Posterior reversible encephalopathy syndrome is a presentation which is diagnosed clinico-radiologically. The primary aetiological processes leading to posterior reversible encephalopathy syndrome are many, which include autoimmune conditions. Polyarteritis nodosa as an aetiological factor for posterior reversible encephalopathy syndrome is rare. We present a case of polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome.
A 26-year-old South-Asian female presented with left sided focal seizures with secondary generalization and visual disturbance for 2 days duration. She had a prior history of arthralgia and weight loss with no medically explainable cause for young onset hypertension. Examination revealed a right claw hand with a palpable vasculitic type of rash involving both the palmar surfaces. Symptoms responded to management with anti-hypertensives and anti-epileptics. Whole blood count, iron studies, erythrocyte sedimentation rate and C-reactive protein values portrayed an ongoing chronic inflammatory process. Serological studies such as Anti-nuclear antibody, Anti -double stranded deoxyribonucleic acid, Anti-neutrophil cytoplasmic antibody and Anti-cyclic citrulinated peptide were negative. Magnetic resonance imaging revealed high signal intensity on T2 in both occipital lobes. Skin biopsy of the palm revealed moderate vessel vasculitis. Renal imaging revealed structurally abnormal kidneys with micro aneurysms in the right renal vasculature. Repeat magnetic resonance imaging of the brain two months later showed marked improvement. A diagnosis of polyarteritis nodosa with posterior reversible encephalopathy syndrome was made.
Posterior reversible encephalopathy syndrome should not be missed. Investigations for an aetio-pathological cause should be considered including the rarer associations like polyarteritis nodosa.
Polyarteritis nodosa; Posterior reversible encephalopathy syndrome; Vasculitis
The electrocardiogram (ECG) is useful in the diagnosis of cardiac and non-cardiac conditions. Rigors due to shivering can cause electrocardiogram artifacts mimicking various cardiac rhythm abnormalities.
We describe an 80-year-old Sri Lankan man with an abnormal electrocardiogram mimicking narrow complex tachycardia during the immediate post-operative period. Electrocardiogram changes caused by muscle tremor during rigors could mimic a narrow complex tachycardia.
Identification of muscle tremor as a cause of electrocardiogram artifact can avoid unnecessary pharmacological and non-pharmacological intervention to prevent arrhythmias.
Arrhythmia; Supra ventricular tachycardia; Rigors
Traumatic dislocation of the interphalangeal of the fifth toe is an unusual foot injury.
We report the case of a 47-year-old woman who sustained a minor foot injury for more than 30 years, resulting in chronic, irreducible dislocation of the proximal interphalangeal joint of the fifth toe. The affected proximal interphalangeal joint was accessed via a dorsal incision over the unstable interphalangeal joint. It was found that the interposed interphalangeal joint capsule and attenuated lateral collateral ligament were reconstructed, and it was stabilized by temporary insertion of a Kirschner wire. The affected joint was found to be stable, well-positioned and pain-free at the 12-month post-surgical check-up.
This unusual presentation of a chronic joint dislocation responded favorably to open reduction, soft tissue reconstruction and restabilization of the affected joint. It is suggested that this approach will provide a good and functional outcome even in cases of very long-standing joint injury.
Interphalangeal joint dislocation; Irreducible; Injury; Foot; Toe
Prostate-specific antigen (PSA) is a widely used specific tumor marker for prostate cancer. We experienced a case of metastatic prostate cancer that was difficult to detect by repeat prostate biopsy despite a markedly elevated serum PSA level.
