Nonmotor features in sex-linked dystonia parkinsonism.

The natural history of sex-linked dystonia parkinsonism (XDP) has been well documented. However, its nonmotor features have not yet been fully described. We reviewed the available literature on the nonmotor features in XDP. We found five articles involving 79 XDP patients, three of which were on cognition and two on mood (anxiety and depression). There were two case reports showing executive dysfunction. The other paper showed impairments in abstract thinking and motor programming. Two articles were on mood (anxiety and depression). The prevalence of anxiety symptoms was 16.7% and 54.8-92.9% had depressive symptoms. The identification of these nonmotor features should lead to early and appropriate management.

Suicide among patients with X-linked dystonia parkinsonism (XDP): A retrospective study

OBJECTIVE: To describe the rate of suicide and explore its possible related factors among patients with X-linked Dystonia Parkinsonism. Specifically, this paper aimed to describe the rate of suicide among patients with XDP based on the Philippine XDP registry and to describe these patients in terms of severity of XDP and psychosocial factors. BACKGROUND: Chronic progressive neurologic conditions have been associated with serious psychosocial stresses. Suicide among patients with X-linked Dystonia Parkinsonism has been previously reported to be high. METHODS: A retrospective chart review was done on XDP patients with deaths attributable to suicide. XDP related variables and available psychosocial factors were noted. RESULTS: The prevalence of suicide among all XDP patients registered is 4.16%. There are 194 deaths in the Philippine XDP registry, 21 of which were attributable to suicide, a proportion of 10.8%. Their mean age at suicide was 44, around 7.76 mean years from the onset of illness. All of the patients were either in generalized dystonia or parkinsonian stage when they had suicide. Psychosocial variables noted were marital and family conflict, and loss of employment. None of the patients had a prior documented psychiatric illness but several of them showed symptoms of depression prior to suicide. CONCLUSIONS: There is a high rate of suicide among patients with XDP which is comparable to other disabling neurodegenerative diseases. It occurs relatively late in the course where the patient is already in the stage of generalized dystonia or parkinsonism. Possible psychosocial risks include poor family support, marital conflict, loss of employment and symptoms suggestive of depression. The present understanding is that depression and suicide in XDP is more likely reactive to the disease rather than part of its clinical feature. This study supports this view.