Adee's syndrome
Contents:
Description:
Special form of defeat of an innervation of a pupil (internal ophthalmoplegia) in the form of a unilateral mydriasis (it is frequent with deformation of a pupil) with loss of reaction of a pupil to light (at safe reaction to installation of a look close) and a pupillotoniya.
Symptoms of the Syndrome of Adee:
Sharply, sometimes after a headache, develop a unilateral mydriasis (as a rule, pupillary pathology unilateral) and deterioration in sight because of accommodation paralysis. The most frequent complaint, except an anisocoria, is sight misting close when examining of close located objects. At 35% of patients in each attempt to look close on the affected eye it is possible to provoke an astigmatism (presumably it is connected with segmented paralysis of a ciliary muscle). The mydriatic pupil does not react to light, but is sharply narrowed at convergence. Both narrowing, and especially the subsequent mydriasis happens very slowly (pupillotoniya). Increase in sensitivity of a pupil (muscles of the struck pupil) to vegetotropny poisons is noted (sharp expansion from an atropine instillation, sharp narrowing from Pilocarpinum while their action on diameter of a normal pupil is insignificant) that is caused (in all cases of a disease) by denervatsionny hypersensitivity. In 60% of cases pathology of a pupil is combined with loss of tendon jerks standing, and sometimes and on hands (a total tendinous areflexia). Why at Adee's syndrome tendon jerks – not clearly are weakened. Hypotheses of widespread polyneuropathy without touch disturbances, about a degeneration of fibers of spinal ganglions, a peculiar form of a myopathy, defects of neurotransmission at the level of spinal synapses are assumed. In an acute stage, is more rare in chronic, there are vision disorders caused by accommodation paralysis. Occasionally after a while there are characteristic disturbances and the second pupil (several cases of the syndrome of Adee having bilateral manifestations, according to some information - from 20% to 50% of patients are described) quite often in combination with the progressing anhidrosis and decrease in tendon jerks standing, a hyperthermia. At introduction of parasympathomimetics these patients also have signs of postdenervatsionny hypersensitivity. Eventually both pupils in many years become narrow and badly react to light. Regarding cases the tendency to normalization of pupillary symptoms, is found more often, however, symptoms are stable.
Formal similarity (the dissociated disturbance of pupillary tests) of Adee's syndrome to Argayl Robertson's syndrome characteristic of neurosyphilis is extremely important. As a rule, Argayl Robertson's syndrome happens bilateral, and, above all, at it there is no pupillotoniya phenomenon. Unlike Argayl Robertson's syndrome at Adee's syndrome the size of a pupil is changeable and changes during the day. The picture, similar to Adee's syndrome, is observed after the infections or injuries affecting a ciliary ganglion in an orbit. In these cases the disease arises sharply, at the beginning of a disease accommodation paralysis is noted that is shown by passing deterioration in sight. The stupid injury of an iris can lead to a rupture of short ciliary branches in a sclera that klinichsk is shown by deformation of pupils, their expansion and disturbance (easing) of a photoharmose. It received the name – a posttraumatic iridoplegia. Tonic reaction of pupils is also observed at Shai-Dreydzhera's syndrome, a segmented hypohydrosis, a diabetes mellitus and an amyloidosis. Ciliary nerves can be surprised at diphtheria, leading to expansion of pupils. Usually it occurs on the 2nd – the 3rd week of a disease and is often combined with paresis of a soft palate. Dysfunction of pupils at diphtheria, as a rule, completely stops. Sometimes the dissociated disturbance of pupillary tests accidentally comes to light at healthy people. For confirmation of the diagnosis enter a drop into each eye divorced (0,125%) Pilocarpinum. The pupil of the affected eye is narrowed (a phenomenon of increase in sensitivity of denervated structures), and normal - does not react.
Reasons of the Syndrome of Adee:
The etiology is unknown. So far to localize the defect concerning an anhidrosis, very difficult. Most likely, defeat of ganglion cilliare (a ciliary node) which is in an eye-socket or postganglionic defeat of parasympathetic pupillary fibers is the cornerstone of pupillary disturbances. But recently it was established that dissociation of reaction of a pupil to light and accommodation can be explained only with diffusion of acetylcholine from a ciliary muscle in the back camera towards a denervated sphincter of a pupil. It is quite probable that diffusion of acetylcholine in watery moisture sposobstvt tensions of movements of an iris at Adee's syndrome, however also the fact that the mentioned dissociation cannot be explained so unambiguously is rather clear. The expressed reaction of a pupil to accommodation, most likely, is caused by pathological regeneration of akkomodatsionny fibers in a pupil sphincter. Surprising ability to regeneration and reinervtion is inherent in nerves of an iris: fruit heart of a rat, pereazhenny in an anterior chamber of an eye of an adult individual, will grow and be reduced in a usual rhythm which can change depending on rhythmic stimulation of a retina. Nerves of a radzhka can grow into heart and set ChSS. In the majority of cases Adee's syndrome is an idiopathic disease. Infections and avitaminosis can be an etiology, however in most cases the etiological moment does not manage to be found out. To syphilis Adee's syndrome of the relation has no. Adee's syndrome can be display of vegetative dysfunction and in rare instances be combined with the progressing generalized anhidrosis, ortostatichesy hypotension, diarrhea, a lock, impotence, local vascular disorders. It is only possible to note that the combination of a syndrome of Adee to an anhidrosis and a hyperthermia – can have homogeny and is a sign of a partial dizavtonomiya. It an image, Adee's syndrome can act as a symptom at a certain stage of development of the progressing vegetative insufficiency, and can sometimes be its first manifestation. Much more often Adee's syndrome is observed at women. The disease in most cases arises in the third decade of life (average age of a disease 32 years). Family cases of a disease are described.
Treatment of the Syndrome of Adee:
Treatment is not developed. At idiopathic forms it is not required. For correction of cosmetic defect it is possible to try an instillation of weak solutions of Pilocarpinum.
