Does spouse participation influence quality of life reporting in patients with Parkinson's disease?

PURPOSE: To investigate whether patient-spouse co-reporting (patient reporting with assistance from their spouse) results in the same ratings of health-related quality of life (HRQoL) as patient ratings without co-reporting, and to assess whether mutuality of the marital relationship is a determinant of co-reported ratings. Patients are the best source of HRQoL; however, co-reporting is common in clinical settings, but has not been compared to independent patient reporting of HRQoL. METHODS: Fifty-nine Parkinson's disease (PD) patient-spouse pairs completed the Short Form Health Status Survey (SF-12), measuring mental and physical HRQoL. Initially, the patient and spouse completed the SF-12 independently (about the patient). Then, patient-spouse pairs completed the SF-12 together assessing the patient's HRQoL. Patients and spouses independently completed the Mutuality Scale rating the intimacy of their relationship. RESULTS: Patients rated physical HRQoL higher (M = 46.6) than spouses alone (M = 43.4, p < 0.01) and co-reporting (M = 44.8, p < 0.05). Co-reporting rated physical HRQoL in between that of patients and spouses, (p < 0.05). Spouses who reported greater mutuality showed greater concordance with the patient regarding the patient's mental HRQoL (B = -0.43, p < 0.05). CONCLUSION: Consistency of the mode of completion of HRQoL instruments is important since co-reporting may alter HRQoL ratings in PD and lead to inaccurate conclusions. Mutuality is a mediator of mental HRQoL.

Impact of belief in neuroprotection on therapeutic intervention in Parkinson's disease.

We explored the hypotheses that an investigator's belief in a putative neuroprotective agent might influence the timing of symptomatic intervention and the assessment of signs and symptoms of patients with Parkinson's disease with the Unified Parkinson's Disease Rating Scale (UPDRS). These hypotheses were tested with Cox and general linear modeling, using data from a previously published double-blind placebo-controlled futility trial of coenzyme Q(10) and GPI-1485. We found the investigators' level of confidence in these agents had no effect on the time to symptomatic therapy or on the change in UPDRS during 12 months of treatment.

Metoclopramide-induced encephalopathy in Parkinson disease.

A case of prolonged encephalopathy and worsened parkinsonism in a Parkinson disease patient exposed to a short course of metoclopramide is described. Parkinson disease (PD) is the second most common neurodegenerative disease in the United States. Because of the increased susceptibility to adverse drug effects, PD presents a special challenge to physicians. Anti-emetic drugs such as metoclopramide are widely used and may be particularly deleterious to PD patients due to blockade of dopamine receptors.

The evolution of disability in Parkinson disease.

The objectives of this study are to assess the level of disease severity associated with disability in Parkinson disease (PD) and the sequence of loss of independence in basic and instrumental activities of daily living (ADLs and IADLs). Six hundred eighteen patients with PD were evaluated for disease severity with the Unified PD Rating Scale (UPDRS) and for disability with the Older Americans Resource and Services Disability Subscale (OARS). The association between patient-reported disability on ADLs and IADLs and level of disease severity on the total UPDRS was examined cross-sectionally. Disability, with loss of independent function is reported between total UPDRS scores 30 to 40, and HY stages II to III. Difficulty with daily activities, without loss of independent function is reported earlier, at UPDRS <20 and HY I to II. Difficulty with walking is initially reported, followed by problems with a number of gait-dependent activities including housework, dressing, transferring in and out of bed, and traveling in the community. The transition from HY stage II to III marks a pivotal milestone in PD, when gait and balance impairment results in disability in many gait-dependent activities. The onset of disability in PD can be identified by asking patients about their walking, housework, dressing, and traveling. While individual patients vary in progression, the benchmarks of disability in this study provide guidance when counseling patients about prognosis. Better understanding of the stages of disability may facilitate the development of novel outcome measures in clinical trials in PD.

Mutuality of the marital relationship in Parkinson's disease.

