1/13. meige syndrome secondary to basal ganglia injury: a potential cause of acute respiratory distress.BACKGROUND: meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on meige syndrome and the relationship between dystonias and respiratory compromise is presented. methods: A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms. RESULTS: Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated. CONCLUSION: This case highlights two interesting aspects of Meige's syndrome: (1) Focal bilateral basal ganglia lesions appear to be responsible for this patient's movement disorder which is consistent with relative overactivity of the direct pathway from striatum to globus pallidus internal and substantia nigra pars reticularis; (2) Respiratory involvement in a primarily craniofacial dystonia to the point of acute airway compromise.- - - - - - - - - - ranking = 1keywords = blepharospasm (Clic here for more details about this article) |
2/13. Botulinum toxin treatment in atypical parkinsonian disorders associated with disabling focal dystonia.We investigated the efficacy of botulinum toxin A (BtxA) therapy in patients with atypical parkinsonian disorders (APD) exhibiting different types of disabling focal dystonia unresponsive to oral drug therapy. Eight patients with functionally disabling focal dystonia out of a series of 60 consecutive patients with APDs regularly treated at our outpatient movement disorders clinic were included. patients were diagnosed according to established criteria and had disabling limb dystonia (n=4) or craniocervical dystonia (n=4) unresponsive to oral pharmacological treatment. Localization and dose of BtxA injections was determined individually based on clinical examination as well as EMG in patients with limb dystonia. BtxA reduced dystonic symptoms in all patients; only one developed a transient local side-effect. BtxA was particularly effective in the long-term treatment (up to 50 months) of blepharospasm associated with progressive supranuclear palsy (PSP). BtxA also alleviated PSP-associated retrocollis and orofacial dystonia with lower lip retraction associated with PSP and multiple system atrophy. BtxA treatment of limb dystonia in corticobasal degeneration (CBD) temporarily improved hand and arm function in early-disease stages while treatment in advanced stages reduced pain, facilitated hygiene and prevented secondary contractures. Limb dystonia was also alleviated by BtxA therapy in one patient with neuronal multisystem degeneration of undetermined cause. The results suggest that BtxA therapy may represent an effective means of alleviating disabling focal dystonia in different APDs. Particularly in early stage APD with disabling limb dystonia local BtxA injections may result in functional improvement.- - - - - - - - - - ranking = 0.5keywords = blepharospasm (Clic here for more details about this article) |
3/13. A familial syndrome of dystonia, blepharospasm, and pigmentary retinopathy.We report the clinical and laboratory findings in 2 siblings with a syndrome of pigmentary retinopathy, blepharospasm, and dystonia. This entity most resembles Hallervorden-Spatz disease, but appears to be a distinct disorder without identifiable neuroimaging or biochemical abnormalities.- - - - - - - - - - ranking = 2.5keywords = blepharospasm (Clic here for more details about this article) |
4/13. Meige's syndrome during long-term neuroleptic treatment.Two patients developed difficulties in eyelid opening following long-term neuroleptic treatment of more than 6-8 years. Tardive dyskinesia and dystonia apart from the face were not found in either case. The symptoms fluctuated in their severities on a daily basis and were easily aggravated by various stimuli, e.g., stress, walking, reading and watching television. Electromyographic studies of their faces clearly indicated that the symptoms resulted from spontaneous blepharospasm and were analogous to idiopathic Meige's syndrome. Therefore, the patients' difficulties in opening their eyes were considered to be the so-called drug-induced Meige's syndrome and/or facial tardive dystonia. It must be stressed that this syndrome is extremely distressing to patients and is a severe complication accompanying a long-term neuroleptic treatment.- - - - - - - - - - ranking = 0.5keywords = blepharospasm (Clic here for more details about this article) |
