ABSTRACT
Depression is a common psychiatric disorder among individuals with Huntington's disease (HD). Depression in HD and major depressive disorder appear to have different pathophysiological mechanisms. Despite the unique pathophysiology, the treatment of depression in HD is based on data from the treatment of major depressive disorder in the general population. The objective of this systematic review was to conduct a comprehensive evaluation of the available evidence. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. Studies on the treatment of depression in HD were identified by searching MEDLINE, Embase, and PsycInfo. The initial search yielded 2,771 records, 41 of which were ultimately included. There were 19 case reports, seven case series, three cross-sectional studies, one qualitative study, nine nonrandomized studies, and two randomized trials among the included studies. The most common assessment tools were the Hospital Anxiety and Depression Scale (N=8), the Beck Depression Inventory (N=6), and the Hamilton Depression Rating Scale (N=6). Only 59% of the included studies assessed depressive symptoms with a scoring system. The pharmacological options for the treatment of depression included antidepressants and antipsychotics. Nonpharmacological approaches were multidisciplinary rehabilitation, psychotherapy, and neurostimulation. Limited evidence on the treatment of depression in HD was available, and this literature consisted mainly of case reports and case series. This systematic review highlights the knowledge gap and the pressing need for HD-specific research to determine the efficacy of treatment approaches for depression in HD.
ABSTRACT
BACKGROUND: Crouch gait, or flexed knee gait, represents a common gait pattern in patients with spastic bilateral cerebral palsy (CP). Distal femoral extension and/or shortening osteotomy (DFEO/DFSO) and patellar tendon advancement (PTA) can be considered as viable options when knee flexion contractures are involved. Better outcomes have been reported after a combination of both, independently of the presence of knee extensor lag. In this study, we evaluated the clinical and kinematic outcomes of these procedures. PATIENTS AND METHODS: We reviewed a cohort of 52 limbs (28 patients) who were treated for crouch gait by DFEO/DFSO alone (group 1, n = 15) or DFEO/DFSO + PTA (group 2, n = 37) as a part of single event multilevel surgery (SEMLS). The mean age at surgery was 14 years, and the mean follow-up time was 18 months. The physical examination data and three-dimensional standardized gait analysis were collected and analyzed before the surgery and postoperatively. RESULTS: Overall knee range of motion improved in all limbs. The knee flexion decreased significantly in both groups at initial, mid, and terminal stance. Hip flexion significantly decreased in mid-stance for limbs in group 2. Both clinical and gait parameters were most improved in limbs who underwent DFEO/DFSO + PTA. Increased pelvic tilt was observed in both groups after surgery. CONCLUSION: Although DFEO/DFSO alone was successful in correcting knee flexion contractures, PTA has helped to improve knee extensor lag and knee extension during gait. LEVEL OF EVIDENCE: Therapeutic level IV.
Subject(s)
Cerebral Palsy , Femur , Gait Disorders, Neurologic , Knee Joint , Osteotomy , Patellar Ligament , Range of Motion, Articular , Humans , Cerebral Palsy/surgery , Cerebral Palsy/complications , Cerebral Palsy/physiopathology , Osteotomy/methods , Male , Female , Adolescent , Range of Motion, Articular/physiology , Femur/surgery , Gait Disorders, Neurologic/surgery , Gait Disorders, Neurologic/etiology , Gait Disorders, Neurologic/physiopathology , Patellar Ligament/surgery , Child , Knee Joint/physiopathology , Knee Joint/surgery , Biomechanical Phenomena , Young Adult , Treatment Outcome , Retrospective Studies , Gait/physiology , Contracture/surgery , Contracture/physiopathologyABSTRACT
Obsessive-compulsive and perseverative behaviors (OCBs/PBs) are characteristic features of Huntington's Disease (HD). Although a few recent research have attempted to discriminate between OCBs and PBs, most of the available evidence on OCBs does not consistently make this distinction. In this article, we aimed to explore the current inconsistencies in assessing and reporting OCBs/PBs and map the body of existing evidence. Up to half of the patients with motor manifest HD can experience OCBs. Separate reporting of PBs in HD patients has been uncommon among the studies and was frequently reported as a part of obsessive-compulsive symptoms. The structural limitation of the currently used rating scales and the overlaps in neuropathology and definition of OCBs and PBs are among the main reasons for the mixed reporting of OCBs/PBs. Perseverative thinking or behavior as a separate item is found in a few assessment tools, such as the Problem Behaviors Assessment - Short form (PBA-s). Even when the item exists, it is commonly reported as a composite score in combination with the obsessive-compulsive item. In addition to the significant psychological burden in individuals with HD, PBs are associated with somatic effects (e.g., cardiovascular symptoms) and high-risk behaviors (e.g., suicide). Recognition and monitoring of PBs in HD can aid in early detection of concerning symptoms and differentiating overlapping illnesses.