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Many previously widely accepted neonatal transfusion practices are changing as neonatologists become more aware of the risks to their patients of multiple blood product transfusions. Recent literature and research on neonatal transfusion practice are here reviewed, and practical guidelines and trigger thresholds for blood products commonly used in neonatal medicine are proposed. [ABSTRACT FROM AUTHOR]

Aims: To assess mortality and cancer morbidity in Canadian petroleum workers and explore exposure-response relations for specific petroleum agents. Methods: A total of 25 292 employees hired between 1964 and 1994 were linked to the Canadian tumour registry and national mortality database. Exposure-response trends were assessed for hydrocarbon solvents/fuels, hydrocarbon lubricants, petroleum coke/spent catalyst, and hydrogen sulphide (H[sub2]S). Results: External comparison analyses (mortality and incidence) showed deficits for all causes and all malignant neoplasms combined and were consistent with expectation for most malignant and non- malignant sites analysed. Gall bladder cancer mortality was increased among males based on four deaths, but cases had no common job assignments and the increase was focused in workers employed >10 years. Mesothelioma incidence was increased. Most exposure-specific analyses were compromised by small numbers. Statistically significant increases were observed for H[sub2]S exposure and a subgroup of accidental deaths as well as for petroleum coke/spent catalyst exposure and lung' cancer. While both findings have a degree of biologic plausibility, the H[sub2]S association, which exhibited a clearer exposure-response pattern, could be subject to unmeasured confounders. Additionally, interpretation was complicated by the high correlation between hydrocarbon and H[sub2]S exposures. With regard to lung cancer, the analysis could not adequately control for smoking, was based on small numbers, and exhibited a tenuous exposure-response pattern. Conclusion: The findings for mesothelioma suggest the need for continued attention to asbestos in the petroleum industry. The relation between accidental deaths and H:S exposure deserves closer scrutiny in similarly exposed populations. Further analyses of lung cancer are underway and will be reported separately. [ABSTRACT FROM AUTHOR]

Neonatal thrombocytopenia is a common clinical problem. Thrombocytopenia presenting in the first 72 hours of life is usually secondary to placental insufficiency and caused by reduced platelet production; fortunately most episodes are mild or moderate and resolve spontaneously. Thrombocytopenia presenting after 72 hours of age is usually secondary to sepsis or necrotising enterocolitis and is usually more severe and prolonged. Platelet transfusion remains the only treatment. There is a need for trials to define the safe lower limit for platelet count and which neonates will benefit from treatment. [ABSTRACT FROM AUTHOR]

Objectives: To update mortality for 34 560 employees from diverse operating segments of a Canadian petroleum company; and to investigate potential relations with occupational factors.Methods: Employees from 1964-83 were linked to the Canadian mortality data base to provide 11 years additional follow up. There were 6760 deaths and 750 683 person-years of follow up compared with 3909 and 428 190, respectively, in the earlier study. Analyses used standardised mortality ratios (SMRs) to compare worker cause specific mortality with the Canadian general population. Duration of employment and latency analyses were performed for causes previously found to be increased in this and other petroleum cohorts, as well as any findings of interest.Results: For the period 1964-94, employees experienced significantly low overall mortality (SMR=0.86 men, SMR=0.80 women). Kidney cancer, which has been increased in some studies of petroleum workers, was not increased. Acute non-lymphocytic leukaemia in exposed operating segments was consistent with the expected or only slightly, non-significantly increased. The most notable finding was increased deaths from mesothelioma among refinery and petrochemical workers (SMR 8.68; 95% confidence interval (95% CI) 5.51 to 13.03), most of whom were long term employees in jobs with presumed exposure to asbestos (mechanical and pipefitters). Deaths from multiple myeloma among marketing and distribution workers, which were previously increased, remained increased (SMR 2.08; 95% CI 0.95 to 3.95) in the update period 1984-94; however, there was no clear pattern by duration of employment or latency. Aortic aneurysms, which also were previously significantly increased among marketing and distribution workers approached the expected in the update period (SMR 1.18; 95% CI 0. 65-1.98). Analyses by duration of employment showed suggestive trends for aortic aneurysms, but earlier studies of this cohort have not found a relation between aortic aneurysms and exposure to hydrocarbons.Conclusion: The additional 2851 deaths and 322 493 person-years of follow up strengthened the assessment of mortality patterns relative to occupational factors. With the exception of mesothelioma, no clear work related increases in disease were identified. [ABSTRACT FROM AUTHOR]

A patient with the Eaton-Lambert syndrome is described in whom no associated condition was discovered. There was clinical and electrical evidence that the defect in neuromuscular transmission became more severe as local temperature was raised. [ABSTRACT FROM AUTHOR]