To assess the association between mutuality of the marital relationship in Parkinson's disease with disease severity, disability, mental health, quality of life, and caregiver burden. Spouses of patients with PD completed questionnaires assessing mutuality of the marital relationship (Mutuality Scale) and caregiver strain (Caregiver Strain Index). Patients and spouses completed scales assessing their mental health (Brief Symptom Inventory-18), medical co-morbidity (Cumulative Illness Rating Scale) and health-related QoL (SF-12v2). PD severity and disability were assessed with the Unified Parkinson's Disease Rating Scale and the Older Americans Resource and Services Disability Scale. The relationships between mutuality and patient and spousal variables were analyzed with univariate correlations and multiple regression. Ninety-six spouse-patient pairs were assessed. Increased mutuality, as reported by the spouse was associated with reduced caregiver burden, less depression of both spouse and patient, and less PD severity. Mutuality was inversely correlated with gait impairment, with lesser correlations for balance, urinary incontinence and motor fluctuations. Greater mutuality between spouses and patients with PD is associated with better mental health of both partners, reduced caregiver burden and improved spousal quality of life. PD severity, especially gait, balance, urinary incontinence and motor fluctuations are particular stressors on the marital relationship.

A comparative study of physical performance measures in Parkinson's disease.

UNLABELLED: The objective of this study is to compare physical performance measures for their ability to discriminate between levels of disability and disease severity in Parkinson's disease (PD). Disability in PD is commonly assessed by patient self-report, which may be limited by patient insight. METHODS: Seventy-nine patients with PD were tested with seven performance measures: Physical Performance Test (PPT), modified Physical Performance Test (mPPT), Short Physical Performance Battery (SPPB), Performance Test of Activities of Daily Living (PADL), Berg Balance Scale (BBS), Timed Up and Go (TUG), and Functional Reach (FR). These measures were compared with patient-reported disability on the Older Americans Resource and Services Disability subscale (OARS) and disease severity on the Unified Parkinson's Disease Rating Scale (UPDRS). The performance measures were more sensitive to levels of disease severity than disability. Four measures discriminated across quartiles of disability (PPT, mPPT, BBS, TUG: P < 0.05), whereas all seven measures discriminated across quartiles of the Total UPDRS (PPT, mPPT, BBS, TUG, FR: P < 0.01; SPPB, PADL: P < 0.05). However, no measure consistently discriminated between subgroups with a range of early and advanced disease severity. The seven physical performance measures showed different profiles of strengths and weaknesses in assessing disability and disease severity. The results of this study will facilitate choosing performance measures for clinical care and clinical trials in PD.

Metatarsal fracture as a consequence of foot dystonia in Parkinson's disease.

We report a 45-year-old man with a 4-year history of Parkinson's disease complicated by the development of left-foot dystonia resulting in a fracture of the fifth metatarsal. Orthopedic injuries are common in Parkinson's disease, but they are usually secondary to falls. Although drug-induced dystonia is a common side effect of pharmacological treatment of Parkinson's disease, this is the first report of a fracture related to these abnormal movements.

Early diagnosis of Parkinson's disease and initiation of treatment.

The diagnosis of Parkinson's disease remains a clinical diagnosis with no confirmatory laboratory or imaging studies available. The classic diagnostic criteria include 2 of 3 cardinal motor features of parkinsonism (resting tremor, cogwheel rigidity, and bradykinesia) on examination. Interest in a "premotor diagnosis" of Parkinson's disease is based on the hope that neuroprotective therapy could be initiated earlier and affect disease course. However, there is no proven method to diagnose Parkinson's disease prior to the onset of motor signs and there is no proven neuroprotective treatment. Once the diagnosis is made, the neurologist must decide, with the patient, whether to institute treatment at the time of diagnosis or whether to institute treatment when functional disability evolves. There are multiple possible initial pharmacologic choices for the initial treatment of Parkinson's disease, including monoamine oxidase type B inhibitors, dopamine receptor agonists, and levodopa/carbidopa.

Progressive supranuclear palsy.

Since progressive supranuclear palsy (PSP) was first reported as a separate clinicopathological entity in 1964, hundreds of other cases have been recorded, and PSP is now one of the most common atypical Parkinson-plus disorders. Diagnostic criteria have been developed by the National Institute of Neurological Disorders and Stroke and the Society for PSP, Inc. Because there is no biological marker for PSP, definitive diagnosis depends on neuropathological examination. Characteristics of PSP include gait disturbances, supranuclear ophthalmoplegia, axial limb rigidity, and frontal lobe dysfunction. Although there are no treatments that alter the natural history of disease in PSP and no drugs that provide significant symptomatic benefits, several supportive measures are available.

Are Patients with Psychogenic Movement Disorders More Likely to be Healthcare Workers?

