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Holmes-Adie syndrome
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求一篇英文文献的汉语翻译，急！要标准！ Holmes-Adie syndrome Paolo Martinelli Holmes-Adie syndrome The Holmes-Adie syndrome is characterised by initially monolateral, then bilateral, enlargement of the pupil with delayed responses to the near vision effort, with delayed redilation. Deep tendon reflexes are absent. The condition was described in two separate reports in 1931.1,2 The clinical features of this case were strongly suggestive of Holmes-Adie Syndrome (HAS). The yearly incidence of this condition has been estimated at 4·7 per 100 000. It is more common in women, with a wide variation in age of onset with a peak incidence in the third decade of life. HAS is usually sporadic, although familial cases have been rarely reported.3–5 The tonic pupil of patients with this syndrome is initially monolateral, but frequently progresses to involve the other eye. Onset is usually gradual and is often noticed by an observer rather than by the patient. On slit lamp examination the pupillary sphincter muscle shows spontaneous movements in some segments that appear worm-like—so-called “iris streaming”. With near effort, segmental palsies of the sphincter may be apparent, with the result that the pupil is not round, but oval shaped, or showing some irregularity. The diameter of the affected pupil may fluctuate, and with time it shows a tendency to become smaller than the contralateral pupil. Immediate pupillary reaction to light is absent, but a slow constriction may occur after 5–10 min exposure to a bright light, and an extremely gradual dilation follows after 15–30 min in a dark room. When the other eye is involved, the appearance of irregular pupils with dissociation between the reaction to light and accommodation mimics Argyll-Robertson pupils.3,4,6 The tonic reaction of the pupil to near vision is delayed, as is subsequent redilation; this accounts for the complaint of blurred vision by some patients with HAS when they change from near to far vision. The greater impairment of reaction to light than to accommodation is explained by the 30-fold excess of neurons to the ciliary body compared with those to the iris sphincter. After lesions in the ciliary ganglion, as in HAS, most accommodative fibres regenerate, albeit with irregular aberrant collateral sprouting to the denervated iris sphincter which results in vermiform movements and a slow pupillary contraction at near vision. With time, a partial recovery of accommodation may occur. In about 10% of cases there is permanent failure of the pupil to react either to light or to near vision. The tonic pupil reacts normally to cocaine, adrenalin, and homatropine, whereas instillation of dilute pilocarpine solution is followed by substantial constriction of the affected pupil, because of denervation hypersensitivity of the iris sphincter.3,4,6 Very few neuropathological examinations are available for patients with this syndrome, but reported findings are consistent, with a partial or total loss of neurons of the ciliary ganglion on the same side as the tonic pupil. In addition to neuronal loss, peripheral glial cell accumulations—forming residual nodules of Nageotte type—are present. The sphincter muscle has been found to be absent in some quadrants or thin in others. The cause of the neuronal degeneration is not known, but the absence of scarring or inflammatory cells and the persistence of myelinated fibres passing through the ganglion, suggests it is unlikely to be the result of infection of conventional bacterial or viral origin or other inflammatory disease.7 The absence of deep tendon reflexes is also characteristic of HAS, with loss of the Achilles tendon reflex being most frequent. The areflexia is acquired, first monolateral, then bilateral, with a bilateralisation rate of 4% per year. Once the areflexia is established, it is permanent. The tonic pupil and loss of tendon reflexes do not tend to be simultaneous, and the possibility of observing both depends on the time of recognition in the course of the disease. No longitudinal electrophysiological studies are available but the observation of tonic pupil and preserved Achilles’ tendon reflexes, leading to later areflexia is suggestive of a progressive disease. Monosynaptic reflex activity assessment has shown that the Achilles’ reflex is absent in most cases, and the electrically evoked H reflex of the soleus muscle is replaced by an attenuated response. Further, the polysynaptic spinal pathways seem to behave normally in patients with this syndrome. Peripheral motor and sensory nerve conduction velocities are normal and the presence of a tonic vibratory reflex suggests that defective conduction in Ia peripheral afferent fibres does not cause the areflexia. The attenuated response to supramaximal motor nerve stimulation has been considered an indirect index