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ABSTRACT Objective: Inhalant users may develop toluene leukoencephalopathy, a devastating neuropsychiatric disorder. We present a case of toluene-induced damage to the corticospinal and the corticonuclear tracts, which presented with involuntary emotional expression dis-order. Methods: Case study of a 20-year-old man with a 3-year history of frequent solvent abuse was admitted to the Neuropsychiatry Unit of the National Institute of Neurology and Neurosurgery because "he could not speak or walk" but would keep "laughing and crying without reason". Results: Neuropsychiatric examination revealed pathological laughter and crying, facial and speech apraxia, a bilateral pyramidal syndrome, and lack of control of urinary sphincter. Magnetic resonance imaging revealed a highly selective bilateral damage to the pyramidal system and the somatosensory pathway. SPECT imaging showed left fronto-parietal hypoperfusion. Conclusions: This document provides support for the understanding of involuntary emotional expression disorders as a differential diagnosis in the clinical practice of psychiatrists, as well as the functional anatomy of these conditions.
RESUMEN Objetivo: Los usuarios de inhalantes pueden contraer leucoencefalopatía por tolueno, un trastorno neuropsiquiátrico devastador. Se presenta un caso de daño inducido por tolueno en el tracto corticoespinal y corticonuclear, que se manifestó con un trastorno involuntario de la expresión emocional. Métodos: Un varón de 20 años con antecedente de 3 años de abuso de solventes ingresó en la Unidad de Neuropsiquiatría del Instituto Nacional de Neurología y Neurocirugía porque «no podía hablar ni caminar¼ y presentaba episodios súbitos de risa y llanto sin razón aparente. Resultados: La valoración neuropsiquiátrica reveló risa y llanto patológicos, apraxia facial y fonatoria, síndrome piramidal bilateral y ausencia de control del esfínter urinario. La resonancia magnética cerebral mostró un daño bilateral muy selectivo del sistema piramidal y la vía somatosensorial. La imagen de tomografía computarizada por emisión monofotónica mostró hipoperfusión frontoparietal izquierda. Conclusiones: Este documento proporciona apoyo para la comprensión de los trastornos de la expresión emocional involuntaria como diagnóstico diferencial en la práctica clínica de los psiquiatras, así como de la anatomía funcional de estas condiciones.
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INTRODUCTION: Pseudobulbar palsy (PBP) is characterized by supranuclear lesions in the corticobulbar pathway. Neoplasia, inflammatory, demyelinating, and stroke are possible etiologies of this disorder. CASE REPORT: We report an elderly female who presented with dysarthria. She was dysarthric with a hypernasal voice, no apraxia or aphasia was observed. Tongue movements were slow with limited amplitude. Her soft palate dropped bilaterally; gag reflex was present. Also, she reported swallowing difficulty and choking with her saliva. Bilateral vertical and horizontal gaze were intact to either voluntary or oculocephalic movements. A cranial CT scan was suggestive of artery of Percheron (AOP) infarction. Brain magnetic resonance imaging showed hypersignal on diffusion-weighted and T2-weighted images and hyposignal on apparent diffusion coefficient in both thalami. CT angiography scan revealed an AOP originating from the left posterior cerebral artery. The swallowing study with a videofluoroscopic demonstrated oral and pharyngeal phases with severe dysfunction. CONCLUSION: To the authors' knowledge, there are two cases of individuals with artery of Percheron infarction who developed PBP associated with other clinical syndromes. Still, isolated PBP following infarction of Percheron's artery was not reported. We hypothesized that the PBP may have occurred because of the existence of vascular territory variations in the perforating arteries that arise from the AOP.
