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Biomedical subjects

T J Day

Publications and source records attributed to T J Day.

16 recordsLinked to original sources

Dominantly inherited proximal myotonic myopathy and leukoencephalopathy in a family with an incidental CLCN1 mutation.

A two generation family of Greek origin with mild myotonia, predominantly proximal muscle weakness, and cataracts compatible with the syndrome of proximal myotonic myopathy, is reported. In addition, brain MRI showed a diffuse leukoencephalopathy in the propositus. Molecular genetic studies showed the R894X mutation in exon 23 of the muscle chloride channel gene in the propositus but in only one of her two clinically affected offspring, indicating that it is not the mutation causing disease in this family.

Abnormalities, Multiple↗

Late radiation associated neurological injury.

Late radiation-associated neurological injury is a recognised and often fatal complication of therapeutic ionising radiation. This retrospective study outlines its highly variable presentation, radiological findings and prognosis over a 6 year period in a major teaching hospital.

Acute Disease↗

RR interval variation and the sympathetic skin response in the assessment of autonomic function in peripheral neuropathy.

The diagnostic value of two simple tests of autonomic function, the RR interval variation and the sympathetic skin response, was evaluated relative to symptoms of dysautonomia in 53 patients with peripheral neuropathy. Of 22 patients with peripheral neuropathy and clinical dysautonomia, 15 showed abnormal results on both tests, and 7 had abnormal results on one test only. In none of the patients with dysautonomia were both tests' results normal. Conversely, all 15 patients with abnormal results of both sympathetic skin response and RR interval variation had symptoms of dysautonomia, while 7 of 15 patients with abnormalities limited to one test had such symptoms. No patient with normal results on both tests had clinical dysautonomia. These data indicate that RR interval variation and sympathetic skin response, both of which can easily be performed in the electromyography laboratory, are helpful in combination in the assessment of autonomic function in peripheral neuropathies.

Adolescent↗

Spatial dispersion of magnetic stimulation in peripheral nerves.

To assess the longitudinal dispersion of the stimulus induced by the magnetic coil, collision experiments were performed in seven normal ulnar nerves. A supramaximal electrical stimulus S1 was delivered at the wrist, and followed by a supramaximal stimulus S2 in the upper arm, which was either electrical (electrical collision studies), or magnetic (magnetic collision studies). The interstimulus interval was varied by 0.2 msec increments from the time of complete cancellation of the S2 evoked motor response onwards, to include the entire span of recovery of that compound motor action potential. Collision curves were obtained for both magnetic and electrical stimuli by plotting the amplitude of the motor response elicited by S2 as a function of the interstimulus interval. In all seven normal ulnar nerves, comparison of the collision curves showed that the S2 evoked motor response is restored significantly more slowly when magnetic stimulation is used. This finding is best explained by longitudinal dispersion of the stimulus induced by the magnetic coil relative to conventional electrical stimulation, the large fibers being stimulated further away from the coil than the small ones. This interpretation is confirmed by the findings obtained with the same method in two cases of ulnar neuropathy, and by comparison of different intensities of magnetic stimulation.

Action Potentials↗

Migraine and other vascular headaches. An overview of diagnosis and management.

The author reviews the diagnosis of various vascular headaches, with an emphasis on migraine, and explains their pharmacological and non pharmacological treatments. In addition, difficult areas such as 'status migranosus', complicated migraine and migraine in pregnancy are discussed. Finally, some unusual headache variants are presented.

Cluster Headache↗

Aortic occlusion and reperfusion and conduction, blood flow, and the blood-nerve barrier of rat sciatic nerve.

We studied the effect of ischemia and reperfusion on blood flow, electrophysiology, and the blood-nerve barrier of the sciatic nerve of the rat. Ischemia for 10 to 60 min, with up to 3 h of reperfusion, was produced by closure and then release of a noose around the abdominal aorta. Nerve blood flow and function were measured serially using microelectrode-hydrogen polarography and electrophysiologic recordings, respectively. The integrity of the blood-nerve barrier was assessed using the permeability surface area product to [14C]sucrose. Ischemia of up to 30 min did not impair reperfusion. One hour of ischemia resulted in reperfusion abnormalities that affected about half of the nerves. The ischemic and reperfusion stresses did not disrupt the blood-nerve barrier to [14C]sucrose nor produce conduction block. Possible mechanisms for this resistance to ischemic and reperfusion injury are discussed.

Animals↗

Analysis of H2 clearance curves used to measure blood flow in rat sciatic nerve.

