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BACKGROUND: Adherence to treatment in Parkinson's disease (PD) is compromised due to the need for multiple therapies, comorbidities related to aging, and the complexity of therapeutic schemes. In the present study, we aimed to explore adherence to treatment in groups of PD patients from six Latin-American (LA) countries and identify its associated demographic and clinical parameters. METHODS: A multicenter, cross-sectional, exploratory study was conducted from September 2016 to March 2017. Treatment adherence was assessed using the simplified medication adherence questionnaire (SMAQ), applied to patients and caregivers. Sociodemographic and clinical variables (MDS-UPDRS Part III-IV, MMSE, Beck Depression Inventory-II (BDI-II)) were recorded. RESULTS: Eight hundred patients from six LA countries were evaluated. Nonadherence was reported in 58.25% of the population, according to patients. The most frequent issues were forgetfulness and correct timing of doses. A high level of agreement in adherence prevalence and most SMAQ items were observed between patients and their caregivers. The nonadherent population had a significantly higher proportion of unemployment, free access to medication, troublesome dyskinesias and off-periods, lesser years of education, and worse motor, cognitive, and mood scores. In multiple logistic and linear regression analyses, MDS-UPDRS Part III, BDI-II, gender, free access to medication, treatment with dopamine agonists alone, years of education, excessive concerns about adverse effects, and beliefs about being well-treated remained significant contributors to adherence measures. CONCLUSION: Educational strategies, greater involvement of PD patients in decision-making, and consideration of their beliefs and values might be of great need to improve medication adherence in this PD population.
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Cumplimiento de la Medicación/estadística & datos numéricos , Enfermedad de Parkinson/terapia , Anciano , Cuidadores , Comorbilidad , Estudios Transversales , Escolaridad , Empleo , Femenino , Humanos , América Latina , Masculino , Cumplimiento de la Medicación/psicología , Persona de Mediana Edad , Enfermedad de Parkinson/psicología , Análisis de Regresión , Índice de Severidad de la Enfermedad , Factores Sociodemográficos , Encuestas y CuestionariosRESUMEN
INTRODUCTION: Meige syndrome is a segmental form of dystonia where botulinum toxins are the preferred treatment option. However, its invasive nature, treatment costs, partial responsiveness, and benefit duration are some of their limitations. METHODS: Six consecutive subjects with Meige syndrome were treated only with aripiprazole. RESULTS: A dramatic response was obtained in all subjects during the first weeks of treatment. Aripiprazole mean ± SD daily dose was 7.9 ± 3.6 mg. Three subjects developed parkinsonism related to aripiprazole treatment; the former improved after reducing the dosage, without significant worsening of cranial dystonia. After a mean ± SD follow-up of 2.0 ± 0.7 years, clinical benefit persists over time, with a mean percentage reduction of Unified Dystonia Rating Score of 75.6% ± 8.4%. CONCLUSIONS: Aripiprazole should be considered as an alternative treatment option among subjects with Meige syndrome, especially in those refractory to botulinum toxin injections. The clinical response shown in our patients may lead to treatment development.
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Distonía , Síndrome de Meige , Aripiprazol/uso terapéutico , Humanos , Síndrome de Meige/inducido químicamente , Síndrome de Meige/tratamiento farmacológico , Resultado del TratamientoRESUMEN
Background: Paroxysmal movement disorders are a heterogeneous group of neurological diseases, better understood in recent years thanks to widely available genetic testing. Case report: A pair of monozygotic twins with dystonia and paroxysmal attacks, resembling paroxysmal non-kinesigenic dyskinesias, due to a novel ATP1A3 variant are reported. The complete resolution of their paroxysms was achieved using levodopa and deep brain stimulation of the internal globus pallidus. Improvement of interictal dystonia was also achieved with this therapy. Discussion: Paroxysmal worsening of movement disorders should be suspected as part of the ATP1A3 spectrum. Treatment outcome might be predicted based on the phenotype.
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Trastornos Distónicos/diagnóstico , Trastornos Distónicos/genética , Variación Genética/genética , ATPasa Intercambiadora de Sodio-Potasio/genética , Gemelos Monocigóticos/genética , Trastornos Distónicos/fisiopatología , Electroencefalografía/métodos , Humanos , Masculino , Adulto JovenRESUMEN
BACKGROUND: Acute disseminated encephalomyelitis and mild encephalopathy with reversible splenial lesion are autoimmune demyelinating disorders of central nervous system. Diagnosis remains clinical, aided by neuroimaging confirmation and excluding other causes. In the absence of a biological marker, the diagnosis of these entities based on clinical and imaging criteria could overlap. METHODS: We describe a 22-year-old woman developing mild neurological signs after an upper tract infection, a brain magnetic resonance image revealed confluent, symmetrical white matter lesions with corpus callosum involvement; after extensive ancillary testing that ruled out secondary causes we concluded that this subject had a post infectious encephalitis sharing clinical and imaging criteria for acute disseminated encephalomyelitis. However, mild encephalopathy with reversible splenial lesion could be an alternate diagnosis for this subject. Treatment with methylprednisolone completely solved both the clinical and image abnormalities without relapsing for more than 3 years of follow-up. CONCLUSION: Both acute disseminated encephalomyelitis and mild encephalopathy with reversible splenial lesion share clinical and radiological features. A biological marker is needed to differentiate among these entities, since overlap is seen according to current criteria.
