RESUMO
By nature, idiopathic interstitial pneumonias have been diagnosed in a multidisciplinary manner. As classifications have been subject to significant refinement over the last decade, the importance of correlating clinical, radiologic, and pathologic information to arrive at a diagnosis, which will predict prognosis in any given patient, has become increasingly recognized. In 2013, the American Thoracic Society and European Respiratory Society updated the idiopathic interstitial pneumonias classification scheme, addressing the most recent updates in the field. The purpose of this review is to highlight the correlations between radiologic and pathologic findings in idiopathic interstitial pneumonias while using updated classification schemes and naming conventions.
Assuntos
Pneumonias Intersticiais Idiopáticas/patologia , Pneumonias Intersticiais Idiopáticas/radioterapia , Síndrome de Meige/patologia , Radiografia , Resultado do Tratamento , Diagnóstico Diferencial , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Síndrome de Meige/diagnóstico , Prognóstico , Radiografia/métodosAssuntos
Reabsorção Óssea/induzido quimicamente , Toxinas Botulínicas Tipo A/administração & dosagem , Côndilo Mandibular/efeitos dos fármacos , Síndrome de Meige/diagnóstico , Fármacos Neuromusculares/administração & dosagem , Transtornos da Articulação Temporomandibular/terapia , Artrocentese , Artroscopia , Diagnóstico Diferencial , Feminino , Humanos , Injeções Intramusculares , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XAssuntos
Antipsicóticos/efeitos adversos , Síndrome de Meige/induzido quimicamente , Palmitato de Paliperidona/efeitos adversos , Antipsicóticos/uso terapêutico , Feminino , Humanos , Síndrome de Meige/diagnóstico , Pessoa de Meia-Idade , Palmitato de Paliperidona/uso terapêutico , Transtornos Psicóticos/tratamento farmacológicoRESUMO
The term "Meige's syndrome" or "Breughel's syndrome" is used by specialists for the description of blepharospasm with the involuntary movements in the lower part of the face and/or masseter muscle. These eponyms "Meige's syndrome" and "Breughel's syndrome" make some mess in the terminology. The term "segmental craniocervical dystonia" joins different blepharospam-plus phenotypes and reflects contemporary perceptions about its genetic and pathophysiological community. Botulinotherapy is in fact the only way of symptomathic treatment of craniocervical dystonias.
Assuntos
Blefarospasmo/classificação , Síndrome de Meige/classificação , Blefarospasmo/diagnóstico , Blefarospasmo/tratamento farmacológico , Toxinas Botulínicas/uso terapêutico , Discinesias/classificação , Discinesias/diagnóstico , Discinesias/tratamento farmacológico , Distonia/classificação , Distonia/diagnóstico , Distonia/tratamento farmacológico , Humanos , Músculo Masseter/fisiopatologia , Síndrome de Meige/diagnóstico , Síndrome de Meige/tratamento farmacológicoRESUMO
Methylphenidate is a short-acting stimulant. In this article, the authors report a 7-year-old male patient who presented with orofacial and limb dyskinesia after his first dose of methylphenidate treatment for a diagnosis of attention-deficit/hyperactivity disorder; he was also receiving sodium valproate treatment for epilepsy. Orofacial dyskinesia appeared 5 hours after methylphenidate administration, persisted for 10 hours, and had completely resolved within 2 days. Although limb dyskinesia after methylphenidate is a commonly reported side effect, to the authors' knowledge this is only the second reported case to develop both orofacial and limb dyskinesia in the acute period after the first dose of methylphenidate. This case is reported to emphasize the potential side effects of methylphenidate, individual differences in drug sensitivities, and drug-receptor interactions via different mechanisms.
