["Critical-illness" polyneuropathy and -myopathy]. / 'Critical-illness'-polyneuropathie en -myopathie.

Critical-illness polyneuropathy and myopathy (CIPNM) can be considered a part of the syndrome of multiple organ dysfunction. CIPNM is the commonest cause of muscle weakness acquired in the intensive care unit. Its incidence is 35-80% during prolonged mechanical ventilation. For a (differential) diagnosis, electrophysiological investigations are usually necessary, and sometimes a muscle biopsy. CIPNM may be induced by triggering of the immune response leading to increased vascular permeability with tissue invasion of inflammatory cells and local damage. There is a relation between myopathy and medication, notably corticosteroids. Clinical improvement usually follows when the CIPNM patient survives the underlying disease, but weaning from artificial ventilation is often difficult, and rehabilitation prolonged.

Critical illness polyneuropathy and myopathy (CIPNM): evidence for local immune activation by cytokine-expression in the muscle tissue.

In a longitudinal prospective study a muscle biopsy was taken from 30/32 (33%) of the 98 patients who developed critical illness polyneuropathy and myopathy (CIPNM). Neuropathic changes were found in 37%, myopathic in 40%, and a combination in 23% of the biopsies. The immunohistopathology showed macrophages and Th-cells in 40% and 60% of the muscle biopsies respectively. Small mainly perivascular infiltrates contained macrophages and Th-cells. ICAM-1, VCAM and MAC were found on the vascular endothelium in 58%, 53% and 79% respectively. In all biopsies there was an upregulation of both HLA-I and HLA-DR. Proinflammatory cytokines and TNFalphaR75 were also produced locally (IL-1beta in 71%, IFN-gamma in 40%, IL-12 in 73%, TNFalphaR75 in 90%). The anti-inflammatory cytokine IL-10 was simultaneously expressed in 96% of the biopsies. HLA-DR, TNFalphaR75 and IL-10 differed significantly when compared with control muscle biopsies. Our data provide evidence that small numbers of activated leukocytes producing both pro- and anti-inflammatory cytokines infiltrate skeletal muscle of CIPNM patients. We propose that the local balance of leukocyte activities is of importance in the pathophysiology of muscle weakness in CIPNM.

[Acute myopathy during treatment of status asthmaticus]. / Acute myopathie tijdens behandeling wegens status asthmaticus.

Two patients with chronic pulmonary disease, a woman of 38 and a man of 54 years old, who had developed a status asthmaticus, had difficulties being weaned from artificial ventilation. They suffered from an acute myopathy caused by a combination of high-dose corticosteroids and muscle relaxants (pancuronium, vecuronium). This acute myopathy is characterised by generalised flaccid quadriplegia with muscle atrophy and areflexia, difficulties being weaned from artificial ventilation, myoglobinuria and high levels of creatine kinase activity in serum. The prognosis is good; almost complete recovery occurs. Muscle biopsy may reveal necrotising myopathy and occasionally, selective loss of thick myofilaments.

Tumor necrosis factor and interleukin-6 in critical illness polyneuromyopathy.

Critical illness polyneuromyopathy (CIPN) occurs in critically ill patients on artificial respiration. The pathophysiology of this disease is unknown. Because of the strong association with sepsis, the levels of cytokines, TNF and IL-6 were measured several times daily in patients having CIPN and in a control group of critically ill patients without CIPN. The diagnosis of CIPN was made on clinical criteria. Patients with CIPN had no significantly elevated levels of TNF or IL-6 as compared to controls.

Congenital oculo-bulbar palsy.

A girl developed progressive weakness of bulbar and ocular muscles starting before the age of two years. Electromyography revealed a widespread subclinical myopathy. An intercostal muscle biopsy showed complex abnormalities including occasional neurofilamentous accumulations and honeycomb-like membranous material in terminal axons. Endplates were small and some secondary synaptic clefts were abnormally deep. Acetylcholine receptors extended unusually deeply into the clefts of the junctional folds. Muscle fibres showed subsarcolemmal vacuolation at some places. This form of congenital oculo-bulbar palsy does not appear to have been described previously.

Critical illness polyneuromyopathy after artificial respiration.

Up to now, 71 critically ill patients have been reported with neuromuscular complications after artificial respiration. The authors review the literature and present data of a personal series of 22 patients all suffering from severe flaccid tetraparesis and muscle atrophy, which developed after an average of two weeks artificial respiration. The prognosis was relatively good in those surviving the primary disease. The multiconditional causes are discussed with emphasis on the combination of polyneuropathy and myopathy. Tumor necrosis factor (TNF), a key mediator of sepsis, which also has an influence on muscle and nerves, is mentioned as a possible cause of this illness.

[Critical illness polyneuropathy]. / 'Critical illness' polyneuropathie.

Critical illness polyneuropathy is a syndrome of neuromuscular complications after artificial respiration. The authors present the data of their own 22 patients all suffering from severe flaccid tetraparesis, areflexia and muscle atrophy, after an average of two weeks on artificial respiration. The prognosis is relatively favourable. The multi-conditional causes are discussed with emphasis on the combination of polyneuropathy and myopathy. The role of plasma factors such as cachectin, which is identical to tumour necrosis factor (TNF), is described. Attention is drawn to this important illness which occurs especially in patients in the intensive care unit with problems in the weaning from artificial respiration.

Intracranial cavernous angiomas: diagnosis and therapy.