A 64-year-old man was referred to our hospital with lumbar back pain and an elevated serum PSA level of 2036 ng/mL. Computed tomography, bone scintigraphy, and magnetic resonance imaging showed systemic lymph node and osteoblastic bone metastases. Digital rectal examination revealed a small, soft prostate without nodules. Ten-core transrectal prostate biopsy yielded negative results. Androgen deprivation therapy (ADT) was started because of the patient’s severe symptoms. Twelve-core repeat transrectal prostate biopsy performed 2 months later, and transurethral resection biopsy performed 5 months later, both yielded negative results. The patient refused further cancer screening because ADT effectively relieved his symptoms. His PSA level initially decreased to 4.8 ng/mL, but he developed castration-resistant prostate cancer 7 months after starting ADT. He died 21 months after the initial prostate biopsy from disseminated intravascular coagulation.
CUP remains a considerable challenge in clinical oncology. Biopsies of metastatic lesions and multimodal approaches were helpful in this case.
Metastatic prostate cancer; Negative prostate biopsy; Elevated PSA level
Autoimmune thrombocytopenic purpura is an immunological disorder characterized by increased platelet destruction due to presence of anti-platelet autoantibodies. Hepatitis C virus infection, which is one of the most common chronic viral infections worldwide, may cause secondary chronic immune thrombocytopenic purpura.
We report a case of a 51-year-old Caucasian female with hepatitis C virus infection who developed a severe, reversible, immune thrombocytopenia. Platelet count was as low as 56.000/mm3, hepatitis C virus serology was positive, serum glutamic oxaloacetic transaminase, serum glutamic pyruvic transaminase and gamma-glutamyltransferase serum levels were elevated. Disorders potentially associated with autoimmune thrombocytopenic purpura were ruled out. A corticosteroid treatment was started and led to an increase in platelet count. The patient was then treated with pegylated-interferon alpha 2a and ribavirin. After four weeks of treatment hepatitis C virus - ribonucleic acid became undetectable and steroid treatment was discontinued. Six months of antiviral therapy achieved a sustained biochemical and virological response together with persistence of normal platelet count.
In our case report hepatitis C virus seemed to play a pathogenic role in autoimmune thrombocytopenic purpura. Moreover, the successful response (negative hepatitis C virus - ribonucleic acid) to tapered steroids and antiviral therapy was useful to revert thrombocytopenia.
HCV; Autoimmune thrombocytopenic purpura; Antiviral therapy; Steroids
We report an interesting case of asymptomatic retinal involvement in an encephalopathic patient enabling early identification of Susac’s syndrome.
A 39-year-old Caucasian lady with hearing loss and encephalopathy was referred for ophthalmic assessment, including screening for branch retinal artery occlusions characteristic of Susac’s syndrome. Clinical features included severe headaches, right-sided hypoacusis, dysphasia and poor memory. Routine blood tests were normal. MRI brain showed numerous hyperintense lesions mainly in corpus callosum. Although she was visually asymptomatic, dilated funduscopy detected bilateral multiple peripheral branch retinal artery occlusions which were confirmed on fluorescein angiography. She was subsequently started on intravenous steroids and pulsed cyclophosphamide which improved her symptoms within 48 hours. Full recovery was made with no new arterial occlusions on four months follow-up.
The case further establishes the crucial role of a detailed ophthalmic examination supported by fluorescein angiography in the assessment of these patients, who are at risk of being misdiagnosed and undertreated.
Susac’s syndrome (SS); Retinopathy; Fluorescein angiography
Pulmonary thromboembolism after upper extremity operation is rare. We report a patient with thromboembolism after debridement open reduction and internal fixation for bilateral open distal radius fractures.
The Japanese patient was an 80-year-old previously healthy female who was able to walk on her own. She fell down and was taken to our hospital. She was diagnosed with bilateral open distal radius fractures and we performed debridement open reduction and internal fixation on the same day. Although she could not walk and was depressed, she was discharged on the ninth postoperative day. However, on the eleventh postoperative day, she returned to our emergency department with complaints of dyspnea and cold sweat. Her serum D-dimer level was 19.0 μg/dl, troponin T was positive, and urgent contrast computed tomography scan of her thorax revealed thrombosis in the bilateral main pulmonary artery. She was diagnosed with pulmonary thromboembolism and admitted to our hospital again. On the second admission, although she had breathing problems, she did not require a respirator. Oxygen was supplied as well as anticoagulants. On the seventh day after being diagnosed with embolism, thrombosis in the bilateral main pulmonary arteries had disappeared.