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BACKGROUND: Homozygous sequestomosome-1 gene mutations have been recently linked to neurodegeneration with dystonia, ataxia and gaze palsy. Seven affected families were identified thus far. OBJECTIVE: To describe four new cases with additional phenotypical features. RESULTS: Four affected patients from two unrelated families were identified. Two compound heterozygous variants of the gene (c.257_259delins35 and c.301+1G > T) were found in one family (cases 1 and 2), and homozygous c.823_824delAG variant was identified in cases 3 and 4. In addition to the previously described syndrome characterized by cerebellar ataxia, dystonia, choreoathetosis, cognitive impairment and gaze palsy, two subjects presented with iridoplegia. Furthermore, we report dysautonomic features such as orthostatic hypotension and sudomotor dysfunction, along with other non-motor symptoms. CONCLUSIONS: We expand the phenotype of dystonia caused by Sequestomosome-1 gene by identifying dysautonomic features along with other non-motor symptoms.
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Ataxia/diagnóstico por imagen , Ataxia/genética , Distonía/diagnóstico por imagen , Distonía/genética , Fenotipo , Proteína Sequestosoma-1/genética , Adulto , Femenino , Fijación Ocular/genética , Humanos , Masculino , Linaje , Adulto JovenRESUMEN
Superficial siderosis (SS) is a rare condition resulting from different sources of bleeding into the subpial space. The most common symptoms are: hypoacusia, ataxia, incontinence, dementia and parkinsonism. Since several neurodegenerative disorders may present with same clinical features, SS is often misdiagnosed. Here we present a case of SS misdiagnosed as idiopathic bilateral neurosensorial deafness.
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Sordera/diagnóstico , Pérdida Auditiva Sensorineural/diagnóstico , Siderosis/diagnóstico , Anciano , Errores Diagnósticos , Humanos , MasculinoRESUMEN
BACKGROUND: D-Decarboxylase inhibitors, such as carbidopa or benserazide, have been used as adjunct therapy in Parkinson disease shortly after levodopa synthesis in the 1960s. These compounds increase intracerebral drug concentration and decrease adverse effects by blocking peripheral conversion to dopamine. Skin rash as part of an allergic reaction was previously described in subjects who were using levodopa in combination with carbidopa or benserazide; however, etiology was never clear. Allergic reactions to carbidopa have not previously been reported. METHODS: We report a case of a 77-year-old woman with a diagnosis of idiopathic Parkinson disease, who developed autonomic and dermatological signs: conjunctival injection, rhinorrhea, excessive sweating, hypertension, and pruritic generalized rash, among others, immediately after carbidopa/levodopa administration regardless of the manufacturer. Treatment with dexamethasone combined with chloropyramine hydrochloride resulted in complete resolution of the hypersensitivity reaction each time it presented. The autonomic and dermatological manifestations did not reappear after treatment was replaced with benserazide/levodopa. CONCLUSIONS: To the best of our knowledge, this is the first case report of an allergic reaction specific to carbidopa. Our case highlights the importance of identifying the source of a hypersensitivity drug response, whether it is caused by the active component or by the excipients.
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Antiparkinsonianos/efectos adversos , Carbidopa/efectos adversos , Hipersensibilidad a las Drogas/etiología , Anciano , Antiparkinsonianos/uso terapéutico , Benserazida/uso terapéutico , Carbidopa/uso terapéutico , Combinación de Medicamentos , Femenino , Humanos , Levodopa/uso terapéutico , Persona de Mediana Edad , Enfermedad de Parkinson/tratamiento farmacológicoRESUMEN
Autosomal dominant leukodystrophy is a neurodegenerative disorder caused by either point mutations or duplication of the lamin B1 gene on chromosome 5q23. The typical clinical picture consists of autonomic symptoms as well as cerebellar and pyramidal signs. Here we present the case of a 57-year-old female referred to our clinic due to cognitive decline. Neurological examination was significant for cognitive impairment as well as pyramidal and cerebellar signs. Brain MRI displayed diffuse hyperintense lesions in the subcortical white matter, pontine nuclei, brachium pontis and restiform body. The diagnosis was confirmed via genetic testing. Autosomal dominant leukodystrophy should be included in the differential diagnosis of patients presenting with cognitive impairment, motor signs, and leukodystrophy-like images.