Assuntos
Transtorno do Deficit de Atenção com Hiperatividade/tratamento farmacológico , Estimulantes do Sistema Nervoso Central/efeitos adversos , Discinesia Induzida por Medicamentos/diagnóstico , Síndrome de Meige/induzido quimicamente , Síndrome de Meige/diagnóstico , Metilfenidato/efeitos adversos , Anticonvulsivantes/efeitos adversos , Anticonvulsivantes/uso terapêutico , Estimulantes do Sistema Nervoso Central/uso terapêutico , Criança , Interações Medicamentosas , Quimioterapia Combinada , Epilepsia do Lobo Frontal/diagnóstico , Epilepsia do Lobo Frontal/tratamento farmacológico , Humanos , Masculino , Metilfenidato/uso terapêutico , Ácido Valproico/efeitos adversos , Ácido Valproico/uso terapêuticoAssuntos
Cerebelo/patologia , Hemorragias Intracranianas/complicações , Mandíbula , Síndrome de Meige/etiologia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/etiologia , Idoso , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Mandíbula/patologia , Músculos da Mastigação/fisiopatologia , Síndrome de Meige/diagnósticoRESUMO
We describe the unusual clinical course of a patient with cranial dystonia (i.e., Meige syndrome) and additional upper limb involvement, who developed sustained relief of motor symptoms following cessation of a prolonged course of bilateral pallidal deep brain stimulation (DBS). Early response to therapy proved titratable and reversible; however, the patient gained independence from DBS in the fifth postoperative year and has since been more than a year without treatment or exacerbation of motor symptoms. Among the potential explanations for these neurological benefits lies the intriguing possibility that DBS therapy may have the capacity to induce plastic change that lessens or obviates the need for further treatment in susceptible patients.
Assuntos
Estimulação Encefálica Profunda , Síndrome de Meige/terapia , Dominância Cerebral/fisiologia , Eletrodos Implantados , Feminino , Seguimentos , Globo Pálido/fisiopatologia , Humanos , Assistência de Longa Duração , Imageamento por Ressonância Magnética , Síndrome de Meige/diagnóstico , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
We describe intermittent or sustained severe involuntary tongue protrusion in patients with a dystonic syndrome. Speech, swallowing, and breathing difficulties can be severe enough to be life threatening. Causes include neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, Lesch-Nyhan syndrome, and postanoxic and tardive dystonia. The pathophysiology of intermittent severe tongue protrusion remains unknown. Tongue protrusion dystonia is often unresponsive to oral drugs but may benefit from botulinum toxin injections into the genioglossus muscle. Bilateral deep brain pallidal stimulation was beneficial in two cases.
Assuntos
Antidiscinéticos/uso terapêutico , Toxinas Botulínicas/uso terapêutico , Distúrbios Distônicos , Síndrome de Meige , Língua , Adolescente , Adulto , Distúrbios Distônicos/diagnóstico , Distúrbios Distônicos/tratamento farmacológico , Distúrbios Distônicos/fisiopatologia , Feminino , Humanos , Masculino , Síndrome de Meige/diagnóstico , Síndrome de Meige/tratamento farmacológico , Síndrome de Meige/fisiopatologia , Pessoa de Meia-IdadeRESUMO
The spontaneous occurrence of blepharospasm and dystonic movements in face muscles, particularly those of the perioral and mandibular regions, has been named as Meige's disease which was first described by Henry Meige in 1910. We report the case of a woman with Meige's syndrome whose symptoms improved with the use of levetiracetam.