The patient did not have any “strong” risk factors as reported in the Japanese Orthopedic Association Clinical Practice Guideline on the Prevention of Venous Thromboembolism in Patients Undergoing Orthopedic Treatments. In general, upper extremity operation carries a low risk for pulmonary thromboembolism. For patients with decreased activity of daily living and depression, we should consider postponing discharge and performing rehabilitation until activity of daily living is improved.
Distal radius fracture; Depression; Pulmonary thromboembolism; Activity of daily living
Few studies have focused on eukaryote community in the human gut. Here, the diversity of microeukaryotes in the gut microbiota of an anorexic patient was investigated using molecular and culture approaches.
A 21-year-old Caucasian woman was admitted in an intensive care unit for severe malnutrition in anorexia nervosa. One stool specimen was collected from the anorexic patient, culture and polymerase chain reaction-based explorations yielded a restricted diversity of fungi but four microeukaryotes Tetratrichomonas sp., Aspergillus ruber, Penicillium solitum and Cladosporium bruhnei previously undescribed in the human gut.
Establishing microeukaryote repertoire in gut microbiota contributes to the understanding of its role in human health.
Anorexia nervosa; Gut; Microeukaryotes; Polymerase chain reaction; Culture
The diagnosis of prosthetic valve endocarditis is challenging. The gold standard for prosthetic valve endocarditis diagnosis is trans-esophageal echocardiography. However, trans-esophageal echocardiography may result in negative findings or yield images difficult to differentiate from thrombus in patients with prosthetic valve endocarditis. Combined computed tomography and fluorodeoxyglucose positron emission tomography is a potentially promising diagnostic tool for several infectious conditions and it has also been employed in patients with prosthetic valve endocarditis but data are still scant.
We reviewed the charts of 6 patients with prosthetic aortic valves evaluated for suspicion of prosthetic valve endocarditis, at two different hospital, over a 3-year period. We found 3 patients with early-onset PVE cases and blood cultures yielding Pseudomonas aeruginosa, Staphylococcus epidermidis and Staphylococcus lugdunensis, respectively; and 3 late-onset cases in the remaining 3 patients with isolation in the blood of Streptococcus bovis, Candida albicans and P. aeruginosa, respectively. Initial trans-esophageal echocardiography was negative in all the patients, while fluorodeoxyglucose positron emission tomography showed images suspicious for prosthetic valve endocarditis. In 4 out of 6 patients valve replacement was done with histology confirming the prosthetic valve endocarditis diagnosis. After an adequate course of antibiotic therapy fluorodeoxyglucose positron emission tomography showed resolution of prosthetic valve endocarditis in all the patients.
Our experience confirms the potential role of fluoroseoxyglucose positron emission tomography in the diagnosis and follow-up of prosthetic valve endocarditis.
FDG-PET; Prosthetic valve endocarditis; Diagnosis; Duke’s criteria
Growth arrest lines can develop within the skeleton after physiological stress or trauma. They are usually evident on radiographs as transverse lines in the metaphyses and have been used in fields from palaeontology to orthopaedics. This report consists of three cases, two of which describe growth arrest lines in an intra-epiphyseal site hitherto rarely documented, and a third demonstrating their clinical application.
Case 1 describes a 9-year-old who suffered a knee hyperflexion injury requiring anterior cruciate ligament and posterior cruciate ligament reattachments. She subsequently developed a marked distal femoral intra-epiphyseal arrest silhouette, as well as metaphyseal arrest lines in the femur, tibia and fibula. Case 2 describes an 8-year-old who sustained a tibial spine fracture and underwent open reduction and internal fixation. Subsequent imaging shows a further example of femoral intra-epiphyseal arrest silhouette as well as tibia and fibula metaphyseal arrest lines. Case 3 describes a 10-year-old who sustained a distal tibia fracture which was managed with open reduction and internal fixation. Subsequently the metaphyseal growth arrest line was parallel to the physis, suggesting no growth arrest (a danger with such a fracture).
