A Systematic Review of Idiopathic Basal Ganglia Calcification for Possible Inclusion in the Social Security Administration's Compassionate Allowances Program

Background:  The Social Security Administration launched the Compassionate Allowances List in 2008. This initiative created a mechanism for identifying diseases and other medical conditions that meet the Social Security Administration’s standards for expedited review and delivery of disability benefits. Idiopathic basal ganglia calcification or Fahr’s disease was selected for review in this study. A review of the condition’s appropriateness for inclusion in the Compassionate Allowance list was undertaken. This project was supported by Policy Research, Inc. as part of the U.S. Social Security Administration's Improving Disability Determination Process Small Grant Program.

Method: Using guidance from the Cochrane methodology, a systematic review of the literature was undertaken. The following databases were searched:  The Cochrane Library, The Health InterNetwork Access to Research Initiative (HINARI), MEDLINE, EMBASE, Applied Social Services Index and Abstracts, Education Resources Information Center, and PsycInfo. The Science Citation Index Expanded was searched as well as the National Technical Information Service. Articles were first identified by condition, and then narrowed down by inclusion criteria. Articles were coded as a “1” (demonstrating that the condition will last beyond 12 months), as a “2” (demonstrating that the presence of the first criteria plus an impact on quality of life or death), and “3” (demonstrating full disability criteria). Once coded, severity thresholds were examined to determine if the condition “invariably” meets the criteria for the Compassionate Allowance list or if the heterogeneous presentation among those affected makes it difficult to include the condition as a whole.

Findings: This systematic review provided an in-depth examination of the extent to which Idiopathic Basal Ganglia Calcification invariably meets or fails to meet the definition of disability provided by the Social Security Administration. Given that symptomatic case are often severe, result in progressive deterioration as well as a myriad of symptoms affecting a variety of different bodily systems, it is recommended that consideration extend to symptomatic idiopathic basal ganglia calcification for inclusion in the Compassionate Allowance List. Given that differentiating between those who are symptomatic and those who are not, among all those diagnosed with IBGC, may include citation of medical evidence, the Quick Disability Determination process may a more applicable expedited review procedure. 

Conclusion: This study demonstrated that Idiopathic Basal Ganglia Calcification does not invariably meet the criteria for inclusion in the Compassionate Allowance List. While some experience this condition in a severe form, the presentation of the condition is too variable to universally recommend its inclusion. Some remain asymptomatic and are diagnosed through CT scans. For these people, disability benefits would be unnecessary. For those who are clinically symptomatic, however, inclusion is recommended. The Compassionate Allowances List provides a benefit to both the Social Security Administration and its respective claimants. By identifying diagnoses that invariably meet the criterion for disability, claimants may experience an expedited review time and the Social Security Administration can expect to see a decrease in their backlog of applications. This systematic review provided one example of a system to identify those diagnoses that may be considered for inclusion in this initiative.