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Encéfalo/diagnóstico por imagem , Infarto Cerebral/diagnóstico por imagem , Imagem de Difusão por Ressonância Magnética/métodos , Disartria/etiologia , Infarto/diagnóstico por imagem , Paralisia Pseudobulbar/complicações , Tálamo/diagnóstico por imagem , Idoso , Artérias/patologia , Infarto Cerebral/complicações , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Infarto/complicações , Imageamento por Ressonância Magnética , Neuroimagem/efeitos adversos , Tálamo/irrigação sanguínea , Tálamo/fisiopatologia , Tomografia Computadorizada por Raios XRESUMO
INTRODUCTION: Foix-Chavany-Marie syndrome (FCMS) is a type of pseudobulbar palsy that affects facio-pharyngo-glosso-masticatory muscles. MATERIALS AND METHODS: A 62-year-old man was admitted to the emergency department after 9 hours of acute dysarthria and dysphagia. MRI showed restricted diffusion in the right operculum on diffusion-weighted imaging (DWI). No thrombolytic therapy was given. The patient had a history of mechanical aortic valve replacement under anticoagulation with a vitamin K antagonist. Work-up demonstrated suboptimal levels of INR. Due to severe dysphagia during hospitalization, a percutaneous endoscopic gastrostomy (PEG) was performed. RESULTS: The patient was discharged 5 days later, with a modified Rankin scale (mRs) score of 3, and secondary stroke prevention. He had achieved an excellent functional outcome (mRs 1) at 6-month follow-up. CONCLUSION: Our patient had a satisfactory recovery due to prompt diagnosis, secondary stroke prevention, and compliance with treatment. LEARNING POINTS: In the presence of acute dysarthria and dysphagia, Foix-Chavany-Marie syndrome (FCMS) should be considered.FCMS may occur in the presence of unilateral opercular stroke.Swallowing and speech therapy play an essential role in rehabilitation after the acute setting.
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Foix-Chavany-Marie syndrome (FCMS) is a cortical-subcortical pseudobulbar palsy characterized by automatic voluntary dissociation of facio-masticatory-pharyngo-glosso-laryngeal movements. FCMS is typically caused by vascular insults on the bilateral anterior opercular or adjacent subcortical areas. Acute onset of FCMS secondary to a unilateral lesion is extremely rare. Herein we present a case of FCMS caused by acute unilateral anterior opercular infarction with preexisting bilateral leukoaraiosis. Our case shows that an acute unilateral anterior opercular lesion can decompensate preexisting corticobulbar-subcortical lesions and cause the typical features of FCMS.
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Resumen La mielinolisis central de la protuberancia o síndrome de desmielinización osmolar, es un padecimiento que se asocia a pacientes con hiponatremia grave, a los cuales se les realiza una corrección brusca del sodio4. Se manifiesta como parálisis pseudobulbar que se presenta con: tetraparesia, encefalopatía que en ocasiones se puede presentar con rigidez, ataxia y movimientos anormales. Se trata de una desmielinización no inflamatoria secundaria al edema neuronal intenso tanto a nivel del puente como en otras zonas extrapontinas10. Es una patología infrecuente, sin embargo tiene mal pronóstico y la terapia con rehabilitación es el único tratamiento. El objetivo de la presente comunicación es informar el caso de un hombre de 63 años con cuadro de déficit neurológico progresivo posterior a un evento quirúrgico y corrección rápida de hiponatremia que presentó en días posteriores, mielinolisis pontina y extrapontina con diagnóstico por imagen y resonancia magnética. El paciente ameritó un largo periodo de hospitalización y rehabilitación, sin embargo las secuelas neurológicas actuales no son incapacitantes. Es imprescindible reconocer la hiponatremia y corregirla de manera correcta para evitar este tipo de complicaciones.
Abstract Central pontine myelinolysis or osmolar demielinization syndrome is a very wellknown disorder in patients with severe hyponatremia that undergo to a rapid sodium replacement. It is clinically described as a pseudobulbar palsy, with tetraparesis, and encephalopathy, that can also show generalized rigidity, ataxia and the presence of abnormal movements. It consists of a non-inflammatory demielinization secondary to neuronal swelling or edema, at the pontine level and in other extrapontine locations. It is an infrequent pathology that carries a bad prognosis and where the only available treatment is prolonged rehabilitation. Herein we inform the case of a 63 year old male, with a progressive neurological deficit following a surgical intervention and rapid replacement of a severe hyponatremia. The patient required a prolonged in hospital stay and further rehabilitation, although the neurological sequels actually are not disabling. It is essential to recognize hyponatremia and to perform an adequate correction of sodium levels to avoid this complication.
