Legacy and current understanding of the often-misunderstood Foix-Alajouanine syndrome. Historical vignette.

Foix-Alajouanine syndrome has become a well-known entity since its initial report in 1926. The traditional understanding of this clinical syndrome is as a progressive spinal cord venous thrombosis related to a spinal vascular lesion, resulting in necrotic myelopathy. However, spinal venous thrombosis is extremely rare and not a feature of any common spinal vascular syndrome.

Venous congestive myelopathy of the cervical spinal cord: an autopsy case showing a rapidly progressive clinical course.

We report a rapidly progressive myelopathy in a 74-year-old Japanese man who was admitted to our hospital with a 4-month history of progressive gait disturbance and died of pneumonia followed by respiratory failure on the 22nd day of admission. During the course of his illness, magnetic resonance imaging (MRI) revealed intramedullary lesions with edematous swelling from the medulla oblongata to the spinal cord at the level of the fourth vertebra. After administration of contrast medium, the ventral portion of the lesion was mildly and irregularly enhanced and a dilated vessel was recognized along the ventral surface of the upper cervical cord.

Foix-Alajouanine syndrome: case report.

In a 52-year-old woman, spinal arteriovenous malformation (AVM) has been associated with what has been known as Foix-Alajouanine syndrome. The pathophysiology of the AV fistula is probably related to increased venous pressure from the AVM plus thrombotic process. The most common initial symptoms are sensory disturbance, pain and leg weakness.

Foix-Alajouanine syndrome: an uncommon cause of myelopathy from an anatomic variant circulation.

Foix-Alajouanine syndrome is a rare cause of myelopathy caused by dural arteriovenous malformation of the spinal cord, mostly lower thoracic and lumbar. Patients are usually over 50 years of age and can present with acute lower extremity dysesthesias or intermittent sciatica. Progression to paraplegia may be slow.

[A 63-year-old man with progressive cauda equina/conus medullaris syndrome].

A 63-year-old man, whose father died of malignant lymphoma, developed subacutely cauda equina/conus medullaris syndrome progressed over 3 months. Initial radicular pain, ascending motor and sensory paralysis without sacral sparing, vesicorectal dysfunction were similar with signs of spinal dural arteriovenous fistula. However, mild inflammatory signs, raised serum LDH, predominantly of LDH 3, lymphocytic pleocytosis and elevated beta 2 microglobulin in CSF suggested neurolymphomatosis.

Dementia resulting from dural arteriovenous fistulas: the pathologic findings of venous hypertensive encephalopathy.

Purpose: Dural arteriovenous fistulas (DAVFs) are acquired arteriovenous shunts located within the dura. The highly variable natural history and symptomatology of DAVFs range from subjective bruit to intracranial hemorrhage and are related to the lesion's pattern of venous drainage and its effect on the drainage of adjacent brain. We examined the prevalence and features of DAVFs in patients with progressive dementia or encephalopathy.

[Congestive myelopathy caused by spinal dural arteriovenous fistulas. Anamnesis, clinical aspects, diagnosis, therapy and prognosis].

Congestive myelopathy, formerly referred to as varicosis spinalis or Foix-Alajouanine syndrome, is caused by a spinal dural arteriovenous fistula (SDAVF). So far, the blood supply from the meningeal arteries draining through the fistula into the medullary venous system can only be verified by spinal angiography. Patients predominantly male and over the age of 60 are afflicted.

Unusual intramedullary vascular lesion: report of two cases.

Objective And Importance: Spinal arteriovenous malformations have been divided by location into dural (Type I), intramedullary glomus (Type II), juvenile (Type III), and perimedullary direct arteriovenous fistulae (Type IV). We report two cases of an unusual intramedullary proliferation of hyalinized capillaries that do not fit into any of these categories.

Clinical Presentation: A 27-year-old woman and a 62-year-old man presented with subacute progressive caudal myelopathy.