This case series describes two examples of rarely described intra-epiphyseal growth arrest silhouettes and demonstrates the usefulness of arrest lines when assessing for growth plate damage.
Growth arrest lines; Orthopaedics; Physis
Renal arteriovenous fistula (RAVF) is a comparatively rare malformation. Here, we report a case of ruptured RAVF that was successfully treated by catheter embolization.
An 89-year-old female was transferred to our institution with massive gross hematuria in March 2011. Plain abdominal computed tomography (CT) revealed dilated left renal pelvis with high-density contents. Hematoma was suspected. Subsequent plain abdominal magnetic resonance imaging revealed left hydronephrosis and blood retention in the dilated left renal pelvis. No renal or ureteral cancer was evident. Hematuria was conservatively treated using hemostatic agents but hematuria persisted. Repeated urinary cytology revealed no malignant cells. On day 9, the patient went into septic and/or hemorrhagic shock. Fluid and catecholamine infusion, blood transfusion, and antibacterial drugs were rapidly initiated, and the patient’s general condition gradually improved. Contrast-enhanced abdominal CT revealed marked expansion of the hematoma in the renal pelvis and microaneurysms in the segmental arteries of the left kidney. Inflammation improved, and a left double-J stent was inserted. Selective renal angiography revealed RAVF with microaneurysms in the left segmental arteries; therefore, catheter embolization using metallic coils was performed, which resolved hematuria.
We report a case of ruptured renal arteriovenous malformation, which was successfully treated by catheter embolization.
Renal arteriovenous malformation; Catheter embolization
Although most reports describing patients infected with methicillin-resistant Staphylococcus aureus enterocolitis have been published in Japan, this concept remains a matter of debate and diagnostic criteria have not yet been defined.
The general status of a 74-year-old Japanese man referred to our hospital (day 1) with severe community-acquired pneumococcal pneumonia gradually improved with antibiotic therapy. Thereafter, up to 4 L/day of acute watery diarrhea that started on day 19 was refractory to metronidazole but responded immediately to oral vancomycin. Gram staining stool samples was positive for abundant fecal leukocytes from which dominant methicillin-resistant Staphylococcus aureus (104 CFU/mL) were isolated, suggesting methicillin-resistant Staphylococcus aureus enterocolitis. High fever with methicillin-resistant Staphylococcus aureus bacteremia was evident at day 30, and suppurative right hip arthritis developed around day 71. All methicillin-resistant Staphylococcus aureus strains isolated from stools, blood and aspirated synovial fluid separated in the same manner on pulsed-field gel electrophoresis, as well as two other strains isolated from sputum, belonged to the same clone as sequence type (ST) 764 (complex clonal 5), and carried SCCmec type II.
The clinical, microbiological and molecular biological findings of this patient indicated methicillin-resistant Staphylococcus aureus enterocolitis that led to septic methicillin-resistant Staphylococcus aureus arthritis.
Methicillin-resistant Staphylococcus aureus; Fecal leukocytes; Multilocus sequence typing
Accidental intravenous administration of an enteral feeding can be fatal or cause complications such as sepsis, acute respiratory and circulatory failure, acute renal failure, hepatic insufficiency, coagulation disorders and severe permanent neurological sequelae. These “wrong route” errors are possible due to compatible connections between enteral feeding systems and intravascular infusion catheters.
We report a six-week-old male infant who received a 5 ml intravenous infusion of breast milk. Within five minutes of administration the child developed tachycardia and tachypnea, accompanied by a sudden decrease in oxygen saturation on pulse oximetry to 69%. The infant received supplemental oxygen via nasal cannula and was transferred to the pediatric intensive care unit. Broad-spectrum antibiotics were administered for 48 hours. Vital signs returned to normal within a few hours. Neurological follow-up through 3 years did not reveal any neurodevelopmental abnormalities.