1. By use of the H2 clearance technique, blood flow was measured in the sciatic nerve of healthy, anaesthetized Sprague-Dawley rats at rest, during inferior vena cava occlusion and during 5-hydroxytryptamine infusion. The purpose was to clarify the mechanisms underlying the biexponential curves which are commonly obtained using this technique. 2. An analysis of the frequency distribution of rate constants of 270 nerve and thirty-three arterial samples indicated that H2 clearance rates cluster below 20 ml min-1 100 g-1 and between 70 and 100 ml min-1 100 g-1. This suggests that at least two compartments are present. 3. The contribution of diffusion was studied by recording H2 clearance immediately following circulatory arrest. Slow clearance rates (median = 2.4 ml min-1 100 g-1) were observed, indicating that diffusion is not likely to contribute significantly to nutritive flow under most situations. 4. The contribution of arteriovenous shunts to H2 clearance was assessed by determining H2 clearance during inferior vena cava occlusion and the infusion of 5-hydroxytryptamine. Both manoeuvres caused abolition of, or a significant reduction in the weight of, the fast component which indicates that this compartment is closely related to arteriovenous shunts in nerve. 5. By use of a multi-compartmental model, it was shown that H2 clearance should follow a multi-exponential course, where the weights of the components reflect the relative volumes of each compartment and the exponents represent the relative flow (i.e. flow per unit volume) in each compartment. 6. By use of other mathematical models, estimates were made for the clearance rates attributable to polarographic oxidation of H2 at the tip of the microelectrode (0.2 ml min-1 100 g-1) and to diffusion to air (2 ml min-1 100 g-1). The latter estimate is very close to the measured value of 2.4 ml min-1 100 g-1. 7. These findings indicate that it is possible to separately assess nutritive and non-nutritive flow by application of biexponential analysis to H2 clearance curves. The data suggest that the fast component of a H2 clearance curve is closely associated with arteriovenous shunts, while the slower component is likely to represent capillary flow. Processes such as diffusion to air or oxidation of H2 by the electrode are very slow and therefore are unlikely to distort the assessment of blood flow by using this technique.

Animals↗

Peripheral sympathetic conduction velocity calculated from surface potentials.

A simple, non-invasive and well tolerated technique is described for measuring autonomic nerve conduction velocity (ANCV) using proximal and distal peripheral autonomic surface potential (PASP) recordings. ANCV were obtained in 90% of subjects, and are comparable to values measured by intraneural recording. This technique may have applications in the assessment of autonomic neuropathies and can be readily performed in a standard EMG laboratory.

Adult↗

CSF in CNS leukaemia.

Explore the source record for details and available documents.

Central Nervous System Diseases↗

Depot-glucagon in the treatment of McArdle's disease.

Glucagon has been reported to improve exercise tolerance in some patients with myophosphorylase deficiency (McArdle's disease) but a placebo effect was not excluded in previous trials. A single-blind controlled trial of glucagon in a patient with myophosphorylase deficiency is described. Although an improved exercise endurance of the forearm muscles was demonstrated with both short-acting and depot-glucagon, this was not statistically significant when compared with placebo or no treatment.

Adult↗

Familial motor neurone disease associated with non-specific organic dementia. A clinico-pathological study of a family.

Two sisters developed motor neurone disease (MND), which was associated with progressive intellectual impairment in one; they survived for 18 and 24 months, respectively. At necropsy, the features of MND were confirmed, and the demented patient had severe non-specific cerebral atrophy. No evidence of Alzheimer's disease was found. Organic dementia of non-specific type can be associated with up to 15% of the familial cases of MND and with about 2% of the sporadic cases of MND in the Western world.

Aged↗

Cerebrospinal-fluid abnormalities associated with migraine.

Cerebrospinal-fluid (CSF) abnormalities have been reported infrequently in patients with migraine. Four cases of CSF pleocytosis and raised pressure found in young women during the course of an attack of protracted prodromal migraine, which was followed by complete recovery, are reported. In the absence of any other detectable cause, the CSF changes were attributed to the effects of the migrainous process on meningeal vascular permeability.

Adult↗

Campylobacter fetus subspecies jejuni isolated from Syrian hamsters with proliferative ileitis.

Two groups of Syrian hamsters received 3 weeks apart from a commercial supplier became ill and were found to have proliferative ileitis (transmissible ileal hyperplasia). Seventy-five of 200 hamsters from one group and 60 of 200 from another group were evaluated by bacteriological and histopathological techniques. Campylobacter fetus subspecies jejuni was isolated from all hamsters showing clinical signs of proliferative ileitis. The organism was recovered from fecal swabs and the ileum. Many asymptomatic animals had microscopic lesions characteristic of proliferative ileitis, and all except one were culture positive for C fetus subspecies jejuni. A group of 48 hamsters from another commercial source and free of the disease were culture negative for C fetus subspecies jejuni. These animals were inoculated by gavage with one of the following: extract of affected ileum (eight hamsters), 1 X 10(8) viable C fetus subspecies jejuni organisms (28 hamsters), or sterile saline (12 hamsters). Ileal-extract inoculated hamsters were culture positive and five of eight developed clinical signs of ileal hyperplasia. Pure-culture-inoculated hamsters had colonization of the ileum by C fetus subspecies jejuni but did not develop hyperplasia of the ileum. With this data and the tendency of C fetus subspecies jejuni to be associated with clinical enteric disease in a variety of animals and man, we suggest that C fetus subspecies jejuni may have an etiologic role in proliferative ileitis of hamsters. Other synergisms or predisposing factors also may be involved.

Animals↗