BACKGROUND: Reported risk factors for the development of a psychogenic movement disorder (PMD) include young age, female gender, history of abuse, current or past psychiatric disorder, lower levels of education and socioeconomic status, and employment as a healthcare worker. Although employment in healthcare is included in several diagnostic criteria for PMD, as well as in many case series, this association has never been validated. METHODS: Using the University of Maryland Movement Disorder Database (UMMDD), we identified PMD cases, as well as patients with isolated focal dystonia as controls. An experienced movement disorder specialist diagnosed all patients, and all cases met criteria for clinically established PMD. Demographic and occupational histories were obtained from medical records and were supplemented by telephone interviews. PMD cases and controls were compared using t tests/χ2 tests. RESULTS: Controls (n = 148) were older than PMD cases (n = 132), with an average age of 61.4 and 52.1 years, respectively (P < 0.001); there were no significant differences between groups with respect to gender, education level, and ethnicity. The proportion of healthcare workers was not significantly different between PMD cases and controls (25% of PMD cases vs. 20% of controls; P = 0.28). CONCLUSIONS: In contrast to traditional teaching, this investigation demonstrates that in our patient population, patients with a PMD were no more likely to be employed as healthcare workers than patients with isolated focal dystonia. This study calls into question the use of employment in healthcare as a reliable criterion to support the diagnosis of PMD.

Psychosis in Parkinson's disease: case studies.

Psychosis is common in patients who have PD and leads to significant disability. Patients often can be managed with non-pharmacologic interventions or with decreasing doses of anti-parkinsonism medications. If these interventions are insufficient, then atypical antipsychotics should be considered. Clozapine is used in more refractory cases and requires stringent monitoring for agranulocytosis.

Motor fluctuations in Parkinson's disease.

Parkinson's disease is characterized by progressive slowness in activities of daily living and is the most common cause of parkinsonism, whose symptoms include resting tremor, cogwheel rigidity, and bradykinesia. The introduction of levodopa and its positive effect on motor dysfunction in Parkinson's disease has allowed neurologists to focus on motor fluctuations. "End-of-dose wearing-off" and "morning akinesia" are terms to describe the transition between a patient's relatively normal motor performance when levodopa is effective and when it has transiently lost its effect on motor responses and parkinsonian symptoms reemerge. The choices available to alleviate these motor fluctuations range from altering the patient's levodopa/carbidopa dosing schedule to the addition of other agents to the regimen, including dopamine receptor agonists, catechol-O-methyltransferase inhibitors, monoamine oxidase inhibitors, and amantadine, as well as implementing dietary changes. Therapeutic decisions can be difficult because older agents have not been compared in head-to-head trials to determine which drugs are better than others and the order in which they should be tried or added to the levodopa regimen; however, all of the available treatments provide a good possibility of benefit to the patient. Deep brain stimulation surgery is an option for patients with medically intractable severe motor fluctuations.

Advances in the diagnosis, treatment, and understanding of Parkinson's disease and parkinsonism.

Highlights from the Twentieth Annual Symposium on the Etiology, Pathogenesis, and Treatment of Parkinson's Disease and Other Movement Disorders October 8, 2006, Chicago, IL. Why do substantia nigra neurons die in patients with Parkinson's disease? Dr. Weiner discusses an interesting hypothesis presented at the 2006 symposium. He also reviews some of the other meeting highlights, including reports on pathologic gambling, the under-recognition of drug-induced parkinsonism, and the latest studies on estrogen replacement therapy, deep brain stimulation, and botulinum toxin.

A differential diagnosis of Parkinsonism.

Parkinsonism is a common, age-related syndrome, characterized by resting tremor, bradykinesias, rigidity, and postural reflex impairment. Though Parkinsonism is not very difficult to recognize, all Parkinsonism is not created equal and it is important to distinguish among the most common identifiable syndromes. This review discusses the key clinical features of these various syndromes, including Parkinson's disease, progressive supranuclear palsy, multiple system atrophy, corticobasal ganglionic degeneration, Lewy body disease, vascular Parkinsonism, and Parkinsonism with no clear etiology. Symptomatology and diagnostic testing for each syndrome are discussed and 4 typical cases are analyzed to offer clinicians guidance in making a differential diagnosis for Parkinsonism.

Multiple mechanisms of rhabdom shedding in the lateral eye of Limulus polyphemus.

