RESUMEN
RATIONALE: Alien Hand syndrome (AHS) is characterized in most patients by seemingly purposeful, involuntary movements of the extremities. It is not well known among physicians on account of its diverse clinical manifestations. PATIENT CONCERNS: We present a 57-year-old Chinese man who could not stop or turn himself around as he involuntarily and uncontrollably walked forward, which had happened frequently in the month prior to treatment. He had been a heavy drinker for thirty years before the onset of the disease, with an alcohol intake of 600 to 800âml/day. DIAGNOSES: History of alcohol intake and the brain magnetic resonance imaging findings indicated a diagnosis of Marchiafava-Bignami disease. The patient was additionally diagnosed with Alien Hand Syndrome according to his clinical symptoms. INTERVENTIONS: The patient was treated with high doses of vitamin B for 1 month. OUTCOMES: The patient's abnormal behaviors never appeared during the treatment, and no instance of recurrence was observed during the 6 months of follow-up. LESSONS: The clinical manifestation of AHS is non-specific. Only by considering its diverse manifestation can doctors better understand the disease and achieve early intervention.
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Fenómeno de la Extremidad Ajena/etiología , Enfermedad de Marchiafava-Bignami/complicaciones , Enfermedad de Marchiafava-Bignami/diagnóstico , Alcoholismo/complicaciones , Fenómeno de la Extremidad Ajena/diagnóstico , Cuerpo Calloso/diagnóstico por imagen , Cuerpo Calloso/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Persona de Mediana Edad , Tiamina/uso terapéutico , Complejo Vitamínico B/uso terapéuticoAsunto(s)
Cuerpo Calloso/diagnóstico por imagen , Imagen por Resonancia Magnética , Enfermedad de Marchiafava-Bignami/diagnóstico por imagen , Cuerpo Calloso/efectos de los fármacos , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Persona de Mediana Edad , Inducción de Remisión/métodos , Complejo Vitamínico B/administración & dosificaciónRESUMEN
RATIONALE: Marchiafava-Bignami disease (MBD) is a rare disease characterized by demyelination of the corpus callosum. It is most commonly seen in patients with chronic alcoholism. The clinical diagnosis of MBD can be difficult due to its nonspecific manifestation. Central pontine myelinolysis (CPM) occurs mostly as a complication of severe and prolonged hyponatremia, especially when corrected too rapidly. However, CPM can be associated with chronic alcoholism and its clinical presentation can be heterogeneous. Because both MBD and CPM can have fatal outcomes, early recognition and treatment can result in a better prognosis. We present a very rare case of simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis in a patient with chronic alcoholism who was diagnosed unexpectedly using brain magnetic resonance imaging and improved after proper treatment. PATIENT CONCERNS: We presented a case of a 39-year-old patient who visited the hospital with general weakness and an altered neurologic condition after a week of vomiting. DIAGNOSIS: The patient was diagnosed with simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis using brain magnetic resonance imaging. INTERVENTION: Administration of a high dose of thiamine. OUTCOMES: The neurologic signs improved after a week of thiamine administration. LESSONS: This case suggests that Marchiafava-Bignami disease and central pontine myelinolysis might have a common pathogenesis, and brain magnetic resonance imaging is of crucial importance in chronic alcoholic patients presenting with nonspecific neurological deterioration. The appropriate administration of thiamine may prevent poor outcomes.
