Perampanel: Medical Alternative for Essential Tremor?

OBJECTIVES: The aim of this study was to assess the safety and efficacy of perampanel in patients with refractory essential tremor (ET). METHODS: We recruited patients from our movement disorders clinic with the diagnosis of severe refractory ET, and perampanel 4 mg at night was initiated.Assessments were conducted at baseline and after 1 month of treatment with perampanel 4 mg/d. Details about tolerance and effectiveness were collected. Clinical evaluation was conducted with the Fahn-Tolosa-Marín scale, and statistical analysis was carried out with Wilcoxon matched pairs signed rank test. RESULTS: This study included 18 patients with severe ET (11 females, 7 males; mean age: 75.1 ± 12.03 years; mean duration of ET: 17.4 ± 17.03 years). Perampanel significantly improved patients' average score with refractory ET ( P ≤ 0.0001). This improvement has been occasionally quite relevant. However, a proportion of patients did not tolerate perampanel because of several adverse effects including dizziness, ataxia, irritability, and instability. CONCLUSIONS: Perampanel had a markedly positive antitremor effect in patients with ET and could be an alternative treatment. However, this drug is not devoid of adverse effects.

Hypomimia in Parkinson's Disease: What Is It Telling Us?

Introduction: Amimia is one of the most typical features of Parkinson's disease (PD). However, its significance and correlation with motor and nonmotor symptoms is unknown. The aim of this study is to evaluate the association between amimia and motor and nonmotor symptoms, including cognitive status, depression, and quality of life in PD patients. We also tested the blink rate as a potential tool for objectively measuring upper facial bradykinesia. Methods: We prospectively studied amimia in PD patients. Clinical evaluation was performed using the Unified Parkinson's Disease Rating Scale (UPDRS) and timed tests. Cognitive status, depression, and quality of life were assessed using the Parkinson's Disease Cognitive Rating Scale (PD-CRS), the 16-Item Quick Inventory of Depressive Symptomatology (QIDS-SR16), and the PDQ-39, respectively. Amimia was clinically evaluated according to item 19 of UPDRS III. Finally, we studied upper facial amimia by measuring resting blink frequency and blink rate during spontaneous conversation. Results: We included 75 patients. Amimia (item 19 UPDRS III) correlated with motor and total UPDRS (r: 0.529 and 0.551 Spearman), and its rigidity, distal bradykinesia, and motor axial subscores (r: 0.472; r: 0.252, and r: 0.508, respectively); Hoehn and Yahr scale (r: 0.392), timed tests, gait freezing, cognitive status (r: 0.29), and quality of life (r: 0.268) correlated with amimia. Blinking frequency correlated with amimia (measured with item 19 UPDRS), motor and total UPDRS. Conclusion: Amimia correlates with motor (especially axial symptoms) and cognitive situations in PD. Amimia could be a useful global marker of overall disease severity, including cognitive decline.

Estimulación cerebral profunda en pacientes parkinsonianos con intolerancia a levodopa / Deep brain stimulation in parkinsonian patients with dopa intolerance

La estimulacion cerebral profunda (ECP) es un tratamiento eficaz para pacientes con enfermedad de Parkinson con complicaciones motoras. El elemento más importante que predice la respuesta a la ECP es la respuesta a la levodopa. Mostramos dos casos de pacientes con enfermedad de Parkinson de comienzo precoz con intolerancia severa a la levodopa y excelente respuesta a ECP. La ECP puede ser una alternativa en pacientes parkinsonianos con intolerancia a la levodopa siempre que la respuesta a apomorfina sea positiva

Deep brain stimulation (DBS) is at present, a useful treatment for patients with advanced Parkinson's disease and motor complications. The crucial step toward consistent DBS outcomes remains careful patient selection; several conditions must be fulfilled including excellent levo dopa response. We report two cases of early onset Parkinson's disease with severe intolerance to levo dopa but excellent and sustained response to DBS. DBS can be a useful alternative for parkinsonian patients with severe intolerance to levo dopa, provided a positive acute response to levo dopa or apomorphine is obtained

Behavioral addictions in early-onset Parkinson disease are associated with DRD3 variants.

