Foix-Alajouanine syndrome: a comprehensive overview of rare but relevant diagnosis

Oday Atallah; Yasser F Almealawy; Roua Arian; Assma Dwebi; Amr Badary; Abbas F Abdul Hussein; Vivek Sanker; Saber Zafarshamspour; Bipin Chaurasia; Amit Agrawal; Santiago Pastrana Brandes; Mohammed A Azab

doi:10.1097/MS9.0000000000002613

Foix-Alajouanine syndrome: a comprehensive overview of rare but relevant diagnosis

Ann Med Surg (Lond). 2024 Sep 30;86(11):6636-6644. doi: 10.1097/MS9.0000000000002613. eCollection 2024 Nov.

Authors

Affiliations

¹ Departemnt of Neurosurgery, Hannover Medical School, Hannover, Germany.
² Faculty of Medicine, University of Kufa, Kufa, Iraq.
³ Faculty of Medicine, University of Aleppo, Aleppo, Syria.
⁴ Faculty of Medicine, Ludwig-Maximilians-Universität München, Munich.
⁵ Departemnt of Neurosurgery, Klinikum Dessau, Dessau-Roßlau, Germany.
⁶ Faculty of Medicine, Babylon University, Hilla, Iraq.
⁷ Department of Neurosurgery, Trivandrum Medical College, Kerala, India.
⁸ Department of Surgery, Rafsanjan University of Medical Sciences, Rafsanjan, Kerman, Iran.
⁹ Department of Neurosurgery, Neurosurgery Clinic, Birgunj, Nepal.
¹⁰ Department of Neurosurgery, All India Institute of Medical Sciences, Saket Nagar, Bhopal, Madhya Pradesh, India.
¹¹ Harvard T.H. Chan School of Public Health, Executive and Continuing Professional Education (ECPE), Boston, Massachusetts, USA.
¹² Departemnt of Neurosurgery, Cairo University Hospital, Cairo, Egypt.

Abstract

Introduction: Foix-Alajouanine syndrome (FAS) is an uncommon neurological disorder marked by the gradual development of spinal cord congestion. First documented by Foix and Alajouanine in 1926. Although it is infrequent, delayed or misdiagnosis is nonetheless prevalent, resulting in inadequate therapy and unfavorable results.

Methods: Using the PubMed database, MEDLINE, and EMBASE, we collected data on FAS patients and conducted a pooled analysis. The term 'FAS' was used to search for related articles. Our search was restricted to previous clinical case reports or series that were published in English. Non-English articles were excluded. We included the articles in the period from 1974 to 2024. Articles were eligible if the radiographic and clinical findings were indicative of FAS. A thorough research analysis was performed, examining case reports that specifically addressed this issue. This study examines the clinical symptoms, difficulties in diagnosis, methods of treatment, and outcomes related to FAS.

Results: FAS predominantly impacts the elderly population. A total of 26 patients were diagnosed with FAS. The median age of affected individuals was 53 (SD ±15.96). The ratio of males to females is roughly 5:1. The clinical manifestations encompass gradual muscle weakness and sensory impairments. The diagnosis is dependent on radiological evaluations, specifically MRI and digital subtraction angiography. Possible treatments include endovascular therapy, surgical closure of arteriovenous fistula, or a combination of the two. Significant improvements in neurological impairments can be achieved by early intervention.

Conclusion: The diagnosis of FAS continues to be difficult due to its infrequency and varied clinical manifestations. Prompt and precise diagnosis is essential for proper intervention, typically utilizing endovascular or surgical methods. Additional research is required to determine prognostic markers and enhance long-term care techniques for this rare neurological condition.

Keywords: Foix–Alajouanine syndrome; arteriovenous fistulas; spinal cord ischemia.

Publication types

Review