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Congestive Myelopathy due to Intradural Spinal AVM Supplied by Artery of Adamkiewicz: Case Report with Brief Literature Review and Analysis of the Foix-Alajouanine Syndrome Definition.

Sood D, Mistry KA, Khatri GD, Chadha V, Garg S, Suthar PP, Patel DG, Patel A - Pol J Radiol (2015)

Bottom Line: Diagnosis is often missed due to its rarity and confusing definitions of the Foix-Alajouanine syndrome.Our patient was treated by embolization of the lesion with 20% glue, after which he showed mild improvement of symptoms.Patients may be treated with surgery or endovascular procedures.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Dr Rajendra Prasad Government Medical College, Kangra, India.

ABSTRACT

Background: Spinal arteriovenous malformations (AVMs) can lead to development of congestive myelopathy (Foix-Alajouanine syndrome). Spinal AVMs are rare and so is this syndrome. Diagnosis is often missed due to its rarity and confusing definitions of the Foix-Alajouanine syndrome.

Case report: We report a case of a 47-year-old male patient suffering from this rare syndrome with an AVM arising from the artery of Adamkiewicz, which is another rarity. Our patient was treated by embolization of the lesion with 20% glue, after which he showed mild improvement of symptoms. We also present a brief review of literature on spinal AVMs and elucidate the evolution of the term Foix-Alajouanine syndrome.

Conclusions: Use of the term "Foix-Alajouanine syndrome" should be restricted to patients with progressive subacute to chronic neurological symptoms due to congestive myelopathy caused by intradural spinal AVMs. CT angiography should supplement DSA as preliminary Imaging modality. Patients may be treated with surgery or endovascular procedures.

No MeSH data available.


Related in: MedlinePlus

Sagittal T2-weighted (TR=3000 ms, TE=88 ms) images (A–D) of the spine demonstrate bulky lower dorsolumbar cord and conus medullaris with increased signal intensity and multiple intradural extramedullary areas of signal void. Grade 1 anterolisthesis of L4 over L5 vertebral body is noted.
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f1-poljradiol-80-337: Sagittal T2-weighted (TR=3000 ms, TE=88 ms) images (A–D) of the spine demonstrate bulky lower dorsolumbar cord and conus medullaris with increased signal intensity and multiple intradural extramedullary areas of signal void. Grade 1 anterolisthesis of L4 over L5 vertebral body is noted.

Mentions: Magnetic resonance imaging (MRI) of the dorsolumbar spine performed with a 1.5T scanner (Signa Excite 1.5T, GE) revealed bulky lower lumbar cord and conus medullaris with high T2 signal. Multiple intradural extramedullary areas signal void were seen from Th6 to S1 level (Figure 1), more abundantly on the left side of Th10–Th12, causing rightward displacement of the lower part of the spinal cord. Spinal cord exhibited intense, relatively homogenous enhancement after intravenous gadolinium injection (Figure 2). Subsequent spinal computed tomography angiography (CTA) was performed with a 16-slice scanner (Philips Brilliance 16, Philips Medical Systems) with nonionic iodinated contrast agent (Iohexol, 350 mg/mL) at dose of 2 mL/kg injected into the antecubital vein through pressure injector at a rate of 4 mL/sec with bolus tracking, with ROI placed over the abdominal aorta (scanning parameters: increment 1.0 mm, reconstruction interval 0.75 mm, slice thickness 2.0 mm, pitch 1.188, rotation time 0.75s, kVp 120, mA 200) CTA revealed an intradural extramedullary arteriovenous malformation (AVM) from Th10 to Th12 level (Figure 3) supplied by a branch of a great radicular artery (artery of Adamkiewicz). In our case it originated from the abdominal aorta on the left side and ran subcostally, entering the spinal canal through the Th12–L1 intervertebral foramen (Figures 4–6). The AVM drained into the right internal iliac vein through an elongated, tortuous venous channel. Adiagnosis of intradural perimedullary AVM with congestive myelopathy (Foix-Alajouanine syndrome) was made based on the findings.


