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1.
J Stroke Cerebrovasc Dis ; 29(10): 105147, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32912540

RESUMO

Hypertrophic olivary degeneration (HOD) is a rare phenomenon that occurs after various insults to the Guillain-mollaret triangle (GMT). HOD is unique because the degeneration of inferior olivary nucleus becomes hypertrophic rather than atrophic. In this study, a 31-year-old woman developed HOD after pontine cavernoma surgery had been performed. The clinical manifestation was involuntary intorsion of right lower extremity during walking, which has not been reported in the literature. The woman also presented with palatal tremor, the most classic symptom of HOD. HOD's imaging trait include olive hypertrophy with increased T2 signal intensity on MRI, which are corresponding to the pathological findings. HOD is a self-limiting disease and excessive treatments are unnecessary.


Assuntos
Distonia/etiologia , Pé/inervação , Perna (Membro)/inervação , Doenças Neurodegenerativas/etiologia , Procedimentos Neurocirúrgicos/efeitos adversos , Núcleo Olivar/patologia , Palato/inervação , Tremor/etiologia , Adulto , Distonia/fisiopatologia , Feminino , Humanos , Hipertrofia , Degeneração Neural , Doenças Neurodegenerativas/diagnóstico por imagem , Doenças Neurodegenerativas/patologia , Doenças Neurodegenerativas/fisiopatologia , Núcleo Olivar/diagnóstico por imagem , Núcleo Olivar/fisiopatologia , Tremor/fisiopatologia
2.
Neurology ; 95(11): e1461-e1470, 2020 09 15.
Artigo em Inglês | MEDLINE | ID: mdl-32651292

RESUMO

OBJECTIVE: We tested the hypothesis that there are 2 distinct phenotypes of Parkinson tremor, based on interindividual differences in the response of resting tremor to dopaminergic medication. We also investigated whether this pattern is specific to tremor by comparing interindividual differences in the dopamine response of tremor to that of bradykinesia. METHODS: In this exploratory study, we performed a levodopa challenge in 76 tremulous patients with Parkinson tremor. Clinical scores (Movement Disorders Society-sponsored version of the Unified Parkinson's Disease Rating Scale part III) were collected "off" and "on" a standardized dopaminergic challenge (200/50 mg dispersible levodopa-benserazide). In both sessions, resting tremor intensity was quantified using accelerometry, both during rest and during cognitive coactivation. Bradykinesia was quantified using a speeded keyboard test. We calculated the distribution of dopamine-responsiveness for resting tremor and bradykinesia. In 41 patients, a double-blinded, placebo-controlled dopaminergic challenge was repeated after approximately 6 months. RESULTS: The dopamine response of resting tremor, but not bradykinesia, significantly departed from a normal distribution. A cluster analysis on 3 clinical and electrophysiologic markers of tremor dopamine-responsiveness revealed 3 clusters: dopamine-responsive, intermediate, and dopamine-resistant tremor. A repeated levodopa challenge after 6 months confirmed this classification. Patients with dopamine-responsive tremor had greater disease severity and tended to have a higher prevalence of dyskinesia. CONCLUSION: Parkinson resting tremor can be divided into 3 partially overlapping phenotypes, based on the dopamine response. These tremor phenotypes may be associated with different underlying pathophysiologic mechanisms, requiring a different therapeutic approach.


Assuntos
Antiparkinsonianos/uso terapêutico , Dopaminérgicos/uso terapêutico , Resistência a Medicamentos/efeitos dos fármacos , Levodopa/uso terapêutico , Doença de Parkinson/tratamento farmacológico , Tremor/tratamento farmacológico , Acelerometria , Adulto , Idoso , Idoso de 80 Anos ou mais , Método Duplo-Cego , Resistência a Medicamentos/fisiologia , Feminino , Seguimentos , Humanos , Hipocinesia/diagnóstico por imagem , Hipocinesia/tratamento farmacológico , Hipocinesia/fisiopatologia , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Doença de Parkinson/diagnóstico por imagem , Doença de Parkinson/fisiopatologia , Resultado do Tratamento , Tremor/diagnóstico por imagem , Tremor/fisiopatologia
3.
Yonsei Med J ; 61(7): 644-646, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32608210

