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

Ten patients who were unable to initiate or sustain eye opening in the absence of overt spasm of the orbicularis oculi, were investigated. In five, the problem was isolated. Three had Parkinson's disease and two progressive supra-nuclear palsy for between one to six years before the eye opening difficulty developed. The clinical features and electrophysiological investigation suggested that the disorder is a variant of blepharospasm due to abnormal contraction in the pre-tarsal orbicularis oculi.
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PMID:A new variant of blepharospasm. 160 9

We studied 104 patients with progressive supranuclear palsy (PSP), 38 of whom were examined by both a neurologist and a neuro-ophthalmologist. Neuro-ophthalmic findings that may help differentiate PSP from Parkinson's disease include vertical supranuclear ophthalmoparesis and fixation instability. Eyelid abnormalities, particularly lid retraction, blepharospasm, and "apraxia" of eyelid opening and closure, were important distinguishing signs. Although downgaze palsy is felt to be the clinical hallmark of PSP, upgaze and downgaze were equally affected at the time of diagnosis in our patients.
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PMID:Neuro-ophthalmic findings in progressive supranuclear palsy. 162 70

The results obtained in a retrospective study on clinical and pharmacological aspects of 41 patients suffering craniocervical dystonia (24 with blepharospasm, 17 with torticollis) and 11 with spasm are here presented. Mean age of symptoms onset was 57.4, 43.8 and 55.8 years old respectively; this variable was comparatively higher in females than in males with torticollis. The prevalence of blepharospasm and hemifacial spasm was higher in females. A 38.7% of patients suffering blepharospasm also presented oromandibular dystonia (Meige's syndrome). Other abnormal movements less frequently associated were cephalic tremor, postural hand tremor and larynx dystonia. In three cases with blepharospasm there was family history of Parkinson's disease and in two cases with torticollis there was family history of essential tremor. The mean age of onset was lower in patients with clonic torticollis and the evolution time of symptoms was longer than in those who presented the tonic type. Clonic torticollis were less frequently associated to pain. Trihexyphenidyl (anticholinergic) was the most efficient drug in craniocervical dystonia, and clonazepam in facial hemispasm. In general, as earliest the age of onset was, as better the therapeutical response was.
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PMID:[Craniocervical dystonia and facial hemispasm: clinical and pharmacological characteristics of 52 patients]. 176 88

Adult-onset dystonia-parkinsonism is a syndrome in search of a pathology. We therefore reviewed the literature on dystonic manifestations in autopsy-proven cases of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and idiopathic Parkinson's disease (PD). Only 6 of 140 autopsy reports of MSA remarked on the presence of dystonia in life, but personal observations suggest prominent antecollis may develop at some stage in up to 1/2 of sufferers. Similarly, very few (15/118) clinicopathologic observations on PSP included convincing dystonic manifestations, in contrast to some clinical reports where blepharospasm and early limb dystonia were prominent. Virtually any form of focal and segmental dystonia may sometimes occur with clinically diagnosed PD, with occasional descriptions of hemidystonia-hemiparkinsonism. However, there is pathologic confirmation of this diagnosis in only 1 case. With many patients thought clinically to have PD proving pathologically to have another cause for their parkinsonism, the true frequency and the range of dystonic manifestations acceptable in PD remain unknown.
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PMID:Dystonia in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy. 221 50

Apraxia of lid opening is a nonparalytic motor abnormality characterized by difficulty in initiating the act of lid elevation. It has been reported with extrapyramidal disorders, including Parkinson's disease, Huntington's chorea, progressive supranuclear palsy, and Shy-Drager syndrome. We found seven cases (7%) of functionally disabling apraxia of lid opening in 100 consecutive blepharospasm patients studied. It is important for physicians treating blepharospasm to be aware of the association between these two visually debilitating disorders.
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PMID:Apraxia of lid opening in blepharospasm. 238 54

We systematically videotaped eyelid movements in a community-based series of 38 patients with progressive supranuclear palsy (PSP). Ten patients (26%) had blepharospasm, "apraxia" of lid opening and/or "apraxia" of lid closing. These patients as a group had more severe upgaze paresis but no greater disease duration than the patients without supranuclear lid dysfunction. Patients used a variety of synkinetic movements to overcome lid-movement abnormalities. One patient displayed "slow blinks," a phenomenon not previously described in PSP. Blink rate in PSP, 3.0/min, was markedly lower than that in patients with Parkinson's disease (PD), 12.5/min, and patients with PSP but not PD increased their blink rate during command versional eye movements.
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PMID:Eyelid movement abnormalities in progressive supranuclear palsy. 205 16

