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Der Nervenarzt Feb 2020Max Nonne, an internationally renowned German neurologist, acted from 1918 to 1924 as president of the (first) Society of German Neurologists (GDN). Appointed honorary... (Review)
Review
Max Nonne, an internationally renowned German neurologist, acted from 1918 to 1924 as president of the (first) Society of German Neurologists (GDN). Appointed honorary president in 1925, he held this position in the (second) German Neurological Society (DGN) until his death. Since 1961, this association has honored 16 neurologists with a commemorative medal named after Nonne. His outstanding findings in various fields of neurology are uncontested and some of them live on as eponyms (Nonne-Apelt syndrome, Nonne-Froin syndrome, Nonne-Milroy-Meige syndrome); however, recent archival studies and an analysis of individual publications deeply darkened the image of the "grey eminence" of German neurology. Records kept at the Hamburg State Archive prove that in a memorandum from 1941/1942 following the example of Binding and Hoche, Nonne firmly approved the killing of "life absolutely unworthy of living". In a report addressed to the District Court of Hamburg he attested in 1946 that many physicians charged with manslaughter acted in accordance with the regulations governing "child euthanasia", resulting in the withdrawal of the accusation. In a further statement from 1949 he confirmed that the killing of children and the "euthanasia program" during the NS era were consistent with the state of medical science. An earlier book chapter authored by Nonne immediately after World War I suggested that his social-Darwinistically colored concept of mankind was developed clearly before the Nazi era. Notwithstanding the arrangement to which he came with the new powers after 1933 and his acceptance of tributes to him by them, he repeatedly stood up for his Jewish colleagues. He was never a Nazi, nevertheless, he engaged in activities that fostered NS "euthanasia" going far beyond a "mentality of approval".
Topics: Eponyms; Euthanasia; Germany; History, 20th Century; National Socialism; Neurologists; Neurology
PubMed: 32067081
DOI: 10.1007/s00115-019-00839-2 -
Heliyon Apr 2024Individual differences were observed in the clinical efficacy of Botulinum toxin A (BoNT-A) in the treatment of the primary Meige syndrome. Our study aimed to explore...
OBJECTIVE
Individual differences were observed in the clinical efficacy of Botulinum toxin A (BoNT-A) in the treatment of the primary Meige syndrome. Our study aimed to explore the potential associations between the clinical efficacy of BoNT-A in the treatment of the primary Meige syndrome and variants of , and , which are involving in the translocation of the BoNT-A in vivo.
METHODS
Patients with the primary Meige syndrome treated with BoNT-A were enrolled. Clinical efficacy was evaluated by the maximum improvement rate of motor symptoms and the duration of efficacy. Variants of , and were obtained by Sanger sequencing. Another cohort diagnosed with primary cervical dystonia was also enrolled in the replication stage.
RESULTS
Among the 104 primary Meige syndrome patients, 80 patients (76.9%) had a good efficacy (the maximum improvement rate of motor symptoms ≥30%) and 24 (23. 1%) had a poor (the maximum improvement rate of motor symptoms <30%). As to the duration of efficacy, 52 patients (50.0%) had a long duration of efficacy (≥4 months), and 52 (50.0%) had a short (<4 months). In terms of primary Meige syndrome, rs6104571 was found associating with the maximum improvement rate of motor symptoms (Genotype: = 0.02, OR = 0.26; Allele: = 0.013, OR = 0.29), and rs31244 was found associating with the duration of efficacy (Genotype: = 0.024, OR = 0.13; Allele: = 0.012, OR = 0.13). Besides, we also conducted the association analyses between the variants and BoNT-A-related adverse reactions. Although, there was no statistical difference between the allele of rs31244 and BoNT-A-related adverse reactions, there was a trend ( = 0.077, OR = 2.56). In the replication stage, we included 39 patients with primary cervical dystonia to further expanding the samples' size. Among the 39 primary cervical dystonia patients, 25 patients (64.1%) had a good efficacy (the maximum improvement rate of motor symptoms ≥50%) and 14 (35.9%) had a poor (the maximum improvement rate of motor symptoms <50%). As to the duration of efficacy, 32 patients (82.1%) had a long duration of efficacy (≥6 months), and 7 (17.9%) had a short (<6 months). Integrating primary Meige syndrome and primary cervical dystonia, rs31244 was still found associating with the duration of efficacy (Genotype: = 0.002, OR = 0. 23; Allele: = 0.001, OR = 0. 25).
CONCLUSION
In our study, rs6104571 was associated with the maximum improvement rate of motor symptoms in patients with primary Meige syndrome treated with BoNT-A, and patients carrying this variant had a lower improvement rate of motor symptoms. rs31244 was associated with duration of treatment in patients with primary Meige syndrome treated with BoNT-A and patients carrying this variant had a shorter duration of treatment. Patients with primary Meige syndrome carrying rs31244 G allele have an increase likelihood of BoNT-A-related adverse reactions. Involving 39 patients with primary cervical dystonia, the results further verify that rs31244 was associated with duration of treatment and patients carrying this variant had a shorter duration of treatment.