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Enfermedades Neurodegenerativas/diagnóstico , Encéfalo/diagnóstico por imagen , Disfunción Cognitiva/diagnóstico , Disfunción Cognitiva/genética , Disfunción Cognitiva/fisiopatología , Diagnóstico Diferencial , Femenino , Humanos , Lamina Tipo B/genética , Persona de Mediana Edad , Enfermedades Neurodegenerativas/genética , Enfermedades Neurodegenerativas/fisiopatología , FenotipoRESUMEN
OBJECTIVES: Impulse control disorder (ICD) is a common adverse effect in patients with Parkinson disease who receive dopamine agonists; however, other factors are involved in its manifestations. To study the frequency and factors involved in the development of this adverse effect in a Latin American population, we conducted a cross-sectional multicenter study. METHODS: Two hundred fifty-five patients in 3 Latin American centers were evaluated by examination and application of scales (Unified Parkinson's Disease Rating Scale, Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease-Rating Scale, Hoehn and Yahr, Clinical Impression of Severity Index for Parkinson's Disease). RESULTS: Of the patients, 27.4% had ICD, most of whom were on dopamine agonists. Other associated risk factors included a younger age at onset of Parkinson disease, moderate symptoms, a shorter evolution of the clinical manifestations, rapid eye movement (REM) sleep disorder behavior, and the consumption of tea, mate, and alcohol. CONCLUSIONS: The frequency of ICD is higher in Latin America than in Anglo-Saxon populations. Consuming tea and mate, in addition to the use of dopamine agonists, is a factor that may demonstrate a genetic link that predisposes patients to the establishment of an ICD.
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Trastornos Disruptivos, del Control de Impulso y de la Conducta/inducido químicamente , Trastornos Disruptivos, del Control de Impulso y de la Conducta/epidemiología , Agonistas de Dopamina/efectos adversos , Enfermedad de Parkinson/epidemiología , Anciano , Estudios Transversales , Trastornos Disruptivos, del Control de Impulso y de la Conducta/complicaciones , Femenino , Humanos , América Latina/epidemiología , Masculino , Enfermedad de Parkinson/tratamiento farmacológico , Factores de RiesgoRESUMEN
We report the case of a 29-year-old male patient with a generalized and progressive dystonia that led him unable to stand. Multiple antidystonic treatments were tried without benefit. Alcohol test was positive with a dramatic improvement. To the best of our knowledge, this is the first reported case of generalized dystonia without other clinical manifestations sensitive to alcohol.
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Alcoholes/efectos adversos , Depresores del Sistema Nervioso Central/efectos adversos , Trastornos Distónicos/inducido químicamente , Adulto , Humanos , Masculino , Índice de Severidad de la EnfermedadRESUMEN
OBJECTIVE: The aim of this study was to report the case of a male patient with Parkinson disease who developed brachial plexopathy (BP) due to varicella-zoster virus, which was successfully treated with human immunoglobulin. METHOD: We report the case of a 75-year-old male subject with a diagnosis of Parkinson disease who came to our hospital complaining of pain, skin lesions, and strength loss in his right arm during the past 2 months. Physical examination revealed vesicular rash compatible with varicella-zoster virus lesions. Nerve conduction studies and magnetic resonance imaging of the brachial plexus showed inflammatory changes at that level. A trial with oral valacyclovir followed by intravenous methylprednisolone bolus was administered without further response. However, human intravenous immunoglobulin resulted in complete recovery of the symptoms. CONCLUSIONS: Human immunoglobulin is effective in BP due to zoster infection and must be considered if standard treatment fails. To the best of our knowledge, this is the first report of BP associated to zoster infection successfully treated with intravenous immunoglobulin.
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Neuropatías del Plexo Braquial/tratamiento farmacológico , Neuropatías del Plexo Braquial/etiología , Encefalitis por Varicela Zóster/complicaciones , Inmunoglobulinas Intravenosas/uso terapéutico , Anciano , Neuropatías del Plexo Braquial/diagnóstico por imagen , Neuropatías del Plexo Braquial/virología , Encefalitis por Varicela Zóster/diagnóstico por imagen , Humanos , Imagen por Resonancia Magnética , Masculino , Conducción Nerviosa/efectos de los fármacos , Enfermedad de Parkinson/complicacionesRESUMEN
OBJECTIVE: This study aimed to report the case of a patient with paroxysmal nonkinesigenic dyskinesias and Fahr syndrome who had a marked response to carbamazepine. METHODS: We present the case of a 57-year-old female patient with episodes of paroxysmal choreoathetoid dyskinesias in the oromandibular region and distal region of upper and lower extremities, with fluctuating dystonic postures in the same distribution; duration was variable ranging from 30 minutes to 3 hours. Laboratory studies were consistent with primary hyperparathyroidism with bilateral brain calcifications. RESULTS: Treatment with low doses of carbamazepine was successful.