RESUMO

A weight-holding tremor is a rare type of hand tremor that occurs only when someone holds some weight. Here we report three cases of isolated weight-holding tremors, of which one patient was diagnosed with Parkinson's disease (PD) and the others as a variant of essential tremor (ET). A 68-year-old woman presented with a left-hand tremor that appeared only when she held objects with some weight. Her tremor was reminiscent of the re-emergent tremor of PD, and dopamine transporter imaging revealed reduced uptake at the right posterior putamen. A 21-year-old man and a 71-year-old woman also presented with similar weight-holding tremors. However, these tremors were not re-emergent, and no signs of parkinsonism were observed during follow-up. Although the underlying etiologies of these tremors differed, all three tremors worsened as the held weight increased. These tremors could be isolated isometric tremors, but further research is needed to clarify the nature of this rare tremor.


Assuntos
Tremor Essencial/diagnóstico , Doença de Parkinson/complicações , Tomografia por Emissão de Pósitrons/métodos , Tremor/diagnóstico por imagem , Tremor/etiologia , Adulto , Idoso , Peso Corporal , Dopamina/metabolismo , Proteínas da Membrana Plasmática de Transporte de Dopamina/metabolismo , Tremor Essencial/fisiopatologia , Feminino , Humanos , Masculino , Tremor/classificação , Tremor/fisiopatologia
4.
World Neurosurg ; 138: 672-679, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32545020

RESUMO

OBJECTIVE: In this study, we considered the treatment of cognitive characteristics of Parkinson's subtypes under resting magnetic resonance imaging scans, and used magnetic resonance imaging to analyze brain activity characteristics of patients with Parkinson's subtypes at rest. METHODS: In this study, patients with neurological Parkinson's disease subtypes were selected: 27 patients in the tremor group, 33 patients in the orthostatic gastric instability group, and 3 patients with mild cognitive impairment and neuropathic Parkinson's disease. Scientific treatment was adopted. RESULTS: Nineteen patients had mild cognitive dysfunction tremor and unstable posture, and 23 of them had mild cognitive dysfunction. Fifteen healthy controls were subjected to resting state functional magnetic resonance imaging by plane echo imaging sequence scanning. Neurological diseases-Regional consistency analysis of brain regions in patients with Parkinson's disease increased, including the right lower lobe, while regional consistency analysis of brain regions decreased, including the right frontal gyrus, right middle anterior gyrus, and lateral cerebellum. CONCLUSIONS: The experimental results show that the local consistency analysis method based on resting magnetic resonance imaging scan can effectively detect the differences in early neural activity in patients with Parkinson's disease subtype cognitive impairment, and can effectively reflect the brain characteristics of Parkinson's disease.


Assuntos
Encéfalo/diagnóstico por imagem , Disfunção Cognitiva/diagnóstico por imagem , Doença de Parkinson/diagnóstico por imagem , Idoso , Estudos de Casos e Controles , Cognição , Disfunção Cognitiva/fisiopatologia , Disfunção Cognitiva/psicologia , Feminino , Neuroimagem Funcional , Transtornos Neurológicos da Marcha/fisiopatologia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/fisiopatologia , Doença de Parkinson/psicologia , Descanso , Tremor/fisiopatologia
5.
BMC Neurol ; 20(1): 145, 2020 Apr 20.
Artigo em Inglês | MEDLINE | ID: mdl-32312236

RESUMO

BACKGROUND: Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late onset, X-linked genetic, neurodegenerative disorder caused by a "premutation (PM)" in the fragile X mental retardation 1 (FMR1) gene. Here we report a case of FXTAS from mainland of China who presented with rare orthostatic tremor. A review of tremor of FXTAS in the literature is also included. CASE PRESENTATION: A 67-year-old right-handed farmer started with tremor of both legs 8 years ago which was present while standing but absent when sitting or lying and progressed with unsteady gait one and a half years ago. The brain MRI showed high intensity signal in the bilateral middle cerebellar peduncles (MCP) in T2-weighted and fluid-attenuated inversion recovery (FLAIR) images and gene test for premutation for FMR1 was positive with 101 CGG repeats. The patient met the the diagnosis of definite FXTAS. Clonazepam and topiramate were administered to control tremor. We reviewed the literature and identified 64 cases with detailed clinical and genetic information. Orthostatic tremor associated with FXTAS is very rare. We found 85.2% patients reported tremor,42.6% with intention tremor,36.1% with kinetic tremor,32.8% with rest tremor and 29.5% with posture tremor. 37.7% of patients who have tremor showed at least two types of tremor. There were 6 patients with isolated rest tremor. There was 2 patient with voice tremor and 6 with head tremor. We also found that 74.6% FXTAS patients had family history of FMR1 gene associated diseases including Fragile X syndrome (FXS), FXTAS or fragile X-associated primary ovarian insufficiency (FXPOI). CONCLUSIONS: Adding our data to the available literature suggests that orthostatic tremor could be a rare initial manifestation of FXTAS and the review will increasing our understanding the phenotype of tremor in FXTAS. Family history of FMR1 gene associated diseases might be an important clue to the diagnosis.


