White matter abnormalities on MRI in neuroacanthocytosis.
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Adult↗
Biomedical subjects
I Sutton
Publications and source records attributed to I Sutton.
Primary biliary cirrhosis: seeking the silent partner of autoimmunity.
Primary biliary cirrhosis is a disorder characterised by an intense inflammatory response in the septal and interlobular bile ducts and is considered to be an autoimmune disease. Evidence to suggest that chronic viral infection could be a crucial element in the development of biliary epithelial cell damage and activation of the associated autoimmune response is reviewed
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗
Restricted IgG1 subclass of anti-Yo antibodies in paraneoplastic cerebellar degeneration.
Paraneoplastic cerebellar degeneration (PCD) occurs as a non-metastatic manifestation of cancer in a small proportion of patients with certain breast or gynaecological tumours, and is characterised by widespread Purkinje cell loss. Antibodies against a Purkinje cell cytoplasmic antigen, called Yo, that is expressed by the tumours, are present in the majority of these patients, but the pathogenic role of the antibodies is not clear. To characterise further the immune response in these cases, 13 anti-Yo positive sera were tested for IgG subclasses by immunohistochemistry and western blotting and, in four cases, PHA-stimulated cytokine secretion by peripheral blood lymphocytes was measured. Surprisingly, anti-Yo antibodies were entirely restricted to the IgG1 subclass, whereas antibodies against the small cell cancer-associated antigen, Hu, were found in all four IgG subclasses. There was a trend towards raised IgG1 levels in the total IgG of the anti-Yo positive patients and, in two, PHA-stimulated peripheral blood lymphocytes secreted raised levels of IFN-gamma. By contrast, in the other two cases tested, raised levels of IL-4 were secreted. Patients with PCD associated with anti-Yo antibodies appear to have strong immune responses that are polarised with respect to the IgG subclass and Th cytokine profiles.
Animals↗
McLeod neuroacanthocytosis: genotype and phenotype.
McLeod syndrome is caused by mutations of XK, an X-chromosomal gene of unknown function. Originally defined as a peculiar Kell blood group variant, the disease affects multiple organs, including the nervous system, but is certainly underdiagnosed. We analyzed the mutations and clinical findings of 22 affected men, aged 27 to 72 years. Fifteen different XK mutations were found, nine of which were novel, including the one of the eponymous case McLeod. Their common result is predicted absence or truncation of the XK protein. All patients showed elevated levels of muscle creatine phosphokinase, but clinical myopathy was less common. A peripheral neuropathy with areflexia was found in all but 2 patients. The central nervous system was affected in 15 patients, as obvious from the occurrence of seizures, cognitive impairment, psychopathology, and choreatic movements. Neuroimaging emphasized the particular involvement of the basal ganglia, which was also detected in 1 asymptomatic young patient. Most features develop with age, mainly after the fourth decade. The resemblance of McLeod syndrome with Huntington's disease and with autosomal recessive chorea-acanthocytosis suggests that the corresponding proteins--XK, huntingtin, and chorein--might belong to a common pathway, the dysfunction of which causes degeneration of the basal ganglia.
Adult↗
The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies.
The clinical and laboratory phenotype of a paraproteinaemic neuropathy syndrome termed chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies is described in a series of 18 cases. Previous single case reports have outlined some features of this syndrome. All 18 cases were defined by the presence of serum IgM antibodies which react principally with NeuAc (alpha2-8)NeuAc(alpha2-3)Gal-configured disialosyl epitopes common to many gangliosides including GDlb, GD3, GTlb and GQlb. In 17 out of 18 cases, the serum contained benign IgM paraproteins, and in four of these cases at least two IgM paraproteins were present. The IgM antibodies were also cold agglutinins in 50% of cases. The clinical picture comprised a chronic neuropathy with marked sensory ataxia and areflexia, and with relatively preserved motor function in the limbs. In addition, 16 out of 18 cases had motor weakness affecting oculomotor and bulbar muscles as fixed or as relapsing-remitting features. When present in their entirety, these clinical features have been described previously under the acronym CANOMAD: chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies. This distribution of clinical features is reminiscent of Miller Fisher syndrome, in which acute-phase anti-disialylated ganglioside IgG antibodies are found. Clinical electrophysiology and nerve biopsy show both demyelinating and axonal features. A partial response to intravenous immunoglobulin and other treatments is reported in some cases.
Adult↗
Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody.
A woman developed brain stem encephalopathy in association with serum anti-Ma2 antibodies and left upper lobe lung mass. T2 weighted MRI of the brain showed abnormalities involving the pons, left middle and superior cerebellar peduncles, and bilateral basal ganglia. Immunohistochemical analysis for serum antineuronal antibodies was confounded by the presence of a non-neuronal specific antinuclear antibody. Immunoblot studies showed the presence of anti-Ma2 antibodies. A premortem tissue diagnosis of the lung mass could not be established despite two CT guided needle biopsies, and the patient died as a result of rapid neurological deterioration. The necropsy showed that the lung lesion was an adenocarcinoma which expressed Ma2 immunoreactive protein. Neuropathological findings included prominent perivascular inflammatory infiltrates, glial nodules, and neuronophagia involving the brain stem, basal ganglia, hippocampus and the dentate nucleus of the cerebellum. Ma2 is an autoantigen previously identified in patients with germ cell tumours of the testis and paraneoplastic brain stem and limbic encephalitis. Our patient's clinical and immunopathological findings indicate that this disorder can affect women with lung adenocarcinoma, and that the encephalitic changes predominate in those regions of the brain known to express high concentrations of Ma proteins.
