Search

Gastric antral vascular ectasia (GAVE) is currently recognized as an important cause of gastrointestinal bleeding. Chronic kidney disease (CKD) is associated with a high incidence of GAVE. We report 3 patients with CKD who presented with severe anemia and were diagnosed with GAVE; they were resistant to endoscopic argon plasma coagulation. However, remission of anemia and improvement in GAVE lesions were observed after the initiation of hemodialysis. The pathogenesis of GAVE remains largely unknown, but mechanical stress of the antrum could play an important role. This stress may be reduced by hemodialysis through improvement of uremia-associated gastrointestinal symptoms. Therefore, the initiation of hemodialysis might be effective for intractable GAVE in CKD patients.

A rare case of subependymoma in a young patient presenting with sensory dysesthesia is reported. Computed tomography scan and magnetic resonance imaging revealed a posterior fossa mass occluding the fourth ventricle with infiltration to the right side immediately behind the pontine tegmentum and impinging on the right spinothalamic tract. Postoperative tumor histopathology revealed the classical appearance of subependymoma. Subependymoma is a rare, asymptomatic, slow-growing, low-grade glioma of the central nervous system. If symptomatic, the clinical features are commonly secondary to hydrocephalus, but subependymoma presenting with sensory dysesthesia has never been reported in the literature.

Postpartum cerebral angiopathy (PCA) is a cerebral vasoconstriction syndrome developing shortly after delivery, without signs of preceding eclampsia. The risk for recurrence of PCA is unknown. Here, we report on a closely monitored, uneventful pregnancy of a woman with a previous severe episode of PCA. In summary, this case report demonstrates that PCA does not necessarily recur in following pregnancies, even after previous severe episodes.

Spinal arterial vascularization is supplied by a large anastomotic net, making spinal ischemic events far less common than ischemic cerebral strokes. Polycythemia vera, due to blood hyperviscosity and activated platelet aggregation, is associated with a higher risk of arterial and venous thrombotic events. We report a patient with spinal cord transient ischemic attacks, a rarely presenting manifestation, and polycythemia vera, which highlights the thrombotic potential of this disease, and the requirement of exhaustive diagnostic workout of a spinal ischemic event.

Background: Progressive multifocal leukoencephalopathy (PML) is a demyelinating central nervous system disease caused by JC virus (JCV) reactivation in immunocompromised patients. The disease course of PML is often progressive, fatal and at present, there are few reports on successful treatment outcomes. Case Report: A 45-year-old man with systemic sarcoidosis presented with rapidly progressive dementia and right hemiparesis. The patient was diagnosed with PML as confirmed via brain biopsy and JCV PCR. With a combination treatment of cidofovir and mirtazapine, there was significant improvement of neurological symptoms without measurable functional deficit. Conclusion: This case suggests that dual therapy with cidofovir and mirtazapine might be an effective treatment option in PML patients with sarcoidosis.

We report the first case of Cremophor EL-induced cutaneous lupus erythematosus-like reaction in a 40-year-old female undergoing treatment for breast cancer. There have been four reported cases of paclitaxel- and four cases of docetaxel-induced cutaneous lupus reactions in the published literature [Dasanu and Alexandrescu: South Med J 2008;101:1161–1162; Adachi and Horikawa: J Dermatol 2007;34:473–476; Lortholary et al: Presse Med 2007;36:1207–1208; Chen et al: J Rheumatol 2004;31:818–820]. Our patient developed findings of a cutaneous lupus-like reaction with administration of paclitaxel which was subsequently discontinued. She was re-challenged with albumin-bound paclitaxel which has no Cremophor EL compound in its formulation. This administration of albumin-bound paclitaxel did not induce further reaction. She did not develop a cutaneous lupus erythematosus-like reaction with three other subsequent administrations of albumin-bound paclitaxel. The diagnosis of lupus-like reaction in our patient was made based on the development of a malar butterfly rash sparing the nasolabial folds, the appearance of this rash in context of recently receiving treatments with paclitaxel, resolution of the rash after discontinuing the paclitaxel, and the presence of autoimmune antibodies in the patient’s serum which resolved with discontinuation of the paclitaxel. This is the first case demonstrating that the cause of the cutaneous lupus erythematosus-like reaction is not likely due to the taxane component of paclitaxel but the chemical composition of Cremophor EL. If the chemotherapeutic agent was causing the reaction then the same reaction should be seen by albumin-bound paclitaxel. We propose that previously reported lupus reactions may actually be due to Cremophor EL, which consists of polyoxyethylated castor oil, and not the chemotherapeutic agent itself.