Body sway and vibration perception in the lower limbs were measured in 32 normal subjects and 25 patients with peripheral neuropathies; nerve conduction studies were also performed in the patients with neuropathies. Body sway was measured by means of force-plate posturography, and three methods were used to assess vibration perception: a neurothesiometer, a semiquantitative tuning fork, and the bone vibrator of a conventional audiometer. Body sway and vibration perception were increased in the patients with peripheral neuropathies and there was significant correlation between these measures.d These findings, together with the lack of correlation between sway and muscle strength, indicate that the main source of unsteadiness in these patients is the loss of proprioceptive information. Vibration perception and body sway did not correlate with the electrophysiological variables, indicating that these measures assess different aspects of peripheral nerve function. In all subjects there was close correlation between vibration perception as assessed by the neurothesiometer and the audiometer could be used to screen proprioceptive function in patients with balance disorders. In normal subjects age correlated with vibration perception (measured with the neurothesiometer and audiometer) and also with body sway standing on foam. This suggests that the increased body sway in elderly people may partly be due to redue proprioception in the lower limbs. [ABSTRACT FROM AUTHOR]

One hundred and fifty patients presenting with small cell lung cancer (SCLC) to chest physicians, were assessed neurologically. Neuromuscular or autonomic deficits were common and occurred in up to 44% of cases. Weakness, dry mouth, and weight loss were not mutually independent and may represent the syndrome formerly described as carcinomatous neuromyopathy. By contrast, undoubted paraneoplastic syndromes were much less commonly detected. Two patients had the Lambert-Eaton myasthenic syndrome (LEMS) and one had subacute sensory neuropathy (SSN). In these patients, neurological symptoms antedated other manifestations of cancer, by between six and 17 months. The 95% confidence interval for the prevalence of LEMS or SSN among SCLC patients was 0-4%, consistent with the results of previous retrospective or smaller studies: summing these, the overall prevalence of LEMS among SCLC patients is close to 3%, which implies about 250 new cases per annum in England and Wales. If LEMS and SSN are the least uncommon neurological paraneoplastic syndromes in SCLC patients, this may reflect the accessibility of motor nerve terminals and dorsal root ganglia to cross-reactive anti-tumour cell antibodies. [ABSTRACT FROM AUTHOR]

Activity in descending motor pathways after scalp electrical and magnetic brain stimulation of the motor cortex was recorded from the exposed cervico-medullary junction in six patients having trans-oral surgery of the upper cervical spine. Recordings during deep anaesthesia without muscle paralysis revealed an initial negative potential (D wave) at about 2 ms with electrical stimulation in five of the six patients. This was followed by a muscle potential which obscured any later waveforms. Magnetic stimulation produced clear potentials in only one patient. The earliest wave to magnetic stimulation during deep anaesthesia was 1-2 ms later than the earliest potential to electrical stimulation. Following lightening of the anaesthetic and the administration of muscle relaxants a series of later negative potentials (I waves) were more clearly seen to both electrical and magnetic stimulation. More I waves were recorded to magnetic stimulation during light anaesthesia than during deep anaesthesia. Increasing the intensity of electrical stimulation also produced an extra late I wave. At the highest intensity of magnetic stimulation the latency of the earliest potential was comparable to the D wave to electrical stimulation. The intervals between these various D and I waves corresponded to those previously described for the timing of single motor unit discharge after cortical stimulation. [ABSTRACT FROM AUTHOR]

The maintenance of axial atonia during REM sleep was monitored in 14 patients with primary torsion dystonia, 10 patients with secondary torsion dystonia, and 10 normal subjects using submental EMG and video EEG telemetry. The excitability of the corticospinal tract during REM sleep was also assessed using scalp magnetic stimulation in seven patients and three controls. During REM sleep dystonic patients had well maintained atonia evidenced by infrequent bursts of submental activity, no episodes of complex semi-purposeful behaviour and reduced motor responses to magnetic stimulation. These findings suggest that the inhibitory centres in the region of the locus coeruleus and their descending pathways to the spinal alpha motor neurons are intact in torsion dystonia. [ABSTRACT FROM AUTHOR]

In a consecutive series of 30 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) minor clinical evidence of CNS involvement was found in five. Cranial magnetic resonance imaging (MRI) was performed in 28 and revealed abnormalities consistent with demyelination in nine patients aged less than 50 years and abnormalities in five aged 50 years or over. Measurements of central motor conduction time (CMCT) were obtained in 18 and showed unilateral or bilateral abnormalities in six. It is concluded that subclinical evidence of central nervous system (CNS) involvement is common, at least in patients with CIDP in the United Kingdom, but that clinically evident signs of CNS disease are infrequent. The association of a multiple sclerosis-like syndrome with CIDP is rare. [ABSTRACT FROM AUTHOR]