PubMed: 38628704
DOI: 10.1016/j.heliyon.2024.e28543 -
Frontiers in Neuroscience 2021Uric acid (URIC) is a natural antioxidant, and it has been shown that low levels of URIC could be a risk factor for the development of Parkinson's disease. Our aim was...
Uric acid (URIC) is a natural antioxidant, and it has been shown that low levels of URIC could be a risk factor for the development of Parkinson's disease. Our aim was to investigate whether URIC also plays a role in Meige's syndrome (MS). We conducted a cohort study to compare serum URIC levels between patients with MS and healthy controls. In addition, we analyzed the impact of URIC on the risk of MS and symptom severity. Compared with normal subjects, URIC content was remarkably decreased in MS patients. In addition, URIC was regarded as a protective factor for MS, as verified by multivariate logistic regression models. We also found non-linear relationships between the levels of serum URIC and the incidence rate of MS and the Burke-Fahn-Marsden dystonia rating scale score. Our study is the first to show a connection between serum URIC levels and MS. Low serum URIC levels indicate an increased risk of MS incidence and more severe clinical symptoms. Our findings provide new insights into the prevention and treatment of MS.
PubMed: 34658782
DOI: 10.3389/fnins.2021.755056 -
Neuroradiology Jun 2024This retrospective study examined the clinical outcomes and complications in 6 cases of compound Meige's syndrome, presenting with blepharospasm and masticatory muscle...
This retrospective study examined the clinical outcomes and complications in 6 cases of compound Meige's syndrome, presenting with blepharospasm and masticatory muscle spasm, following treatment with CT-guided radiofrequency ablation targeting bilateral facial nerves and mandibular branches of the trigeminal nerve. After the operation, the symptoms of eyelid spasm and masticatory muscle spasm resolved, but mild facial paralysis and numbness of mandibular nerve innervation persisted. Follow-up for 4-28 months showed that the symptoms of facial paralysis resolved within 2-5 (3.17 ± 0.94) months after the operation, whereas the numbness in the mandibular region persisted, accompanied by a decrease in masticatory function. During the follow-up period, none of the 6 patients experienced a recurrence of Meige's syndrome. These findings suggest that CT-guided radiofrequency ablation of the bilateral facial nerve and mandibular branches of the trigeminal nerve may offer a promising approach to treating compound Meige's syndrome.
PubMed: 38844696
DOI: 10.1007/s00234-024-03392-1 -
[Zhonghua Yan Ke Za Zhi] Chinese... Jan 2023To evaluate the efficacy and safety of nerve loop blocking in the treatment of blepharospasm caused by Meige syndrome. It was a retrospective case series study....
To evaluate the efficacy and safety of nerve loop blocking in the treatment of blepharospasm caused by Meige syndrome. It was a retrospective case series study. Patients with Meige syndrome characterized by blepharospasm or blepharospasm-oromandibular dystonia who underwent nerve loop blocking in the Ophthalmology Department of Henan No. 3 Provincial People's Hospital from April 2018 to January 2020 were included. Before and after surgery, blepharospasm was graded, and the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to score the symptoms of ocular and oral dystonia. The improvement rate of ocular spasm was calculated after surgery according to the scores. The postoperative complications were observed. The Wilcoxon rank test was used to compare the BFMDRS movement subscale scores before and after surgery. The independent sample Mann-Whitney test was used to compare the improvement rates of eye spasm between male and female patients and between patients with and without combined oromandibular dystonia. Among the 199 patients included, 64 (32.2%) were males, and 135 (67.8%) were females, aged 58 (51, 64) years (22-79 years). The postoperative follow-up period was 24 (21, 28) months. Preoperatively, blepharospasm was graded as grade 3 in 12 patients (6.0%) and grade 4 in 187 patients (94.0%), while the postoperative blepharospasm grades were grade 0 in 100 patients (50.3%), grade 1 in 64 patients (32.2%), grade 2 in 31 patients (15.6%), and grade 3 in 4 patients (2.0%). There was statistically significant difference in the BFMDRS scores of ocular dystonia before and after surgery [8.0 (8.0, 8.0) . 0.0 (0.0, 1.0); =-12.41, <0.001]. The improvement rate of blepharospasm in all patients was 100% (87.5%, 100%), ranging from 43.8% to 100%, with no statistically significant difference between patients of different genders and between patients with and without combined oral dystonia (both >0.05). Statistically significant difference existed in the scores of oral dystonia before and after surgery [2.0 (0.5, 4.5) . 1.0 (0.5, 2.0); =-4.38, <0.001], with 25 of 65 patients (38.5%) having their oral symptoms improved. Postoperative complications included eyelid valgus (7.5%, 15/199), frontal numbness (100%, 199/199) and tearing (89.9%, 179/199). Nerve loop blocking is a relatively safe and effective method in the treatment of blepharospasm symptoms of Meige syndrome.
