Unilateral Flaccid Paraplegia Associated with Chronic Granulomatous Disease

Document Type : Case Report


Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.


Chronic Granulomatous Disease (CGD) can be associated with several neurological complications. Abscess in the brain in patients diagnosed with CGD has been reported in several publications. Different pathogens have been linked with brain abscess development including fungal or bacterial infections. Other neurologic complications may include white matter disease and formation of a granulomatous lesion in the central nervous system. In addition to these common reports, observation of leptomeningeal, along with focal brain infiltration by pigmented, lipid-laden macrophages, fungal brain infection, Aspergillus abscess resembling a brain tumor, spinal cord infection by Aspergillus, and fungal granuloma of the brain have also been described. Physicians should be aware of Streptococcus- and Candida-induced meningitis in a selected group of CGD patients as well. Herein, we report a case of the attenuated ill-defined lesion in the right cerebellar hemisphere in a CGD patient without involvement of the sinuses or lungs.


1. Marciano BE, Spalding C, Fitzgerald A, Mann D, Brown T, Osgood S, et al. Common severe infections in chronic granulomatous disease. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. 2015 Apr 15;60(8):1176- 83. PubMed PMID: 25537876. Pubmed Central PMCID: 4400412. 2. Martire B, Rondelli R, Soresina A, Pignata C, Broccoletti T, Finocchi A, et al. Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: an Italian multicenter study. Clinical immunology. 2008 Feb;126(2):155-64. PubMed PMID: 18037347. 3. Drouin-Garraud V, Belgrand M, Grunewald S, Seta N, Dacher JN, Henocq A, et alNeurological presentation of a congenital disorder of glycosylation CDG-Ia: implications for diagnosis and genetic counseling. American journal of medical genetics. 2001 Jun 1;101(1):46-9. PubMed PMID: 11343337. 4. Ma JS, Chen PY, Lau YJ, Chi CS. Brain abscess caused by Salmonella enterica subspecies houtenae in a patient with chronic granulomatous disease. Journal of microbiology, immunology, and infection = Wei mian yu gan ran za zhi. 2003 Dec;36(4):282-4. PubMed PMID: 14723260. 5. Segal BH, Veys P, Malech H, Cowan MJ. Chronic granulomatous disease: lessons from a rare disorder. Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation. 2011 Jan;17(1 Suppl):S123-31. PubMed PMID: 21195301. Pubmed Central PMCID: 3052948. 6. Schwenkenbecher P, Neyazi A, Donnerstag F, Ringshausen FC, Jacobs R, Stoll M, et al. Chronic Granulomatous Disease First Diagnosed in Adulthood Presenting With Spinal Cord Infection. Frontiers in immunology. 2018;9:1258. PubMed PMID: 29915596. Pubmed Central PMCID: 5994559. 7. Koker MY, Camcioglu Y, van Leeuwen K, Kilic SS, Barlan I, Yilmaz M, et al. Clinical, functional, and genetic characterization of chronic granulomatous disease in 89 Turkish patients. The Journal of allergy and clinical immunology. 2013 Nov;132(5):1156-63 e5. PubMed PMID: 23910690. 8. Rawat A, Bhattad S, Singh S. Chronic Granulomatous Disease. Indian journal of pediatrics. 2016 Apr;83(4):345-53. PubMed PMID: 26865172. 9. Nwawka OK, Nadgir R, Fujita A, Sakai O. Granulomatous disease in the head and neck: developing a differential diagnosis. Radiographics : a review publication of the Radiological Society of North America, Inc. 2014 Sep-Oct;34(5):1240-56. PubMed PMID: 25208278. 10. Danziger RN, Goren AT, Becker J, Greene JM, Douglas SD. Outpatient management with oral corticosteroid therapy for obstructive conditions in chronic granulomatous disease. The Journal of pediatrics. 1993 Feb;122(2):303-5. PubMed PMID: 8429451.