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PURPOSE: Interest in the association of epilepsy and pseudobulbar palsy was rekindled since the identification through magnetic resonance imaging (MRI) of bilateral perisylvian polymicrogyria (PMG). Seizures are often intractable, but resective epilepsy surgery has not been recommended. However, a similar clinical picture can be encountered in patients with bilateral perisylvian destructive lesions, which fit the description of ulegyria (ULG). We report a series of patients with epilepsy and pseudobulbar palsy due to bilateral perisylvian ULG (BP-ULG), show that hippocampal sclerosis (HS) is often associated and highlight the fact that in this entity, unlike in malformative bilateral perisylvian PMG, seizures may be surgically treated. METHODS: The motor, cognitive, epileptologic, and imaging features of 12 patients with perisylvian ULG followed at three institutions are described. For patients with refractory seizures, we detail extracranial and intracranial electrographic recordings, surgical strategies, histopathologic analyses of the resected tissue, and outcome of surgical treatment. Descriptive statistics were used for quantitative and categorical variables. Student's t-test was used to compare means, and a p < 0.05 was considered significant. KEY FINDINGS: Pseudobulbar palsy and mental retardation were present in all patients with symmetrical BP-ULG. Five had refractory seizures. There was no relationship between the severity of the pseudobulbar palsy or of the mental retardation and the degree of seizure control with medication. The five patients in whom seizures were refractory to medication had significantly earlier age of onset and longer duration of epilepsy (p < 0.05). Dual pathology with associated unilateral HS was present in four. One patient with dual pathology had a temporolimbic electroclinical picture and had an anterior temporal lobectomy (ATL) based upon noninvasive evaluation. The other four had ictal semiology suggesting involvement of both temporolimbic and perisylvian cortex. Intracranial electroencephalography (EEG) showed concomitant seizure onset in the anterior temporal region and in the ipsilateral ULG in three of the four with dual pathology and in the ulegyric cortex in the one without HS. Resection guided by a combination of semiology, MRI, and extra and intracranial EEG led to complete seizure control in two and almost complete seizure control (Engel class II) in two other patients. The only surgical failure was an isolated ATL in a patient with dual pathology, and concomitant seizure onset in both lesions according to semiology and intracranial EEG. SIGNIFICANCE: Our findings suggest that BP-ULG mimics the clinical features of bilateral perisylvian PMG. In patients with refractory seizures, recognition of this entity should lead to consideration of resective surgery despite the bilateral ULG.
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Córtex Cerebral/cirurgia , Epilepsia/complicações , Epilepsia/cirurgia , Deficiência Intelectual/complicações , Malformações do Desenvolvimento Cortical/complicações , Malformações do Sistema Nervoso/complicações , Anormalidades Múltiplas/cirurgia , Adolescente , Adulto , Córtex Cerebral/patologia , Eletroencefalografia , Epilepsia/diagnóstico , Feminino , Proteína Glial Fibrilar Ácida/metabolismo , Humanos , Recém-Nascido , Deficiência Intelectual/cirurgia , Imageamento por Ressonância Magnética , Masculino , Malformações do Desenvolvimento Cortical/cirurgia , Malformações do Sistema Nervoso/cirurgia , Proteínas de Neurofilamentos/metabolismo , Testes Neuropsicológicos , Procedimentos Neurocirúrgicos/métodos , Paralisia Pseudobulbar/complicações , Paralisia Pseudobulbar/cirurgia , Resultado do Tratamento , Adulto JovemRESUMO
A síndrome pseudobulbar caracteriza-se por disartria, disfagia e disfonia, deficiência dos movimentos voluntários da língua e músculos faciais e por labilidade emocional. E causada pelo comprometimento bilateral das aferências bulbares supranucleares determinada por diferentes fato¬res etiológicos, entre os quais transtornos cerebrovasculares, traumatismo cranioencefálico, doenças do neurônio motor superior e esclerose múltipla. Considerando as dificuldades encontradas pelos fonoaudiológos em compreender os mecanismos de controle neural da deglutição e da fala, que são essenciais para o diagnostico e terapia dos distúrbios destes sistemas, este estudo foi realizado com o objetivo de revisar a literatura especializada sobre a síndrome pseudobulbar, enfocando seus aspectos anatomofisiológicos, fisiopatológicos, clínicos e fonoaudiológicos.
The pseudobulbar syndrome is characterized by dysarthria, dysphagia and disphonia, deficiency of the voluntary movements of the tongue and facial muscles and emotional lability. It is caused by bilateral injury of the supranuclear bulbar afferences determined by different etiological factors like cerebrovascular disorders, traumatic brain injury, diseases of the upper motor neuron and multiple sclerosis. The difficulties found by the speech-language pathologists in understanding the mechanisms of the neural control of deglutition and speech, which are essential for the diagnosis and therapy of these systems disturbances, is the reason of this review of the specialized literature about the pseudobulbar syndrome, focusing its anatomophysiological, physiopathological, clinical and speech-language pathological features.