Topics: Humans; Male; Female; Blepharospasm; Meige Syndrome; Dystonia; Retrospective Studies; Deep Brain Stimulation; Postoperative Complications; Spasm; Treatment Outcome
PubMed: 36631055
DOI: 10.3760/cma.j.cn112142-20220726-00361 -
Journal of Neurosurgery Jun 2024Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has demonstrated efficacy against multiple types of dystonia, but only a few case reports and small-sample...
OBJECTIVE
Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has demonstrated efficacy against multiple types of dystonia, but only a few case reports and small-sample studies have investigated the clinical utility of STN-DBS for Meige syndrome, a rare but distressing form of craniofacial dystonia. Furthermore, the effects of DBS on critical neuropsychological sequelae, such as depression and anxiety, are rarely examined. In this study, the authors investigated the therapeutic efficacy of STN-DBS for both motor and psychiatric symptoms of Meige syndrome.
METHODS
The authors retrospectively reviewed consecutive patients with Meige syndrome receiving bilateral STN-DBS at their institution from January 2016 to June 2023. Motor performance and nonmotor features including mood, cognitive function, and quality of life (QOL) were evaluated using standardized rating scales at baseline and at final postoperative follow-up. Clinical and demographic factors influencing postoperative motor outcome were evaluated by uni- and multivariable linear regression models.
RESULTS
Fifty-one patients were ultimately included, with a mean ± SD follow-up duration of 27.3 ± 18.0 months. The mean Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score improved from 12.9 ± 5.2 before surgery to 5.3 ± 4.2 at the last follow-up (mean improvement 58.9%, p < 0.001) and the mean BFMDRS disability score improved from 5.6 ± 3.3 to 2.9 ± 2.9 (mean improvement 44.6%, p < 0.001). Hamilton Depression and Anxiety Rating Scale scores also improved by 35.3% and 34.2%, respectively, and the postoperative 36-item Short-Form Health Survey score indicated substantial QOL enhancement. Global cognition remained stable after treatment. Multiple linear regression analysis identified disease duration (β = -0.241, p = 0.027), preoperative anxiety severity (β = -0.386, p = 0.001), and volume of activated tissue within the dorsolateral (sensorimotor) STN (β = 0.483, p < 0.001) as independent predictors of motor outcome.
CONCLUSIONS
These findings support STN-DBS as an effective and promising therapy for both motor and nonmotor symptoms of Meige syndrome. Timely diagnosis, treatment of preoperative anxiety, and precise electrode placement within the dorsolateral STN are essential for optimal clinical outcome.
Topics: Humans; Deep Brain Stimulation; Subthalamic Nucleus; Male; Female; Middle Aged; Retrospective Studies; Meige Syndrome; Treatment Outcome; Adult; Quality of Life; Aged; Follow-Up Studies; Anxiety
PubMed: 38241667
DOI: 10.3171/2023.11.JNS232075 -
Consensus of the Iberoamerican Oculoplastic Society for diagnosis and management of facial dystonia.Archivos de La Sociedad Espanola de... Sep 2019To propose guidelines for the diagnosis and treatment of facial dystonia prepared by a group of experts in orbit and oculoplastics from the Iberoamerican Oculoplastic... (Review)
Review
OBJECTIVE
To propose guidelines for the diagnosis and treatment of facial dystonia prepared by a group of experts in orbit and oculoplastics from the Iberoamerican Oculoplastic Society.
MATERIAL AND METHODS
An interactive discussion between the expert panel and those attending the 6th Iberoamerican Society of Oculoplastics Congress, which took place at the Hospital Nuestra Señora de la Luz in Mexico City on 22 October 2018, providing their personal experience based on evidence for diagnosis and treatment of facial dystonia. Around 200 ophthalmologists specialised in oculoplastics from North, Central and South America, Spain, and Portugal were involved. Discussion was focused on the following themes: pathophysiology, diagnosis, medical management, and surgical management.
CONCLUSIONS
Facial dystonia diagnosis is clinical; therefore, image studies are rarely needed. The ophthalmologist is generally the first physician to be consulted, and is able to be the treating physician, with the exception of specific cases of hemifacial spasm where management with neurosurgery may be beneficial. Botulinum toxin is the treatment of choice. Treatment with oral neuroleptics and myectomy of the orbicularis oculi muscle are reserved for refractory cases, since these do not have an adequate clinical response as first choice treatments. Persistent use of botulinum toxin does not modify the natural course of the disease.