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Enfermedad de Marchiafava-Bignami/complicaciones , Enfermedad de Marchiafava-Bignami/diagnóstico por imagen , Mielinólisis Pontino Central/complicaciones , Mielinólisis Pontino Central/diagnóstico por imagen , Adulto , Alcoholismo/complicaciones , Humanos , Imagen por Resonancia Magnética , Masculino , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Mielinólisis Pontino Central/tratamiento farmacológico , Tiamina/uso terapéutico , Complejo Vitamínico B/uso terapéuticoAsunto(s)
Pedúnculo Cerebral/diagnóstico por imagen , Cuerpo Calloso/diagnóstico por imagen , Enfermedad de Marchiafava-Bignami/diagnóstico por imagen , Imagen de Difusión por Resonancia Magnética , Humanos , Masculino , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Persona de Mediana Edad , Fármacos Neuroprotectores/uso terapéutico , Piracetam/uso terapéutico , Ácido Valproico/uso terapéutico , Complejo Vitamínico B/uso terapéuticoRESUMEN
Ethanol is an important risk factor for the occurrence of several brain disorders that depend on the amount, period and frequency of its consumption. Chronic use of ethanol often leads to the development of neurodegenerative syndromes, which cause morphological and functional impairments such as foetal alcohol syndrome in newborns exposed to ethanol during pregnancy, Wernicke-Korsakoff Syndrome and, more rarely, Marchiafava-Bignami disease (MBD). MBD is characterized by primary degeneration of the corpus callosum, without inflammation and is associated with oxidative stress and hypovitaminosis, as well as altered mental status, to mention dementia, seizures, depression and so on. This review discusses MBD and poor nutrition as a risk factor for the development of such alcoholic syndrome, with focus on diagnosis, pathogenic aspects, signs and symptoms, as well as therapeutic perspectives. On the basis of the inclusion/exclusion criteria adopted, the performed search in scientific databases (Pubmed, Scielo and Google Scholar) resulted in 100 studies that are being presented and discussed in the present work. Review, case-control and cohort studies on alcoholism-associated hypovitaminosis, oxidative stress, MBD and ethanol metabolism pathways were admitted as relevant. We highlight that MBD is a poorly described, diagnosed, insidious and progressive condition, for which evidence suggests a synergism between ethanol-induced neurotoxic effects and hypovitaminosis B. Present treatment consists of vitamin B1(thiamine) supplementation. Nonetheless, other strategies such as the inclusion of antidepressants or steroidal anti-inflammatories as add-on therapies have been employed as an attempt to improve the damage. Indeed, both the diagnosis and treatment are difficult, and death occurs within few years.
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Consumo de Bebidas Alcohólicas/efectos adversos , Alcoholismo/sangre , Etanol/efectos adversos , Enfermedad de Marchiafava-Bignami/sangre , Deficiencia de Tiamina/sangre , Consumo de Bebidas Alcohólicas/sangre , Alcoholismo/complicaciones , Alcoholismo/tratamiento farmacológico , Enfermedad de Marchiafava-Bignami/diagnóstico , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Enfermedad de Marchiafava-Bignami/etiología , Síndromes de Neurotoxicidad/sangre , Síndromes de Neurotoxicidad/tratamiento farmacológico , Síndromes de Neurotoxicidad/etiología , Estrés Oxidativo , Tiamina/farmacología , Deficiencia de Tiamina/complicaciones , Deficiencia de Tiamina/tratamiento farmacológico , Complejo Vitamínico B/farmacologíaRESUMEN
Rare metabolic diseases may sometimes arise acutely and endanger human life if not immediately recognized and treated. Marchiafava Bignami disease is an uncommon neurologic disorder described in alcohol abusers and characterized by an acute severe damage of brain white matter. Even more rarely, it has been reported in non-alcohol addicted patients, but never in vegetarian people. This is a case report of a young vegetarian woman, accustomed to drink high amounts of tea, who, three weeks after her first natural childbirth, developed serious motor and cognitive disturbances. A timely brain magnetic resonance (MR) allowed us to identify Marchiafava Bignami disease and she healed few hours after the administration of parenteral steroids and vitamins. We advise to suspect Marchiafava Bignami Disease in all patients presenting with non-obvious acute generalized motor and cognitive disturbances, also if non alcoholics, and to collect the nutritional habits in all patients with suspected symptoms. In these cases a timely brain MRI is warranted, since brain imaging is typical and patients may recover after a prompt treatment.
