hypertrophy was seen more frequently, and the course of the disease was more severe in autosomal recessive and sporadic cases. The pectoralis, iliopsoas and gluteal muscles, hip adductors and hamstrings were the most affected muscles. Distal muscle involvement occurred late in the course of the disease. Facial weakness was a rare phenomenon. The severity of the clinical picture was correlated with a deteriorating lung function. All autosomal dominantly inherited cases showed a mild course, although in two families life-expectancy was reduced because of concomitant cardiac involvement.

Adolescent↗

[Use of alternative treatments by patients with myasthenia gravis].

OBJECTIVE: To analyse the use of alternative treatments by patients before and after myasthenia gravis was diagnosed, and the influence on the diagnostic delay. DESIGN: Retrospective study. SETTING: University Hospital Groningen, Netherlands. METHOD: A questionnaire was sent to 90 consecutive patients with myasthenia gravis to inquire about their use of alternative treatments. RESULTS: Of the 72 respondents 18 had used alternative treatments. Of these 11 had even done so before diagnosis. The most important reason for the use was the lack of understanding and trust that these patients said they experienced from their regular doctors. Almost all patients discontinued alternative treatment when it became clear that it had no effect. Compared with those not using alternatives, the diagnosis was delayed in the group of patients using alternative treatments, but not significantly. CONCLUSION: More attention for patients with chronic and undefined complaints may shorten the delay in diagnosing and treating myasthenia gravis adequately.

Adolescent↗

[Unterberger's test not useful in testing of vesitibular function].

OBJECTIVE: Quantification of the stepping test in routine neurological examination. SETTING: University Hospital Groningen, the Netherlands. DESIGN: Prospective, controlled study. METHODS: The stepping test was assessed in 41 healthy subjects, in 7 subjects with artificially induced vertigo and in 83 patients with disturbances of gait or equilibrium resulting from different affections of the central nervous system. Displacement and rotation to the right or the left side were measured. The different groups were then compared with each other. RESULTS: We found a great inter- and intra-individual variation in direction and width of rotation and in displacement. The results of the test are not influenced by right(+)-or left(-)-handedness or by dominance of the right or the left leg. CONCLUSION: The current test does not appear to be useful for detection of abnormalities in the vestibular system or for distinguishing normal individuals from patients.

Caloric Tests↗

Familial hypokalemic periodic paralysis. Clinical, diagnostic and therapeutic aspects.

Five generations of a family with hypokalemic periodic paralysis (HOPP) were studied. Of the 120 screened family members, 64 were found to have HOPP of which 38 were suffering from attacks. In the other 26 the diagnosis was made on account of vacuolar myopathy, a reduced muscle fiber conduction velocity or the signs of permanent muscle weakness (PMW) in combination with (grand)children with attacks. Applying these criteria skipping of generations did not occur in this family. When defined properly, PMW was found in all patients at older age, independent of the previous occurrence of paralytic attacks. Dynamometry and muscle CT-scanning appeared valuable in the diagnosis and the progression of PMW. In 2 patients autopsy was performed. Characteristics vacuolization was found in the striated muscle tissue in various degrees. Heart and smooth muscle tissue were not involved. Therapy is limited. Potassium salts shortening and preventing the paralytic attacks are tolerated well. Acetazolamide is more effective in the prevention of the paralytic attacks, but is not tolerated very well. HOPP can be considered as a myopathy characterized by PMW at older age in all patients, combined with paralytic attacks in more than half the patients.

Acetazolamide↗

Antibodies to skeletal muscle in myasthenia gravis. Part 3. Relation with clinical course and therapy.

Fluctuations of anti skeletal muscle antibodies (AMA) were studied in relation to clinical changes and fluctuations in anti acetylcholine receptor antibodies (a-AChR). Forty-two patients with generalized myasthenia gravis were studied in clinical and serological follow-up during several years under various conditions. Results from this study demonstrate that AMA fluctuate in strong relation to a-AChR, clinical course and immunosuppressive therapy. Thymomectomy resulted in an increase or de novo appearance of AMA in 10 of the 12 patients who did not receive immunosuppressive medication.

Adult↗

Antibodies to skeletal muscle in myasthenia gravis. Part 2. Prevalence in non-thymoma patients.

Anti-skeletal muscle antibodies (AMA) were measured in 340 non-thymoma patients with myasthenia gravis (MG) by both an ELISA with citric acid muscle extract and by immunofluorescence assay. Three a-AChR negative but AMA-positive patients were found. The occurrence of AMA was related to the age at onset in patients with an onset of MG beyond 40 years and with duration of disease in patients with an earlier onset of MG. This stresses the heterogeneity of late onset and early onset MG. Serial measurements in individual patients revealed no evidence for a thymic role in the generation of AMA. There was no difference in specificity of AMA in early and late onset patients. The presence of AMA in CT-negative patients is not necessarily suggestive of a radiological shortcoming in the detection of thymoma since in 16 AMA-positive patients from our series no thymoma was found at operation or obduction.

Adolescent↗