Assuntos
Ataxia , Síndrome do Cromossomo X Frágil , Tremor , Idoso , Anticonvulsivantes/uso terapêutico , Ataxia/diagnóstico , Ataxia/tratamento farmacológico , Ataxia/genética , Ataxia/fisiopatologia , Encéfalo/diagnóstico por imagem , Encéfalo/fisiopatologia , Clonazepam/uso terapêutico , Síndrome do Cromossomo X Frágil/diagnóstico , Síndrome do Cromossomo X Frágil/tratamento farmacológico , Síndrome do Cromossomo X Frágil/genética , Síndrome do Cromossomo X Frágil/fisiopatologia , Humanos , Imagem por Ressonância Magnética , Masculino , Topiramato/uso terapêutico , Tremor/diagnóstico , Tremor/tratamento farmacológico , Tremor/genética , Tremor/fisiopatologia
7.
Zhonghua Yi Xue Za Zhi ; 100(3): 207-212, 2020 Jan 21.
Artigo em Chinês | MEDLINE | ID: mdl-32008288

RESUMO

Objective: To find more objective evidence and support for clinical classification of Parkinson's disease by means of tremor analysis in patients with early confirmed Parkinson's disease. Methods: A cross-sectional study was conducted to collect 65 patients with early Parkinson's disease treated in the Third Hospital of Peking University from January 2015 to December 2016. Unified Parkinson's Disease Rating Scale (UPDRS), Hoehn-Yahr scale (H-Y scale), Mini-mental state examination (MMSE), Hamilton depression scale (HAMD) were recorded in all patients. According to the ratio of UPDRS rigidity score to tremor score, the patients were divided into tremor dominant (TD), postural instability/gait difficulty (PIGD) and mixed types. All patients were examined by tremor analysis and the results were analyzed and compared. Results: Among the 65 patients, the mean age of onset was (63±10) years, the course of disease was (14±8) months. Twenty-one patients were classified to TD group, 28 patients were classified to PIGD type, and 16 patients with mixed type. There was no significant difference in frequency and amplitude of tremor between the three groups (P>0.05), but the proportion of alternating contraction and harmonic resonance of active and antagonistic muscles in TD group was significantly higher (P<0.05). Twenty patients (71.4%) in TD group showed typical Parkinson's disease manifestations in tremor analysis. Only four patients (14.3%) in PIGD group showed the typical manifestations. More patients in PIGD group showed no clear main peak of tremor at rest. Some patients showed 6-8 Hz/sec peak frequency in posture position and simultaneous contraction of the active and antagonist muscles. These two manifestations occured simultaneously in mixed type patients, including 10 cases (62.5%) with typical Parkinson's disease manifestations. Conclusion: As an objective electrophysiological method to evaluate tremor type, tremor analysis can be used as an important assistant method for clinical classification of Parkinson's disease. It can provide information of the pathway of pathological loss in different types and give important hints in prognosis and treatment.


Assuntos
Transtornos Neurológicos da Marcha/fisiopatologia , Doença de Parkinson/classificação , Doença de Parkinson/fisiopatologia , Tremor/fisiopatologia , Idade de Início , Idoso , Estudos Transversais , Feminino , Marcha , Transtornos Neurológicos da Marcha/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico , Tremor/etiologia
8.
Sci Rep ; 10(1): 2195, 2020 02 10.
Artigo em Inglês | MEDLINE | ID: mdl-32042111

