Neurologic Complications of Common Variable Immunodeficiency

Abstract

Common variable immunodeficiency is a rare disorder of immunity associated with a myriad of clinical manifestations including recurrent infections, autoimmunity, and malignancy. Though rare, neurologic complications have been described in a small number of case reports and case series of CVID patients. In this article, we present a patient with CVID who suffered significant neurologic morbidity and categorize the reported range of neurologic complications associated with CVID. Our case highlights the complex nature of neurologic manifestations in CVID patients, and our review of the current database suggests that infection and inflammatory neurologic disorders are the cause of most neurologic presentations.

Central Nervous System Involvement in Common Variable Immunodeficiency: A Case of Acute Unilateral Optic Neuritis in a 26-Year-Old Italian Patient

“…In conclusion, neurological manifestations in CVID are challenging for both their atypical presentation and for the nature of the disease itself, which can provoke or mimic several diseases ( 2 , 9 ). Autoimmune diseases, especially cytopenias, occur in 25% of CVID patients, and splenomegaly is also a common finding in such patients.…”

“…Among autoimmune disorders, the most common are thrombocytopenia, hemolytic anemia, and rheumatologic disorders ( 4 , 6 – 8 ). Neurologic complications are rare, and include a variety of central nervous system (CNS) infections, the most common being bacterial meningitis and viral meningoencephalitis, autoimmune/inflammatory encephalomyelitis or peripheral neuropathies ( 9 ). Only one case of bilateral posterior optic neuritis is reported ( 10 ).…”

Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management

“…Neurologic and liver diseases are less commonly reported in CVID patients [127]. Infectious etiologies of the nervous system have been described in 43 cases and represent the largest class of neurologic dysfunction [128,129], followed by autoimmune/inflammatory myelitis [130,131].…”

Infection, Immunodeficiency, and Inflammatory Diseases in Autoimmune Neurology

“…25,29 While CVID-associated granulomatous disease is still not commonly recognized by physicians, 29 understanding of neurological involvement and complications is even less common. 30,31 It is common for CVID to be confused with sarcoidosis, as the granulomas in CVID are noncaseating. There are no current guidelines for the standard practice of diagnosing and treating noncaseating granulomatous disease; however, perhaps patients diagnosed with sarcoidosis should have serum immunoglobulins measured as part of their evaluation and initiation of immunotherapy to rule out CVID as an etiology of the granulomatous disease.…”