Cogan syndrome
As oculomotor apraxia (also: Cogan's syndrome II or COMA ) is defined as the inability to put eye movements for fixation recording, so-called eye target movements in transition. Spontaneous rapid eye movements ( saccades ) may be obtained, as well as the ability to trigger the optokinetic and vestibular nystagmus. The disease was first described in 1952 by the U.S. ophthalmologist David G. Cogan.
Symptoms
The oculomotor apraxia is very common congenital. In most cases, the horizontal eye movements are affected, whereas in the acquired variant, the vertical viewing target movements are disturbed. In infants, especially the gaze is striking. Later it comes to a characteristic movement of the spin head. Here, this is jerky out on turns only in the desired direction, the eyes are advised by the mechanism of vestibulo-ocular reflex to the mutual attack and virtually " taken ". Thereafter, the head is moved back a little and the eyes are subject to the stick to be considered. Depending on the desired look direction, the spin head movements can turn horizontally or vertically.
Often, the children also suffer from cerebellar ataxia and motor development delays.
Cause
The pathogenesis of congenital disorder is still unclear. In acquired oculomotor apraxia, which occurs much less frequently, a neurological inspection in each case is carried out to clarify supranuclear lesions. In acquired forms a number of other diseases also delineate the differential diagnosis.
Treatment and prognosis
The oculomotor apraxia can improve by itself until age 20 spontaneously. Adults need to supply only a small movement of the head, which is necessary to bring the eye movement in the passage. But already at school age, improvements can set.
Demarcation
In contrast to the one here Cogan's syndrome II, the oculomotor apraxia, a Cogan I syndrome is known to be an autoimmune disease with involvement of eyes and ears. The only common feature of these two diseases is their first describer the U.S. ophthalmologists David. G. Cogan.