Oculomotor apraxia

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Classification according to ICD-10
H51.8 Other specified disorders of eye movements
ICD-10 online (WHO version 2019)

As oculomotor apraxia (also: Cogan II syndrome or COMA ) is defined as the inability to eye movements to fixation recording , so-called eye target moves to set in motion. Spontaneous rapid eye movements ( saccades ) can be preserved, as well as the possibility of triggering the optokinetic and vestibular nystagmus . The clinical picture was first described in 1952 by the US ophthalmologist David G. Cogan .

Symptoms

The oculomotor apraxia is very common congenital. The horizontal eye movements are mostly affected, while in the acquired variant the vertical eye target movements are also disturbed. The rigid gaze is particularly noticeable in babies. Later there is a characteristic slinging movement of the head. Here, this is a jerk out only in the desired direction overturned , with the eyes through the mechanism of vestibuloocular reflex will come to the mutual stop and "taken" quasi. Then the head is moved back a little and the eyes remain attached to the object that is to be viewed. Depending on the desired direction of view, the head-flinging movements can be horizontal or vertical.

Often the children also suffer from cerebellar ataxia and motor developmental delays .

root cause

The pathogenesis for the congenital disorder is still unclear. In acquired oculomotor apraxia, which is much less common, a neurological inspection should be performed in any case to clarify supranuclear lesions. In the case of acquired forms, a number of other clinical pictures must also be differentiated diagnostically.

Therapy and prognosis

The oculomotor apraxia may improve age 20 spontaneously self. Adults sometimes only need a small head movement, which is necessary to get the eye movement going. But improvements can also occur at school age.

Demarcation

In contrast to the Cogan II syndrome dealt with here , oculomotor apraxia , there is also known Cogan I syndrome , an autoimmune disease that affects the eyes and ears . The only thing these two clinical pictures have in common is the first person to describe them, the US ophthalmologist David. G. Cogan.

Web links

  • M. Steinlin, L. Thun-Hohenstein, E. Boltshauser: Congenital oculomotor apraxia - presentation - developmental problems - differential diagnosis. In: Clinical monthly sheets for ophthalmology. 200, 1992, pp. 623-625, doi : 10.1055 / s-2008-1045846 .

literature

  • Herbert Kaufmann (Ed.): Strabismus. With the collaboration of Wilfried de Decker et al. Enke, Stuttgart 1986, ISBN 3-432-95391-7 .

Individual evidence

  1. Commission "Guidelines of the German Society for Neurology" (Ed.): Guidelines for Diagnostics and Therapy in Neurology. 3rd, revised edition. Georg Thieme, Stuttgart et al. 2005, ISBN 3-13-132413-9 , keyword: Peripheral eye muscle and nerve paresis ; AWMF guidelines register: No. 030/033.