Central motor conduction to small hand muscles was measured using magnetic stimulation of the motor cortex and electrical stimulation of proximal motor roots in 11 patients with Friedreich's ataxia, 10 patients with early onset cerebellar ataxia with retained tendon reflexes (EOCA) and 13 patients with late onset degenerative cerebellar disease (LOCD). Central motor conduction was abnormal in 91% with Friedreich's ataxia, 70% with EOCA and 38% with LOCD. Central motor conduction abnormalities were not specific to individual disorders but were more severe and were related to disease duration in Friedreich's ataxia and EOCA. [ABSTRACT FROM AUTHOR]

A magnetic stimulator was used for direct transcutaneous stimulation of the intracranial portion of the facial nerve in 15 normal subjects and in patients with Bell's palsy, demyelinating neuropathy, traumatic facial palsy and pontine glioma. Compound muscle action potentials (CMAPs) thus elicited in the orbicularis oris muscle of controls were of similar amplitude but longer latency (1.3 SD 0.15 ms) compared with CMAPs produced by conventional electrical stimulation at the stylomastoid foramen. No response to magnetic stimulation could be recorded from the affected side in 15 of 16 patients with Bell's palsy. Serial studies in two patients demonstrated that the facial nerve remained inexcitable by magnetic stimulation despite marked improvement in clinical function. In the patient with a pontine glioma, the CMAP elicited by transcranial magnetic stimulation was of low amplitude but normal latency. In six of seven patients with demyelinating neuropathy, the response to intracranial magnetic stimulation was significantly delayed. Magnetic stimulation produced no response in either patient with traumatic facial palsy. Although the precise site of facial nerve stimulation is uncertain, evidence points to the labyrinthine segment of the facial canal as the most likely location. [ABSTRACT FROM AUTHOR]

A case of combined multiple sclerosis and demyelinating neuropathy is presented. Percutaneous electrical stimulation of the cortex and spinal cord has shown that pyramidal tract conduction time was prolonged and conduction velocity in the cord was 4 m/s. Motor conduction velocity in proximal segments of peripheral nerves was slowed to the same extent as in distal segments. [ABSTRACT FROM AUTHOR]

Seven out of 12 normal subjects could deliberately produce abnormal pattern-reversal visual evoked potentials (VEPs) which simulated disorders of the anterior visual pathways without detection. In six the mechanism was near-point accommodation and in one eccentric fixation. If voluntary suppression of the VEPs is suspected various modifications to the recording technique may be of value. These include the use of a large check size and stimulus field and binocular stimulation. [ABSTRACT FROM AUTHOR]

The clinical features of 53 British-born patients with Gilles de la Tourette syndrome are described. The mean age at onset of body tics was seven years and for vocalisations 11 years. Coprolalia was present in 39%, copropraxia in 21%, echolalia in 46% and echopraxia in 21%. Complicated antics and mannerisms were also common, often involving the compulsive touching of objects or self-injurious behaviour. Forty-six per cent of cases had a family history of tics in a single close relative and in two individuals a further member of the family had Gilles de la Tourette syndrome. Focal dystonia was present in four patients who had never received neuroleptics drugs and chorea was seen in two other untreated patients. In three patients acoustic startle consistently induced brief eye blink followed by a whole body jerk or jump. Rapid repetitive movements of the hands increased the frequency and severity of tics in 13 patients, but the performance of mental arithmetic under time pressure had a much more unpredictable effect. Electroencephalographic abnormalities occurred in eight (13%) but no definite CT brain scan abnormalities were detected. The incidence of left handedness did not differ from that in the general population and no evidence to suggest organic impairment was found on neuropsychological testing. This study provides no support for the notion that Gilles de la Tourette syndrome is a degenerative disorder of the central nervous system but provides some evidence for heterogeneity. [ABSTRACT FROM AUTHOR]

Two cases of spinal myoclonus are described; in both patients myoclonus was responsive to stimuli and absent during sleep. The first patient was considered to have viral neuronitis and the condition resolved spontaneously. The second patient had spinal cord ischaemia; there was electro-physiological evidence of abnormal alpha motor neurone activity and histological study of the spinal cord revealed a severe reduction in small and intermediate neurones. This supports the theory that spinal myoclonus may result from abnormal activity of alpha motor neurones released from control by spinal internuncial neurones. [ABSTRACT FROM AUTHOR]

Serial spinal somatosensory evoked potentials were recorded in six patients undergoing radiotherapy involving incidental spinal radiation. Two patients developed transient radiation myelopathy. No abnormality was found in the somatosensory evoked potentials throughout the study. [ABSTRACT FROM AUTHOR]