Rhabdom shedding in horseshoe crab lateral eye photoreceptors was studied with anti-opsin and anti-arrestin immunocytochemistry. Two, possibly three, distinct shedding mechanisms were revealed in animals maintained in natural lighting. Transient rhabdom shedding, triggered by dawn, is a brief, synchronous event that removes up to 10% of the rhabdom membrane. Whorls of rhabdomeral membrane break into vesicles and form compact multivesicular bodies. These debris particles are immunoreactive for opsin and are of a relatively uniform size, averaging approximately 2 microm(2) in area. Transient shedding requires that input from circadian efferent fibers to the retina precedes the light trigger, and cutting the optic nerve blocks efferent input and transient shedding. Light-driven rhabdom shedding is a progressive process. Rhabdomeral membrane is removed by coated vesicles that accumulate into loosely packed multivesicular bodies. These debris particles label with antibodies directed against opsin, arrestin, and adaptin, and they have a large distribution of sizes, averaging almost 6 microm(2) in area and ranging up to 25 microm(2) or more. The amount of rhabdomeral membrane removed by light-driven shedding has seasonal variation and depends on latitude. Light-driven shedding does not require circadian efferent input. A possible third shedding mechanism, light-independent shedding, is observed when transient shedding is blocked either by 48 hours of darkness or by cutting the optic nerve. Small particles, averaging 1.8 microm(2) in area, exhibiting opsin but not arrestin immunoreactivity can then be found in the cytoplasm surrounding the rhabdom. The nature of light-independent shedding is not yet clear.

Pramipexole vs levodopa as initial treatment for Parkinson disease: a 4-year randomized controlled trial.

BACKGROUND: The best way to initiate dopaminergic therapy for early Parkinson disease remains unclear. OBJECTIVE: To compare initial treatment with pramipexole vs levodopa in early Parkinson disease, followed by levodopa supplementation, with respect to the development of dopaminergic motor complications, other adverse events, and functional and quality-of-life outcomes. DESIGN: Multicenter, parallel-group, double-blind, randomized controlled trial. SETTING: Academic movement disorders clinics at 22 sites in the United States and Canada. PATIENTS: Patients with early Parkinson disease (N = 301) who required dopaminergic therapy to treat emerging disability, enrolled between October 1996 and August 1997 and observed until August 2001. INTERVENTION: Subjects were randomly assigned to receive 0.5 mg of pramipexole 3 times per day with levodopa placebo (n = 151) or 25/100 mg of carbidopa/levodopa 3 times per day with pramipexole placebo (n = 150). Dosage was escalated during the first 10 weeks for patients with ongoing disability. Thereafter, investigators were permitted to add open-label levodopa or other antiparkinsonian medications to treat ongoing or emerging disability. MAIN OUTCOME MEASURES: Time to the first occurrence of dopaminergic complications: wearing off, dyskinesias, on-off fluctuations, and freezing; changes in the Unified Parkinson's Disease Rating Scale and quality-of-life scales; and adverse events. RESULTS: Initial pramipexole treatment resulted in a significant reduction in the risk of developing dyskinesias (24.5% vs 54%; hazard ratio, 0.37; 95% confidence interval [CI], 0.25-0.56; P<.001) and wearing off (47% vs 62.7%; hazard ratio, 0.68; 95% CI, 0.49-0.63; P =.02). Initial levodopa treatment resulted in a significant reduction in the risk of freezing (25.3% vs 37.1%; hazard ratio, 1.7; 95% CI, 1.11-2.59; P =.01). By 48 months, the occurrence of disabling dyskinesias was uncommon and did not significantly differ between the 2 groups. The mean improvement in the total Unified Parkinson's Disease Rating Scale score from baseline to 48 months was greater in the levodopa group than in the pramipexole group (2 +/- 15.4 points vs -3.2 +/- 17.3 points, P =.003). Somnolence (36% vs 21%, P =.005) and edema (42% vs 15%, P<.001) were more common in pramipexole-treated subjects than in levodopa-treated subjects. Mean changes in quality-of-life scores did not differ between the groups. CONCLUSIONS: Initial treatment with pramipexole resulted in lower incidences of dyskinesias and wearing off compared with initial treatment with levodopa. Initial treatment with levodopa resulted in lower incidences of freezing, somnolence, and edema and provided for better symptomatic control, as measured by the Unified Parkinson's Disease Rating Scale, compared with initial treatment with pramipexole. Both options resulted in similar quality of life. Levodopa and pramipexole both appear to be reasonable options as initial dopaminergic therapy for Parkinson disease, but they are associated with different efficacy and adverse-effect profiles.

Transient movement disorders and multiple sclerosis.

Movement disorders associated with multiple sclerosis (MS) are uncommon, except for tremor. We report two patients with relapsing-remitting MS, who developed either dystonia or chorea during clinical exacerbation of their MS. The movement disorders resolved during treatment with adrenocorticotropin hormone (ACTH). Acute exacerbations of MS may be associated with transient movement disorders, which are responsive to ACTH.