SUMMARY We describe two patients with metachromatic leucodystrophy who presented with peripheral neuropathy and had no evidence of central nervous system involvement at the time of presentation. Such an occurrence is not well recognized and the diagnosis is usually missed until the other features of the disease manifest themselves. Early recognition of this disease may become of practical importance from the point of view of therapy, judging from recent developments, and is also important from the point of view of genetic counselling. We suggest that cases of peripheral neuropathy in infancy and childhood, should be subjected to peripheral nerve biopsy when the aetiology is not obvious.Although it is known that the peripheral nerves share in the pathological changes that occur in metachromatic leucodystrophy (
SUMMARY The hyperglycaemic response to intramuscular glucagon was studied in 19 migraine subjects and 17 matched controls. Venous blood sugar 30 minutes after the injection, and the maximum rise from fasting levels, were significantly reduced in migraine subjects. This diminished sensitivity to the hyperglycaemic action of glucagon may be at least one factor in the hypoglycaemia unresponsiveness shown in migraine.Migraine might perhaps be regarded as a reaction determined by a lowered threshold of central cerebral mechanisms, in combination with a wide range of external precipitating factors (Pearce, 1969(Pearce, , 1971aRao and Pearce, 1971). So far attempts to demonstrate or measure a central disorder of threshold have been unsuccessful, but the more precise delineation of external precipitating mechanisms and of endogenous biochemical anomalies susceptible to these triggers is important.It is well known that in certain migraine subjects attacks can be precipitated by hunger and fasting (Critchley and Ferguson, 1933;Blau and Cumings, 1966;Pearce, 1971a). Migraine subjects also show a tendency towards a 'flat glucose tolerance curve' (Gray and Burtness, 1935;Wilkinson, 1949), but Pearce (1971b) showed that it is not hypoglycaemia per se which is responsible for precipitating migraine attacks in susceptible subjects. Rao and Pearce (1971) found that, during an extended standard insulin hypoglycaemia test, migraine subjects showed a hypoglycaemia unresponsiveness. This investigation also effectively excluded a defect of the corticotrophin-cortisol part of the hypothalamopituitary-adrenal axis, and one of the remaining possible explanations was a defect of hepatic glycogen breakdown. The present study aims to assess hepatic glycogenolysis by the glucagon test, in migraine and control subjects. METHODSThirty-six volunteers, consisting of 19 migraine patients and 17 controls, were studied. The age range in both groups was 20 to 45 years. The migraine subjects consisted of 14 females and five males, and the controls consisted of 12 females and five males. The selection was weighted in favour of females because of their higher prevalence rate for migraine. The body weights were slightly less on average in the migraine group; this would if anything increase the hyperglycaemic effect of glucagon.All subjects were fasted and requested to abstain from smoking from midnight. Venous blood samples were obtained through an indwelling heparinized cannula. The first blood sample was obtained immediately before the intramuscular injection of 1 mg glucagon at 9.00 a.m. Further samples were then obtained at 30 minute intervals for two hours. Blood sugar was estimated by the Technicon auto-analyser method using alkaline-ferricyanide. Throughout the test the subjects were kept under continuous observation. RESULTSThe results are summarized and the statistical significance of the differences between the two groups are given in the Table. At 30 minutes after the injection (at which point the maximal hyperglycaemic response to glucag...
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