Calcified cervical disc in a three year old.
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Biomedical subjects
Publications and source records attributed to J Acheson.
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Patients with hereditary apolipoprotein AI (apoAI) amyloidosis often have extensive visceral amyloid deposits, and many develop end-stage renal failure as young adults. Solid organ transplantation to replace failing organ function in systemic amyloidosis is controversial due to the multisystem and progressive nature of the disease and the risk of recurrence of amyloid in the graft. We report the outcome of solid organ transplantation, including dual transplants in 4 cases, among 10 patients with apoAI amyloidosis who were followed for a median (range) of 16 (4-28) and 9 (0.2-27) years from diagnosis of amyloidosis and transplantation, respectively. Eight of 10 patients were alive, seven with a functioning graft at censor. Two patients died, one of disseminated cytomegalovirus infection 2 months after renal transplantation and the other of multisystem failure following severe trauma more than 13 years after renal transplantation. The renal transplant of one patient failed due to recurrence of amyloid after 25 years. Amyloid disease progression was very slow and the natural history of the condition was favorably altered in both cases in which the liver was transplanted. Failing organs in hereditary apoAI amyloidosis should be replaced since graft survival is excellent and confers substantial survival benefit.
PURPOSE: To investigate changes in visual motion perception after treatment with botulinum toxin in patients with unilateral chronic lateral rectus muscle palsy. METHODS: Five patients and control subjects were asked to report the perceived drift direction of a sinusoidal grating that was initially stationary and then began to accelerate at 0.09 degrees /sec2 in a horizontal direction. The grating had a field size of 18.5 degrees and was presented monocularly with a contrast just above threshold for visibility for central vision. Both the paretic and non-affected eyes were tested. Psychophysical testing was performed under the following conditions: 1) before treatment and testing, patients occluded their paretic eye for at least three days to avoid diplopia. 2) After treatment with botulinum toxin, alignment was corrected and patients stopped occluding their paretic eye for at least three days before testing. The control subjects occluded their non-dominant eye for three days before testing. RESULTS: In condition 1, no differences in motion detection values between patients and control subjects were found. In condition 2, motion detection thresholds were raised approximately 0.15 degrees /sec as compared to pre-treatment values and compared to the control group. CONCLUSIONS: After treatment, a raised threshold for motion detection is one mechanism used to avoid oscillopsia and visuo-vestibular disorientation during head movements in patients with chronic paralytic squint. This study lends evidence that perceptual-adaptive, compensatory mechanisms develop to reduce oscillopsia and disorientation rather than being caused by abnormal cortical motion processing or defective eye muscle action.
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Acute urinary retention in women is uncommon. A 63 year old woman presented with suprapubic pain, a palpable bladder, and multiple grouped vesicles on the right buttock. Catheterisation showed a residual of 2000 ml. A case is reported of acute urinary retention secondary to herpes zoster infection of the sacral nerves (S2-4).
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This review of optic nerve and chiasmal disease briefly outlines the clinical assessment and the use of diagnostic testing in the topical diagnosis of lesions of the anterior visual pathways. The commoner pathological entities including inflammatory, vascular, heredofamilial and compressive lesions are then summarised with specific reference to important points in the diagnosis and management. Specific disorders described include optic neuritis, papilloedema, ischaemic optic neuropathy, giant cell arteritis, Leber's hereditary optic neuropathy and pituitary tumours.
BACKGROUND: Chronic infection with the hepatitis C virus has been reported to cause a wide variety of ophthalmic lesions. The incidence and significance of these lesions in an unselected population has not been assessed. METHODS: We studied a group of unselected patients with chronic hepatitis C and performed a full ophthalmic examination on each. As a control group we studied patients with chronic hepatitis B infection. RESULTS: In 25 patients with chronic hepatitis C we found no increase in the prevalence of significant ocular disease when compared with a cohort of patients with chronic hepatitis B. CONCLUSION: Chronic heptatitis C does not cause any marked increase in the incidence of ocular disease.
The peptidergic sensory innervation of cranial blood vessels may play an important part in vascular head pain. The neuropeptides calcitonin gene-related peptide (CGRP) and substance P in sensory fibres are dependent on nerve growth factor (NGF) produced by the blood vessels, and when released from nerve terminals mediate neurogenic inflammation. NGF is increased in inflamed tissues, and acts via its high affinity receptor trk A on nociceptor fibres to produce hyperalgesia. CGRP and trk A immunoreactive nerve fibres have therefore been studied, for the first time, in inflamed (n=7) and non-inflamed (n=10) temporal arteries biopsied from patients with headache and suspected giant cell arteritis. CGRP immunoreactivity was markedly decreased to absent in adventitial nerve fibres in inflamed regions of vessels, which may reflect secretion from nerve terminals, as CGRP immunoreactivity could still be seen in nerve trunks in periadventitial tissue. Trk A immunoreactive nerve fibres were found in a similar distribution to CGRP containing nerve fibres in non-inflamed vessels, and the trk A immunoreactivity was virtually unchanged in inflamed vessels. The evidence supports a role for NGF related mechanisms in inflammatory vascular head pain. Anti-NGF or anti-trk A agents may represent novel analgesics in this condition.
Iris mammillations are rarely described, distinctive villiform protuberances that can cover the iris. In the majority of reported cases they are unilateral and sporadic, and are seen in association with oculodermal melanosis. In past literature and current clinical practice they are frequently confused with the iris nodules seen in neurofibromatosis type 1. Their clinical significance is not established, although it has been suggested that iris mammillations may be an external sign of ocular hypertension or intraocular malignancy. We report a series of 9 patients between the ages of 3 and 28 years with iris mammillations. The mammillations appear as regularly spaced, deep brown, smooth, conical elevations on the iris, of uniform height or increasing in height as the pupil margin is approached. They often overlie a naevus or an exceptionally deeply pigmented iris, such as that seen in melanosis oculi. One case had an associated ciliary body mass. They tend to occur in more highly pigmented ethnic groups and can be dominantly inherited. Iris mammillations may occur in association with systemic conditions including phakomatosis pigmentovascularis type IIb and neurofibromatosis type 1 when they may even coexist with iris hamartomas.
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A controlled double-blind study of the effect of dipyridamole was performed in 169 patients with established cerebral vascular disease. A dose of 400 mg. was used initially, given daily for an average of 14 months; the dose was then increased to 800 mg. daily for a further average period of 11 months. When the incidence of cerebral ischaemic episodes during treatment was compared in the drug-treated and placebo-treated groups no significant difference was found.
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