of impaired transmission of Ia afferents to motor neurons in their proximal part.5 Morphological examination of the lumbar and thoracic ganglia have shown a decreased numbers of nerve cells in patients with HAS and there is evidence of nerve-cell destruction, loss of myelin sheaths in the posterior roots and in the medial part of the posterior funiculi of the spinal cord, but without any substantial change in grey or white matter.7 Thus, electrophysiological and anatomical data suggest that the pathophysiology of areflexia is the result of impaired spinal monosynaptic connections, probably due to structural damage of Ia afferents to motor neurons in their proximal tracts. Histological features common to both affected ciliary and spinal ganglia (spiralling of the stainable part of the proximal axon, neuronophagias, and residual bodies of Nageotte’s type) support the concept of HAS as a progressive disease.7 The association between HAS and more widespread autonomic dysfunction remains controversial. Single case reports or series of HAS have been reported, associated with sweating or cardiovascular dysfunction, diarrhoea, and coughing. Tonic pupil, areflexia, and anhydrosis are frequently recognised in published work as Ross’s syndrome. In these cases the tonic pupil is more often bilateral, whereas the loss of flushing and sweating has been detected in a different pattern distribution: segmental, following a dermatomeric distribution, or localised to half of the body. The possible link between HAS and Ross’s syndrome remains ill-defined, since abnormalities of sweating in a high proportion of HAS patients have been reported. The finding of cholinergic supersensitivity in the iris muscles of patients with the Harlequin syndrome (a syndrome of local autonomic failure affecting the face and the arm) may also indicate that Harlequin syndrome is linked with HAS. Cardiovascular system dysfunctions are not always associated with the sweating abnormalities, but they are more prevalent with increasing duration of the condition. HAS may also be detected in a symptomatic form during the course of different diseases of the nervous system, most frequently polyneuropathies. Hereditary, inflammatory, paraneoplastic, diabetic, amyloidotic, and alcohol-related neuropathies can all be complicated by HAS, as can those associated with Sjøgren’s syndrome, rheumatoid arthritis, and temporal arthritis. Sporadic reports have described an association with myopathy, as well as with migraine. Historical notes Sir Gordon Morgan Holmes was born in Castlebellingham, Ireland in 1876 and gained his MD with a gold metal at Trinity College, Dublin, Ireland. After being resident medical officer at the Richmond Hospital, Dublin for a short period, he then moved to the Anatomical Institute of Frankfurt to study human and animal comparative anatomy of the nervous system. 2-years later Holmes came to England and turned to neurological medicine. He covered, successively, the roles of resident medical officer, pathologist, and finally physician to the National Hospital in London. Holmes’ interests covered a wide range of clinical manifestations and morbid anatomical correlates of the diseases of the nervous system, with a special interest in cerebellar defects. A comprehensive study of cerebellar tumours was made by Holmes and Stewart in the first years of the century, and the study and analysis of cerebellar defects was amplified by the observations made during the First World War on acute injuries from gunshot wounds. Sir Gordon Morgan Holmes died on Dec 29, 1965. The syndrome of partial iridoplegia associated with symptoms of other diseases in the nervous system was described by Holmes in 1931, and named Holmes-Adie syndrome by Bramwell in 1936. William John Adie was born at Geelong, Australia, in 1886 and graduated at Edinburgh in 1911. During the First World War he served as medical officer and went to France with the British Forces. At the end of the war Adie became medical registrar at Charing Cross Hospital and in the following years obtained higher qualifications: MRCP in 1919, MD with gold medal in 1920, and was elected FRCP in 1925. During his medical career, Adie served on the staff of several hospitals in London: the National Hospital, the Royal London Ophthalmic Hospital, and Mount Vernon Hospital. A careful clinical observation followed by the ability to summarise a complex problem to the core, was the basis of Adie’s work. His neurological scientific production covered different subjects—eg, pituitary tumours, disseminated sclerosis, and idiopathic narcolepsy. Adie published his account of the association of tonic pupil and absent tendon reflexes in the same year as Holmes, but there has been considerable dispute about the priority of his report, and whether his name should be attached to the disorder.7 He died in March, 1935. |
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