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Encéfalo/diagnóstico por imagen , Dieta Vegetariana/efectos adversos , Enfermedad de Marchiafava-Bignami/diagnóstico por imagen , Enfermedad de Marchiafava-Bignami/etiología , Té/efectos adversos , Adulto , Femenino , Humanos , Imagen por Resonancia Magnética , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Metilprednisolona/uso terapéutico , Resultado del Tratamiento , Complejo Vitamínico B/uso terapéuticoRESUMEN
No disponible
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Humanos , Femenino , Persona de Mediana Edad , Enfermedad de Marchiafava-Bignami/complicaciones , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Enfermedad de Marchiafava-Bignami , Complicaciones de la Diabetes/diagnóstico , Complicaciones de la Diabetes/terapia , Tiamina/uso terapéutico , Hiperglucemia/complicaciones , Hiperglucemia/diagnóstico , Hiperglucemia/tratamiento farmacológico , Trastornos de la Memoria/complicaciones , Glucemia/análisis , Imagen por Resonancia Magnética/métodos , Hipoglucemiantes/uso terapéutico , Prednisona/uso terapéutico , Factores de RiesgoRESUMEN
Marchiafava-Bignami disease (MBD) is a form of toxic demyelinating disease more often seen in chronic alcoholics. The disease process typically involves the corpus callosum and clinically often presents with altered sensorium, neurocognitive defects or seizures with acute cases often deteriorating to comatose state. The death rate is high. We report a rare case of MBD with complete clinical recovery. A 50-year-old male patient presented in an unconscious state and underwent MRI of the brain which showed significant lesions involving the corpus callosum. Following treatment with thiamine and supportive therapy, he improved clinically and a follow-up MRI revealed significant resolution of the earlier lesions. Diffusion-weighted MRI showed the changes more conspicuously as compared with conventional imaging. The clinical resolution corresponded well with the MRI pattern. The case highlights that diffusion-weighted MRI is an extremely useful tool in evaluation and prognostication of MBD.
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Imagen de Difusión por Resonancia Magnética , Enfermedad de Marchiafava-Bignami/diagnóstico , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Humanos , Masculino , Persona de Mediana Edad , Tiamina/uso terapéutico , Complejo Vitamínico B/uso terapéuticoAsunto(s)
Corticoesteroides/uso terapéutico , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Cuerpo Calloso/efectos de los fármacos , Cuerpo Calloso/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Enfermedad de Marchiafava-Bignami/diagnóstico , Persona de Mediana EdadRESUMEN
OBJECTIVE: Marchiafava-Bignami disease (MBD) is a rare condition mainly associated with alcoholism, although it may be mimicked by several other disorders that cause corpus callosum lesions. Our objective was to identify helpful features for differential diagnosis and assess whether any treatment can be recommended. METHODS: We reviewed 122 reports containing data on 153 subjects with confirmed MBD that was associated with either alcoholism or malnutrition, and 20 reports with data on 53 subjects with conditions mimicking MBD. All the cases had been verified antemortem by brain imaging. Unconditional logistic regression was used to demonstrate factors that were associated with the outcome of MBD. RESULTS: The mimicking conditions were differentiated from MBD by the occurrence of solitary and rapidly disappearing splenial lesions; fewer signs and symptoms with exception of seizures, hemiparesis and tetraparesis; nystagmus; and rapid and complete recovery. MBD occurred most frequently among alcoholics, but it was also reported in 11 non-alcoholics (7.2% of all the MBD cases). A better outcome was observed among those who were treated within 2 weeks after onset of symptoms with parenteral thiamine (p=0.033). CONCLUSIONS: As thiamine deficiency is frequently associated with alcoholism, malnutrition and prolonged vomiting; we recommend prompt treatment of MBD with parenteral thiamine in such subjects. Recovery should be followed by repeated neuropsychological and MRI examinations, preferably using diffusion tensor imaging.
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Enfermedad de Marchiafava-Bignami/diagnóstico , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Tiamina/uso terapéutico , Alcoholismo/complicaciones , Alcoholismo/diagnóstico , Alcoholismo/tratamiento farmacológico , Cuerpo Calloso/patología , Diagnóstico Diferencial , Humanos , Imagen por Resonancia Magnética , Enfermedad de Marchiafava-Bignami/complicaciones , Enfermedad de Marchiafava-Bignami/patología , Imagen Multimodal , Neuroimagen , Pronóstico , Esteroides/uso terapéutico , Deficiencia de Tiamina/complicaciones , Deficiencia de Tiamina/tratamiento farmacológico , Tomografía Computarizada por Rayos XRESUMEN
Marchiafava-Bignami disease (MBD) is a rare alcohol-associated disorder characterized by demyelization and necrosis of the corpus callosum. Diffusion-weighted (DW) MRI may reveal the lesions in patients showing serious clinical manifestations, high mortality rate and severe cognitive sequelae, though some cases with good outcome have been reported. We describe a case of a man with a history of chronic alcohol abuse associated with malabsorption; the man presented mild clinical signs on the first neurological exam, despite the presence of DW-MRI lesions, bilaterally involving entire corpus callosum, that are compatible with MBD diagnosis.