Aged↗
Limbic encephalitis and antibodies to Ma2: a paraneoplastic presentation of breast cancer.
A patient with atypical medullary breast cancer is described who presented with symptoms of limbic encephalitis. The patient's serum and CSF contained antibodies that reacted with the nervous system and the tumour. These antibodies recognised Ma2, a neuronal protein related to paraneoplastic limbic and brainstem encephalitis in men with testicular tumours. This report highlights the importance of testing for paraneoplastic antineuronal antibodies in cases of unexplained limbic encephalitis and suggests screening for breast cancer in women with antibodies predominantly directed to Ma2.
Amnesia↗
Spinal anaesthesia for Caesarean section in a patient with a cervical arteriovenous malformation.
PURPOSE: Arteriovenous malformations (AVM) of the spinal cord are rare. We report the successful management of a patient with a cervical spinal cord AVM undergoing Caesarean section delivery, using a spinal anaesthetic. CLINICAL FEATURES: Based on previous radiological investigations, the patient was known to have an AVM at the third cervical level of her spinal cord. After application of monitors and intravenous administration of 1 L normal saline, a 25 g Whitacre needle was inserted into the subarachnoid space at the L3-4 interspace. Spinal anaesthesia was established with a solution consisting of hyperbaric spinal bupivacaine 12 mg, fentanyl 12.5 micrograms and epidural morphine 0.25 mg. There was no neurological deficit during hospital stay or after discharge. CONCLUSION: The safe outcome of spinal anaesthesia for our patient is encouraging. The presence of spinal cord AVM at the cervical region is not an absolute contraindication to spinal anaesthesia.
Adult↗
A technique for simultaneously comparing the release of noradrenaline evoked by field stimulation and by propagated nerve impulses in rabbit ear arteries.
1. A technique has been described which utilized radiotracer methods to measure the release of transmitter noradrenaline, simultaneously, from segments of rabbit ear artery subjected to field stimulation and propagated nerve impulses. 2. The release of radioactivity from arteries labelled with 3H-noradrenaline was much greater in the segment subjected to field stimulation than in the segment stimulated by propagated nerve impulses. 3. The release of radioactivity from segments invaded by nerve impulses decreased progressively with increases in frequency through the range of 10-50 Hz, using 150 pulses at 10, 15, 20, 30 and 50 Hz. However, the release remained constant in the field stimulated segments throughout the frequency range used.
Animals↗
Comparison of the effects of phenoxybenzamine and uptake blockade on noradrenaline efflux from rabbit ear arteries evoked by field stimulation and propagated nerve impulses.
1. A comparison has been made of the effects of blockade of prejunctional alpha-adrenoreceptors and blockade of transmitter noradrenaline uptake in segments of rabbit ear arteries subjected to field stimulation or neuronally propagated impulses. 2. The relationship between evoked release and frequency of stimulation differed in artery segments subjected to field stimulation and those receiving propagated nerve impulses. However, the effectiveness of phenoxybenzamine in increasing stimulation-induced efflux of radioactivity decreased as the frequency of stimulation increased in artery segments subjected to either field stimulation or neuronally propagated impulses. 3. Blockade of neuronal and extraneuronal uptake had no effect on evoked efflux from field-stimulated artery segments but it did produce a marked and significant enhancement of release evoked by propagated nerve impulses.
Animals↗
Prejunctional alpha-adrenoreceptors subserve a physiological role in cardiac noradrenergic transmission.
1 The question whether prejunctional alpha-adrenoreceptors subserve a physiological role in a noradrenaline-mediated autoinhibitory feedback loop has been studied in guinea-pig isolated atria by stimulating the accelerans nerve and measuring chronotropic responses and the release of radioactivity after labelling transmitter stores with 3H-noradrenaline. 2 Phentolamine (0.3 micrometer) significantly enhanced chronotropic responses when stimulation was with 0.5 Hz for 30 s, but the increase in the release of radioactivity was too small to be measured reliably. When the frequency of stimulation was increased to 4 Hz for 30 s, phentolamine significantly increased the release of radioactivity but the chronotropic response to stimulation was near maximal and phentolamine had no significant effect on it. 3 With prolonged stimulation (12 min) at the lower frequency (0.5 Hz), both the release of radioactivity and the chronotropic response to stimulation were significantly enhanced by phentolamine (3 micrometers). 4 The results support a physiological role for prejunctional alpha-adrenoreceptors in guinea-pig isolated atria.
Animals↗
The big unisex wards controversy: more laughter, less tension; vive la différence!
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Adult↗
Effects of acute and chronic pentobarbitone on the gamma-aminobutyric acid system in rat brain.
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Aminobutyrates↗
The selective blockade by diaminobutyric acid of neuronal uptake of (3H)GABA in rat brain in vivo.
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Amino Acids, Diamino↗
The cardiovascular actions of nicotine in the rat.
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Adrenal Glands↗