Subacute painful quadriplegia and purpura.
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Fatal Outcome↗
Biomedical subjects
M F van der Meulen
Publications and source records attributed to M F van der Meulen.
[Brain tumor or stroke?].
Sometimes, the clinical presentation of a brain tumour mimics that of stroke or vice versa, as exemplified in the following three patients. In a 73-year-old patient the initial clinical picture was compatible with a brachial plexus lesion, as the weakness in his right hand appeared to have a traumatic, and not a central nervous system related, cause. When he experienced a focal seizure, the CT scan of the brain revealed a lesion in the motor cortex. This was presumed to be an infarction due to the lack of mass effect and the absence of contrast enhancement. Shortly afterwards the patient deteriorated and a follow-up scan revealed a large contrast-enhancing lesion. During surgery this proved to be a glioblastoma multiforme. A 76-year-old man was suffering from a progressive neurological deficit. An MRI scan of the brain revealed a contrast-enhancing lesion and a chest X-ray revealed an asymptomatic lung tumour; the diagnosis 'brain metastasis' was made. The surgeon removed the lung tumour, which proved to be a carcinoma. Later, when the patient was referred to the neurosurgeon for extirpation of the presumed brain metastasis, the MRI scan revealed that the lesion had decreased in size and no longer exhibited contrast enhancement. The metastasis proved to be an infarction. A 53-year-old man presented with sudden loss of consciousness due to a haemorrhage in the occipital lobe. An angiogram did not reveal a vascular malformation and during surgery no abnormal tissue was seen. The patient almost made a complete recovery. However, several months later he developed an elevated intracranial pressure due to a large occipital high-grade glioma, which had caused the original haemorrhage.
Aged↗
Rimmed vacuoles and the added value of SMI-31 staining in diagnosing sporadic inclusion body myositis.
Problems in diagnosing sporadic inclusion body myositis may arise if all clinical features fit a diagnosis of polymyositis, but the muscle biopsy shows some rimmed vacuoles. Recently, immunohistochemistry with an antibody directed against phosphorylated neurofilament (SMI-31) has been advocated as a diagnostic test for sporadic inclusion body myositis. The aims of the present study were to define a quantitative criterion to differentiate sporadic inclusion body myositis from polymyositis based on the detection of rimmed vacuoles in the haematoxylin-eosin staining and to evaluate the additional diagnostic value of the SMI-31 staining. Based on clinical criteria and creatine kinase levels in patients with endomysial infiltrates, 18 patients complied with the diagnosis of sporadic inclusion body myositis, and 17 with the diagnosis of polymyositis. A blinded observer counted the abnormal fibres in haematoxylin-eosin-stained sections and in SMI-31-stained sections. The optimal cut-off in the haematoxylin-eosin test was 0.3% vacuolated fibres. Adding the SMI-31 staining significantly increased the positive predictive value from 87 to 100%, but increased the negative predictive value only to small extent. We conclude that (1) patients with clinical and laboratory features of polymyositis, including response to treatment, may show rimmed vacuoles in their muscle biopsy and that (2) adding the SMI-31 stain can be helpful in differentiating patients who respond to treatment from patients who do not.
Adult↗
Oral pulsed high-dose dexamethasone for myositis.
To study the short-term effect of oral pulsed high-dose dexamethasone for myositis we treated eight newly diagnosed patients with three 28-day cycles of oral dexamethasone. Primary outcome measures were muscle strength, pain, and serum creatine kinase activity. Six patients responded. Side effects were mild. At follow-up five responders were still in remission, without medication. Pulsed high-dose dexamethasone seems beneficial in myositis. A larger, prednisone-controlled trial is justified to analyze long-term efficacy.
Administration, Oral↗
A man with progressive weakness in his legs.
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Arteriovenous Fistula↗
Absence of characteristic features in two patients with inclusion body myositis.
According to recently published criteria a diagnosis of definite sporadic inclusion body myositis is made if the typical histopathological abnormalities (rimmed vacuoles and abnormal accumulations of proteins, in addition to mononuclear cell infiltrates) are present. The two women described here presented with myositis which was unresponsive to treatment. Patient 1 had features of non-progressive sporadic inclusion body myositis clinically, whereas patient 2 had a very slowly progressive limb girdle syndrome. The cryostat sections of the first biopsies did not show rimmed vacuoles, even in retrospect. Only a repeated biopsy, 12 years after presentation in one patient and 18 years after presentation in the other, disclosed the typical features of sporadic inclusion body myositis. The initial absence of abnormal fibres probably represents a real absence or scarcity rather then a sampling error due to a multifocal nature of the histological abnormalities. It is of importance for the clinician to realise that some patients with myositis unresponsive to treatment, even if both clinical and histological features do not suggest sporadic inclusion body myositis, may prove to have the disease on repeated histopathological examination.
Adult↗