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Enfermedad de Marchiafava-Bignami/patología , Anciano , Alcoholismo/complicaciones , Cuerpo Calloso/patología , Imagen de Difusión por Resonancia Magnética , Trastornos Neurológicos de la Marcha/etiología , Humanos , Procesamiento de Imagen Asistido por Computador , Síndromes de Malabsorción/complicaciones , Síndromes de Malabsorción/tratamiento farmacológico , Síndromes de Malabsorción/patología , Masculino , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Enfermedad de Marchiafava-Bignami/psicología , Examen Neurológico , Pruebas Neuropsicológicas , Pronóstico , Resultado del Tratamiento , Vitaminas/administración & dosificación , Vitaminas/uso terapéuticoRESUMEN
We report a 37-year-old woman with no history of alcohol consumption or malnutrition who had Marchiafava-Bignami disease (MBD) as a complication of diabetes mellitus. The patient suddenly developed dizziness and could not speak words fluently. Neurological examination revealed acalculia, agraphia, left blepharoptosis, and mild left facial palsy. Her blood glucose was 391 mg/dL, and her glycated hemoglobin (HbA1c) was 16.0%. Her brain MRI revealed hyperintense changes in the corpus callosum on T2-weighted, fluid-attenuated inversion recovery and diffusion-weighted images. In addition to therapy for diabetes mellitus, prednisolone was commenced. All of the symptoms gradually improved, and after three months, only slight dysarthria, acalculia, and agraphia were observed. Brain MRI also revealed a reduction in lesion size. In conclusion, MBD may occur even with metabolic disorders. It is important to diagnose MBD in the early stages with MRI and to treat the symptoms with cortico steroids.
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Cuerpo Calloso/patología , Complicaciones de la Diabetes/patología , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Enfermedad de Marchiafava-Bignami/etiología , Fibras Nerviosas Mielínicas/patología , Prednisolona/uso terapéutico , Adulto , Antiinflamatorios/uso terapéutico , Cuerpo Calloso/fisiopatología , Complicaciones de la Diabetes/tratamiento farmacológico , Complicaciones de la Diabetes/fisiopatología , Femenino , Humanos , Enfermedad de Marchiafava-Bignami/fisiopatología , Resultado del TratamientoRESUMEN
A 65-year-old woman with dermatomyositis (DM) was admitted because of disorientation, mental dysfunction, and disturbance of consciousness. Prior to admission, she suffered from septic and hypovolemic shock. There was no evidence of active DM on physical examination and laboratory tests. Cerebrospinal fluid examination revealed no signs of meningitis. Because of clinical symptoms and findings on magnetic resonance images, such as the lesion in the splenium of the corpus callosum that was a low-intensity area on T1-weighted images and a high intensity on T2-weighted images; she was diagnosed as Marchiafava-Bignami disease (MBD). She received a combination of vitamin B, vitamin E, vitamin C, and nicotinic acid. Her symptoms improved gradually, and she was discharged at 1.5 months after admission. There has been no report of a case of DM with MBD. This report may provide useful data with regard to the mechanisms of central nervous system (CNS) disorders in patients with DM.
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Encéfalo/patología , Dermatomiositis/complicaciones , Enfermedad de Marchiafava-Bignami/complicaciones , Enfermedad de Marchiafava-Bignami/diagnóstico , Anciano , Ácido Ascórbico/uso terapéutico , Dermatomiositis/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Pruebas Neuropsicológicas , Resultado del Tratamiento , Complejo Vitamínico B/uso terapéutico , Vitamina E/uso terapéuticoAsunto(s)
Vasculitis por Lupus del Sistema Nervioso Central/diagnóstico , Enfermedad de Marchiafava-Bignami/diagnóstico , Administración Oral , Adulto , Anemia Hemolítica Autoinmune/diagnóstico , Anemia Hemolítica Autoinmune/tratamiento farmacológico , Antiinflamatorios/uso terapéutico , Anticuerpos Antinucleares/sangre , Cuerpo Calloso/patología , Diagnóstico Diferencial , Imagen de Difusión por Resonancia Magnética , Femenino , Estudios de Seguimiento , Humanos , Vasculitis por Lupus del Sistema Nervioso Central/tratamiento farmacológico , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Metilprednisolona/uso terapéutico , Necrosis , Prednisolona/uso terapéutico , Proteinuria/diagnóstico , Quimioterapia por PulsoRESUMEN
