Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030567 (Parkinson's disease)
 63,064 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with Parkinson's disease (PD) often show impaired manual dexterity even when being only minimally bradykinetic, suggesting that they may have limb kinetic apraxia (LKA), that is, a loss of fine motor skill not explained by elemental motor deficits. To explore this dissociation, we investigated the differential dopaminergic responsiveness of dexterity and bradykinesia in PD. Twelve patients with PD (4 women, age 64.4 +/- 8.3, mean + SD) and 12 matched healthy controls (64.8 +/- 8.9) were tested twice in ON vs. OFF and 1st vs. 2nd trial, respectively. A coin rotation (CR) task was applied to assess dexterity and a finger tapping (FT) task to assess bradykinesia. Performance was followed by video recording and analyzed by measuring the frequency of CR and FT during three 10-second periods. Statistical analysis was done by a mixed factorial design with group (PD vs. controls) as between-subject factor and medication (ON- vs. OFF-state or 1st vs. 2nd trial), task (FT vs. CR), and handedness (dominant vs. nondominant) as within-subject factors. In patients with PD, regardless of hand involved, dopaminergic treatment only mildly improved CR performance, in contrast to the strong increase in FT scores (up to the level of controls), as demonstrated by the significant triple interaction of the factors group, medication, and task (F(1,22) = 7.9, P = 0.01; eta(2) = 0.26). Furthermore, CR scores were considerably lower, both in OFF and ON, than in normal controls, pointing to a substantial impairment of dexterity in PD (P < 0.001). In conclusion, impaired manual dexterity showing significantly diminished response to dopaminergic treatment suggests that dextrous deficits in PD are related to LKA rather than bradykinesia.
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PMID:Poor dopaminergic response of impaired dexterity in Parkinson's disease: Bradykinesia or limb kinetic apraxia? 1864 88

Botulinum toxin (BoNT) has been used for over a quarter of century for the treatment of well over 100 different indications. Many of the symptoms for which BoNT has been found to be effective occur in a variety of neurological disorders. One neurodegenerative disorder in which BoNT has been used extensively to treat various symptoms is Parkinson's disease (PD). This review will highlight the following therapeutic applications of BoNT in conditions associated with PD: limb dystonia, blepharospasm and lid apraxia, bruxism, cervical dystonia (anterocollis), camptocormia, hand and jaw tremor, rigidity (painful shoulder), freezing of gait, sialorrhea, dysphagia (achalasia), seborrhea, hyperhidrosis, overactive bladder, and constipation.
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PMID:Disease-oriented approach to botulinum toxin use. 1907 3

Corticobasal degeneration (CBD) is a neurodegenerative disorder characterized clinically by a combination of cortical and basal ganglia signs. Pathologically, it is classified as a tauopathy. The most distinctive clinical feature is its unilateral or markedly asymmetric presentation; among parkinsonian syndromes, with rare exceptions, only Parkinson's disease presents with such asymmetry. The most common presenting cortical features include apraxia (patients often complain of a "useless" limb), aphasia (usually nonfluent), parietal lobe sensory signs (agraphesthesia, extinction, astereognosis), frontal dementia, or myoclonus. Basal ganglia signs include rigidity, akinesia, limb dystonia, and postural instability. The diagnosis is often challenging for three reasons: 1) The full complement of findings are rarely seen at presentation; 2) If CBD is not suspected, subtle but relevant findings (eg, extinction, language impairment, myoclonus, or apraxia) may not be searched for or appreciated; 3) The clinical picture of CBD has substantial overlap with a variety of other parkinsonian and dementing illnesses. The differential diagnosis includes Parkinson's disease, progressive supranuclear palsy, frontotemporal dementia, primary progressive aphasia, and Alzheimer's disease. The clinical diagnosis is not confirmed pathologically in up to half of cases, so the term corticobasal syndrome is often preferred during life, reserving the term corticobasal degeneration for pathologically verified cases. Treatment of CBD is primarily supportive, and most patients die within 10 years of onset. Parkinsonian signs may improve to a modest degree with levodopa, clonazepam can suppress myoclonus, and botulinum toxin can relieve dystonia. Early speech therapy, physical therapy, and occupational therapy, as well as assist devices such as a rolling walker may improve functioning and reduce complications such as aspiration pneumonia and falls. With time, however, most patients lose their independence and mobility. Throughout the course of the illness (particularly when it is advanced), caring for the caregiver is as important as caring for the patient.
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PMID:Corticobasal degeneration. 1936 52

