Foix–Alajouanine syndrome, also called subacute ascending necrotizing myelitis, is a disease caused by an

arteriovenous malformation Arteriovenous malformation is an abnormal connection between arteries and veins, bypassing the capillary system. This vascular anomaly is widely known because of its occurrence in the central nervous system (usually cerebral AVM), but can appea ...

of the

spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spi ...

. In particular, most cases involve dural arteriovenous malformations that present in the lower thoracic or lumbar spinal cord. The condition is named after

Charles Foix Charles Foix (; 1 February 1882 – 22 March 1927) was a French internist and neurologist. Charles Foix was born in Salies-de-Béarn, Pyrénées-Atlantiques. He studied medicine at the University of Paris and was a pupil of Pierre Marie at the S ...

and

Théophile Alajouanine Théophile Alajouanine (; 12 June 1890 – 2 May 1980) was a French neurologist. Théophile Alajouanine was born in Verneix, Allier. He was a student of Joseph Jules Dejerine and a colleague of Georges Guillain and Charles Foix. He was a pro ...

who first described the condition in 1926.

Signs and symptoms

The patients can present with symptoms indicating spinal cord involvement such as (

paralysis Paralysis (also known as plegia) is a loss of motor function in one or more muscles. Paralysis can also be accompanied by a loss of feeling (sensory loss) in the affected area if there is sensory damage. In the United States, roughly 1 in 50 ...

of arms and legs, numbness and loss of sensation and

sphincter A sphincter is a circular muscle that normally maintains constriction of a natural body passage or orifice and which relaxes as required by normal physiological functioning. Sphincters are found in many animals. There are over 60 types in the hum ...

dysfunction), and pathological examination reveals disseminated

nerve cell A neuron, neurone, or nerve cell is an electrically excitable cell that communicates with other cells via specialized connections called synapses. The neuron is the main component of nervous tissue in all animals except sponges and placozoa. No ...

death in the spinal cord.

Diagnosis

Clinically, the patient may present with neurological symptoms such as numbness, weakness, loss of reflexes, or even sudden or progressive paralysis. The affected portion of the body will correlate to where the lesion lies within the spinal cord. The disease typically has an insidious onset, but symptoms may manifest suddenly. A thorough physical exam may lead a physician toward targeted imaging, with

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...

being the most appropriate imaging modality for initial diagnosis. A spinal MRA will serve as a superior imaging technique to visualize the extent of the arteriovenous malformation within the cord and may be especially useful if surgical treatment is attempted.

Treatment

Surgical treatment may be attempted with

endovascular embolization Interventional radiology (IR) is a medical specialty that performs various minimally-invasive procedures using medical imaging guidance, such as x-ray fluoroscopy, computed tomography, magnetic resonance imaging, or ultrasound. IR performs both ...

or ligation of the

within the spinal cord.

Corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involv ...

s may be used acutely to help slow the progression of symptoms or they may be used chronically in a poor surgical candidate. In either case,

physical therapy Physical therapy (PT), also known as physiotherapy, is one of the allied health professions. It is provided by physical therapists who promote, maintain, or restore health through physical examination, diagnosis, management, prognosis, patient ...

will be an important part of the recovery process in helping the patient regain strength and coordination.

References

External links

* * Spinal cord disorders Syndromes affecting the nervous system {{Nervoussystem-disease-stub

Signs and symptoms

Diagnosis

Treatment

See also

References

External links