Congestive Myelopathy due to Intradural Spinal AVM Supplied by Artery of Adamkiewicz: Case Report with Brief Literature Review and Analysis of the Foix-Alajouanine Syndrome Definition.

Sood D, Mistry KA, Khatri GD, Chadha V, Garg S, Suthar PP, Patel DG, Patel A - Pol J Radiol (2015)

Sagittal T2-weighted (TR=3000 ms, TE=88 ms) images (A–D) of the spine demonstrate bulky lower dorsolumbar cord and conus medullaris with increased signal intensity and multiple intradural extramedullary areas of signal void. Grade 1 anterolisthesis of L4 over L5 vertebral body is noted.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4492508&req=5

f1-poljradiol-80-337: Sagittal T2-weighted (TR=3000 ms, TE=88 ms) images (A–D) of the spine demonstrate bulky lower dorsolumbar cord and conus medullaris with increased signal intensity and multiple intradural extramedullary areas of signal void. Grade 1 anterolisthesis of L4 over L5 vertebral body is noted.
Mentions: Magnetic resonance imaging (MRI) of the dorsolumbar spine performed with a 1.5T scanner (Signa Excite 1.5T, GE) revealed bulky lower lumbar cord and conus medullaris with high T2 signal. Multiple intradural extramedullary areas signal void were seen from Th6 to S1 level (Figure 1), more abundantly on the left side of Th10–Th12, causing rightward displacement of the lower part of the spinal cord. Spinal cord exhibited intense, relatively homogenous enhancement after intravenous gadolinium injection (Figure 2). Subsequent spinal computed tomography angiography (CTA) was performed with a 16-slice scanner (Philips Brilliance 16, Philips Medical Systems) with nonionic iodinated contrast agent (Iohexol, 350 mg/mL) at dose of 2 mL/kg injected into the antecubital vein through pressure injector at a rate of 4 mL/sec with bolus tracking, with ROI placed over the abdominal aorta (scanning parameters: increment 1.0 mm, reconstruction interval 0.75 mm, slice thickness 2.0 mm, pitch 1.188, rotation time 0.75s, kVp 120, mA 200) CTA revealed an intradural extramedullary arteriovenous malformation (AVM) from Th10 to Th12 level (Figure 3) supplied by a branch of a great radicular artery (artery of Adamkiewicz). In our case it originated from the abdominal aorta on the left side and ran subcostally, entering the spinal canal through the Th12–L1 intervertebral foramen (Figures 4–6). The AVM drained into the right internal iliac vein through an elongated, tortuous venous channel. Adiagnosis of intradural perimedullary AVM with congestive myelopathy (Foix-Alajouanine syndrome) was made based on the findings.

Bottom Line: Diagnosis is often missed due to its rarity and confusing definitions of the Foix-Alajouanine syndrome.Our patient was treated by embolization of the lesion with 20% glue, after which he showed mild improvement of symptoms.Patients may be treated with surgery or endovascular procedures.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Dr Rajendra Prasad Government Medical College, Kangra, India.

ABSTRACT

Background: Spinal arteriovenous malformations (AVMs) can lead to development of congestive myelopathy (Foix-Alajouanine syndrome). Spinal AVMs are rare and so is this syndrome. Diagnosis is often missed due to its rarity and confusing definitions of the Foix-Alajouanine syndrome.

Case report: We report a case of a 47-year-old male patient suffering from this rare syndrome with an AVM arising from the artery of Adamkiewicz, which is another rarity. Our patient was treated by embolization of the lesion with 20% glue, after which he showed mild improvement of symptoms. We also present a brief review of literature on spinal AVMs and elucidate the evolution of the term Foix-Alajouanine syndrome.

Conclusions: Use of the term "Foix-Alajouanine syndrome" should be restricted to patients with progressive subacute to chronic neurological symptoms due to congestive myelopathy caused by intradural spinal AVMs. CT angiography should supplement DSA as preliminary Imaging modality. Patients may be treated with surgery or endovascular procedures.

No MeSH data available.


Related in: MedlinePlus