Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0003635 (apraxia)
 2,817 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present the case of a 72-year-old woman with a history of a bipolar mood disorder chronically treated with lithium. Upon having the dose increased, she developed an acute confusional state accompanied by blepharospasm (BS) and apraxia of eyelid opening. Gait instability with frequent falls, pyramid tract signs, and postural tremor in both hands were also evident. On withdrawing lithium, symptoms remitted within 2 weeks. This patient illustrates that BS and apraxia of eyelid opening may be triggered by lithium overdose. Our case warrants the inclusion of lithium in the list of drugs liable to induce such movement disorders.
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PMID:Blepharospasm and apraxia of eyelid opening in lithium intoxication. 1036 83

The authors report the underestimated cognitive, mood, and behavioral complications in patients who have undergone bilateral contemporaneous pallidotomy, as seen in their early experience with functional neurosurgery for Parkinson's disease (PD) that is accompanied by severe motor fluctuations before pallidal stimulation. Four patients, not suffering from dementia, with advanced (Hoehn and Yahr Stages III-IV), medically untreatable PD featuring severe "on-off" fluctuations underwent bilateral contemporaneous posteroventral pallidotomy (PVP). All patients were evaluated according to the Core Assessment Program for Intracerebral Transplantations (CAPIT) protocol without positron emission tomography scans but with additional neuropsychological cognitive, mood, and behavior testing. For the first 3 to 6 months postoperatively, all patients showed a mean improvement of motor scores on the Unified Parkinson's Disease Rating Scale (UPDRS), in the best "on" (21%) and worst "off" (40%) UPDRS III motor subscale, a mean 30% improvement in the UPDRS II activities of daily living (ADL) subscore, and 60% on the UPDRS IV complications of treatment subscale. Dyskinesia disappeared almost completely, and the mean daily duration of the off time was reduced by an average of 60%. Despite these good results in the CAPIT scores, one patient experienced a partially regressive corticobulbar syndrome with dysphagia, dysarthria, and increased drooling. No emotional lability was found in this patient, but he did demonstrate severe bilateral postoperative pretarsal blepharospasm (apraxia of eyelid opening), which interfered with walking and which required treatment with high-dose subcutaneous injections of botulinum toxin. No patient showed visual field defects or hemiparesis, but postoperative depression, changes in personality, behavior, and executive functions were seen in two individuals. Postoperative abulia was reported by the family of one patient, who lost his preoperative aggressiveness and drive in terms of ADL, speech, business, family life, and hobbies, and became more sleepy and fatigued. One patient reported postoperative mental automatisms, such as compulsive mental counting, and circular thoughts and reasoning during off phases; postoperative depression was found in two patients. However, none of the patients demonstrated these symptoms during intraoperative microelectrode stimulation. These findings are compatible with previous reports on bilateral pallidal lesions. A progressive lowering of UPDRS subscores was seen after 12 months, consistent with the progression of the disease. Bilateral simultaneous pallidotomy may be followed by emotional, behavioral, and cognitive deficits such as depression, obsessive-compulsive disorders, and loss of psychic autoactivation-abulia, as well as disabling corticobulbar dysfunction and apraxia of eyelid opening, in addition to previously described motor and visual field deficits, which make this surgery undesirable even though significant improvement in motor deficits can be achieved.
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PMID:Bilateral contemporaneous posteroventral pallidotomy for the treatment of Parkinson's disease: neuropsychological and neurological side effects. Report of four cases and review of the literature. 1070 52

We report a case of a unique eye sign following right hemispheric infarction. This patient was a 78 year old right-handed woman. There was a history of a left hemispheric stroke 1 year previously. On admission, she showed left hemianopia, dysarthria, mild left central facial paresis, bilateral sensory deficit and quadriparesis which were marked on the left side. Babinski sign was elicited on the left. She did not have anosognosia or visual neglect. She had mild orofacial apraxia, but ideomotor and ideational apraxia was absent. There was no motor impersistence. Magnetic resonance imaging of the brain revealed a recent infarction in the territory of the right middle cerebral artery and an old infarction in the left tempro-parietal lobe. The patient could not open her eyelids to verbal command or voluntarily until about two weeks later, when she became able to open her eyes but showed difficulty keeping her left eye closed. She was aware of this problem and could repeat the command and comprehend what was requested to her. On verbal command to close the eyes, her right eye would be closed continuously and excessively and the left eye would only blink. When requested to blink, however, she could blink correctly without excessive eye closure. Spontaneous, reflex and voluntary blinking were normal. Her eyes were closed normally during sleep. Blepharospasm was not seen. The patient showed a striking dissociation between a failure to close her eyes continuously and a preserved ability to blink voluntarily. We suggest that her ability to contract palpebral portion of her left orbicularis oculi muscle is preserved. Regarding the mechanism of the voluntary eye closure system, separate control mechanisms should exist on closing eyes continuously and blinking.
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PMID:[Dissociation of voluntary eye closure--to keep the eyes closed and to blink--following right hemisphere stroke]. 1087 26

Apraxia of eyelid opening (ALO) is a non-paralytic motor abnormality characterised by difficulty in initiating the act of eyelid opening without blepharospasm. We found that wearing goggles improved the difficulty of opening eyes in two patients with ALO with parkinsonism. Wearing goggles is a simple method for improving daily life in patients with ALO.
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PMID:Improvement of apraxia of eyelid opening by wearing goggles. 1125 3