Involuntary closure of eyelids (ICE), a phenomenon variously interpreted as blepharospasm and apraxia of lid opening, is occasionally observed in parkinsonism. Nine patients (4 with Parkinson's disease, 2 with post-encephalitic parkinsonism, and 3 with supranuclear palsy) with prominent ICE, were studied by electromyographic recording of the eye muscles. ICE episodes were shown to be dependent upon prolonged, irregular inhibition of the normal tonic activity of the levator palpebrae superioris (LPS) muscle causing drooping of the upper eyelid without any corresponding activation of the orbicularis oculi (OO) muscle. Nevertheless, some degree of excessive, widely fluctuating OO activity was present in seven of the patients. Blepharocolysis (from Gr. blepharon, eyelid, and kolysis inhibition) is put forward as the term to designate ICE episodes resulting from abnormally long inhibition of the LPS muscles and should be differentiated electrophysiologically from blepharospasm, excessive OO muscles activity. Abnormal influences from basal ganglia acting on brainstem structures that regulate blinking may falicitate either of the two components of normal blinking resulting in ICE due to the predominance of LPS inhibition (blepharocolysis), the predominance of OO activation (blepharospasm) or a combination of the two.
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PMID:Involuntary closure of eyelids in parkinsonism. Electrophysiological evidence for prolonged inhibition of the levator palpebrae muscles. 321 29

Most classifications of movement disorders emphasize their differential diagnosis and epidemiology according to clinical history and neurological examination. This review of movement disorders is organized according to the hypothesis of basal ganglia neurotransmitter imbalance in order to emphasize current research based on the pharmacology of these disorders. Specifically, dopamine (DA) excess and acetylcholine (ACh) deficiency may characterize part of the pathology of several hyperkinetic movement disorders including tardive dyskinesia, Huntington disease, Gilles de la Tourette syndrome, l-dopa dyskinesias, tardive Tourette syndrome, and toxic Tourette syndrome. The mirror image of this paradigm, namely DA deficiency and ACh excess, may characterize several rigid-dystonic movement disorders including Parkinson disease, drug-induced dystonias, and dystonia musculorum deformans. Finally, the unique combination of DA excess with ACh excess may characterize idiopathic orofacial dyskinesia (also known as Meige dystonia, Brueghel syndrome, and blepharospasm-oromandibular dystonia). Evidence supporting this formulation of movement disorders is reviewed, the limitations of this hypothesis are discussed, and new data from our own studies are presented.
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PMID:The neuropharmacology of tardive dyskinesia, spontaneous dyskinesia, and other dystonias. 612 51

Dystonia is a persistent attitude or posture in one or other of the extremes of athetoid movement. It may take the form of an over-extension or over-flexion of the hand, torsion of the spine, with arching and twisting of the back or forceful closure of the eyes and a fixed grimace. Dystonia is classified into idiopathic and symptomatic dystonia. Idiopathic dystonia is further divided into generalized, focal and segmental dystonia. Generalized dystonia covers classical torsion dystonia, paradoxical dystonia, myoclonic dystonia, dystonia with diurnal variation and Dopa-responsive dystonia. Dystonic tic, paroxysmal dystonia and hypnotic dystonia show a dystonic posture, although they are also accompanied by various other involuntary movements such as athetosis or chorea. Torticollis, writer's cramp or blepharospasm is assigned to the focal dystonia and Meige syndrome to the segmental dystonia. Symptomatic dystonia is observed in various neurological disorders, including cerebrovascular diseases, Parkinson's disease and Wilson's disease.
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PMID:[Dystonia]. 827 58

The clinical, pathophysiological and therapeutic approaches to the representative involuntary movements encountered in the aged are described. The prevalence rates of Parkinson disease and essential tremor are very high, and their diagnoses and treatments are quite important. Recent advances in treating Parkinson disease with anti-parkinsonian medications and essential tremor with beta-adrenergic blockers were presented. Blepharospasms, though uncommon, but occasionally seen in the aged persons, are disabled conditions. The botulinus toxin injections to the orbicularis oculi muscles proved to show dramatic therapeutic effects, greatly contributing to these patients' ADL. The importance of neuroscience in the coming 21st century is also stressed.
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PMID:[Motor dysfunction in the aged--approach to involuntary movements]. 926 46


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