Foix-Alajouanine Syndrome

Classification according to ICD-10
G37.4	Subacute necrotizing myelitis [Foix-Alajouanine syndrome]
ICD-10 online (WHO version 2019)

The Foix-Alajouanine syndrome describes a clinical picture with neurological failures due to congenital malformations of the spinal vessels.

Synonyms are:

Alajouanine Foix Syndrome
Posterior spinal vascular syndrome
Angioma racemosum of the spinal cord
Subacute necrotizing myelitis
Myelopathia necrotisans
Vascular myelopathy
Spinal varicosis

The name is named after the first describe it in 1926, the French neurologists Charles Foix and Théophile Alajouanine .

distribution

The male sex is affected somewhat more often, the disease usually occurs later in life.

root cause

The disease is caused by congenital malformations of the vessels supplying the spinal cord , mostly arteriovenous short-circuit connections.

Clinical manifestations

Initially, unspecific complaints appear in the lumbar region, followed by cauda equina symptoms . The initially changing and reversible complaints turn into permanent disorders, mainly of the ability to walk.

Investigation methods

In the liquor there is an increase in protein without pleocytosis .

diagnosis

Whereas in the past these AV malformations had to be detected angiographically , a search using magnetic resonance tomography is now possible at an early stage .

therapy

Treatment can be by embolization or surgery .

Prospect of healing

The prognosis depends on the time of discovery and treatment.

literature

A. Buchholz, U. Müller: Angiodysgenic necrotizing myelopathy (Foix-Alajouanine). In: Psychiatry, Neurology, and Medical Psychology. Volume 25, Number 11, November 1973, pp. 661-667, ISSN 0033-2739 . PMID 4778471 .

Individual evidence

↑ ^a ^b ^c B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger, 7th edition. Urban & Schwarzenberg 1990, ISBN 3-541-01727-9
^ C. Foix, T. Alajouanine: La myélite nécrotique subaiguë (Myélite centrale angiohypertrophique à évolution progressive). Paraplégie amyotrophique lentement ascendante d'abord spasmodique, puis flasque. Revue neurologique, Paris, 1926, 2, pp. 1-42.
^ ^A ^b ^c C. Koch, HC Hansen, M. Westphal, T. Kucinski, H. Zeumer: The congestive myelopathy through spinal dural arteriovenous fistulas. In: The neurologist. Volume 69, Number 4, April 1998, pp. 279-286, ISSN 0028-2804 . PMID 9606677 .

[Leiber-1] B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger, 7th edition. Urban & Schwarzenberg 1990, ISBN 3-541-01727-9

[2] C. Foix, T. Alajouanine: La myélite nécrotique subaiguë (Myélite centrale angiohypertrophique à évolution progressive). Paraplégie amyotrophique lentement ascendante d'abord spasmodique, puis flasque. Revue neurologique, Paris, 1926, 2, pp. 1-42.

[Koch-3] A ^b ^c C. Koch, HC Hansen, M. Westphal, T. Kucinski, H. Zeumer: The congestive myelopathy through spinal dural arteriovenous fistulas. In: The neurologist. Volume 69, Number 4, April 1998, pp. 279-286, ISSN 0028-2804 . PMID 9606677 .