Journal of medical case reports
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Paraneoplastic pruritus is defined as pruritus that occurs before or during the natural evolution of a hematologic disease. The reported prevalence is 30% in patients with Hodgkin's lymphoma. The severity of this pruritus has a very negative impact on patients' quality of life. Very few studies have been made to examine the efficacy of pharmacological treatments for this type of pruritus. One drug that appears to be effective in this respect is off-label aprepitant, a neurokinin 1 receptor antagonist. ⋯ Several cases and case series have been reported on the use of aprepitant for paraneoplastic pruritus, but none have referred to its use for Hodgkin's lymphoma. A prospective study was carried out to evaluate the efficacy of this drug in refractory pruritus secondary to Sezary syndrome, and other authors have studied the effectiveness of aprepitant against pruritus, secondary to biological therapy with erlotinib. In our case report, treatment was started with daily doses of aprepitant 80 mg. Pruritus improvement appeared to be attributable exclusively to the administration of aprepitant.
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Systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease is an extremely rare disorder and classically arises following primary acute or chronic active Epstein-Barr virus infection. It is characterized by clonal proliferation of Epstein-Barr virus-infected T-cells with an activated cytotoxic phenotype. This disease has a rapid clinical course and is more frequent in Asia and South America, with relatively few cases being reported in Western countries. The clinical and pathological features of the disease overlap with other conditions including infectious mononucleosis, chronic active Epstein-Barr virus infection, hemophagocytic lymphohistiocytosis and natural killer cell malignancies. We describe the rare case of systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease in a 16-year-old Malay boy. ⋯ Although systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease is a disorder which is rarely encountered in clinical practice, our case report underlines the importance of a comprehensive diagnostic approach in the management of this disease. A high level of awareness of the disease throughout the diagnosis process for young patients who present with systemic illness and hemophagocytic syndrome may be of great help for the clinical diagnosis of this disease.
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Ganglioneuromas are rare benign tumors arising from the neural crest tissue and are most commonly located in the posterior mediastinum and retroperitoneum; they are rarely found in the adrenal gland. This tumor is usually asymptomatic and in the majority of cases is detected incidentally. Although the characteristics of adrenal ganglioneuroma on computerized tomography and magnetic resonance imaging have been well described, the exact diagnosis is difficult. Histopathological examination is currently the mainstay of diagnosis. Ganglioneuromas have a very good prognosis with surgical removal. We report the case of a male patient with an incidentally identified adrenal ganglioneuroma with high standardized uptake values in a positron emission tomography scan. ⋯ Ganglioneuroma may sometimes be similar to other adrenal malignancies. Careful evaluation with endocrine tests and imaging procedures is necessary to provide an accurate diagnosis. Definitive diagnosis can be made by histological examination. The prognosis is very good with surgical removal.
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Red ear syndrome is a rare condition characterized by episodic attacks of erythema of the ear accompanied by burning ear pain. Symptoms are brought on by touch, exertion, heat or cold, stress, neck movements and washing or brushing of hair. Diagnosis and treatment of this condition are challenging. The case we report here involves a woman whose symptoms were brought on by a dietary trigger: orange juice as well as stress, causing significant physical and psychological morbidity. Avoidance of triggers resulted in symptomatic improvement. ⋯ Red ear syndrome is a little-known clinical syndrome of unknown etiology and management. To the best of our knowledge, our present case report is the first to describe primary red ear syndrome triggered by orange juice. Clinical benefit derived from avoidance of this trigger, which is already known to precipitate migraines, gives some insight into the pathogenesis of red ear syndrome.
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Case Reports
Facial nerve compression by the posterior inferior cerebellar artery causing facial pain and swelling: a case report.
We report an unusual case of facial pain and swelling caused by compression of the facial and vestibulocochlear cranial nerves due to the tortuous course of a branch of the posterior inferior cerebellar artery. Although anterior inferior cerebellar artery compression has been well documented in the literature, compression caused by the posterior inferior cerebellar artery is rare. This case provided a diagnostic dilemma, requiring expertise from a number of specialties, and proved to be a learning point to clinicians from a variety of backgrounds. We describe the case in detail and discuss the differential diagnoses. ⋯ Hemifacial spasm is characterized by unilateral clonic twitching, although our patient presented with more unusual symptoms of pain and swelling. Onset of symptoms is mostly in middle age and women are more commonly affected. Differential diagnoses include trigeminal neuralgia, temporomandibular joint dysfunction, salivary gland pathology and migrainous headache. Botulinum toxin injection is recognized as an effective treatment option for primary hemifacial spasm. Microvascular decompression is a relatively safe procedure with a high success rate. Although a rare pathology, posterior inferior cerebellar artery compression causing facial pain, swelling and hearing loss should be considered as a differential diagnosis in similar cases.