RESUMO

The global aging phenomenon has increased the number of individuals with age-related neurological movement disorders including Parkinson's Disease (PD) and Essential Tremor (ET). Pathological Hand Tremor (PHT), which is considered among the most common motor symptoms of such disorders, can severely affect patients' independence and quality of life. To develop advanced rehabilitation and assistive technologies, accurate estimation/prediction of nonstationary PHT is critical, however, the required level of accuracy has not yet been achieved. The lack of sizable datasets and generalizable modeling techniques that can fully represent the spectrotemporal characteristics of PHT have been a critical bottleneck in attaining this goal. This paper addresses this unmet need through establishing a deep recurrent model to predict and eliminate the PHT component of hand motion. More specifically, we propose a machine learning-based, assumption-free, and real-time PHT elimination framework, the PHTNet, by incorporating deep bidirectional recurrent neural networks. The PHTNet is developed over a hand motion dataset of 81 ET and PD patients collected systematically in a movement disorders clinic over 3 years. The PHTNet is the first intelligent systems model developed on this scale for PHT elimination that maximizes the resolution of estimation and allows for prediction of future and upcoming sub-movements.


Assuntos
Mãos/fisiopatologia , Tremor/diagnóstico , Tremor/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Tremor Essencial/fisiopatologia , Feminino , Humanos , Aprendizado de Máquina/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Movimento (Física) , Movimento , Redes Neurais de Computação , Doença de Parkinson/diagnóstico , Prognóstico , Qualidade de Vida
10.
Nat Commun ; 11(1): 63, 2020 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-31896743

RESUMO

Each vestibular sensory epithelium in the inner ear is divided morphologically and physiologically into two zones, called the striola and extrastriola in otolith organ maculae, and the central and peripheral zones in semicircular canal cristae. We found that formation of striolar/central zones during embryogenesis requires Cytochrome P450 26b1 (Cyp26b1)-mediated degradation of retinoic acid (RA). In Cyp26b1 conditional knockout mice, formation of striolar/central zones is compromised, such that they resemble extrastriolar/peripheral zones in multiple features. Mutants have deficient vestibular evoked potential (VsEP) responses to jerk stimuli, head tremor and deficits in balance beam tests that are consistent with abnormal vestibular input, but normal vestibulo-ocular reflexes and apparently normal motor performance during swimming. Thus, degradation of RA during embryogenesis is required for formation of highly specialized regions of the vestibular sensory epithelia with specific functions in detecting head motions.


Assuntos
Membrana dos Otólitos/embriologia , Ácido Retinoico 4 Hidroxilase/metabolismo , Tretinoína/metabolismo , Animais , Potenciais Evocados/genética , Potenciais Evocados/fisiologia , Feminino , Regulação da Expressão Gênica no Desenvolvimento , Cabeça/fisiopatologia , Camundongos Endogâmicos C57BL , Camundongos Knockout , Osteopontina/metabolismo , Membrana dos Otólitos/citologia , Membrana dos Otólitos/metabolismo , Retinal Desidrogenase/genética , Retinal Desidrogenase/metabolismo , Ácido Retinoico 4 Hidroxilase/genética , Sáculo e Utrículo/citologia , Sáculo e Utrículo/embriologia , Tremor/genética , Tremor/fisiopatologia , Testes de Função Vestibular , Vestíbulo do Labirinto/embriologia , Vestíbulo do Labirinto/metabolismo
11.
Neurology ; 94(6): e639-e650, 2020 02 11.
Artigo em Inglês | MEDLINE | ID: mdl-31937622

RESUMO

OBJECTIVE: To determine whether different phenotypes of cervical dystonia (CD) express different types and levels of somatosensory impairment. METHODS: We assessed somatosensory function in patients with CD with and without tremor (n = 12 each) and in healthy age-matched controls (n = 22) by measuring tactile temporal discrimination thresholds of the nondystonic forearm and proprioceptive acuity in both the dystonic (head/neck) and nondystonic body segments (forearm/hand) using a joint position-matching task. The head or the wrist was passively displaced along different axes to distinct joint positions by the experimenter or through a robotic exoskeleton. Participants actively reproduced the experienced joint position, and the absolute joint position-matching error between the target and the reproduced positions served as a marker of proprioceptive acuity. RESULTS: Tactile temporal discrimination thresholds were significantly elevated in both CD subgroups compared to controls. Proprioceptive acuity of both the dystonic and nondystonic body segments was elevated in patients with CD and tremor with respect to both healthy controls and patients with CD without tremor. That is, tactile abnormalities were a shared dysfunction of both CD phenotypes, while proprioceptive dysfunction was observed in patients with CD with tremor. CONCLUSIONS: Our findings suggest that the pathophysiology in CD can be characterized by 2 abnormal neural processes: a dysfunctional somatosensory gating mechanism involving the basal ganglia that triggers involuntary muscle spasms and abnormal processing of proprioceptive information within a defective corticocerebellar loop, likely affecting the feedback and feedforward control of head positioning. This dysfunction is expressed mainly in CD with tremor.