The main objective in the treatment of blepharospasm is to decrease or cease the unwanted, repeated forced closure of the eyelids. This is best achieved by the use of botulinum toxin. In a minority of patients, botulinum toxin is either ineffective or poorly tolerated. In this group of patients, a trial with oral medication in the following order is warranted: trihexyphenidyl, baclofen, clonazepam, and tetrabenazine. Before going to the next medication, each of these drugs should be administered at the highest tolerated dosage for a period of 1 or 2 months. If, as often happens, all pharmacologic treatment attempts fail, and the patient is too disabled to remain untreated, he or she can be referred to an experienced plastic surgeon for a myectomy of the eyelid protractors. For treatment of apraxia of eyelid opening, botulinum toxin should be administered as the first treatment. If this fails, and vision is significantly impaired, the patient may be referred to a plastic surgeon for a frontalis suspension of the eyelid. Treatments of hemifacial spasm are aimed at decreasing or ending the annoying twitches of one side of the face. In this disorder, interference with vision is not a problem unless the contralateral eye is amblyopic. Despite isolated reports of spasm relief by drugs such as carbamazepine, oral medication is unlikely to be helpful. Botulinum toxin is the preferred treatment in hemifacial spasm patients. In some patients, relief from spasms can only be obtained at the cost of an ipsilateral upper lip droop of varying severity. Patients who are dissatisfied with the results of treatment with botulinum toxin, and are not willing to tolerate their condition, can be referred to an experienced neurosurgeon for microvascular decompression of the facial nerve. Pending success of ongoing attempts to reduce adverse effects, we believe that doxorubicin chemomyectomy, a recent treatment that has been used for both facial spasm and blepharospasm, is best administered in a research setting.
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PMID:Blepharospasm and Hemifacial Spasm. 1109 64

The authors ascertained the prevalence of primary blepharospasm (BSP) in a community located in Puglia, a region in Southern Italy, by focusing on neurologists and ophthalmologists. The crude prevalence rates were 133 per million (95% CI, 61--153) for both focal and segmental BSP, 74 per million (95% CI, 24--173) for focal BSP, and 59 per million (95% CI, 16--151) for segmental BSP. Prevalence rate increased with age. Apraxia of eyelid opening and BSP coexisted in one third of cases.
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PMID:Prevalence of primary blepharospasm in a community of Puglia region, Southern Italy. 1140 21

Lid movement and EMG of the orbicularis oculi (OOc) were analyzed in 10 patients with apraxia of lid opening associated with blepharospasm before and after botulinum toxin treatment. The latencies to onset and to complete the eye opening and the time during which eye opening was sustained were studied in relation to OOc activity and compared with control values obtained in 12 healthy subjects. Following treatment there was an improvement of all lid opening measurements, a decrease of the abnormally prolonged OOc activity, and a reduction of the functional disability.
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PMID:Botulinum toxin improves lid opening delays in blepharospasm-associated apraxia of lid opening. 1208 88

We ascertained the prevalence of apraxia of eyelid opening (AEO) in a community located in Puglia, a region of southern Italy. The crude prevalence rate was 59 per million (95% confidence interval, 24-173). AEO coexisted with adult onset blepharospasm in 75% of cases, with atypical parkinsonism in 25% of cases. Among the overall patient population seen at our movement disorders clinic from 1987 to 1997, AEO was isolated in 10 otherwise healthy individuals, associated with adult-onset dystonia in 13 cases, and associated with a parkinsonian syndrome in 9 cases. The frequency of AEO was 10.8% in the dystonia group, and 2.1% in the overall parkinsonian group (Parkinson's disease, 0.7%; progressive supranuclear palsy, 33.3%). In two patients with possible progressive supranuclear palsy, AEO worsened after increasing levodopa dosage or acute apomorphine challenge and disappeared following levodopa discontinuation. AEO developing in the setting of a parkinsonian syndrome may be either disease- or drug-related.
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PMID:Frequency of apraxia of eyelid opening in the general population and in patients with extrapyramidal disorders. 1254 54

We studied the clinical features, the natural history and disability in 47 progressive supranuclear palsy patients and brain imaging aspects by routinely performed MRI in a subgroup of 25. Unexplained falls together with atypical parkinsonism (symmetric, levodopa unresponsive without resting tremor) are good clinical pointers of the early diagnosis, since they occurred within the first year. Cognitive slowness and unspecific visual complains are also early symptoms, while usual cardinal signs such as supranuclear palsy are more delayed. Blepharospasm and eyelid opening apraxia as well as deep sighs are also quite characteristic clinical features (1/3 of cases). Cardinal signs (falls, pseudobulbar signs, supranuclear gaze palsy) worsened rapidly (20 to 30 months) towards a major disability. In the 20 patients deceased during follow-up, the mean survival time was about 5 years. The MRI study showed typical cortical fronto-temporo-parietal atrophy, mesencephalic and quadrigeminal plate atrophy with third ventricle dilatation. In conclusion, unexplained falls associated with atypical parkinsonism are contributive for the early clinical diagnosis. Non specific visual complains could be useful pointers in the absence of supranuclear ophthalmoplegia. MRI contributes to the clinical diagnosis even in the first 3 years of the disease course.
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PMID:[Progressive supranuclear palsy: a clinical, natural history and disability study]. 1261 51

Primary blepharospasm is an adult-onset focal dystonia characterised by involuntary contractions of the orbicularis oculi muscles. Patients may have various types of movements arising from the different parts of the orbicularis oculi muscle. These include typical blepharospasm associated with Charcot's sign, pretarsal blepharospasm and flickering of the eyelids. Primary blepharospasm may be associated with so-called apraxia of eyelid opening as well as dystonia in the lower face, jaw or cervical muscles. Unless there are clinical clues to a symptomatic cause, adults presenting with blepharospasm do not require extensive aetiological investigation because the condition is rarely due to an identifiable condition. As the aetiology of primary blepharospasm is largely unknown, therapeutic approaches are symptomatic, with type A botulinum toxin being the treatment of choice.
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PMID:Primary blepharospasm: diagnosis and management. 1487 Nov 68


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