Neuroimaging Pearls from the MDS Congress Video Challenge. Part 2: Acquired Disorders.

The MDS Video Challenge continues to be the one of most widely attended sessions at the International Congress. Although the primary focus of this event is the presentation of complex and challenging cases through videos, a number of cases over the years have also presented an unusual or important neuroimaging finding related to the case. We reviewed the previous Video Challenge cases and present here a selection of those cases which incorporated such imaging findings. We have compiled these "imaging pearls" into two anthologies. The first focuses on pearls where the underlying diagnosis was a genetic condition. This second anthology focuses on imaging pearls in cases where the underlying condition was acquired. For each case we present brief clinical details along with neuroimaging findings, the characteristic imaging findings of that disorder and, finally, the differential diagnosis for the imaging findings seen.

Functional outcomes of an integrated Parkinson's Disease Wellbeing Program.

INTRODUCTION: There is growing evidence that exercise provides benefit in treating motor and non-motor symptoms in Parkinson's disease (PD). OBJECTIVES: The aims of this study were to determine (a) whether a 5-week PD-specific program resulted in sustained physical and psychosocial benefits, and (b) the relationship between patient characteristics, exercise, falls and physical and psychosocial parameters. DESIGN: Single-centre prospective observational study. METHODS: A total of 135 consecutive patients with mild-to-moderate PD underwent a 5-week PD-specific education and exercise program from August 2013 to March 2015. Gait, mobility and psychosocial measures were compared at baseline, 6 weeks and 12 months. RESULTS: Significant improvements in physical (walking distance in 2 minutes, number of "Sit To Stands" in 30 seconds, time in seconds taken to "Timed Up and Go," fast gait velocity over 10 m, Berg Balance Scale [BBS]) and psychosocial (quality of life (QoL) [PDQ-39], depression and anxiety [DASS-21], and fatigue [PSF-16]) measures were seen at 6 weeks (all P < .01) with physical improvements sustained at 12 months (all P < .001). The number of patients at 12 months with ≥1 fall reduced from 66% to 33%, and the number not exercising reduced from 42% to 21%. A lack of exercise correlated with ≥1 fall at 12 months (OR 3.39, 95% CI 1.36-8.39, P = .009). It was also associated with poorer balance and psychosocial parameters at 12 months (all P < .05). CONCLUSIONS: Patients recruited into a 5-week Parkinson's disease education and exercise program achieved significant 12-month benefits in physical but not psychosocial measures. Patients with ≥1 fall post-treatment were less likely to have been exercising at 12-month follow-up.

Impact of small vessel disease on severity of motor and cognitive impairment in Parkinson's disease.

BACKGROUND: Subcortical small vessel disease and vascular risk factors are associated with motor and cognitive impairment. In this study we examined the relationship between these factors and the severity of motor impairment and dementia in pathologically-confirmed Parkinson's disease (PD). METHODS: The extent and severity of small vessel disease (SVD) was assessed pathologically in 77 patients with PD. The severity of motor impairment was determined using a cumulative index derived from longitudinal measures of Hoehn and Yahr score. The presence of dementia was scored using the Clinical Dementia Rating. The presence or absence of vascular risk factors and stroke were also recorded. Interactions were assessed using stepwise multiple regression analyses. RESULTS: Significant correlations were demonstrated between perivascular pallor in the globus pallidus interna and the Hoehn and Yahr stage and between increasing Braak PD stage, the number of vascular risk factors and dementia. Among the vascular risk factors, hypertension was the only variable to independently correlate with dementia. SVD pathology did not correlate with dementia in our cohort. CONCLUSIONS: This study demonstrates an association between SVD and motor impairment, and between vascular risk factors, particularly hypertension, and dementia in PD and highlights the need to manage vascular co-morbidities in PD patients.

The Effect of Biofeedback as a Psychological Intervention in Multiple Sclerosis: A Randomized Controlled Study.

BACKGROUND: Relaxation, mindfulness, social support, and education (RMSSE) have been shown to improve emotional symptoms, coping, and fatigue in multiple sclerosis (MS). Biofeedback was trialed as a psychological intervention, designed to improve self-control, in two groups of patients with MS. Both groups received RMSSE, and one group additionally received biofeedback. METHODS: Forty people with relapsing-remitting MS were recruited from three sites in Sydney, Australia. The mean disability score (Expanded Disability Status Scale; EDSS) was 2.41 ± 1.46 (95% confidence interval [CI], 1.46-3.36); the mean age in years was 45.9 ± 12.42 (95% CI, 41.92-49.87). Participants were randomly assigned to two active treatment groups (n = 20 per group). All participants received one 1-hour session per week for 3 weeks of RMSSE, while biofeedback equipment measured breathing rate and muscle tension. Members of one group used biofeedback screens to regulate physiological response. RESULTS: Whole-group pre- and post-treatment scores demonstrated a reduction of 38% for anxiety and 39% for depression scores (P = .007 and P = .009, respectively). A post-treatment comparison failed to demonstrate any significant difference between the two active treatment groups in anxiety and depression scores. The biofeedback group showed significant pre- to post-treatment improvement or trends toward improvement in anxiety, fatigue, and stress (P = .05, .02, and .03, respectively). Comparison of pre-post treatment results between groups showed improvements for the biofeedback group in breathing rate and muscle tension (P = .06 and .09). CONCLUSIONS: For relapsing-remitting MS patients receiving biofeedback in addition to RMSSE, there was a demonstrable reduction in anxiety, fatigue, and stress. There was also a trend toward significant reduction of breathing rate and muscle tension in favor of biofeedback.

