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Depression in adult patients with biotin responsive basal ganglia disease
Dalal K Bubshait1, Asif Rashid, Mohammed A Al-Owain
1Department of Medical Genetics, King Faisal Specialist Hospital and Research center.
Insights
Biotin responsive basal ganglia disease (BBGD) is treatable, but long-term outcomes are unknown. Treated BBGD patients may develop depression later in life, indicating a potential chronic complication.
Area of Science:
- Neurology
- Metabolic Disorders
- Genetics
Background:
- Biotin responsive basal ganglia disease (BBGD) is an inherited metabolic disorder.
- Early diagnosis and treatment lead to good childhood recovery.
- Long-term outcomes in treated adult patients remain underreported.
Observation:
- Two adult patients with BBGD, stable on treatment, developed depression.
- This suggests a potential link between BBGD and mood disorders.
Findings:
- Depression can manifest as a long-term complication in individuals with BBGD.
- The study highlights the importance of monitoring mental health in treated BBGD patients.
Implications:
- This research underscores the need for comprehensive, long-term follow-up of BBGD patients.
- It suggests that neurological and psychiatric monitoring should be integral to managing BBGD throughout life.
- Further research is warranted to understand the mechanisms linking BBGD and late-onset depression.
Abstract:
Biotin responsive basal ganglia disease (BBGD), is a potentially treatable inherited metabolic disorder which clinically presents as sub-acute encephalopathy in children. Early diagnosis and treatment of this disorder results in good clinical recovery in childhood. However, there is no report in the literature on the long term outcome of these treated patients in adult life. We report two patients with BBGD who were metabolically stable on treatment and developed depression later in life. These cases highlight the association of depression with basal ganglia disorders and demonstrate that depression is the potential long term complication of BBGD.
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