Topics: Antipsychotic Agents; Blepharospasm; Botulinum Toxins; Diagnostic Techniques, Ophthalmological; Disease Management; Dystonic Disorders; Face; Hemifacial Spasm; Humans; Microvascular Decompression Surgery; Muscle, Skeletal; Prognosis
PubMed: 31272707
DOI: 10.1016/j.oftal.2019.03.018 -
Journal of the Neurological Sciences Aug 2022Blepharospasm is one of the most common subtypes of dystonia, and often spreads to other body regions. Despite published guidelines, the approach to diagnosis and...
BACKGROUND
Blepharospasm is one of the most common subtypes of dystonia, and often spreads to other body regions. Despite published guidelines, the approach to diagnosis and classification of affected body regions varies among clinicians.
OBJECTIVE
To delineate the clinical features used by movement disorder specialists in the diagnosis and classification of blepharospasm according to body regions affected, and to develop recommendations for a more consistent approach.
METHODS
Cross-sectional data for subjects diagnosed with all types of isolated dystonia were acquired from the Dystonia Coalition, an international, multicenter collaborative research network. Data were evaluated to determine how examinations recorded by movement disorder specialists were used to classify blepharospasm as focal, segmental, or multifocal.
RESULTS
Among all 3222 participants with isolated dystonia, 210 (6.5%) had a diagnosis of focal blepharospasm. Among these 210 participants, 34 (16.2%) had dystonia outside of upper face region. Factors such as dystonia severity across different body regions and number of body regions affected influenced the classification of blepharospasm as focal, segmental, or multifocal.
CONCLUSIONS
Although focal blepharospasm is the second most common type of dystonia, a high percentage of individuals given this diagnosis had dystonia outside of the eye/upper face region. These findings are not consistent with existing guidelines for the diagnosis and classification of focal blepharospasm, and point to the need for more specific guidelines for more consistent application of existing recommendations for diagnosis and classification.
Topics: Blepharospasm; Cross-Sectional Studies; Dystonia; Dystonic Disorders; Humans
PubMed: 35716653
DOI: 10.1016/j.jns.2022.120319 -
Neurological Sciences : Official... Nov 2021Chronic acquired hepatocerebral degeneration (CAHD) is a rare neurologic syndrome occurring in patients with chronic liver disease, resulting in the combination of...
Chronic acquired hepatocerebral degeneration (CAHD) is a rare neurologic syndrome occurring in patients with chronic liver disease, resulting in the combination of movement disorders and cognitive\behavioral changes. Its pathogenesis is debated and the symptoms are poorly responsive to medical therapy. Meige's syndrome is a form of cranial dystonia, considered an idiopathic form of adult onset dystonia. We report a 60-year-old man with HCV-related liver cirrhosis and hepatocarcinoma who developed Meige's syndrome associated with cognitive and behavioral manifestations, unrelated to acute metabolic derangement. CAHD was diagnosed. Liver transplantation reversed the clinical picture and MR abnormalities, reinforcing the idea that CAHD is a potentially reversible syndrome, which may be healed by liver transplantation and should not be considered a contraindication for this operation.
Topics: Adult; Behavioral Symptoms; Hepatolenticular Degeneration; Humans; Liver Transplantation; Male; Meige Syndrome; Middle Aged
PubMed: 34278516
DOI: 10.1007/s10072-021-05475-w -
Neuromodulation : Journal of the... Feb 2021To investigate the efficacy of bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) in patients with Meige syndrome.
OBJECTIVE
To investigate the efficacy of bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) in patients with Meige syndrome.
MATERIALS AND METHODS
Fifteen consecutive patients who underwent STN-DBS at the Peking University People's Hospital between September 2017 and June 2018 were included in this study. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score and the BFMDRS disability score were obtained prior to surgery, and at specific time points after surgery. Patients' sleep status was also assessed before and after surgery.
RESULTS
The BFMDRS movement scores decreased from 15.3 ± 4.6 to 5.2 ± 6.2 after STN-DBS, with a mean improvement of 68.6% (p < 0.05). The BFMDRS disability scores were also significantly decreased, from 6.9 ± 3.3 to 3.5 ± 2.9, with a mean improvement of 51.7% (p < 0.05). The eye, mouth, speech, and swallowing movement scores also decreased significantly after STN-DBS compared to baseline (p < 0.05). The sleep quality of the patients was also improved after surgery.
CONCLUSIONS
These findings demonstrate that the STN is an effective brain target for the treatment of patients with Meige syndrome. STN-DBS was not only able to improve patients' motor symptoms, but also their sleep status.
Topics: Deep Brain Stimulation; Follow-Up Studies; Globus Pallidus; Humans; Meige Syndrome; Subthalamic Nucleus; Treatment Outcome
PubMed: 32476223
DOI: 10.1111/ner.13174