A 43-year-old woman reported acute onset of right retroorbital headache followed by ptosis, paralysis of extraocular muscles, and subsequently amaurosis, characterizing an orbital apex syndrome 1 . Brain MRI showed diffuse pachymeningitis with leptomeningeal and parenchymatous involvement (Figure 1). CSF analysis was unremarkable. ACE and
RESUMO -Descrevemos caso de uma paciente de 40 anos com quadro de degeneração cerebelar subaguda paraneoplásica associada a tumor ovariano. Apresentamos breve revisão sobre as manifestações clínicas e laboratoriais desta síndrome, enfatizando a importância do seu reconhecimento, o que possibilita muitas vezes a detecção e tratamento precoce da doença primária.PALAVRAS-CHAVE: síndromes paraneoplásicas, degeneração cerebelar subaguda, câncer de ovário. Paraneoplastic subacute cerebellar degeneration: case reportABSTRACT -We report a 40-year-old woman with subacute cerebellar degeneration associated with ovarian cancer. We briefly review the clinical and laboratory features of this syndrome, and emphasize the importance of its prompt recognition, which many times makes possible the early detection and treatment of the primary disorder.KEY WORDS: paraneoplastic syndromes, subacute cerebellar degeneration, ovarian cancer.Complicações neurológicas atingem cerca de 50% dos pacientes com câncer durante o curso da doença. As neoplasias originadas fora do sistema nervoso podem afetar suas porções central e periférica de duas maneiras: 1) mecanismos diretos (invasão direta ou metástases); 2) mecanismos indiretos (obstrução vascular, déficit nutricional ou metabólico, efeitos tóxicos do tratamento, síndromes paraneoplásicas).Síndromes paraneoplásicas são doenças neurológicas com fisiopatologia relacionada ao câncer, mas não atribuíveis a metástases ou outros mecanismos indiretos citados acima. São as complicações mais raras nos pacientes com câncer, e sua freqüência é desconhecida. Podem afetar qualquer nível do sistema nervoso e freqüentemente antecedem a detecção do tumor. Seu diagnóstico correto depende de alto grau de suspeição por parte do médico, além do conhecimento de suas manifestações clínicas e dos tumores tipicamente a elas associados. Por anteceder o diagnóstico da neoplasia, sua importância reside na oportunidade de identificar e tratar precocemente o tumor, que muitas vezes é pequeno e escapa à detecção mesmo após avaliações clínicas repetidas.Relata-se caso de uma paciente com adenocarcinoma de ovário diagnosticado durante avaliação de síndrome pancerebelar subaguda. Detecção relativamente precoce da neoplasia e tratamento curativo só foram possíveis pela identificação da síndrome paraneoplásica e reconhecimento das patologias a ela associadas, possibilitando atuação rápida e direcionada da equipe.
Marchiafava-Bignami disease (MBD) is a neurological disorder that has been found to be associated with chronic alcoholism and malnutrition. We report a 45 year old man, chronic alcoholic that developed discouragement for activities involving daily living, changes in retrograde memory in addition to mutism and gait instability. Brain MRI showed central atrophy of the corpus callosum(CC), hypointensity(necrosis) and ventricular dilation(white matter and subcortical region involvement). Pathological characteristics include isolated demyelination and axonal loss in the central layer of the CC sparing the dorsal and ventral layer. This pattern of necrosis of the middle layer of the CCis a typical finding in the disease. The clinical diagnosis has considerably changed during recent decades after brain MRI provided the opportunity of a reliable in-vivo diagnosis. With early detection and treatment, the prognosis of MBD may be good. Marchiafava-Bignami disease (MBD) is a rare complication related to alcohol abuse and chronic malnutrition which results in demyelination and symmetrical necrosis of the central layer of the corpus callosum [1]. Serum anion gap and osmotic gap can be observed in patients ingesting etanol. The serum anion gap still might be high, which could lead to the demyelination syndrome. The increase in serum osmolarity produced by alcohol is relied onserum concentration, molecular weights, and metabolic rate. Toxic effects of alcohol and its metabolites can cause demyelination of the corpus callosum [2]. Despite alcohol is the principal element, there are other causes that can be factors such as cyanide and CO poisoning, sepsis, sickle cell disease and Plasmodium infection [3]. The clinical presentation is variable, including mental confusion, dementia, psychosis, spasticity and dysarthria [4]. Since the symptoms are not specific, the clinical diagnosis can be difficult, being the magnetic resonance imaging (MRI) of pivotal value in the investigation [5]. Diffuse thickening of the CC andT1 and T2 prolonged areas in the CC are considered characteristic MRI findings at the acute phase. It has been reported that the first change on MRI is diffuse swelling of the corpus callosum, followed by a genu lesion, and finally by a splenium lesion, sparing the rostrum [5,6]. T1-weighted MRI in the subacute to chronic stage shows hypointense cystic-necrotic lesions in the corpus callosum in addition to callosal atrophy. Extracallosal lesions have been reported involving predominantly the periventricular white matter or the basal ganglia [7].
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