Gait apraxia is most commonly a part of the Hakimov triad (gait apraxia, urinary incontinence, dementia) in normotensive hydrocephalus (NPH), although it may be a symptom of some other conditions. In our case the patient was a long term Parkinson's disease sufferer who developed normotensive hydrocephalus and consequently gait apraxia. Only after a third successive evacuation of the CSF his gait apraxia improved (Fig. 1, Ref. 15). Full Text (Free, PDF) www.bmj.sk.
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PMID:Gait apraxia. 2042 24

Progressive supranuclear palsy is a rare, degenerative brain disorder and the second most common syndrome in which the patient exhibits 'parkinsonism', that is, a variety of symptoms involving problems with movement. General symptoms include difficulties with gait and balance; the patient walking clumsily and often falling backwards. The syndrome can be difficult to diagnose and visual signs and symptoms can help to separate it from closely related movement disorders such as Parkinson's disease, multiple system atrophy, dementia with Lewy bodies and corticobasal degeneration. A combination of the presence of vertical supranuclear gaze palsy, fixation instability, lid retraction, blepharospasm and apraxia of eyelid opening and closing may be useful visual signs in the identification of progressive supranuclear palsy. As primary eye-care practitioners, optometrists should be able to identify the visual problems of patients with this disorder and be expected to work with patients and their carers to manage their visual welfare.
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PMID:Visual signs and symptoms of progressive supranuclear palsy. 2062 67

Impairment of hand dexterity in Parkinson's disease (PD) is usually attributed to bradykinesia. Recently, behavioral studies illustrated that decreased dexterity might also be due to limb-kinetic apraxia (LkA), as demonstrated by impaired performance in a coin rotation task. Here, we provide a first investigation on whether functional magnetic resonance imaging (fMRI) may reveal specific brain activation patterns for PD patients with impaired performance in a coin rotation task. We compared coin rotation as an apraxia task to simple finger tapping as a bradykinesia task in ten PD patients OFF medication and matched healthy controls. In addition to a tendency for general overactivation, PD patients showed a perirolandic dissociation with precentral overactivation and postcentral underactivation. This finding significantly separated PD patients from healthy controls.
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PMID:FMRI correlates of apraxia in Parkinson's disease patients OFF medication. 2065 52

The atypical parkinsonian disorders (APD) embrace a heterogeneous group of movement disorders all characterized by prominent parkinsonism, accompanied by specific additional features such as cerebellar ataxia, early autonomic dysfunction, early dementia, pyramidal tract signs, myoclonus, supranuclear gaze palsy, apraxia which are atypical for idiopathic Parkinson's disease (PD). Beside these features, rapid disease progression and poor or absent response to L-Dopa therapy both raise the suspicion of an APD. Currently, multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and dementia with Lewy bodies (DLB) are referred to as APD. Clinical diagnosis can be difficult in early stages and although the predictive value of the widely established, diagnostic criteria is high at first neurological evaluation sensitivity tends to be poor and may be less than 30%. In this review, we will discuss diagnostic issues in MSA and PSP.
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PMID:Diagnostic criteria for multiple system atrophy and progressive supranuclear palsy. 2081 85

