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Brain Stimulation 2022
Topics: Humans; Meige Syndrome; Transcranial Magnetic Stimulation
PubMed: 34800698
DOI: 10.1016/j.brs.2021.11.013 -
Psychiatry and Clinical Neurosciences Aug 1998A 52-year-old schizophrenic patient acutely showed blepharospasm and oromandibular dystonia following neuroleptic-induced akathisia. She had suffered from schizophrenia...
A 52-year-old schizophrenic patient acutely showed blepharospasm and oromandibular dystonia following neuroleptic-induced akathisia. She had suffered from schizophrenia and been treated with neuroleptics for 15 years and had manifested tardive dyskinesia 5 years ago. Following a change in her neuroleptic medication, severe akathisia developed. Two days after the appearance of akathisia, blepharospasm and oromandibular dystonia appeared. After the disappearance of akathisia, the disorder continued. The frequency of blepharospasm ranged from 30 to 40 (times/min). The oral administration of trihexyphenidyl (6 mg/day), perphenazine (12 mg/day), and fluphenazine (12 mg/day) significantly decreased the frequency of blepharospasm, whereas carbamazepine (600 mg/day) and sulpiride (1200 mg/day) did not. These results suggest that overactivity of both cholinergic and dopaminergic functions in the striatum may be involved in this patient. Our patient, who showed acute onset of Meige's syndrome following neuroleptic-induced akathisia, is of interest to those studying the pathogenesis of Meige's syndrome.
Topics: Akathisia, Drug-Induced; Antipsychotic Agents; Female; Humans; Meige Syndrome; Middle Aged; Receptors, Dopamine; Schizophrenia; Visual Cortex
PubMed: 9766696
DOI: 10.1046/j.1440-1819.1998.00408.x -
Movement Disorders Clinical Practice Jul 2021
PubMed: 34307753
DOI: 10.1002/mdc3.13233 -
Journal of the Neurological Sciences May 2024Cranial dystonias (CrD) are challenging to treat. Oral pharmacotherapy is often sub-optimal, while delicate anatomy and limited availability of skilled botulinum toxin...
INTRODUCTION
Cranial dystonias (CrD) are challenging to treat. Oral pharmacotherapy is often sub-optimal, while delicate anatomy and limited availability of skilled botulinum toxin injectors makes this approach risky, and often difficult to access; neurosurgical options e.g. deep brain stimulation, are high-risk in the elderly populations most affected. We observed significant improvement in CrD in 2 patients prescribed Zolpidem+Melatonin combination treatment for insomnia, and therefore trialled this treatment in a further 4 patients with CrD.
METHODS
Six patients were treated with Zolpidem+Melatonin. Pre- and post-treatment videotaped clinical examinations were blindly rated by an independent assessor (EM) and scored using the 'Facial and Oral Movements' section of the abnormal involuntary movements scale (AIMS), as well as the Jankovic rating scale for blepharospasm.
RESULTS
Dystonic features, as measured by the abnormal involuntary movements scale (AIMS) improved by an average of 75% after treatment (6.5±3.1 before treatment to 1.7 +/- 0.8 after treatment). Improvements were also observed in blepharospasm severity scores, and in cervical dystonic features.
CONCLUSION
Zolpidem+Melatonin combination treatment represents a safe and effective treatment for CrD. Low cost and wide availability makes it an attractive option, particularly in resource-constrained healthcare settings, or in patients who have failed, or lack access to alternatives.
Topics: Humans; Zolpidem; Female; Melatonin; Pyridines; Male; Aged; Middle Aged; Treatment Outcome; Drug Therapy, Combination; Video Recording; Dystonia; Dystonic Disorders; Adult
PubMed: 38583390
DOI: 10.1016/j.jns.2024.122986 -
Journal of Anaesthesiology, Clinical... Jan 2012Meige's syndrome is rare form of orofacial dystonia. There is unfortunately no cure, but occasionally patients may improve with time. We present the successful...
Meige's syndrome is rare form of orofacial dystonia. There is unfortunately no cure, but occasionally patients may improve with time. We present the successful management of a palladial deep brain stimulator (DBS) implantation for Meige's syndrome. Dexmedetomidine infusion was used for sedation. The procedure lasted for around 12 h and the patient was comfortable, responsive, and cooperative over the extended period of time. The surgeons were comfortable with electrophysiologic brain mapping and clinical testing. DBS were implanted, through a burr hole, into the globus pallidus neurophysiological testing under guidance. The pulse generator battery was subcutaneously implanted into the chest wall under general anesthesia. The implanted pulse generator battery was started 2 days later and the patient showed dramatic improvement in his symptoms.
