ABSTRACT
Fabry disease is an X-linked lysosomal storage disorder which can result in renal, cardiac, and cerebrovascular disease. Patients are at increased risk of stroke and neuroimaging studies note cerebrovascular pathology. This study provides a cognitive profile of a cohort of individuals with Fabry disease and investigates the impact of pain, age, renal, cardiac, and cerebrovascular functioning on cognition and psychological functioning. Seventeen Fabry patients (12 males) with ages ranging 25 to 60 years (M = 46.6+11.8), and 15 age-matched healthy controls (M = 46.2+12.7) were administered a comprehensive neuropsychological battery. Fabry males demonstrated slower speed of information processing, reduced performance on measures of executive functions (verbal generation, reasoning, problem solving, perseveration), were more likely to show clinically significant reductions, and were more likely to report symptoms of anxiety and depression. Conversely, Fabry females performed at a similar level to controls. Correlational analyses indicated a link between cognitive and clinical measures of disease severity.
Subject(s)
Cognition Disorders/diagnosis , Executive Function/physiology , Fabry Disease/complications , Psychomotor Performance/physiology , Adult , Anxiety/psychology , Cognition Disorders/etiology , Depression/psychology , Fabry Disease/psychology , Female , Humans , Male , Middle Aged , Neuropsychological Tests , Severity of Illness Index , Sex Factors , Stress, Psychological/psychologyABSTRACT
Psychiatric symptoms are historically thought a relative contraindication to DBS for advanced Parkinson's disease (PD). However, in the case of drug-induced mental illness, DBS may provide an acceptable alternative for the treatment of motor symptoms. This allows reduction of pharmacological dopaminergic therapy that might otherwise cause negative psychiatric consequences. For example, DBS is increasingly used to ameliorate specific complications of PD treatment, such as impulse control disorders. We present a series of 3 cases of young male patients who developed Othello syndrome (OS) during treatment with dopamine agonists. In each case, the OS resolved with withdrawal of the offending drug. Subsequent treatment with bilateral STN DBS improved motor symptoms and allowed reduction in their dopaminergic drug regimen. We therefore propose that drug-induced psychopathology may be an indication (rather than a contraindication) for DBS in selected cases.
