At current presentation, he was unable to stand, even with support. Bilateral foot drop was present. Right lower limb power was grade 3/5 in hip flexors and knee extensors and 2/5 in knee flexors, ankle plantar flexors, and hip extensors. [ncbi.nlm.nih.gov]

Foix-Alajouanine syndrome refers to presentation of spinal AVMs with progressive neurological deterioration. Initially, patients have a spastic paraplegia which progresses to flaccidity, loss of sphincter control and ascending sensory level. [radiopaedia.org]

Owing to the rarity of this syndrome and the varied clinical presentations, multidisciplinary teams may best serve the patient. [emedicine.medscape.com]

The skin lesions of Cobb syndrome typically are present as port wine stains or angiomas, but reports exist of angiokeratomas, angiolipomas, and lymphangioma circumscriptum. [dixion-healthcare.com]

A translation and review of the original 42-page French report revealed 2 young men who had presented with progressive and unrelenting myelopathy ultimately leading to their deaths. [thejns.org]

• Hyperthermia

  There are no indications that malignant hyperthermia occurs; however, avoiding trigger anesthetic agents during the acute phase of neuromuscular degeneration is recommended. [accessanesthesiology.mhmedical.com]

• Choking

  Another proposed mechanism is that remittent leakage of blood from the arteriovenous malformation promotes progressive adhesive arachnoiditis resulting in iterative “chokes” of the spinal cord. [accessanesthesiology.mhmedical.com]

• Leg Pain

  His dysesthesias and leg and gluteal pain also disappeared. A repeat MRI done at 5 months follow-up showed disappearance of flow voids as well as cord T 2 hyperintensity [ Figure 4 ]. [ncbi.nlm.nih.gov]

• Buttock Pain

  This affliction of the lower part of the cord leads to the commonly observed symptoms of dysesthesias, non-dermatomal sensory loss, walking difficulty, wasting, weakness of lower limbs, buttock pain, and sphincter dysfunction.[ 1, 2, 3 ] Often this condition [ncbi.nlm.nih.gov]

• Flaccid Paralysis

  Affected patients initially are spastic but eventually develop flaccid paralysis of the limbs and may become wheelchair bound. [accessanesthesiology.mhmedical.com]

  Prognosis Foix-Alajouanine syndrome is a subacute disorder that gradually evolves over 1-5 years. [2] Affected patients initially are spastic but eventually develop flaccid paralysis of the limbs and may become wheelchair bound. [emedicine.medscape.com]

• Neglect

  Dysdiadochokinesia Intention tremor ) medial ( Cerebellar ataxia ) Basal ganglia Chorea Dystonia Parkinson's disease Cortex ACA syndrome MCA syndrome PCA syndrome frontal lobe : Expressive aphasia Aboulia parietal lobe : Receptive aphasia Hemispatial neglect [en.wikipedia.org]

• Expressive Aphasia

  aphasia Aboulia parietal lobe : Receptive aphasia Hemispatial neglect Gerstmann syndrome Astereognosis occipital lobe : Bálint's syndrome Cortical blindness Pure alexia temporal lobe : Cortical deafness Prosopagnosia Thalamus Thalamic syndrome Other [en.wikipedia.org]

• Gerstmann Syndrome

  Basal ganglia Chorea Dystonia Parkinson's disease Cortex ACA syndrome MCA syndrome PCA syndrome frontal lobe : Expressive aphasia Aboulia parietal lobe : Receptive aphasia Hemispatial neglect Gerstmann syndrome Astereognosis occipital lobe : Bálint's [en.wikipedia.org]

• Foot Drop

  Bilateral foot drop was present. Right lower limb power was grade 3/5 in hip flexors and knee extensors and 2/5 in knee flexors, ankle plantar flexors, and hip extensors. [ncbi.nlm.nih.gov]

(See Workup.) Gross photograph of the dorsal surface of the spinal cord showing dilated and tortuous vessels. [emedicine.medscape.com]