A 57-year-old man with a history of more than 10 years of bronchial asthma and chronic sinusitis complained of double vision which developed 18 days after cardiac tamponade with eosinophil-rich fluid (eosinophils 30%). He had oculomotor nerve palsy, and a blood test revealed eosinophilia (12,700/mm 3 ) and elevation of both C-reactive protein and rheumatoid factor. He was diagnosed as having Churg-Strauss syndrome. His symptoms were relieved by corticosteroid therapy. Our case and previous cases in the literature revealed that oculomotor nerve palsy in Churg-Strauss syndrome is associated with pupil involvement and may be relieved by corticosteroid treatment.

Signet ring cell carcinoma (SRCC) of the prostate is rare, with approximately 100 case reports to date. Here we report a very aggressive case of SRCC of the prostate in a Japanese man. The patient received estramustine, docetaxel, and carboplatin combination chemotherapy, followed by TS-1 and CPT-11 combination therapy. Unfortunately, the disease progressed, and he died of general metastatic disease treated over 16 month with systemic chemotherapy.

Acquired palsy of the lateral rectus presents with horizontal diplopia and has a broad differential. Herpes zoster ophthalmicus- (HZO) related cranial nerve palsy is a transient and self-limiting condition. Systemic antiviral treatment is administered in order to prevent sight-threatening complications. In suspected cases, zosteriform rash should be questioned. One should keep in mind that acquired esotropia in the elderly may sometimes present following HZO.

The clinical differential diagnosis between ischemic stroke and postictal deficit is sometimes challenging. If the clinical presentation is inconclusive, perfusion imaging can help to identify stroke patients for thrombolysis therapy. However, also epileptic phenomena may alter cerebral perfusion. Hypoperfusion spreading beyond the borders of cerebrovascular territories is usually considered suggestive of an etiology other than stroke. We present a patient whose clinical symptoms suggested a postictal deficit rather than an acute stroke. CT perfusion imaging showed hypoperfusion of the entire left cerebral hemisphere covering all vascular territories. CT angiography revealed occlusions in the ipsilateral internal carotid artery and in the circle of Willis as the cause of the global hypoperfusion. The patient was treated with i.v. thrombolysis and recovered with moderate disability. This is the first description of hyperacute ischemia of an entire cerebral hemisphere and its treatment with thrombolysis. It demonstrates the potential of modern neuroimaging in identifying atypically presenting strokes and shows that i.v. thrombolysis can be effectively and safely used to treat such potentially fatal insults.

Purpose: To report a case of tarantula hairs found in the cornea and discuss treatment. Case Report: A 16-year-old male presented with a 6-week history of right ocular irritation that began after letting his pet tarantula crawl on his face. Slit-lamp examination of the right eye revealed the presence of approximately 16 dark foreign bodies that had the appearance of small hairs. The foreign bodies were removed from the nasal region of the right cornea using Jewelers forceps, and the patient was prescribed a combination neomycin, polymyxin B, and dexamethasone ointment (Maxitrol®), given 4 times per day. Results: The patient presented for follow-up 2 weeks later, with resolution of symptoms. Conclusion: Effective treatment of keratitis caused by tarantula hairs includes taking a detailed history, conducting a careful slit-lamp examination, removal of any accessible hairs, and initiation of treatment with a topical steroid as determined by the clinical picture.