The long-term efficacy and safety of deep brain stimulation (DBS) implant for Parkinson's disease (PD) is described in several recent papers. This procedure has been reported to permit a stable reduction of dopaminergic therapy requirements for up to 5 years, although some expectation of deterioration in non-dopaminergic signs has been recently stated. Our aim is to perform a literature-based review of papers available describing long-term post-operative follow-up after a bilateral implant for subthalamic DBS (STN-DBS). Only peer-reviewed published papers with a post-operative follow-up of at least 5 years were considered. Clinical outcome, disease progression and side effects were assessed at baseline and 2 (or 3 years) and 5 years after surgery. Seven papers were included in the review. A total of 238 patients were analyzed. STN-DBS was confirmed to be an effective treatment for selected patients with PD. In all studies, off-related motor symptoms improved dramatically, compared with pre-implant, at 2 (or 3, according to the study) years and this result persisted at 5-year evaluations. Antiparkinsonian drug reductions, improvements in motor fluctuations and dyskinesias, functional measures and the progression of underlying PD were also reported in all series. Some axial scores, in particular postural stability and speech, improved transiently. Persisting adverse effects included eyelid opening apraxia, weight gain, psychiatric disorders, depression, dysarthria, dyskinesias, and apathy. The present review of the 5-year observations confirms that STN-DBS is a powerful method in the management of PD, but its long-term effects must be thoroughly assessed.
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PMID:Dopaminergic therapy and subthalamic stimulation in Parkinson's disease: a review of 5-year reports. 2108 Jan 93

Deficiency in repair of nuclear and mitochondrial DNA damage has been linked to several neurodegenerative disorders. Many recent experimental results indicate that the post-mitotic neurons are particularly prone to accumulation of unrepaired DNA lesions potentially leading to progressive neurodegeneration. Nucleotide excision repair is the cellular pathway responsible for removing helix-distorting DNA damage and deficiency in such repair is found in a number of diseases with neurodegenerative phenotypes, including Xeroderma Pigmentosum and Cockayne syndrome. The main pathway for repairing oxidative base lesions is base excision repair, and such repair is crucial for neurons given their high rates of oxygen metabolism. Mismatch repair corrects base mispairs generated during replication and evidence indicates that oxidative DNA damage can cause this pathway to expand trinucleotide repeats, thereby causing Huntington's disease. Single-strand breaks are common DNA lesions and are associated with the neurodegenerative diseases, ataxia-oculomotor apraxia-1 and spinocerebellar ataxia with axonal neuropathy-1. DNA double-strand breaks are toxic lesions and two main pathways exist for their repair: homologous recombination and non-homologous end-joining. Ataxia telangiectasia and related disorders with defects in these pathways illustrate that such defects can lead to early childhood neurodegeneration. Aging is a risk factor for neurodegeneration and accumulation of oxidative mitochondrial DNA damage may be linked with the age-associated neurodegenerative disorders Alzheimer's disease, Parkinson's disease and amyotrophic lateral sclerosis. Mutation in the WRN protein leads to the premature aging disease Werner syndrome, a disorder that features neurodegeneration. In this article we review the evidence linking deficiencies in the DNA repair pathways with neurodegeneration.
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PMID:DNA repair deficiency in neurodegeneration. 2155 Mar 79

Progressive supranuclear palsy (PSP) has been described as a clinical syndrome characterized by an impairment of voluntary control of gaze (supranuclear palsy), postural and gait instability, and behavioral and cognitive deficits including a frontal syndrome and psychic retardation. However, in the recent years, at least four other clinical forms of PSP have been recognized: PSP-Parkinsonism, "pure akinesia with gait freezing", PSP with cortico-basal syndrome, and PSP with speech apraxia. PSP-Parkinsonism mimics the signs and symptoms of idiopathic Parkinson's disease, including a significant reactivity to levodopa. "Pure akinesia with gait freezing" is characterized by a difficulty of self-initiation of motor programs, usually walking program. PSP with cortico-basal syndrome mimics cortico-basal degeneration (CBD) in that unilateral or asymmetric limb dystonia and apraxia are prominent signs. PSP with speech apraxia is an isolated syndrome of progressive anarthria. All these clinical syndromes are due to brain accumulation of phosphorylated tau protein. The differences in clinical expression within the framework of PSP can be explained by the differences in the topographical distribution of the lesions. PSP is considered as a primary tau disease ("tauopathy") such as CBD and some forms of fronto-temporal lobar degeneration. At the level of neuropathology, the pattern of tau abnormal inclusions differentiates PSP from other tau diseases, but some overlaps are reported. Moreover, several of the clinical forms of PSP partially or fully overlap with the other tauopathies. As a whole, the emergence of new clinical forms of PSP challenges the nosology of tauopathies and our understanding of these diseases.
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PMID:[Progressive supranuclear palsy: what's new?]. 2169 28


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