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Anticonvulsivantes/uso terapéutico , Enfermedades de los Ganglios Basales/tratamiento farmacológico , Calcinosis/tratamiento farmacológico , Carbamazepina/uso terapéutico , Corea/tratamiento farmacológico , Enfermedades de los Ganglios Basales/complicaciones , Enfermedades de los Ganglios Basales/diagnóstico por imagen , Calcinosis/complicaciones , Calcinosis/diagnóstico por imagen , Corea/complicaciones , Corea/diagnóstico por imagen , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana EdadRESUMEN
OBJECTIVES: The aims of the study were to report the case of a male patient who developed a first episode of Pisa syndrome (PS) to the right side and a second episode to the left side and to discuss the hypothesis that states that denervation is one of the main mechanisms implicated in the development of PS. METHODS: We report on the case of a 71-year-old patient with Parkinson disease who developed PS to the right side while on dopaminergic treatment with pramipexol and levodopa. The dopamine agonist was discontinued and the postural abnormality was corrected increasing the levodopa dose. Six years later, while on ropinirole and levodopa, he developed PS again but this time the lean was to the left. Even though the dopamine agonist was discontinued, this condition failed to improve. CONCLUSIONS: Mechanisms other than denervation and its relationship with the more or less affected side contribute to the development of the syndrome.This is the first report of a case of recurrent alternating PS and highlights the need for research on this topic to better understand this disorder.
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Benzotiazoles/efectos adversos , Agonistas de Dopamina/efectos adversos , Trastornos Distónicos/inducido químicamente , Levodopa/efectos adversos , Equilibrio Postural , Trastornos de la Sensación/inducido químicamente , Anciano , Humanos , Masculino , Enfermedad de Parkinson/tratamiento farmacológico , PramipexolRESUMEN
Parkinson's disease (PD) is not a single entity but rather a heterogeneous neurodegenerative disorder. The present study aims to conduct a critical systematic review of the literature to describe the main pharmacological strategies to treat cognitive dysfunction and major depressive disorder in PD patients. We performed a search of articles cited in PubMed from 2004 to 2014 using the following MeSH terms (Medical subject headings) "Parkinson disease"; "Delirium," "Dementia," "Amnestic," "Cognitive disorders," and "Parkinson disease"; "depression," "major depressive disorder," "drug therapy." We found a total of 71 studies related to pharmacological treatment in cognitive dysfunction and 279 studies for pharmacological treatment in major depressive disorder. After fulfillment of all the inclusion and exclusion criteria, 13 articles remained for cognitive dysfunction and 11 for major depressive disorder, which are presented and discussed in this study. Further research into non-motor symptoms of PD may provide insights into mechanisms of neurodegeneration, and provide better quality of life by using rational drugs.
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OBJECTIVE: To evaluate if type 2 diabetes mellitus (DM) constitutes a prognostic factor for death and severe disability in patients with aneurysm clipping after subarachnoid hemorrhage (ASH), in an Intensive Care Unit (ICU). MATERIAL AND METHODS: This is a cohort study in patients who were admitted to the ICU between December-2009 and June-2010; 20 with DM (exposed group) and 40 without DM (non-exposed group). Mortality was quantified during ICU stay. At ICU discharge, severe disability was measured through the Glasgow Outcome Scale (category 2); and Glasgow Coma Scale was used to estimate the difference in consciousness level between ICU arrival and discharge. Descriptive statistics and Kaplan Meier survival curves were performed. RESULTS: Mean age was similar between groups (55.8 +/- 11 and 55.6 +/- 15 years, respectively, p = 0.40). A vegetative state was present in one patient without DM. The Glasgow Coma Scale score at ICU entry was 14.1 +/- 1.4 and at discharge, 12.0 +/- 3.6 in the exposed group (p = 0.01); and 13.9 +/- 2.0 us. 13.5 +/- 2.6, in the non-exposed group, respectively (p = 0.45). There were 3 deaths in patients with DM and 5, in patients without DM (p > 0.05); survival time was 12 (95%CI 7, 16) and 10 days (95%CI 7, 13), respectively. Mean glucose remained higher in patients who died at the ICU (p < 0.001). Hydrocephaly was present in 6 exposed patients and 2, non-exposed (p = 0.007). Additionally, 7 and 5 with and without DM, respectively registered a positive blood culture (p = 0.04). CONCLUSIONS: DM was not associated with higher mortality in ICU patients, but hyperglycemia was; thus, it is essential that the intensive care provider watches closely the glycemic control.