Assuntos
Propriocepção , Distúrbios Somatossensoriais/fisiopatologia , Torcicolo/fisiopatologia , Tato , Tremor/fisiopatologia , Idoso , Estudos de Casos e Controles , Limiar Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Estimulação Física , Filtro Sensorial , Limiar Sensorial , Distúrbios Somatossensoriais/complicações , Torcicolo/complicações , Tremor/complicações
12.
Brain ; 143(1): 94-111, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31855247

RESUMO

Cerebral choline metabolism is crucial for normal brain function, and its homoeostasis depends on carrier-mediated transport. Here, we report on four individuals from three families with neurodegenerative disease and homozygous frameshift mutations (Asp517Metfs*19, Ser126Metfs*8, and Lys90Metfs*18) in the SLC44A1 gene encoding choline transporter-like protein 1. Clinical features included progressive ataxia, tremor, cognitive decline, dysphagia, optic atrophy, dysarthria, as well as urinary and bowel incontinence. Brain MRI demonstrated cerebellar atrophy and leukoencephalopathy. Moreover, low signal intensity in globus pallidus with hyperintensive streaking and low signal intensity in substantia nigra were seen in two individuals. The Asp517Metfs*19 and Ser126Metfs*8 fibroblasts were structurally and functionally indistinguishable. The most prominent ultrastructural changes of the mutant fibroblasts were reduced presence of free ribosomes, the appearance of elongated endoplasmic reticulum and strikingly increased number of mitochondria and small vesicles. When chronically treated with choline, those characteristics disappeared and mutant ultrastructure resembled healthy control cells. Functional analysis revealed diminished choline transport yet the membrane phosphatidylcholine content remained unchanged. As part of the mechanism to preserve choline and phosphatidylcholine, choline transporter deficiency was implicated in impaired membrane homeostasis of other phospholipids. Choline treatments could restore the membrane lipids, repair cellular organelles and protect mutant cells from acute iron overload. In conclusion, we describe a novel childhood-onset neurometabolic disease caused by choline transporter deficiency with autosomal recessive inheritance.


Assuntos
Antígenos CD/genética , Transtornos Heredodegenerativos do Sistema Nervoso/genética , Proteínas de Transporte de Cátions Orgânicos/genética , Adolescente , Ataxia/genética , Ataxia/fisiopatologia , Atrofia , Cerebelo/diagnóstico por imagem , Cerebelo/patologia , Colina/farmacologia , Disfunção Cognitiva/genética , Disfunção Cognitiva/fisiopatologia , Vesículas Citoplasmáticas/efeitos dos fármacos , Vesículas Citoplasmáticas/ultraestrutura , Transtornos de Deglutição/genética , Transtornos de Deglutição/fisiopatologia , Disartria/genética , Disartria/fisiopatologia , Retículo Endoplasmático/efeitos dos fármacos , Retículo Endoplasmático/ultraestrutura , Incontinência Fecal/genética , Incontinência Fecal/fisiopatologia , Feminino , Fibroblastos/efeitos dos fármacos , Fibroblastos/ultraestrutura , Mutação da Fase de Leitura , Globo Pálido/diagnóstico por imagem , Transtornos Heredodegenerativos do Sistema Nervoso/diagnóstico por imagem , Transtornos Heredodegenerativos do Sistema Nervoso/patologia , Transtornos Heredodegenerativos do Sistema Nervoso/fisiopatologia , Homozigoto , Humanos , Leucoencefalopatias/diagnóstico por imagem , Leucoencefalopatias/genética , Leucoencefalopatias/fisiopatologia , Imagem por Ressonância Magnética , Masculino , Microscopia Eletrônica , Mitocôndrias/efeitos dos fármacos , Mitocôndrias/ultraestrutura , Nootrópicos/farmacologia , Atrofia Óptica/genética , Atrofia Óptica/fisiopatologia , Linhagem , Ribossomos/efeitos dos fármacos , Ribossomos/ultraestrutura , Substância Negra/diagnóstico por imagem , Síndrome , Tremor/genética , Tremor/fisiopatologia , Incontinência Urinária/genética , Incontinência Urinária/fisiopatologia
13.
Artigo em Inglês | MEDLINE | ID: mdl-31832265