5/13. Phenothiazine-induced oculogyric crisis.Extrapyramidal symptoms such as torticollis, oculogyric crisis and blepharospasm are typical manifestations of phenothiazine-induced drug reactions. Widespread use of such drugs, especially in children make these symptoms relatively commonplace. A case of phenothiazine-induced oculogyric crisis is presented. Included is a discussion of phenothiazine pharmacology.- - - - - - - - - - ranking = 0.5keywords = blepharospasm (Clic here for more details about this article) |
6/13. Meige's syndrome associated with neuroleptic treatment.Meige's syndrome is characterized by blepharospasm and oromandibular dystonia. Three cases are presented; two were associated with long-term neuroleptic administration. This drug-induced syndrome may be a variant of tardive dystonia, and prompt discontinuation of neuroleptic treatment may be therapeutic.- - - - - - - - - - ranking = 0.5keywords = blepharospasm (Clic here for more details about this article) |
7/13. Use of botulinum toxin in Meige's disease.Four patients with severe Meige's disease (blepharospasm-oromandibular dystonia) have been treated, after having given an informed consent, by local injections of purified botulinum toxin type "A". Previous systemic therapy with anticholinergics, dopamine antagonists and other drugs had been unsuccessful in all these subjects. Each patient was treated by saline solution injected with the same method as botulinum toxin, just once. The self-evaluation of patients and the clinical evaluation that some of us- unaware of the kind of therapy which had been performed- gave to the symptoms on the basis of videotapes, for each session of injection, showed that the injections of botulinum toxin are effective in the treatment of such disorder. The duration of the beneficial effect was slightly shorter in these patients than in patients with blepharospasm treated by the same method.- - - - - - - - - - ranking = 1keywords = blepharospasm (Clic here for more details about this article) |
8/13. blepharospasm accompanying hypoxic encephalopathy.Severe bilateral blepharospasm with left hemiplegia occurred in a 50-year-old woman, who developed cardiac arrest following ventricular aneurysmectomy. The blepharospasm was present constantly, even during sleep, and increased during attempts at voluntary eye opening. CT scan revealed multiple hypodense areas involving basal ganglia bilaterally and the right perisylvian area. The blepharospasm improved gradually over a 6-month period, although the left hemiplegia remained unchanged.- - - - - - - - - - ranking = 1.5keywords = blepharospasm (Clic here for more details about this article) |
9/13. Coexistent Meige's syndrome and myasthenia gravis. A relationship between blinking and extraocular muscle fatigue?We studied five patients with a combination of Meige's syndrome (blepharospasm-oromandibular dystonia) and myasthenia gravis. The coexistence of two disorders impairing eyelid opening led to diagnostic confusion and delayed appropriate therapy. Detailed oculographic monitoring of one patient indicated that eye position drifting due to myasthenic oculomotor fatigue was corrected by eye blinks, and that blinks tended to occur with slower saccades. Our observations suggest that fatigue of extraocular muscles may lead to synkinetic blinking and perhaps eventually to autonomous blepharospasm.- - - - - - - - - - ranking = 1keywords = blepharospasm (Clic here for more details about this article) |
10/13. Late-onset Hallervorden-Spatz disease presenting as familial parkinsonism.We studied a 68-year-old man who died after 13 years of progressive dementia, rigidity, bradykinesia, mild tremor, stooped posture, slow and shuffling gait, dystonia, blepharospasm, apraxia of eyelid opening, anarthria, aphonia, and incontinence. At autopsy, he had generalized brain atrophy with large deposits of iron pigment in the globus pallidus, caudate, and substantia nigra. Axonal spheroids were found in the globus pallidus, substantia nigra, medulla, and spinal cord. The neurochemical analysis of the brain revealed marked loss of dopamine in the nigral-striatal areas, with relative preservation of dopamine in the limbic areas. This is the oldest case of familial Hallervorden-Spatz disease reported and the first with neurochemical analysis of the brain.- - - - - - - - - - ranking = 0.5keywords = blepharospasm (Clic here for more details about this article) |
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