Optic atrophy
| Classification according to ICD-10 | |
|---|---|
| H47.2 | Optic atrophy |
| ICD-10 online (WHO version 2019) | |
The optic atrophy is a degenerative disease of the optic nerve , the away characterized by the loss of nerve cells for much of its route and mostly as a result of state unterschiedlichster disease processes or as a primary disease of the nervous occurs itself. It is therefore an important symptom in many neurological diseases . Optic atrophy is considered irreversible and is a common cause of blindness .
Depending on the localization of the underlying disorder, the optic nerve can atrophy upwards (e.g. in the case of retinal diseases ) and downwards (e.g. in cerebral events) . The degeneration in children and adolescents develops significantly faster than in adults and older people.
causes
In principle, all lesions and diseases of the optic nerve that (cannot) be treated are potential triggers of optic atrophy. These can include:
- an increased intracranial pressure with congestive papilla
- a glaucoma ( glaucoma )
- traumatic lesions on the optic nerve
- an optic neuritis
- decreased blood flow ( ischemia )
- a compression of the nerve, z. B. in the superior orbital fissure syndrome , meningioma of the optic sheath or in tumors, for example in the area of the optic nerve junction
- toxic causes, e.g. from cassava , alcohol or tobacco , but also quinine , arsenic , lead and bromine
In a number of syndromes , optic atrophy is one of the main characteristics:
- 3-methylglutacon aciduria
- Behr syndrome I.
- Biotinidase deficiency
- Chiasm Syndrome
- DIDMOAD syndrome
- Dysosteosclerosis
- Foster Kennedy Syndrome
- GAPO syndrome
- Goltz-Gorlin syndrome
- Infantile Neuroaxonal Dystrophy Seitelberger
- Irvine Gass Syndrome
- Craniodiaphyseal dysplasia
- Leigh syndrome
- Austin metachromatic leukodystrophy
- Mohr-Tranebjaerg syndrome
- Canavan's disease
- Krabbe's disease
- Neuromyelitis optica
- Neural ceroid lipofuscinosis
- Oculo-cerebro-facial syndrome
- Paine Syndrome
- Pyruvate dehydrogenase deficiency
- Rosenberg-Chutorian syndrome
to form
Several forms can be distinguished:
- Simple optic atrophy with sharp atrophy borders, mostly non-inflammatory
- Secondary forms after inflammation
- Incomplete atrophy only in sections, with temporal papilla bleaching
- Hereditary forms ( ADOA , Leber optic atrophy , Arts syndrome , Behr syndrome I , hereditary motor-sensitive neuropathy VI , girdle dystrophy 2O , Tay-Sachs syndrome , Cohen syndrome , metachromatic leukodystrophy )
Symptoms and Diagnosis
The most common symptoms are visual field deficits of varying degrees with increasing loss of central visual acuity up to blindness. In addition, there may be impaired color perception and dark adaptation , as well as occasionally a disorder of the afferent pupillary reaction. Examination of the fundus using ophthalmoscopy usually reveals a pale coloration of the papilla . Further examinations using imaging techniques and VECP are indicated.
The optic nerve hypoplasia is to be adjoined in the differential diagnosis .
Therapy and prognosis
Both the treatment options and the expected course depend on the underlying disease. Once damage has occurred to the optic nerve, it is irreversible. However, if the underlying disease is treated early, further progression of the optic atrophy can possibly be prevented.
See also
Web links
literature
- Franz Grehn: Ophthalmology. 30th edition. Springer Verlag, Berlin 2008, ISBN 978-3-540-75264-6 .
- Th. Axenfeld (conception), H. Pau (ed.): Textbook and atlas of ophthalmology . With the collaboration of R. Sachsenweger and others Gustav Fischer Verlag, Stuttgart 1980, ISBN 3-437-00255-4 .
- Gesa-Astrid Hahn: Short textbook ophthalmology . Georg Thieme Verlag, 2012, ISBN 978-3-13-171471-8 , p. 193.
- Jack J. Kanski: Textbook of Clinical Ophthalmology. 2nd Edition. Thieme, 1996, ISBN 3-13-683402-X .
- Rudolf Sachsenweger: Neuroophthalmology. 3. Edition. Thieme Verlag, Stuttgart 1983, ISBN 3-13-531003-5 , p. 102 ff.
- Pschyrembel clinical dictionary. With clinical syndromes and nouns anatomica. (= Clinical dictionary). Edited by the publisher's dictionary editor under the direction of Christoph Zink. 256th, revised edition. de Gruyter, Berlin et al. 1990, ISBN 3-11-010881-X .
Individual evidence
- ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