Introducción. La enfermedad de Marchiafava-Bignami(EMB) es una rara complicación del alcoholismo crónico, caracterizadapor desmielinización y necrosis del cuerpo callosoy usualmente con mal pronóstico. Actualmente, la tomografíacomputarizada (TC) y la resonancia magnética (RM)permiten diagnosticarla en vida.Casos clínicos. Describimos los dos primeros casos deEMB en Uruguay. La depresión de conciencia, la hipertonía ylos signos frontales fueron los signos clínicos dominantes.Un paciente asoció una neuropatía óptica. El otro caso presentóalteraciones neuropsiquiátricas, precediendo a la instalacióndel coma, y asociaba lesiones dérmicas pelagroides.La TC y RM evidenciaron lesiones de sustancia blanca localizadasfundamentalmente en el cuerpo calloso.Conclusiones. La potencial existencia de la EMB debeinvestigarse en todo paciente con alcoholismo crónico quese presente con síndrome confusional prolongado, estupor ocoma. La presencia de alteraciones neuropsiquiátricas puedeconstituir un pródromo, por lo cual deberían explorarse exhaustivamentelas funciones neurosicológicas, incluyendo labúsqueda de signos de desconexión interhemisférica. Laatenta observación del cuerpo calloso en la imagen es fundamentalpara el diagnóstico de esta enfermedad probablementesubdiagnosticada (AU)
Introduction. Marchiafava-Bignami disease (MBD)is a rare complication of chronic alcoholism characteri characterizedby demyelination and necrosis of the corpus callosumthat usually has a poor prognosis. It has an extensiveclinical spectrum presentation and can presently bediagnosed in vivo with the computed tomography (CT)and magnetic resonance imaging (MRI).Clinical cases. We describe two cases of MBD diseasewith fatal outcome that presented with acute onsetcoma. Impairment of consciousness, hypertonia, andfrontal release signs were the dominant clinical signs.One of the patients had optic neuropathy and the otherhad neuropsychiatric symptoms before onset of comaand pellagra-like skin lesion. The CT scan and MRI showedlesions of the white matter that were fundamentallyin the corpus callosum.Conclusions. The possibility of the existence of MBDshould be investigated in all patients with chronic alcoholismwho have prolonged confusional syndrome, stuporor coma. A prodromal stage with neuropsychiatricsymptoms should be thoroughly investigated with a neuropsychologicalexploration including interhemisphericdisconnection signs. Careful attention should be given tothe corpus callosum in the image for the diagnosis ofthis probably underdiagnosed disease (AU)
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Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Enfermedad de Marchiafava-Bignami/diagnóstico , Alcoholismo/complicaciones , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Cuerpo Calloso , UruguayRESUMEN
Marchiafava-Bignami disease (MBD) is characterized by demyelination and necrosis of corpus callosum encountered in chronic alcoholic patients. Etiology is the deficiency of vitamin B complex. Magnetic resonance imaging (MRI) in MBD typically reveals focal lesions of high T2 and FLAIR signal intensity in the corpus callosum. We here present a 42-year-old male alcoholic diagnosed as MBD on the basis of MRI and diffusion-weighted imaging (DWI) features. The patient totally recovered following appropriate vitamin B complex replacement therapy, despite reduced diffusion in the initial setting. This case report emphasizes on the important role played by MRI and DWI in the early diagnosis and follow-up of this potentially fatal disease.
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Imagen de Difusión por Resonancia Magnética , Enfermedad de Marchiafava-Bignami/diagnóstico , Adulto , Humanos , Masculino , Enfermedad de Marchiafava-Bignami/tratamiento farmacológico , Piridoxina/administración & dosificación , Tiamina/administración & dosificación , Vitamina B 12/administración & dosificaciónRESUMEN
We report the first known case of anorexia nervosa (AN) with Marchiafava-Bignami Disease (MBD) that responded to high-dose intravenous corticosteroid administration. A 16-year-old Japanese female with AN was diagnosed with MBD after rapid weight loss. During the acute stage, she suffered from a sudden onset of coma. After regaining consciousness, she presented with lack of movement, apathy, labile affect, and poverty of speech. On admission, magnetic resonance imaging showed an area of demyelination in the splenium of the corpus callosum. Positron emission tomography obtained 7 days after admission showed areas of hypoperfusion in the medial temporal lobe and in regions anterior and posterior to the central sulcus.