PubMed: 22345957
DOI: 10.4103/0970-9185.92459 -
The Laryngoscope Jan 2010Spasmodic dysphonia (SD) is a primary focal dystonia of unknown pathophysiology, characterized by involuntary spasms in the laryngeal muscles during speech production....
Spasmodic dysphonia (SD) is a primary focal dystonia of unknown pathophysiology, characterized by involuntary spasms in the laryngeal muscles during speech production. We examined two rare cases of postmortem brainstem tissue from SD patients compared to four controls. In the SD patients, small clusters of inflammation were found in the reticular formation surrounding solitary tract, spinal trigeminal, and ambigual nuclei, inferior olive, and pyramids. Mild neuronal degeneration and depigmentation were observed in the substantia nigra and locus coeruleus. No abnormal protein accumulations and no demyelination or axonal degeneration were found. These neuropathological findings may provide insights into the pathophysiology of SD.
Topics: Aged; Aged, 80 and over; Autopsy; Brain Stem; Dysphonia; Female; Humans; Male; Meige Syndrome; Reticular Formation
PubMed: 19795469
DOI: 10.1002/lary.20677 -
Journal of Neurology Jun 2008Deep brain stimulation (DBS) has emerged as a useful therapeutic option for patients with insufficient benefit from conservative treatment.
OBJECTIVE
Deep brain stimulation (DBS) has emerged as a useful therapeutic option for patients with insufficient benefit from conservative treatment.
METHODS
Nine patients with chronic DBS who suffered from cervical dystonia (4), generalized dystonia (2), hemidystonia (1), paroxysmal dystonia (1) and Meige syndrome (1) were available for formal follow-up at three years postoperatively, and beyond up to 10 years. All patients had undergone pallidal stimulation except one patient with paroxysmal dystonia who underwent thalamic stimulation.
RESULTS
Maintained improvement was seen in all patients with pallidal stimulation up to 10 years after surgery except in one patient who had a relative loss of benefit in dystonia ratings but continued to have improved disability scores. After nine years of chronic thalamic stimulation there was a mild loss of efficacy which was regained when the target was changed to the pallidum in the patient with paroxysmal dystonia. There were no major complications related to surgery or to chronic stimulation. Pacemakers had to be replaced within 1.5 to 2 years, in general.
CONCLUSION
DBS maintains marked long-term symptomatic and functional improvement in the majority of patients with dystonia.
Topics: Adult; Brain; Deep Brain Stimulation; Dystonia; Dystonic Disorders; Female; Follow-Up Studies; Globus Pallidus; Humans; Male; Meige Syndrome; Middle Aged; Neural Pathways; Pacemaker, Artificial; Thalamus; Time; Torticollis; Treatment Outcome
PubMed: 18338193
DOI: 10.1007/s00415-008-0798-6 -
Journal of Movement Disorders Apr 2024
PubMed: 38253477
DOI: 10.14802/jmd.23265 -
The British Journal of Ophthalmology May 1986A 3 1/2-year-old female presented with Turner's syndrome and Nonne-Milroy-Meige disease. Ocular findings included strabismus and bilateral chemosis which was unchanging...
A 3 1/2-year-old female presented with Turner's syndrome and Nonne-Milroy-Meige disease. Ocular findings included strabismus and bilateral chemosis which was unchanging and persisted throughout the four years the patient was followed up. Histopathological findings included diffuse lymphangiectasia and dense connective tissue surrounding the dilated lymph channels. Although the association between congenital lymphoedema and Turner's syndrome is common, the lymphoedema usually disappears by the first year of life. The persistence of the lymphoedema beyond this age is rare, as is the presence of the persistent chemosis. This report represents the first histopathological documentation of congenital lymphangiectasis in association with Turner's syndrome.
Topics: Child, Preschool; Conjunctiva; Connective Tissue; Female; Humans; Lymphangiectasis; Lymphatic System; Lymphedema; Turner Syndrome
PubMed: 3964640
DOI: 10.1136/bjo.70.5.396 -
Journal of Neurology, Neurosurgery, and... Jun 1983
Topics: Aged; Basal Ganglia Diseases; Deglutition Disorders; Humans; Male; Spasm
PubMed: 6875603
DOI: 10.1136/jnnp.46.6.589