Biochemical investigations in geophagia.
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Biomedical subjects
Publications and source records attributed to R F Gledhill.
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Lesions of the spinal cord causing paroxysmal kinesigenic choreoathetosis are rare and most of the reported cases have been because of multiple sclerosis. We now describe this movement disorder occurring in a patient who developed a myelitis of unknown aetiology. A typically striking remission followed treatment with carbamazepine. It is suggested that the effect of the drug and the disorder itself may both be explained on the basis of altered sodium channels.
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Sandifer syndrome is a dystonic movement disorder described in children with severe gastro-oesophageal reflux. We now report a patient who had the features of Sandifer syndrome first developing in adult life. Onset of dystonic episodes followed closely the occurrence of a Bell's palsy, while symptoms of peptic oesophagitis had been present for several months beforehand. Successful symptomatic treatment of gastro-oesophageal reflux was accompanied by cessation of the dystonic episodes. Possible pathophysiological mechanisms of the abnormal movements in Sandifer syndrome are discussed.
Phaeohyphomycosis is an uncommon disorder caused by a variety of saprophytic fungi having distinctive morphologic features. Central nervous system infection typically occurs in the absence of predisposing factors and usually manifest symptoms and signs of abscess formation. We describe an otherwise healthy young man whose presentation with cerebral phaeohyphomycosis was subacute meningitis and stroke. Neuroimaging studies revealed multiple parenchymal lesions having the characteristics of recent infarcts; several vascular territories were involved. The nature of these lesions was confirmed histologically at autopsy. To our knowledge, such radiologic appearances have not previously been reported in this condition.
An association between syringomyelia and spinal syphilis was described in the early literature but has not been the subject of reports subsequently. We give details of a contemporary case, affirmed by magnetic resonance imaging. The patient showed significant clinical recovery following penicillin treatment, while the imaging appearances became less pronounced.
Focal spinal cord lesions have been present in all previously reported cases of MRI appearances in myelopathy complicating vitamin B12 deficiency. We describe two further cases showing mild atrophy only and review the salient features of the previous 11 publications. MRI findings reflect quite closely the known pathological changes in this condition.
Reversible electrophysiologic abnormalities of sensory nerve function were found by chance in three patients with hypokalemic periodic paralysis, a disorder previously considered to affect the function of muscle membranes only. A formal, prospective study was therefore conducted. Serial nerve conduction studies were done in ten additional patients. Amplitude of sensory action potentials was significantly smaller during paralytic attacks, but did not differ from controls after normalization of serum potassium concentration. These apparently novel findings might be explained by previous electrodiagnostic studies either not involving the testing of sensory nerves at all, or not being repeated after recovery from an attack. Involvement of sensory nerves in hypokalemic periodic paralysis is suggested to arise through dorsal root ganglia having an incomplete blood-nerve barrier and sensory neurons being particularly vulnerable to derangements affecting nerve cell metabolism. Neuronal inexcitability is postulated to occur consequent upon possible inactivation of the sodium-potassium pump by the low concentration of extracellular potassium. In patients with acute areflexic limb weakness, the diagnosis of hypokalemic periodic paralysis should not be excluded by abnormal results of sensory nerve conduction studies.
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In a previous retrospective study, 4 of 9 patients with benign intracranial hypertension were unexpectedly positive for intrathecal synthesis of immunoglobulin (Ig) G by quantitative measurement (log IgG index). This was remarkable as the only disease among many studied that showed such a discrepancy. A further study was done, now prospectively. Log IgG index values were elevated in 2 of the 11 new cases. As before, qualitative measurement (isoelectric focusing) gave uniformly negative results. Five of the 6 instances where the log IgG index was elevated could be accounted for, in fact, by abnormal values of constituent variables other than cerebrospinal fluid IgG. Quantitative tests for intrathecal synthesis of IgG can give misleading results on their own. Immunological mechanisms most probably are not involved in the pathogenesis of benign intracranial hypertension.
The clinical presentation, symptoms, and signs in 20 new patients with the painful legs and moving toes syndrome are presented. Painful legs and moving toes may develop in the setting of spinal cord and cauda equina trauma, lumbar root lesions, injuries to bony or soft tissues of the feet, and peripheral neuropathy. In 4 of the 20 cases in the present study, no definite cause was found. Pain preceded the onset of toe movements in 18 cases, but in 2 the reverse sequence occurred. The pain had many of the characteristics of causalgia, but none of the patients exhibited the full picture of reflex sympathetic dystrophy, and peripheral trauma was the trigger in only 5 cases. Several patients reported that the occurrence of toe movements was closely related to the pain, although abolition of pain with lumbar sympathetic blocks was not necessarily associated with disappearance of the movements. Several features suggest a central origin for the movements. Symptoms may begin on one side and become bilateral; movements may be momentarily suppressed by voluntary action or exacerbated by changing posture; and electromyography reveals complex patterns of rhythmic activity with normal recruitment of motor units involving several myotomes. Three other patients with similar moving toes but no pain are also described. The occurrence of similar movements in the absence of pain raises the possibility that these cases represent examples at one end of a spectrum of disorders, with pain alone (causalgia) at the other end and the syndrome of painful legs and moving toes in between.(ABSTRACT TRUNCATED AT 250 WORDS)
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Cardiovascular autonomic function was assessed in 9 subjects with Raynaud's phenomenon. The underlying diseases were systemic lupus erythematosus (n = 5), systemic sclerosis (n = 3) and rheumatoid arthritis (n = 1). Five standard non-invasive tests, 3 of heart rate and 2 of blood pressure, were employed. Compared with age and sex matched controls (n = 25), the number of values abnormal was 24 of 45 (53%) overall and between one and 4 (median, 2) individually. Significant differences were present for 3 tests, two of heart rate and one of blood pressure. The subjects were given triiodothyronine, 60 to 80 micrograms per day, for vasospastic attacks. Autonomic function was reassessed between weeks 4 and 9 (9 subjects) and between weeks 12 and 18 (8 subjects) after introduction of triiodothyronine. Test results showed a considerable improvement. At the second reassessment, the number of values abnormal was now 5 of 40 (12.5%) overall and nil (n = 4) or one (n = 4) individually. Significant differences remained for one heart rate test only. Adverse side effects to triiodothyronine occurred in a single subject and were readily controlled. Evidence of somatic neuropathy was present electrophysiologically in all 9 subjects and clinically in 8. Triiodothyronine may have corrected autonomic dysfunction by increasing blood flow to ischaemic peripheral nerves or by acting on the autonomic system more directly. Further study of triiodothyronine in autonomic insufficiency appears merited.
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