The authors reviewed the case histories of 10 patients with intracranial cavernous angiomas treated from 1985 till 1987. Two patients are described in detail and are illustrated by CT scan, MRI scan and angiography. The diagnostic and therapeutic problems of intracranial cavernous angiomas are discussed.

Cerebral haemorrhage in a pregnant woman with a multiple endocrine neoplasia syndrome (type 2A or Sipple's syndrome).

We present the case history of a 30-yr-old woman who suffered a cerebral haemorrhage toward the end of pregnancy (at 35 wk). The pregnancy was terminated by Caesarean section because aggravation of cerebral bleeding was feared. Extensive studies revealed that the patient was suffering from a multiple endocrine neoplasia syndrome (type 2A or Sipple's syndrome) with bilateral pheochromocytomas and a medullary thyroid carcinoma. She made an uneventful recovery. The relevant literature is discussed.

Respiratory failure as initial symptom of acid maltase deficiency.

Nine patients with adult onset acid maltase deficiency were seen at the Nijmegen University Hospital and the St Elisabeth Hospital, Tilburg , during the period 1970-1982. Five of these patients developed respiratory failure, and in four this was the initial symptom. The occurrence of respiratory failure as an early symptom of this muscular disease is discussed.

Multivariate logistic analysis of risk factors for stroke in Tilburg, The Netherlands.

By means of a case-control study conducted between October 1, 1978, and July 31, 1981, in Tilburg, The Netherlands, various characteristics and events, including personal data, health-related behavior, and medical history, were evaluated as risk factors for stroke. The study subjects included 132 stroke patients and 239 age- and sex-matched control patients interviewed at the two city hospitals. To assess joint effects and possible interactions, and to control for multiple confounding factors, a series of multivariate logistic models for matched data were studied. From this analysis, it appeared that hypertension, acute myocardial infarction, cardiac arrhythmias, transient cerebral ischemic attacks, obesity, physical activity during leisure time, education of head of household, and Rhesus factor were all significant stroke risk factors. These risk determinants demonstrated a multiplicative effect in general; however, the influence of some variables on stroke risk was not constant with age (hypertension, acute myocardial infarction, cardiac arrhythmias, obesity, and Rhesus factor) and sex (hypertension and education of head of household). The relationship of diabetes mellitus to stroke slightly decreased and became nonsignificant after adjustment for factors besides age and sex. Stroke risk was not associated with cigarette and alcohol use, family history of stroke and related disorders, marital status, and ABO blood typing.

Progressive poliodystrophy (Alpers' disease) with a defect in cytochrome aa3 in muscle: a report of two unrelated patients.

We present two unrelated patients, a boy and a girl, with a progressive neurologic disorder, characterized by psychomotor retardation, seizures and paresis, the illness being exacerbated during stressful periods. Lactate levels in serum and cerebrospinal fluid were elevated in both patients. Histopathologic studies of muscle tissue revealed mitochondrial abnormalities in the boy; in the girl, slight neuronal degeneration was observed. A cerebral biopsy in the girl showed abnormalities compatible with progressive poliodystrophy. Autopsy in the boy demonstrated progressive poliodystrophy. Biochemical studies in muscle tissue showed a defect of cytochrome aa3 in both patients, connected with a defect of cytochrome b in the girl. The association of defective pyruvate metabolism and progressive poliodystrophy is discussed.

An evaluation of risk factors for stroke in a Dutch community.

The objective of the present epidemiological study was to reevaluate the relationship of various suspect risk factors to stroke within a Dutch community. The Tilburg study lends support to findings in other countries concerning the greater stroke risk of persons with high blood pressure, heart disorders, and diabetes mellitus. Significant interactions of both age and a history of myocardial infarction with hypertension on stroke risk were noted. The presence of overweight and possible transient ischemic attacks (TIA) were found related to stroke onset. No increase in stroke risk was associated with cigarette smoking. The extent of leisure time physical activity during one's lifetime was found inversely related to one's chances for stroke.

Successful treatment by plasmapheresis of respiratory insufficiency in myasthenia gravis.

A 27-year-old man with myasthenia gravis developed a severe myasthenic crisis with respiratory insufficiency. In spite of high iv doses of pyridostigminebromide and corticosteroids, mechanical ventilation remained necessary. Treatment with plasma exchange grossly decreased the serum level of antibodies against acetylcholine receptor protein and caused an immediate clinical improvement. The respiratory assistance could be terminated and muscle strength increased substantially. Plasmapheresis seemed to be of definite benefit in the treatment of this acute crisis of myasthenia gravis.

A patient with so-called primary reticulum cell sarcoma of the brain with 6.5 years' survival, treated as 'meningeal leukaemia'.

This paper describes the case history of a 43 year-old patient with so-called primary reticulum cell sarcoma of the brain. The CSF contained cells suggestive of leukaemia which in view of an assumed myelofibrosis, initially seemed also possible to originate from a haematopoietic focus. The patient complained of intermittent violent headaches, which were controlled by spinal taps to release highly cellularised CSF under high pressure and by intrathecal MTX injection, every 4-6 weeks over a period of 4.5 years. In the terminal stage the patient developed paraplegia which, partly on the basis of neuropathological findings, was ascribed to the large accumulated dose of MTX. The tumour proved to be localised in the fornix; the localisation adjacent to the ventricular system made the intermittent cell eruptions in the CSF possible. On the basis of an erroneous diagnostic assumption, a therapy was instituted which resulted in a survival of 6.5 years, which is exceptionally long for a patient with 'primary reticulum cell sarcoma' of the brain.