Assuntos
Anticonvulsivantes/administração & dosagem , Músculos Faciais/fisiopatologia , Síndrome de Meige/tratamento farmacológico , Piracetam/análogos & derivados , Toxinas Botulínicas Tipo A/efeitos adversos , Encéfalo/efeitos dos fármacos , Encéfalo/fisiopatologia , Distúrbios do Sono por Sonolência Excessiva/induzido quimicamente , Relação Dose-Resposta a Droga , Eletromiografia/efeitos dos fármacos , Músculos Faciais/inervação , Feminino , Humanos , Canais Iônicos/efeitos dos fármacos , Canais Iônicos/metabolismo , Levetiracetam , Síndrome de Meige/diagnóstico , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade , Chaperonas Moleculares/genética , Piracetam/administração & dosagem , Resultado do TratamentoRESUMO
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary vascular disease that usually begins with migraine, followed by repeated strokes and progressive dementia. We describe an unusual clinical presentation of this condition in members of a Chilean family with an established NOTCH3 mutation. We report early clinical, neuropsychological, transcranial ultrasound, magnetic resonance imaging (MRI), cerebral blood flow, and skin biopsy findings on these patients. Of the patients, 2 presented with facial dystonia, 1 of whom had abnormal single photon emission computed tomography and transcranial ultrasound studies after normal brain MRI scans. Our report emphasizes that CADASIL must be considered in the study of patients with secondary dystonia.
Assuntos
CADASIL/genética , Síndrome de Meige/genética , Receptores Notch/genética , Adulto , Idoso , Biópsia , Encéfalo/patologia , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/genética , CADASIL/diagnóstico , Ventrículos Cerebrais/patologia , Chile , Análise Mutacional de DNA , Demência/diagnóstico , Demência/genética , Diagnóstico Diferencial , Diagnóstico por Imagem , Progressão da Doença , Endotélio Vascular/patologia , Éxons , Músculos Faciais , Feminino , Seguimentos , Giro do Cíngulo/patologia , Humanos , Processamento de Imagem Assistida por Computador , Imageamento Tridimensional , Masculino , Síndrome de Meige/diagnóstico , Microcirculação/patologia , Microscopia Eletrônica de Transmissão , Testes Neuropsicológicos , Linhagem , Receptor Notch3 , Pele/irrigação sanguínea , Pele/patologia , Lobo Temporal/patologiaRESUMO
We report on a 38-year-old patient with rapid-onset dystonia-parkinsonism (RDP) with a missense mutation in the Na/K-ATPase alpha3 subunit (ATP1A3). Asymmetrical parkinsonian symptoms evolved over a year. After a stable episode of another 2.5 years, overnight he developed oromandibular dystonia and more severe parkinsonian symptoms. We conclude that RDP should be considered as a rare cause of levodopa-unresponsive parkinsonism even if there is no family history and the classic presentation is lacking.
Assuntos
Distúrbios Distônicos/diagnóstico , Síndrome de Meige/diagnóstico , Doença de Parkinson/diagnóstico , Transtornos Parkinsonianos/diagnóstico , Adulto , Diagnóstico Diferencial , Dominância Cerebral/genética , Disartria/diagnóstico , Disartria/genética , Distúrbios Distônicos/genética , Seguimentos , Humanos , Masculino , Síndrome de Meige/genética , Mutação de Sentido Incorreto , Exame Neurológico , Doença de Parkinson/genética , Transtornos Parkinsonianos/genética , Fenótipo , Recidiva , ATPase Trocadora de Sódio-Potássio/genéticaRESUMO
We report a 67-year-old woman with idiopathic oromandibular dystonia (OMD). She could neither open the mouth nor take meals due to involuntarily strong mouth-closing. The movement of face, pharynx and tongue were normal, and she could open the mouth slightly when jaw and cheek were touched (sensory trick). Chvostek sign and Trousseau sign were negative, and opisthotonus was not recognized. The laboratory data including calcium, phosphorous and cerebrospinal fluid were within normal limits, head and cervical MRI, temporomandibular joints-Xp and needle electromyography were normal. The surface electromyography revealed that masseter and chin muscles contracted synchronously. This result meant dystonia around the mouth. The clinical course and physical examination did not support the diagnosis of tetanus, tetany or bulldog response. She was diagnosed as OMD. She had peroral administration of baclofen, because this drug is a GABA-derivative and acts as a muscular relaxant. Her clinical symptoms and dystonic pattern on the surface electromyography improved markedly after the administration. Baclofen is an effective drug for treatment of oromandibular dystonia.
