RESUMO
Acute toxic encephalopathy (ATE) is a widely recognized medical emergency with an expansive differential. One particular known etiology for ATE is elevated ammonia, a powerful neurotoxin that often presents with clinical findings of confusion, disorientation, tremors, and in severe cases, coma and death. Hyperammonemia is most commonly associated with liver disease and presents as hepatic encephalopathy in the setting of decompensated cirrhosis; however, in rare cases, a patient may suffer from non-cirrhotic hyperammonemic encephalopathy. We describe the case of a 61-year-old male with metastatic gastrointestinal stromal tumor who was diagnosed with non-cirrhotic hyperammonemic encephalopathy, and briefly explore the literature describing its mechanisms.
RESUMO
Clinical worsening or new manifestation of cryptococcal disease following initiation of anti-retroviral therapy (ART) in an HIV patient is a hallmark of cryptococcal immune reconstitution inflammatory syndrome (C-IRIS). However, it can be difficult to distinguish IRIS from worsening or new infection. Here, we present a case of severe C-IRIS involving multiple cerebellar, spinal, and intradural abscesses and spinal arachnoiditis 7 months after ART initiation in an AIDS patient with uncertain prior ART compliance. He had multiple prior episodes of cryptococcal meningitis with complications necessitating ventriculoperitoneal shunt placement and was on suppressive fluconazole when he developed worsening brain manifestations. He received empiric anti-cryptococcal re-induction without improvement. All cerebrospinal fluid cultures remained sterile, with negative Cryptococcus PCR testing, and his condition continued to worsen prior to corticosteroid initiation. Ultimately, C-IRIS was diagnosed by brain biopsy. This case demonstrates an extreme in severity of C-IRIS and in the timeline of presentation after ART initiation.
Assuntos
Síndrome da Imunodeficiência Adquirida/tratamento farmacológico , Antirretrovirais/uso terapêutico , Antifúngicos/uso terapêutico , Abscesso Encefálico/diagnóstico por imagem , Empiema Subdural/diagnóstico por imagem , Síndrome Inflamatória da Reconstituição Imune/diagnóstico por imagem , Meningite Criptocócica/tratamento farmacológico , Anfotericina B/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Aracnoide-Máter/patologia , Aracnoidite/congênito , Aracnoidite/diagnóstico por imagem , Aracnoidite/tratamento farmacológico , Biópsia , Encéfalo/patologia , Abscesso Encefálico/tratamento farmacológico , Edema Encefálico/diagnóstico por imagem , Doenças Cerebelares/diagnóstico por imagem , Doenças Cerebelares/tratamento farmacológico , Empiema Subdural/tratamento farmacológico , Fluconazol/uso terapêutico , Flucitosina/uso terapêutico , Infecções por HIV/tratamento farmacológico , Humanos , Síndrome Inflamatória da Reconstituição Imune/tratamento farmacológico , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Prednisona/uso terapêutico , RecidivaRESUMO
Background. Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a diagnosis of exclusion in the clinical scenario of increased intracranial pressure without an identifiable cause. This case report provides a brief review of current literature on IIH, important differential considerations to rule out prior to diagnosis, and relevant imaging findings of IIH. Case Presentation. An 18-year-old male presenting with headache and blurry vision was found to have signs of increased intracranial pressure on imaging without other abnormalities to explain the cause, suggesting IIH as a diagnosis. Conclusion. IIH is classically seen in overweight females of childbearing age but should be considered in all obese patients, including the pediatric population given the increasing rate of childhood obesity.
RESUMO
Hemophagocytic lymphohistiocytosis (HLH) is a rare immune hyperactivation syndrome which may be primary (genetic) or secondary to various immune-related conditions including infection, immunodeficiency, and malignancies. Rapid diagnosis and treatment are essential because it can be associated with significant morbidity and mortality. Epstein-Barr virus (EBV) is a known infectious cause of acquired HLH, but EBV-associated HLH involving the central nervous system is rare and not well characterized neuropathologically. We report a case of fatal EBV-associated HLH with severe involvement of the central nervous system showing florid hemophagocytosis in the choroid plexus, with extensive neuron loss and gliosis in the cerebrum, cerebellum, and brainstem.
Assuntos
Encéfalo/virologia , Viroses do Sistema Nervoso Central/virologia , Infecções por Vírus Epstein-Barr/virologia , Herpesvirus Humano 4/isolamento & purificação , Linfo-Histiocitose Hemofagocítica/virologia , Neurônios/virologia , Medula Espinal/virologia , Adulto , Autopsia , Biópsia , Encéfalo/patologia , Viroses do Sistema Nervoso Central/diagnóstico , Viroses do Sistema Nervoso Central/terapia , Infecções por Vírus Epstein-Barr/diagnóstico , Infecções por Vírus Epstein-Barr/terapia , Evolução Fatal , Gliose , Herpesvirus Humano 4/genética , Humanos , Imuno-Histoquímica , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/terapia , Imageamento por Ressonância Magnética , Masculino , Neurônios/patologia , RNA Viral/genética , Índice de Gravidade de Doença , Medula Espinal/patologiaRESUMO
A middle-aged woman who experienced recurrent episodes of unilateral vision loss and eye pain. On presentation, magnetic resonance imaging (MRI) demonstrated left optic nerve enhancement with patchy hyperintensities in the white matter of both frontal lobes and ill-defined enhancement in a lenticulostriate distribution. Ophthalmologic examination revealed left optic disc edema with a macular scar consistent with neuroretinitis. Her subsequent clinical course was notable for 2 episodes of painful vision loss, without associated neurologic symptoms, which resolved with intravenous and oral steroids. More than 1 year after her initial presentation, the patient developed right facial weakness and slurred speech, and shortly thereafter suffered a fatal intracerebral hemorrhage. Histopathology on autopsy confirmed a diagnosis of primary angiitis of the central nervous system (PACNS). This is an unusual case of PACNS presenting with recurrent unilateral optic neuritis. The vascular enhancement pattern on MRI suggesting inflamed cerebral blood vessels is a rarely described pattern, which likely reflects intracerebral extension of the ocular pathology. The combination of neuroretinitis and perivascular MRI enhancement pattern may represent a subtype of PACNS.
Assuntos
Neurite Óptica/fisiopatologia , Vasculite do Sistema Nervoso Central/complicações , Vasculite do Sistema Nervoso Central/diagnóstico , Transtornos da Visão/etiologia , Feminino , Lateralidade Funcional , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Esteroides/uso terapêutico , Vasculite do Sistema Nervoso Central/tratamento farmacológico , Transtornos da Visão/tratamento farmacológicoAssuntos
Artérias Cerebrais/patologia , Artérias Cerebrais/fisiopatologia , Cérebro/patologia , Cérebro/fisiopatologia , Vasculite do Sistema Nervoso Central/patologia , Vasculite do Sistema Nervoso Central/fisiopatologia , Cérebro/irrigação sanguínea , Lateralidade Funcional/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Vasculite do Sistema Nervoso Central/terapiaRESUMO
In acquired immunodeficiency syndrome (AIDS) patients, differentiating toxoplasmosis and primary central nervous system (CNS) lymphoma remains a clinical and radiographic dilemma. The presence of butterfly lesions crossing the corpus callosum is customarily used to exclude the possibility of toxoplasmosis. We present an AIDS patient who had Epstein-Barr virus (EBV) polymerase chain reaction (PCR) -positive cerebrospinal fluid studies with a butterfly toxoplasmosis lesion confirmed by multiple methods signifying the importance of including toxoplasmosis in the differential diagnosis of butterfly lesions.