A 37-year-old woman presented to the Internal Medicine Clinic with complaints of abdominal pain and constipation which had begun 3 months earlier. A colonoscopy was performed, and wall thickening of the sigmoid colon was detected. A biopsy of the sigmoid colon revealed a poorly differentiated, mucin-producing adenocarcinoma with a signet-ring pattern. No distant metastasis was detected. The patient was treated with chemotherapy consisting of 5-fluorouracil, leucovorin, and oxaliplatin. One and a half years later, a painless mass, which was not fixed to the skin, measuring 1 cm in diameter, was found in the lower outer quadrant of the left breast. A core biopsy of the mass was performed, and a histopathological report confirmed metastasis to the breast from mucinous adenocarcinoma of an intestinal primary.

The association of unilateral trochlear nerve palsy with Claude Bernard-Horner syndrome represents a rare clinical condition. We present the case of a patient with this unusual presentation. The investigation performed implicated cerebrovascular disease as the underlying cause of the condition in this patient.

Intra-arterial (IA) chemotherapy for head and neck cancer is effective and multiple IA concurrent chemoradiation (CCRT) protocols have been reported. However, the role of IA CCRT in the multimodality treatment of head and neck cancer is still controversial. We have treated 5 cases of unresectable T4 maxillary sinus squamous cell carcinoma with IA cisplatin (CDDP) and docetaxel (DOC) and CCRT with oral S-1. We report our experience and the effectiveness and feasibility of this combination as an alternative choice of treatment for inoperable head and neck cancer. The patients received an IA infusion of CDDP (50-70 mg/m(2)) and DOC (50-60 mg/m(2)) through the femoral artery, followed by CCRT with oral S-1. The IA infusion was repeated up to 3 times and the radiation was dosed at up to 60-70 Gy. Complete response was achieved in 4 patients and partial response in one, giving an overall response rate of 100%. The most common grade 3 or 4 toxicities were anorexia (80%), mucositis (80%) and leukopenia (80%), all of which were manageable. CCRT with IA CDDP/DOC and oral S-1 was effective and tolerated. Although preliminary, the response rate encourages further pursuit and definitive evaluation of this combination for the treatment of inoperable advanced head and neck cancer.

The distribution of neuromyelitis optica (NMO)-characteristic brain lesions corresponds to sites of high aquaporin-4 (AQP4) expression, and the brainstem and hypothalamus lesions that express high levels of AQP4 protein are relatively characteristic of NMO. The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is one of the important causes of hyponatremia and results from an abnormal production or sustained secretion of antidiuretic hormone (ADH). SIADH has been associated with many clinical states or syndromes, and the hypothalamic-neurohypophyseal system regulates the feedback control system for ADH secretion. We report the case of a 63-year-old man with NMO, whose initial manifestation was hyponatremia caused by SIADH. Retrospective analysis revealed that the serum anti-AQP4 antibody was positive, and an MRI scan showed a unilateral lesion in the hypothalamus. SIADH recovered completely with regression of the hypothalamic lesion. As such, NMO should even be considered in patients who develop SIADH and have no optic nerve or spinal cord lesions but have MRI-documented hypothalamic lesions.

Imatinib mesylate, as treatment for gastrointestinal stromal tumors (GIST), has dramatically changed the prognosis for survival - not only because it is efficacious, but also because it attracted attention to this malignant disease. GIST is now a well-known disease entity and a paradigm for targeted therapies in malignant diseases. A now 74-year-old patient presented with recurrence of a primary duodenal GIST (initial diagnosis and primary resection in 1998; diameter 10 cm, KIT exon 11 mutation, PM V559D) and liver metastasis after a second surgical resection was performed in 2000. Conventional chemotherapy with adriamycin and ifosfamide failed to control growth of the relapsed tumor and liver metastasis. In July 2001, compassionate use of imatinib was started. Tumor regression was observed at continuous follow-ups (every 2 months for the first 6 months, and 6 months thereafter) and persisted until now. The patient's physical performance has remained in good condition. Side effects consisted of periorbital edema and sudden muscle cramps of toes and fingers, pain of bones and joints, an intentional tremor, a paler color of the skin, as well as a slight anemia. Imatinib is the first orally administered anticancer drug. Our case shows that a sustained response is possible with continuous therapy over a long time, if the drug is well tolerated. This implies a high compliance of the patient and suggests that resistance to imatinib does not have to develop. Exon 11 (point) mutation might not only represent a positive predictor for imatinib response in general, but especially for imatinib response on long-term. peerReviewed