RESUMO

Background: Orthostatic tremor (OT) is defined as tremor in the legs and trunk evoked during standing. While the classical description is tremor of ≥13 Hz, slower frequencies are recognized. There is disagreement as to whether the latter represents a slow variant of classical OT, or different tremor disorder(s) given frequent coexistent neurological disease. Methods: A systematic literature search of PubMed was performed in February 2019 for "slow orthostatic tremor" and related terms which generated 573 abstracts, of which 61 were included. Results: Between 1970 and 2019, there were 70 cases of electrophysiologically confirmed slow OT. Two-thirds were female, of mean age 60 years (range 26-86), and mean disease duration 6 years (range 0-32). One-third of cases were isolated, and two-thirds had a coexistent disorder(s), including parkinsonism (30%), ataxia (12%), and dystonia (10%). Postural arm tremor was present in 34%. Median tremor frequency was 6-7 Hz (range 3-12). Tremor bursts ranged from 50 to 150 ms duration, and were alternating or synchronous in antagonistic and/or analogous muscles. Low and high coherences were reported. Five cases (7%) had coexistent classical OT. Clonazepam was the most effective medication across all frequencies, and levodopa was effective for 4-7 Hz OT with coexistent parkinsonism. Two cases resolved with the treatment of Graves' disease. Electrophysiology and imaging predominantly support a central tremor generator. Discussion: While multiple lines of evidence separate slow OT from classical OT, clinical and electrophysiological overlap may occur. Primary and secondary causes are identified, similar to classical OT. Further exploration to clarify these slow OT subtypes, clinically and neurophysiologically, is proposed.


Assuntos
Tontura/diagnóstico , Tontura/fisiopatologia , Eletromiografia/tendências , Medicina Baseada em Evidências/tendências , Tremor/diagnóstico , Tremor/fisiopatologia , Eletromiografia/métodos , Medicina Baseada em Evidências/métodos , Humanos , Postura/fisiologia
14.
Artigo em Inglês | MEDLINE | ID: mdl-31673479

RESUMO

Background: Primary orthostatic tremor (POT) is a rare disorder for which current treatments are largely ineffective. Following up on our recent report of complete resolution of POT symptoms in a patient using low doses of perampanel, we describe our experience of perampanel in 20 patients. Methods: Twenty patients whose neurologists prescribed perampanel were recruited. Initial dose was 2 mg/day, which was increased to 4 mg/day after the first month. Treatment efficacy was self-scored from +3 to -3 at 1 and 3 months. Results: Eight patients withdrew due to adverse effects. Of the 12 patients who completed the study, 92% indicated that their POT symptoms had improved after 1 month, with 75% indicating moderate to marked improvement (mean score 1.9 ± 0.9). This improvement was not sustained by follow-up at 3 months (mean score 0.9 ± 1.3). A rebound of POT symptoms that lasted 2-6 weeks was observed in most patients who withdrew. Discussion: Our experience with this series of cases points to the potential of low-dose perampanel as a treatment for POT, although poor tolerance and the possibility of a non-persistent therapeutic benefit need to be considered. Controlled studies are needed to confirm these findings.


Assuntos
Anticonvulsivantes/administração & dosagem , Tontura/diagnóstico , Tontura/tratamento farmacológico , Piridonas/administração & dosagem , Tremor/diagnóstico , Tremor/tratamento farmacológico , Idoso , Tontura/fisiopatologia , Eletromiografia/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Tremor/fisiopatologia
15.
Stereotact Funct Neurosurg ; 97(4): 241-243, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31743916

RESUMO

The beneficial effect of thalamic deep brain stimulation (DBS) on action tremor has been reported in a few cases of spinocerebellar ataxia (SCA); however, several factors should be taken into account regarding the indication for DBS in advanced cases. We performed DBS of the ventral intermediate nucleus (Vim) of the thalamus for treatment of coarse action tremor in a patient with SCA2 (spinocerebellar ataxia type 2) in the wheelchair-bound stage. Although improvement of the tremor of the proximal part was incomplete, the patient regained substantial parts of daily functioning. The effect lasted for more than 6 years, and the suppression of tremor significantly contributed to maintaining the level of the patient's expression into the bedridden stage. Vim DBS can be a treatment option for tremor in SCA patients, even in the advanced stage, as long as the tremor is depriving the patient of behavioral expression. As residual proximal tremor may hamper functional recovery, DBS of other targets or multi-targets should be further explored to attain a better outcome.