BACKGROUND: Impulse control disorders (ICDs) comprise abnormal behaviors frequently found in patients with Parkinson's disease (PD) receiving antiparkinsonian medication. ICDs in PD would develop when dopaminergic treatment overstimulates the dopamine receptor D3 (DR3). Here we studied whether DR3 gene (DRD3) is associated to ICD in PD patients with early-onset (EOPD). METHODS: We performed association analysis of the rs6280 DRD3 single nucleotide variation (SNV) (Ser9Gly) in a clinical sample of 126 non early-onset PD (NEOPD) and 73 EOPD (age at onset < 45). ICD was evaluated using the Questionnaire for Impulsive-Compulsive Disorders (QUIP) in PD. RESULTS: In the total sample, we found that a younger onset of PD is linked to ICD traits with a potentially addictive reinforcement (ICDARs, behavioral addictions) (p = .017) and a trend for total ICDs (p = .078) while punding was not associated (p = .75). EOPD sample showed an increase of DRD3 C+ genotype for ICD (p = .022) and ICDARs (p = .043) but not for punding (p = .170). The post-hoc analyses including the time of evolution and Pramipexol or Ropinirole treatments, confirmed the independent effect of the DRD3 upon ICDs (p = .028) and ICDARs (p = .041) as well as the interaction between DRD3 and Pramipexol treatment upon ICDARs (OR = 4.60, 95% CI 1.20-17.632, p = .026). The NEOPD group showed no association between DRD3 and ICDs. CONCLUSIONS: We found that behavioral addictions in PD are associated with an early onset of the disease, the rs6280 DRD3 SNV and the type of dopamine agonist. Further investigation in independent samples is warranted.

Amimia en la enfermedad de Parkinson. Significado y correlación con la clínica / Amimia in Parkinson’s disease. Significance and correlation with the clinical features

Introducción. La amimia o reducción de la expresión facial es una de las características más típicas de la enfermedad de Parkinson (EP), y se puede definir como bradicinesia facial. A pesar de ser un elemento clásico, la amimia no se conoce bien, no se sabe con precisión su fisiopatología, su significado patológico ni su correlación con otros síntomas motores o no motores, incluyendo la depresión. Pacientes y métodos. Se ha analizado la amimia en un grupo de 84 pacientes con EP evaluados de forma prospectiva desde su diagnóstico hasta el quinto año de evolución, y también la correlación entre la amimia basal y la depresión en un subgrupo de 30 pacientes con EP. Resultados. La valoración basal (antes del tratamiento) y las evaluaciones de seguimiento se realizaron mediante la Unified Parkinson’s Disease Rating Scale (UPDRS). La amimia se evaluó mediante el ítem 19 de la UPDRS. La amimia basal se correlacionó con la UPDRS basal total y motora. Además, la amimia basal se correlacionó con la UPDRS total y motora a los cinco años de evolución. Sin embargo, la amimia basal no se relacionó con la presencia de complicaciones motoras (fluctuaciones motoras, discinesias o bloqueos) o no motoras. La correlación entre amimia y depresión se analizó mediante el Quick Inventory of Depressive Symptoms (QIDS-SR16). La amimia no se correlacionó con las puntuaciones del QIDS-SR16. Conclusión. Este estudio sugiere que la amimia basal se correlaciona con la situación basal general (UPDRS) e incluso con la valoración clínica a los cinco años, aunque no predice la tasa de complicaciones a medio plazo (AU)

Introduction. Reduced facial expression or amimia is one of the most typical characteristics of Parkinson’s disease (PD). Despite being described in classic texts, its significance, physiopathology and correlation with motor and non-motor symptoms is largely unknown. Patients and methods. We have studied facial bradykinesia in a group of 84 de novo PD patients prospectively evaluated for five years. We also studied the relationship of facial bradykinesia with depression in a subgroup of 30 patients. Results. Baseline and follow-up assessments were performed with the Unified Parkinson’s Disease Rating Scale (UPDRS). Baseline facial bradykinesia was rated according to item 19 of UPDRS. Baseline facial bradykinesia correlated with total and motor baseline UPDRS. In addition, baseline bradykinesia correlated with total and motor UPDRS at five years. However baseline bradykinesia did not influence the presence of motor (motor fluctuation, dyskinesias and freezing of gait) or non-motor complications (delusion, behavior abnormalities and dementia) at five years. Finally a subgroup of 30 patients completed the self-report version of the Quick Inventory of Depressive Symptoms (QIDS-SR16) questionnaire, facial bradykinesia did not correlate with QIDS-SR16 scores. Conclusion. Our study suggests that baseline facial bradykinesia correlates with general baseline situation in PD and even might predict the motor and functional status at five years (AU)

[Deep brain stimulation in parkinsonian patients with dopa intolerance]. / Estimulación cerebral profunda en pacientes parkinsonianos con intolerancia a levodopa.

Deep brain stimulation (DBS) is at present, a useful treatment for patients with advanced Parkinson's disease and motor complications. The crucial step toward consistent DBS outcomes remains careful patient selection; several conditions must be fulfilled including excellent levo dopa response. We report two cases of early onset Parkinson's disease with severe intolerance to levo dopa but excellent and sustained response to DBS. DBS can be a useful alternative for parkinsonian patients with severe intolerance to levo dopa, provided a positive acute response to levo dopa or apomorphine is obtained.

Enfermedad de Parkinson senil benigna / Benign late onset Parkinsons disease

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