Cases reported "Meige Syndrome"

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11/36. Sporadic rapid-onset dystonia-parkinsonism presenting as Parkinson's disease.

    We report on a 38-year-old patient with rapid-onset dystonia-parkinsonism (RDP) with a missense mutation in the Na/K-ATPase alpha3 subunit (ATP1A3). Asymmetrical parkinsonian symptoms evolved over a year. After a stable episode of another 2.5 years, overnight he developed oromandibular dystonia and more severe parkinsonian symptoms. We conclude that RDP should be considered as a rare cause of levodopa-unresponsive parkinsonism even if there is no family history and the classic presentation is lacking.
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ranking = 1
keywords = dystonia
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12/36. Postherpes simplex type 1 neuralgia simulating postherpetic neuralgia.

    patients with prodromal neuralgia associated with recurrent herpes simplex type 1 (HST1) infection and chronic facial pain following years of relapsing HST1 have been described. Chronic neuralgia following a single clinical HST1 infection and simulating postherpetic neuralgia has not been previously reported. Such a case is described: A 49-yr-old woman with a 2-mo history of oral-facial dyskinesia developed burning pain and hypersensitivity of the left side of the tongue, lower gum, and inner cheek, followed 1 day later by a vesicular rash in the same painful distribution. Viral cultures of the lesions identified HST1 but not herpes zoster. cerebrospinal fluid analyses during the vesicular lesion stage and 1 mo later were normal with no viral growth. Oral and facial lesions resolved after 10 days; acyclovir was given for 3 wk. brain and brainstem magnetic resonance imaging (MRI), electroencephalogram, and brainstem evoked potential studies were normal. Hyperesthesias, allodynia, and burning pain persisted despite nonsteroidal antiinflammatory agents, codeine and hydrocodone. Oral opioids were administered until sedation occurred, with no relief of pain. The burning pain and hyperesthesia resolved after the 16th day of amitriptyline use, 75 mg/day. A trial off amitriptyline 6 mo later resulted in recurrence of pain, and amitriptyline was restarted with good pain control. Post-HST1 neuralgia may simulate postherpetic neuralgia clinically, and painful symptoms may respond to amitriptyline.
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ranking = 0.015312892437012
keywords = dyskinesia
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13/36. cadasil presenting with a movement disorder: a clinical study of a Chilean kindred.

    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil) is a hereditary vascular disease that usually begins with migraine, followed by repeated strokes and progressive dementia. We describe an unusual clinical presentation of this condition in members of a Chilean family with an established NOTCH3 mutation. We report early clinical, neuropsychological, transcranial ultrasound, magnetic resonance imaging (MRI), cerebral blood flow, and skin biopsy findings on these patients. Of the patients, 2 presented with facial dystonia, 1 of whom had abnormal single photon emission computed tomography and transcranial ultrasound studies after normal brain MRI scans. Our report emphasizes that cadasil must be considered in the study of patients with secondary dystonia.
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ranking = 0.33333333333333
keywords = dystonia
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14/36. Severe tongue protrusion dystonia: clinical syndromes and possible treatment.

    We describe intermittent or sustained severe involuntary tongue protrusion in patients with a dystonic syndrome. speech, swallowing, and breathing difficulties can be severe enough to be life threatening. Causes include neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, lesch-nyhan syndrome, and postanoxic and tardive dystonia. The pathophysiology of intermittent severe tongue protrusion remains unknown. tongue protrusion dystonia is often unresponsive to oral drugs but may benefit from botulinum toxin injections into the genioglossus muscle. Bilateral deep brain pallidal stimulation was beneficial in two cases.
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ranking = 1
keywords = dystonia
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15/36. Cerebellar degeneration and Meige's syndrome.

    We have reported a case of Meige's syndrome in a middle-aged man who eventually had a cerebellar degeneration syndrome. The extrapyramidal symptoms preceded cerebellar signs and symptoms by 5 years. Most patients with idiopathic Meige's syndrome show some improvement with high-dose anticholinergic therapy. Our patient's lack of response to such agents and his subsequent cerebellar symptoms are reminiscent of the situation seen with parkinsonian patients who do not respond to medications, indicating a more widespread degenerative disease. The association of extrapyramidal symptoms with some spinocerebellar disorders, and the pathologic changes seen in the one reported autopsy case, should place the group of spinocerebellar disorders high on the differential list.
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ranking = 0.00010937781903803
keywords = idiopathic
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16/36. Idiopathic blepharospasm-oromandibular dystonia syndrome (Meige's syndrome) presenting as chronic temporomandibular joint dislocation.

    A case of idiopathic blepharospasm-oromandibular dystonia (Meige's syndrome) is reported, presenting as chronic bilateral dislocation of the temporomandibular joints. The nature of the syndrome is discussed, together with the difficulties in diagnosis and management.
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ranking = 2107.7165202131
keywords = blepharospasm-oromandibular dystonia, blepharospasm-oromandibular, dystonia, idiopathic
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17/36. blepharospasm-oromandibular dystonia associated with a left cerebellopontine angle meningioma.

    blepharospasm-oromandibular dystonia is characterized by the presence of spasms of the orbicularis oculi (blepharospasm) and of the lower facial or oromandibular muscles. A patient with this syndrome is presented in which a left cerebellopontine angle meningioma appeared to act as a triggering mechanism for the development of this disorder. On the basis of this report, we recommend that physicians search for this tumor in patients with this disorder.
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ranking = 0.83333333333333
keywords = dystonia
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18/36. 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.
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ranking = 0.34875560358938
keywords = dystonia, dyskinesia, idiopathic
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19/36. 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.
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ranking = 0.33333333333333
keywords = dystonia
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20/36. meige syndrome: an unusual cause of involuntary facial movements.

    Dental and allied health professionals are on occasion confronted with patients who exhibit abnormal facial movements. These patients may be seeking a diagnosis or may relate a specific problem resulting from the uncontrolled and involuntary orofacial movements. A complete description of the various conditions associated with abnormal facial movements is beyond the scope of this article. Instead, these authors present a case with dental symptoms that were masking a more serious underlying progressive neurologic disorder. Appropriate referral to the neurology service is essential so that treatment of the underlying cause may precede, rather than follow, empiric management of these patients' symptoms.
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ranking = 0.0043930540401989
keywords = orofacial
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