Four hundred and three patients were considered for entry into a trial of intravenous streptokinase in suspected myocardial infarction. Three hundred and sixty seven (91%) were excluded. Two hundred and sixty (65%) did not meet the inclusion criteria and 45 of the remaining 143 (35%) patients had contraindications to thrombolysis. This left 98 (24%) patients who were suitable for thrombolysis and 42 of them were over 70 years, the upper age limit. Thus according to this trial protocol 56 (14%) patients were eligible for recruitment; 36 (9%) patients were finally randomised. These data suggest that treatment with intravenous streptokinase may be applicable to only a small proportion of patients with myocardial infarction. [ABSTRACT FROM PUBLISHER]

A family having two affected siblings with congenital dyserythropoietic anaemia type II (HEMPAS) is described. The proband was diagnosed after referral for investigation of haemolytic anaemia. Clinical evaluation and in vivo red cell (RBC) survival and the sequestration studies in the proband indicated that the anaemia was due to a combination of ineffective erythropoiesis and premature destruction of RBCs in the spleen. Scanning electron microscopic examination of peripheral RBCs was undertaken and is reported. The polypeptide composition of RBC membranes was also examined using polyacrylamide gel electrophoresis after solubilisation in sodium dodecyl sulphate. These results are also reported. [ABSTRACT FROM PUBLISHER]

A patient with primary B cell non-Hodgkin's lymphoma of the sciatic nerve is described. He presented with neuropathic symptoms in the left leg, initially diagnosed as tarsal tunnel syndrome. Magnetic resonance imaging (MRI) identified the abnormality in the sciatic nerve. A fascicular biopsy of the sciatic nerve showed a diffuse large B cell non-Hodgkin's lymphoma. The patient was treated with chemotherapy and rituximab (anti-CD20 monoclonal antibody). Four months later he was in remission, and remains so 48 months from presentation. Primary lymphoma of single peripheral nerves may be a unique subtype of extranodal lymphoma, which usually follows an aggressive course and has a variable response to current therapeutic strategies. MRI is useful, alongside electrophysiological studies, in patients with atypical peripheral nerve symptoms. [ABSTRACT FROM AUTHOR]

A 35 year old woman with clinical features of hyperkalaemic periodic paralysis confirmed on provocative exercise testing was investigated. DNA sequence analysis of the gene for the alpha-subunit of the skeletal muscle voltage gated sodium channel (SCN4A) on chromosome 17q23 identified a point mutation at nucleotide position 2188. This results in a threonine to methionine substitution at amino acid position 704. The patient was intolerant of diuretic medication but showed a striking clinical and electrophysiological improvement with salbutamol therapy. Treatment with beta-adrenergic agents should be considered in patients with hyperkalaemic periodic paralysis who are intolerant of, or resistant to, diuretic medications. [ABSTRACT FROM AUTHOR]

A patient with multiple sclerosis is reported in whom neck flexion produced a reversible deterioration in gait and an increase in the degree of pyramidal weakness of the lower limbs. The name McArdle's sign is proposed. [ABSTRACT FROM AUTHOR]

Serum or IgM fraction from two patients with a demyelinating neuropathy and IgM monoclonal antibodies to myelin-associated glycoprotein were injected in three different animal species. There were no clinical, electrophysiological or morphological signs of demyelination in either chronic or acute passive transfer experiments. These results suggest that the pathogenesis of this human demyelinating neuropathy may be more complex than has been assumed. [ABSTRACT FROM AUTHOR]

A 25 year old man with no history of heart disease presented with sweats and rigors. Echocardiography showed a large vegetation on the pulmonary valve and blood cultures grew Escherichia coli. Because of recurrent pulmonary emboli a large vegetation on the anterior leaflet of the pulmonary valve was excised. He recovered after a full course of antibiotics. [ABSTRACT FROM PUBLISHER]

Twenty days after a streptokinase infusion given for myocardial infarction, a patient developed a group G streptococcal throat infection. Thirteen days later he presented with a serum sickness type illness and progressive renal failure. Renal biopsy showed crescentic glomerulonephritis. [ABSTRACT FROM PUBLISHER]

A letter to the editor is presented which discusses the burden of ocular comorbidity in cataract patients in West Africa.

Although recurrent rheumatic fever in adults is uncommon and the recurrence rate declines with age and with the interval from the attack, we describe a patient who has had four attacks of rheumatic fever, two in childhood and two in adult life. [ABSTRACT FROM PUBLISHER]

A preterm baby, whose mother received chemotherapy for acute leukaemia during pregnancy, required intensive care because of profound anaemia and neutropenia. Haemopoietic progenitor cell studies showed fetal marrow suppression. Those caring for such mothers and babies should know the possible serious effects chemotherapy for malignancies can have on a developing fetus. Long term follow up of the baby is imperative. [ABSTRACT FROM PUBLISHER]

Presents a case study on effect of drugs on diseases in Europe. Development of acute renal failure following recombinant interferon treatment; Observation of nicoumalone related skin disorders; Association of skin lesion with intravenous streptokinase administration.