Assuntos
Estimulação Encefálica Profunda/métodos , Ataxias Espinocerebelares/diagnóstico por imagem , Ataxias Espinocerebelares/terapia , Tremor/diagnóstico por imagem , Tremor/terapia , Núcleos Ventrais do Tálamo/diagnóstico por imagem , Feminino , Humanos , Pessoa de Meia-Idade , Ataxias Espinocerebelares/fisiopatologia , Fatores de Tempo , Tremor/fisiopatologia , Núcleos Ventrais do Tálamo/fisiopatologia
16.
Ann Neurol ; 86(6): 812-820, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31614012

RESUMO

OBJECTIVE: Holmes tremor is a debilitating movement disorder with limited treatment options. Lesions causing Holmes tremor can occur in multiple different brain locations, leaving the neuroanatomical substrate unclear. Here, we test whether lesion locations that cause Holmes tremor map to a connected brain circuit and whether this circuit might serve as a useful therapeutic target. METHODS: Case reports of Holmes tremor caused by focal brain lesions were identified through a systematic literature search. Connectivity between each lesion location and the rest of the brain was computed using resting state functional connectivity magnetic resonance imaging data from 1,000 healthy volunteers. Commonalities across lesion locations were identified. This Holmes tremor circuit was then compared to neurosurgical treatment targets and clinical efficacy. RESULTS: We identified 36 lesions causing Holmes tremor, which were scattered across multiple different brain regions. However, all lesion locations were connected to a common brain circuit with nodes in the red nucleus, thalamus, globus pallidus, and cerebellum. In cases with effective neurosurgical treatment, the treatment target was connected with the lesion location, indicating that a second hit to the same circuit might be beneficial. Commonly used deep brain stimulation targets such as the ventral intermediate nucleus and subthalamic nucleus fell outside our Holmes tremor circuit, whereas the globus pallidus target was close, consistent with published clinical response rates for these targets. INTERPRETATION: Lesions causing Holmes tremor are part of a single connected brain circuit that may serve as an improved therapeutic target. ANN NEUROL 2019;86:812-820.


Assuntos
Mapeamento Encefálico/métodos , Encéfalo/diagnóstico por imagem , Conectoma/métodos , Rede Nervosa/diagnóstico por imagem , Tremor/diagnóstico por imagem , Encéfalo/fisiopatologia , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Rede Nervosa/fisiopatologia , Tremor/fisiopatologia
17.
Parkinsonism Relat Disord ; 67: 14-20, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31621599

RESUMO

INTRODUCTION: Apomorphine is a dopamine agonist used in Parkinson's disease (PD), which matches levodopa in terms of the magnitude of effect on the cardinal motor features, such as tremor and bradykinesia. The beneficial effect of this treatment on PD patients with tremor-dominant has widely been demonstrated, although the underlying neural correlates are unknown. We sought to examine the effects of apomorphine on topological characteristics of resting-state functional connectivity networks in tremor-dominant PD (tdPD) patients. METHODS: Sixteen tdPD patients were examined using a combined electromyography-functional magnetic resonance imaging approach. Patients were scanned twice following either placebo (subcutaneous injection of 1 mL saline solution) or 1 mg of apomorphine injection. Graph analysis methods were employed to investigate the modular organization of functional connectivity networks before and after drug treatment. RESULTS: After injection of apomorphine, evident reduction of tremor symptoms was mirrored by a significant increase in overall connectivity strength and reorganization of the modular structure of the basal ganglia and of the fronto-striatal module. Moreover, we found an increase in the centrality of motor and premotor regions. No differences were found between pre- and post-placebo sessions. CONCLUSION: These results provide new evidence about the effects of apomorphine at a large-scale neural network level showing that drug treatment modifies the brain functional organization of tdPD, increasing the overall resting-state functional connectivity strength, the segregation of striato-frontal regions and the integrative role of motor areas.