[The cerebellar cortical atrophy syndrome].

The results of the observation of 44 patients with cerebellar syndrome of different etiology are presented. Together with careful study of anamnestic and clinical data some additional examinations were performed: senso- and pallesthesiometry, thermovisional investigation, vestibulometry, electroencephalo-, electromyography, computer tomography. The study allowed to reveal both the cause of the disease and to refer etiologically late cerebellar cortical ataxia to alcohol factor.

Case report: spontaneous thrombosis of a spinal dural AVM (Foix-Alajouanine syndrome)--magnetic resonance appearance.

We describe a patient with a classical presentation of a spinal dural arteriovenous malformation which probably underwent spontaneous thrombosis. This is known as the Foix-Alajouanine syndrome. The diagnosis was not made in this patient until after cord infarction had occurred.

An autopsy case of spinal arteriovenous malformation (Foix-Alajouanine syndrome).

An autopsy case of spinal arteriovenous malformation (AVM) was reported. The patient was a 75-year-old male and his initial neurologic symptoms were paraplegia, paresthesia below the umbilical level and urination difficulty. Subsequently night delirium and parkinsonism also appeared.

[Angiodysgenetic necrotizing myelopathy (the Foix-Alajouanine syndrome)].

One observation of a rare disease--angiodysgenetic necrotizing myelopathy--in a 50-year-old patient is described. The diagnosis was established at necropsy and did not confirm suspected spinal cord tumor and myelitis. The disease is angiodysplasia with resulting necrotizing myelopathy.

[Spinal angiomatous malformation: clinical course and limits of technical diagnosis].

The sensitivity of the diagnostic procedures in space occupying lesions of the spine have been markedly improved in the recent past, mainly due to the application of CT and MRI--technology and the introduction of superselective angiography. Despite these 'high-tech'-investigations, neurological examination still provides the sole in neurological diagnosis. To show the discrepancies between the techniques mentioned, we present a case of a vascular malformation of the spine with secondary myelopathy (so-called "Foix-Alajouanine-syndrome").

Reversible acute and subacute myelopathy in patients with dural arteriovenous fistulas. Foix-Alajouanine syndrome reconsidered.

Acute or subacute neurological deterioration without evidence of hemorrhage in a patient with a spinal arteriovenous (AV) malformation has been referred to as "Foix-Alajouanine syndrome." This clinical entity has been considered to be the result of progressive vascular thrombosis resulting in a necrotic myelopathy; it has therefore been thought to be largely irreversible and hence untreatable. The authors report five patients with dural AV fistulas who presented in this manner, and who improved substantially after embolic and surgical therapy.

Myelopathy due to intracranial dural arteriovenous fistulas draining intrathecally into spinal medullary veins. Report of three cases.

Arteriovenous malformations (AVM's) of the spine commonly cause progressive myelopathy. Occasionally, myelography reveals serpentine filling defects characteristic of a spinal AVM, but an AVM or arteriovenous (AV) fistula cannot be demonstrated arteriographically, despite selective catheterization of all vessels known to have the potential of supplying the spinal cord and spinal dura. Often, and particularly in the setting of subacute or acute deterioration, this has been attributed to spontaneous thrombosis of the veins (the Foix-Alajouanine syndrome).

Vascular malformations of the central nervous system: a morphological overview.

Vascular malformations of the central nervous system (C.N.S.

[Foix-Alajouanine disease in childhood (clinico-morphologic case)].

It is the first report in the Soviet literature on Foix-Alajouanine's disease in childhood. The disease is marked by congenital multiple angiomatosis of the spinal vessels attended by gliosis and cavity formation. A thorough morphological analysis of the case was performed.

A case of necrotic myelopathy due to a spinal vascular malformation (Foix-Alajouanine's disease).

A woman of 65 years, after a period of painful paraesthesiae in the legs, developed a polyneuritic syndrome and also sphincter disturbance. At first the c.s.