Assuntos
Baclofeno/administração & dosagem , Agonistas GABAérgicos/administração & dosagem , Síndrome de Meige/tratamento farmacológico , Relaxantes Musculares Centrais/administração & dosagem , Idoso , Eletromiografia , Feminino , Humanos , Síndrome de Meige/diagnóstico , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
INTRODUCTION: The Meige's syndrom is characterized by the presence of bilateral, symmetrical, dystonic cramp of face muscles or muscles of middle line of body, the respiratory muscles and muscles of throat. The etiology of Meige's syndrome is uncertain. The disorders of basal ganglia function and neurotransmitters' imbalance (dopamine and acetylocholine) can be with reason of pronouncement of symptoms presumably. MATERIAL AND METHOD: Our aim was to introduce a case of 71 years old patient in whom we diagnosed spasmodic dysphonia in course of Meige's Syndrom. Patient has been treated by 3 years with Botulin toxin. The spasmodic Dysphonia occurred after over 2 years from appearing of first syndrom's symptoms. RESULTS: Sonorous voice during rehabilitation was got during expressing syllables and short bisyllabic words. Patient stays still under phoniatric care. CONCLUSIONS: Patients with spasmodic dysphonia ought to be examined by a interdisciplinary medical team.
Assuntos
Síndrome de Meige/complicações , Síndrome de Meige/diagnóstico , Espasmo/etiologia , Distúrbios da Voz/etiologia , Idoso , Toxinas Botulínicas/uso terapêutico , Humanos , Masculino , Síndrome de Meige/tratamento farmacológico , Espasmo/tratamento farmacológico , Distúrbios da Voz/tratamento farmacológico , Qualidade da Voz , Treinamento da VozRESUMO
Herein, a rare case of ovarian granulosa cell tumor, presenting as Meigs' syndrome, with elevated carbohydrate antigen 125 (CA125), is reported. A 69-year-old woman was admitted for the investigation of abdominal fullness and dyspnea. A preoperative examination revealed a huge pelvic tumor and an abdominopelvic magnetic resonance image (MRI) assumed ovarian cancer. A chest computed tomography (CT) scan revealed pleural effusion. A laparotomy confirmed the huge mass to be an ovarian tumor. A total abdominal hysterectomy (TAH), with a bilateral salpingo-oophorectomy (BSO) and partial omentectomy, was performed. Although short-term intrathoracic drainage was required, the hydrothorax and ascites rapidly resolved in the postoperative period.
Assuntos
Antígeno Ca-125/sangue , Tumor de Células da Granulosa/diagnóstico , Síndrome de Meige/diagnóstico , Neoplasias Ovarianas/diagnóstico , Idoso , Diagnóstico Diferencial , Feminino , HumanosRESUMO
The liability to develop parkinsonian symptoms was evaluated in 105 outpatients with idiopathic blepharospasm (IBS; 54 cases) or IBS associated to oromandibular dystonia (Meige's syndrome; 51 cases) mean age 70.3 +/- 9.6 years, and compared with an age- and sex-matched population. Eleven patients developed Parkinson's disease in the blepharospasm group, whereas only 2 of 105 patients were affected in the control group. Our results suggest that patients with IBS either isolated or associated with oromandibular dystonia are more prone to develop parkinsonian symptoms.
Assuntos
Síndrome de Meige/epidemiologia , Doença de Parkinson/epidemiologia , Doença de Parkinson/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Toxinas Botulínicas Tipo A/uso terapêutico , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Encéfalo/fisiopatologia , Progressão da Doença , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Síndrome de Meige/diagnóstico , Síndrome de Meige/tratamento farmacológico , Fármacos Neuromusculares/uso terapêutico , Doença de Parkinson/diagnóstico , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios XRESUMO
We report on a woman with idiopathic Meige's syndrome whose dystonia improved with the use of levetiracetam (LEV, Keppra, UCB Pharma, Smyrna, GA). This report and data from an animal model of paroxysmal dystonia suggest that LEV might be helpful in the treatment of dystonia.