Treatment of spinal cord vascular malformations by surgical excision. J. Neurosurgery, 30, 427–435. Article Google Scholar 16 Logue, V (1976). Practical aspects of surgical treatment. In Spinal Angiomas, pp. 131–136. M. J. Aminoff (ed.). [nature.com]

Operations, if successful, are permanent modalities of treatment. [emedicine.medscape.com]

Endovascular and surgical treatment of spinal dural arteriovenous fistulas. Neuroradiology 2008;50(10):869–876. HTML PDF Published: 2020-11-10 Issue: Vol 7 No 12 (view) Section: Articles How to cite: 1. Rathnam AS, Memon AB. [ejcrim.com]

Table 3 Summary of cases of filum terminale AV fistulas Both surgical and endovascular treatment options are mentioned in literature. [ncbi.nlm.nih.gov]

Cited by 1 article Arteriovenous fistula of the filum terminale: diagnosis, treatment, and literature review. Fischer S, Aguilar Perez M, Bassiouni H, Hopf N, Bäzner H, Henkes H. Fischer S, et al. [pubmed.ncbi.nlm.nih.gov]

Prognosis likely depends on the severity of the spinal pathology and the timeliness of diagnosis and intervention. Patients who present with rapidly progressing neurological deficits may have a worse prognosis. Early diagnosis is critical. [emedicine.medscape.com]

Early recognition and surgical intervention can result in a good prognosis. VIEW THE ENTIRE ARTICLE HTML PDF References Foix C, Alajouanine T. La myélite nécrotique subaigue. Rev Neurol (Paris) 1926;2:1–42. Jellema K, Tijssen CC, van Gijn J. [ejcrim.com]

Factors determining prognosis are said to be duration of illness, sphincter involvement at presentation and location of fistula however, there is no consensus on any of these.[ 1 ] Before our case, only one such has been published in Indian literature [ncbi.nlm.nih.gov]

Etiology The etiology of Foix-Alajouanine syndrome is not well understood. Most patients have an AV fistula in the lower thoracic dura. [emedicine.medscape.com]

No classification has been possible in the face of ignorance of etiologic factors; pathogenesis has been equally uncertain, and the interpretation of pathologic material has produced no uniformity of opinion regarding the nature of the morbid changes. [jamanetwork.com]

The etiology of the Foix-Alajouanine syndrome remains unknown but the lesions are probably acquired. Access options Buy single article Instant access to the full article PDF. 43,34 € Tax calculation will be finalised during checkout. [link.springer.com]

Drugs are used in neurological recovery depending on the etiological classification of FCMS. [en.wikipedia.org]

(See Etiology and Treatment.) [2, 5] Epidemiology Frequency United States No recent data exists describing the incidence or prevalence of patients with or treated for Foix-Alajouanine Syndrome in the United States or across other countries. [emedicine.medscape.com]

Pathophysiology The enlarged, abnormal veins are associated with dural AV shunts or fistulas, usually intradurally, but rarely extradurally. [1] These AV shunts are associated with reflux of arterial blood into the venous drainage of the cord. [emedicine.medscape.com]

Spinal intradural arteriovenous fistulas acquired in late adulthood: Absent spinal venous drainage in pathogenesis and pathophysiology. Report of two cases. J Neurosurg Spine. 2005; 3 :488–94. [ PubMed ] [ Google Scholar ] [ncbi.nlm.nih.gov]

*'a: clinical diagnosis d: pathophysiology e: EEG, EMG, neuroradiologic procedures, high function tests f: biochemistry and pharmacology g: nosography Symptoms o f PSP reported as appearing within the first year from the onset. [slideheaven.com]

The inclusion of thrombosis in the clinical picture of this syndrome is not only incorrect but may leave one with the impression of therapeutic futility, thus possibly preventing successful surgical or endovascular therapy. [thejns.org]

The spinal vascular lesions can cause neurologic deficits, including paralysis. [2, 3] Early recognition of the association between the vascular skin lesions and associated spinal lesions may prevent or minimize neurological sequela. [emedicine.medscape.com]