Progressive Multifocal Leukoencephalopathy Associated With Idiopathic CD8+ Lymphocytopenia.

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system (CNS) caused by the polyoma John Cunningham (JC) virus. This virus is rarely pathogenic in immunocompetent individuals, being associated with profound cellular immunosuppression. We present a case of a 72-year-old woman with schizoaffective disorder who presented to the emergency department with dysarthria and right hemiataxia. The initial computer tomography was normal and the diagnosis of ischemic stroke was first assumed. However, during hospitalization there was a progressive worsening of symptoms with cerebellar ataxia, and the magnetic resonance revealed a lesion in the right middle cerebellar peduncle hypointense in T1 and hyperintense on T2/fluid attenuated inversion recovery (FLAIR) sequence, suggestive of PML. Although the first cerebrospinal fluid analysis was negative, the second one was positive for the JC virus. Furthermore, due to radiological and clinical progression, mirtazapine was started and the patient underwent a course of intravenous immunoglobulin, with no response. In parallel, causes of immunosuppression were investigated, which led to the diagnosis of idiopathic CD8+ lymphocytopenia. Due to rapid progression of symptoms and radiological worsening of lesions, pembrolizumab was administered. After the first administration of pembrolizumab there was a transitory clinical stabilization. However, shortly after the second administration of pembrolizumab, the patient developed stridor with bilateral vocal cord paralysis and subsequent symptom progression, which led to the death of the patient three months after the appearance of initial symptoms. In conclusion, we report a case of a PML in a patient with idiopathic CD8+ lymphocytopenia, enhancing the need for a high suspicion index for this entity as well as for occult and less frequent forms of immunosuppression. Although there have been various case reports of favourable outcomes with pembrolizumab for PML, more research is needed, particularly to identify patient factors that might be associated with better responses to this therapy.

Metamizole-Induced Type I Kounis Syndrome.

Kounis syndrome (KS) is defined as acute coronary syndrome (ACS) triggered by mast cell and platelet activation in the setting of allergic or anaphylactic insults. KS is a unique and complex cause of ACS and many cases may be missed due to its highly variable clinical manifestations. In this report, we present a case of KS type I triggered by metamizole in the absence of a previous history of allergy to this drug. Following the administration of metamizole, the patient developed generalized acute urticaria, chest pain and diaphoresis. Electrocardiography (ECG) showed ST-segment elevation suggestive of myocardial infarction complicated by ventricular tachycardia. No coronary disease was observed on coronary angiography. The cardiac manifestations of KS may be life-threatening, and so it is important to appropriately recognize and treat this condition. LEARNING POINTS: Kounis syndrome (KS) diagnosis requires a high index of suspicion and should be considered in patients who present with acute coronary syndrome (ACS) soon after the administration of a new medication or possible allergic stimulus.Treatment should be administered carefully, since some drugs used to treat the cardiac manifestations of KS can worsen the allergic reaction.The prognosis is generally good with appropriate treatment, but some complications may occur, such as malignant arrhythmia.

Drug reaction with eosinophilia and systemic symptoms syndrome in a patient taking lamotrigine.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare adverse drug reaction characterised by skin eruption and multiple organ involvement. Diagnosing this entity is challenging due to the variability of clinical manifestations, late onset and relapse even after stopping the causative drug. It is potentially life-threatening; thus, it must be promptly recognised and the causative drug withdrawn. We describe a case of a 50-year-old man with an acute diffuse rash, fever and eosinophilia 4 weeks after having started lamotrigine. The suspected eliciting drug was suspended and systemic corticoid treatment was initiated (prednisolone 0.5 mg/kg/day). Symptoms relapsed under corticoid tapering with greater severity. The patient developed an exuberant rash associated with peripheral lymphadenopathies, marked eosinophilia and hepatic cytolysis. The diagnosis of DRESS syndrome to lamotrigine was made. Prednisolone dosage was increased to 1 mg/kg/day, and the subsequent taper was performed slowly over the course of 10 weeks. Full clinical remission was observed.

[Cryptogenic organizing pneumonia -- case report]. / Pneumonia em organização critogénica -- caso clínico.

Cryptogenic organizing pneumonia (COP) is one of the new recognized histological patterns of idiopathic interstitial pneumonias, in witch no cause or association is identified. Idiopathic bronchiolitis obliterans-organizing pneumonia (BOOP) is a synonymous. This rare entity is characterized by involvement of alveoli and bronchioles, which are filled by intraluminal polyps of fibroblastic tissue (Masson bodies). The clinical presentation often mimics that of community-acquired pneumonia. Persistent non-productive cough, dyspnoea with exertion and weight loss are common features, and in one-half of the cases the onset is heralded by a flu-like syndrome. Definitive diagnosis depends on histological data, and video-assisted thoracoscopic (VAT) has become the established technique. Spontaneous recovery is rare; corticosteroid therapy provides cure in two thirds of cases. The authors present a case of a patient whose lung biopsy by VAT confirmed the diagnosis. She was treated with corticoids without recurrence in a two year follow-up.