Rebound exacerbation multiple sclerosis following cessation of oral treatment.

BACKGROUND: Multiple sclerosis (MS) management is changing, revolutionized by oral agents. Immune Reconstitution Inflammatory Syndrome (IRIS) describes exaggerated response to both exogenous (infective) and endogenous (non-infective) antigens. METHODS: This paper reports two cases of MS "rebound" following withdrawal of oral treatments. RESULTS: Two patients, with suboptimal response to interferons, trialled oral MS treatment (fingolimod and BG12) with excellent response. Upon cessation both experienced MS "rebound" which responded to steroids. CONCLUSIONS: "Rebound" may occur following withdrawal of oral MS therapies. Patients should be advised accordingly and possibly started on alternative treatment before the immunomodulating effect of therapy has subsided.

Small-vessel disease in patients with Parkinson's disease: a clinicopathological study.

Few studies have examined the relationship between cerebrovascular disease, vascular risk factors, and Parkinson's disease (PD), although 1 study found small-vessel disease (SVD) to be the main subtype of cerebrovascular disease. In this study we compared the extent and topography of SVD and assessed associated vascular risk factors in autopsy-proven PD cases and community-dwelling controls. Seventy-seven PD and 32 control brains from the Sydney Brain Bank were assessed microscopically by a single examiner blinded to the diagnosis. SVD was assessed by grading perivascular pallor, gliosis, hyaline thickening, and enlargement of perivascular spaces in the white matter underlying the superior frontal and primary motor cortices, basal ganglia, and white matter tracts. A history of vascular risk factors (hypertension, heart disease, diabetes, and cigarette smoking) was obtained. Groups were compared using stepwise multiple regression analysis. There was significantly greater frontal pallor (P = .004) and widening of perivascular spaces in the globus pallidus interna (P = .007) in controls compared with PD. Hyaline thickening and widening of perivascular spaces in the frontal white matter, hyaline thickening in the motor white matter, and widening of perivascular spaces in the caudate nucleus were more common in the control group, but did not reach significance. The prevalence of vascular risk factors and SVD pathology was significantly lower in autopsy-proven PD compared with controls (P = .03) living in the same community. The results of this study support the need for further research in this area.

Positional vomiting due to a thoracic spinal dural arteriovenous fistula. Case report.

The authors report the unique case of a patient with a thoracic spinal dural arteriovenous fistula (DAVF) causing remote brainstem symptoms of positional vomiting and minimal vertigo. Magnetic resonance (MR) imaging of the brain demonstrated high signal abnormality in the medulla, presumably related to venous hypertension, and spinal MR imaging revealed markedly dilated veins along the dorsal aspect of the cord. Spinal angiography confirmed the presence of a thoracic spinal DAVF. Disconnection of the DAVF from the spine resulted in a marked improvement in symptoms and resolution of the preoperative MR imaging-documented abnormalities. The authors highlight the rare syndrome of positional vomiting as a brainstem symptom and conclude that spinal DAVFs should be considered in the differential diagnosis of high signal MR imaging abnormalities localized to the brainstem.

Evoked potentials elicited by stimulation of the lateral and anterior femoral cutaneous nerves in meralgia paresthetica.

Seventy-five consecutive patients with clinical symptoms and signs of meralgia paresthetica underwent bilateral somatosensory evoked potential (SEP) studies involving stimulation of skin areas innervated by the lateral and anterior femoral cutaneous nerves of the thighs. The most common abnormality was an absolute lateral femoral cutaneous SEP latency > 40 ms in 35 patients (47%), followed by an absent response in 14 patients (19%), an absolute latency < 40 ms but amplitude reduction > 50% compared with the contralateral response in 8 patients (11%), and an absolute latency < 40 ms but > 5 ms interside latency difference in 5 patients (7%). Anterior femoral cutaneous SEPs were of value in distinguishing meralgia paresthetica from a proximal lumbar radiculopathy in an additional 4 patients and confirming bilateral meralgia paresthetica in 10 patients.