The "eye of the tiger" sign in pure akinesia with gait freezing.

The "eye of the tiger" is a neuroradiologic sign due to iron deposition in the globus pallidus: it appears as diffuse low signal intensity with a central area of high signal intensity confined to the globus pallidus. The "eye of the tiger" sign has been associated with neurodegeneration with brain iron accumulation type 1 (NBIA1), a condition caused by mutations in the gene encoding pantothenate kinase 2 (PANK2). However, the specificity of this neuroradiologic sign has been already challenged and it has been described in other neurodegenerative diseases. Here, we report the first case of a patient suffering from pure akinesia with gait freezing with the "eye of the tiger" sign in T2-weighted MRI sequences. All clinical, laboratory and radiologic data excluded other diagnosis and genetic testing excluded PANK2 mutations suggesting that the "eye of the tiger" is not specific for NBIA1 and may also occur in other movement disorders.

Comparative neuropsychological profile of pathological gambling, hypersexuality, and compulsive eating in Parkinson's disease.

BACKGROUND: Impulse control disorders (ICDs), in particular pathological gambling, hypersexuality, and compulsive eating, are being increasingly identified in Parkinson's disease (PD) patients. Pathological gambling has been associated with frontal/executive dysfunctions, whereas hypersexuality and compulsive eating, and their relation with cognitive dysfunctions, have not been investigated in PD. METHODS: We investigated cognitive correlates underpinning pathological gambling, hypersexuality, and compulsive eating in PD. PD outpatients were screened for pathological gambling, hypersexuality, and compulsive eating. Based on clinical criteria, we identified 13 patients with hypersexuality, 12 with compulsive eating, 14 with pathological gambling, and 10 with multiple ICDs. Fourteen PD patients matched for age and education without ICDs served as controls. Clinical features and neuropsychiatric and neuropsychological functioning were assessed in the 5 groups. RESULTS: Demographic, clinical, neuropsychiatric, and neurological aspects did not differ among groups. All 4 groups of ICD patients were impaired on tasks exploring spatial-planning and set-shifting tasks compared with the controls. The main difference among patients with pathological gambling, hypersexuality, and compulsive eating was that patients with hypersexuality were more impaired on the Stroop test than patients with pathological gambling. Individuals with hypersexuality, compulsive eating, and multiple ICDs performed worse on verbal learning and memory tests than did patients with pathological gambling. DISCUSSION: ICDs are associated with impaired cognitive functions; the severity of impairment decreased in the order multiple ICDs and hypersexuality > compulsive eating > pathological gambling. Our findings support the idea that hypersexuality is associated with prefrontal and memory dysfunctions, whereas pathological gambling seems to be related only to frontal dysfunction. NS065070

Multiple system atrophy is associated with changes in peripheral insulin-like growth factor system.

Insulin-like growth factors (IGF-I and IGF-II) have been shown to have several neurotrophic actions and IGF system may be impaired in neurodegenerative disorders. The aim of this study was to investigate the IGF system in patients with MSA and to evaluate correlations between this endocrine system and clinical features of the disease. Serum levels of IGF-I, IGF-II, insulin, IGF-binding protein 1 (BP1), and IGF-binding protein 3 (BP3) were measured in 25 patients with probable MSA and 25 age, sex and BMI-matched healthy controls. Clinical status of each patient was evaluated with the Unified Multiple System Atrophy Rating Scale (UMSARS) Part II and the Hoehn and Yahr rating scale. IGF-I levels were significantly higher in MSA (164.1 + 66.2 µg/L) than in healthy controls (111.7 + 60.3 µg/L; p = 0.001). Insulin levels were significantly higher in MSA patients (21.9 ± 14.4 µU/mL) than in healthy controls (13.3 ± 5.1 µU/mL, p = 0.048). No significant difference was found in serum IGF-II, IGF-BP1, and IGF- BP3 levels between patients with MSA and healthy controls. There was a trend toward significantly higher IGF-II levels in MSA patients with UMSARS score <26 (1026.3 ± 442.6 µg/L) than MSA patients with UMSARS score >26 (796.1 ± 234 µg/L, p = 0.055). The results of this study demonstrated that IGF system is altered in MSA. The degenerative process in MSA could lead to a compensatory increase in IGF-I and insulin in an attempt to provide additional support to degenerating neurons.

Dopamine receptor agonists and depression in Parkinson's disease.

Depression is one of the most common non-motor symptoms in Parkinson's disease (PD). It is associated with a more rapid progression of physical symptoms, greater decline in cognitive skills, and a poorer quality of life. Despite the high prevalence of depression and antidepressant use in PD, validated guidelines for the treatment of PD-associated depression (dPD) are lacking. Several methodological limitations have been recognized in the available studies examining the treatment of dPD. Some studies support a relevant role of the catecholaminergic systems in the pathogenesis of dPD. In open-label studies, the dopamine receptor agonists pramipexole and pergolide have shown antidepressant effects in PD patients. A placebo-controlled study of pramipexole in dPD is ongoing. The combined results of data from animal models and evidence in human studies support the strategy of dopaminergic stimulation as a treatment of depression. Treatment strategies for depressive symptoms in PD should include optimization of dopaminergic treatment prior to the addition of classic antidepressant drugs, thus reducing the risk of side-effects associated with multi-drug therapies.