The presence of isolated splenic metastasis in rectal carcinoma is uncommon and usually presents as an asymptomatic mass, noted incidentally on imaging. Splenectomy is usually performed with the goal of curing metastatic disease. It is unclear if adjuvant chemotherapy affords any benefit, and the prognosis is unknown. The case of a young woman is reported, in whom an isolated metastatic lesion in the spleen was discovered 9 months after adjuvant chemotherapy for stage III rectal adenocarcinoma. The patient has remained disease-free for nearly 5 years following splenectomy and chemotherapy. To our knowledge, this is the fourth reported case in the English literature of an isolated splenic metastatic lesion from rectal cancer. We discuss the unique presentation, the importance of post-treatment surveillance, and the implementation of multi-modality treatment strategies in this young patient.

A 28-year-old man with bilateral central serous chorioretinopathy (CSCR) and body weight gain was diagnosed with Cushing’s syndrome secondary to an adrenocortical adenoma. The patient had high levels of free cortisol and the tumor was confirmed by histopathology. After surgery, cortisol levels decreased and the CSCR spontaneously resolved. This case highlights the role of cortisol in the pathogenesis of CSCR. A diagnosis of endogenous Cushing’s syndrome should be considered in patients with CSCR as an initial symptom.

A 68-year-old man with a clinical diagnosis of Alzheimer's disease (AD) later developed amyotrophic lateral sclerosis (ALS), which was confirmed at autopsy at age 72 years. Because neuronal loss and AD-type pathologies (Braak stage II for neurofibrillary tangles) were scant, TDP-43-positive intracytoplasmic inclusions in hippocampal dentate granular cells and in neurons in the subiculum and amygdala, even though small in amount, may represent the earliest lesions of ALS-related dementia and could be the cause of dementia in this patient. Although the persistent elevation of creatine kinase from the onset could be a pointer to the presence of motor involvement, more accurate characterization of dementia, which may differentiate ALS-related dementia and AD, is necessary.

Ischemic stroke is a serious disease leading to significant morbidity and mortality. Multifocal and recurrent strokes are usually caused by embolic diseases, i.e. atrial fibrillation, but rare causes like cerebral vasculitis and clotting disorders are also well known. Here we report on two patients suffering from the very rare intravascular large B-cell lymphoma leading to multifocal and recurrent strokes in the brain and spinal cord as the prominent neurological symptom. The difficulties and the need for diagnostic brain biopsy in making an ‘in vivo’ diagnosis in this particular disease are outlined. Furthermore, the prerequisite for an interdisciplinary approach in these patients is strongly emphasized. Delayed diagnosis for several reasons was the most probable cause for cerebral relapse leading to death in one patient a few months after diagnosis. Conversely, early initiation of immunochemotherapy with a classical lymphoma schedule (R-CHOP) led to long-lasting remission of the disease in the other patient. With this report we like to improve alertness to intravascular large B-cell lymphoma as a cause for multifocal and recurrent strokes.

Bone involvement is uncommon in epithelial ovarian cancer. In this report, a case of bone metastases from ovarian cancer is described and the potential risk factors for such a rare occurrence are discussed. It is possible that such an event will become more common in the future as patients with advanced ovarian cancer experience prolonged survival but the cancer is not eliminated.