Assuntos
Apomorfina/farmacologia , Agonistas de Dopamina/farmacologia , Lobo Frontal/efeitos dos fármacos , Neostriado/efeitos dos fármacos , Doença de Parkinson/tratamento farmacológico , Tremor/tratamento farmacológico , Idoso , Apomorfina/uso terapêutico , Agonistas de Dopamina/uso terapêutico , Eletromiografia , Feminino , Lobo Frontal/diagnóstico por imagem , Lobo Frontal/fisiopatologia , Neuroimagem Funcional , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neostriado/diagnóstico por imagem , Neostriado/fisiopatologia , Vias Neurais/diagnóstico por imagem , Vias Neurais/efeitos dos fármacos , Vias Neurais/fisiopatologia , Doença de Parkinson/diagnóstico por imagem , Doença de Parkinson/fisiopatologia , Método Simples-Cego , Tremor/diagnóstico por imagem , Tremor/fisiopatologia
18.
Artigo em Inglês | MEDLINE | ID: mdl-31572622

RESUMO

Background: We investigated whether the characteristics of Parkinson's disease (PD) patients differ based on the primary indication for deep brain stimulation (DBS). Methods: We reviewed data for 149 consecutive PD patients who underwent DBS at the University of Virginia. Patients were categorized based on primary surgical indication, and clinical characteristics were compared between groups. Results: Twenty-nine (93.5%) of 31 PD patients who underwent DBS for medication refractory tremor were men, and 66 (62.3%) of 106 PD patients who underwent DBS for motor fluctuations were men (p = 0.001). Other primary indications for DBS were tremor and fluctuations (n = 5), medication intolerance (n = 5), and dystonia (n = 2). Discussion: Patients who underwent DBS for medication refractory tremor were predominantly men, while patients who had DBS for motor fluctuations approximated the gender distribution of PD. Possible explanations are that men with PD are more likely to develop medication refractory tremor or undergo surgery for medication refractory tremor in PD compared to women.


Assuntos
Estimulação Encefálica Profunda/estatística & dados numéricos , Doença de Parkinson/fisiopatologia , Doença de Parkinson/terapia , Tremor/fisiopatologia , Tremor/terapia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/complicações , Estudos Retrospectivos , Fatores Sexuais , Tremor/etiologia
19.
Artigo em Inglês | MEDLINE | ID: mdl-31572623

RESUMO

Background: Orthostatic tremor (OT) is a rare symmetric tremor disorder occasionally observed in association with other movement disorders. Case report: We report the presence of a fast OT in a case of Parkinson's disease (PD), and provide a comprehensive review of the literature. Discussion: A fast OT presenting as unsteadiness may be a presenting symptom of PD. This symptom may be nonresponsive to levodopa, and benzodiazepines should be prescribed to adequately control the OT and reduce disability.


Assuntos
Doença de Parkinson/fisiopatologia , Postura/fisiologia , Tremor/fisiopatologia , Idoso , Feminino , Humanos , Doença de Parkinson/complicações , Tremor/etiologia
20.
J Vis ; 19(11): 8, 2019 09 03.
Artigo em Inglês | MEDLINE | ID: mdl-31532470

RESUMO

The study of fixational eye motion has implications for the neural and computational underpinnings of vision. One component of fixational eye motion is tremor, a high-frequency oscillatory jitter reported to be anywhere from ∼11-60 arcseconds in amplitude. In order to isolate the effects of tremor on the retinal image directly and in the absence of optical blur, high-frequency, high-resolution eye traces were collected in six subjects from videos recorded with an adaptive optics scanning laser ophthalmoscope. Videos were acquired while subjects engaged in an active fixation task where they fixated on a tumbling E stimulus and reported changes in its orientation. Spectral analysis was conducted on periods of ocular drift, with all drifts being concatenated together after removal of saccades from the trace. The resultant amplitude spectra showed a slight deviation from the traditional 1/f nature of optical drift in the frequency range of 50-100 Hz, which is indicative of tremor. However, this deviation rarely exceeded 1 arcsecond and the consequent standard deviation of retinal image motion over the tremor band (50-100 Hz) was just over 5 arcseconds. Given such a small amplitude, it is unlikely tremor will contribute in any meaningful way to the visual percept.


Assuntos
Fixação Ocular/fisiologia , Retina/fisiologia , Movimentos Sacádicos/fisiologia , Tremor/fisiopatologia , Visão Ocular/fisiologia , Adaptação Fisiológica/fisiologia , Adulto , Humanos , Movimento (Física) , Orientação Espacial/fisiologia , Gravação em Vídeo
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