Development of specific enteral feeding connections, which are incompatible with intravascular catheter connections, is needed urgently to prevent a misconnection with potential morbidity or mortality of children.
Woman milk; Intravenous administering; Misconnection; Enteral feeding systems
Fabry disease is an X-linked inherited metabolic condition where the deficit of the α-galactosidase A enzyme, encoded by the GLA gene, leads to glycosphingolipid storage, mainly globotriaosylceramide. To date, more than 600 mutations have been identified in human GLA gene that are responsible for FD, including missense and nonsense mutations, small and large deletions. Such mutations are usually inherited, and cases of de novo onset occur rarely.
In this article we report an interesting case of a 44-year-old male patient suffering from a severe form of Fabry disease, with negative family history. The patient showed signs such as cornea verticillata, angiokeratomas, cardiac and neurological manifestations, an end-stage renal disease and he had low α-galactosidase A activity. We detected, in this subject, the mutation c.493 G > C in the third exon of the GLA gene which causes the amino acid substitution D165H in the protein. This mutation affects the amino acid - belonging to the group of buried residues - involved, probably, in the preservation of the protein folding. Moreover, studies of multiple sequence alignment indicate that this amino acid is highly conserved, thus strengthening the hypothesis that it is a key amino acid to the enzyme functionality.
The study of the relatives of the patient showed that, surprisingly, none of the members of his family of origin had this genetic alteration, suggesting a de novo mutation. Only his 11-year-old daughter - showing acroparaesthesias and heat intolerance with reduced enzymatic activity - had the same mutation.
We suggest that a non-inherited mutation of the α-galactosidase A gene is responsible for Fabry disease in the patient who had reduced enzyme activity and classical clinical manifestations of the disease. In a family, it is rare to find only one Fabry disease affected subject with a de novo mutation. These findings emphasize the importance of early diagnosis, genetic counselling, studying the genealogical tree of the patients and starting enzyme replacement therapy to prevent irreversible vital organ damage that occurs during the course of the disease.
Fabry disease; α-galactosidase A; GLA gene; D165H mutation; De novo mutation
Meningitis is an uncommon complication of an untreated pituitary macroadenoma. Meningitis may occur in patients with macroadenomas who have undergone transsphenoidal surgery and radiotherapy and is usually associated with rhinorrhea. Less commonly, cerebrospinal fluid rhinorrhea has been reported as a complication of treatment of prolactinomas by dopamine agonists. Cerebrospinal fluid rhinorrhea in cases of untreated pituitary macroadenoma is reported only in isolated cases. Acute bacterial meningitis without rhinorrhea in patients with an untreated pituitary macroadenoma is an exceptional finding with only three previously reported cases.
A 31-year-old male was urgently admitted for headache, fever and visual loss. Neuroimaging disclosed an invasive pituitary lesion. Cerebrospinal fluid leakage was not clinically detected. Lumbar puncture showed acute meningitis. Blood tests revealed increased inflammatory markers, a serum prolactin of 9000 ng/ml (2.5-11 ng/ml) and panhypopituitarism. Intravenous antibiotics and hydrocortisone replacement therapy were initiated, leading to a favorable clinical outcome. An endoscopic transsphenoidal debulking procedure was performed, it showed that the sphenoid floor was destroyed and the sinus occluded by a massive tumor.
Meningitis should be ruled out in patients with a pituitary mass who present with headache and increased inflammatory tests, even in the absence of rhinorrhea.
Macroprolactinoma; Meningitis; Pituitary apoplexy; Rhinorrhea
Rampant caries is an advanced and severe dental disease that affects multiple teeth. This case describes the management of rampant caries in a young teenager suffering from chronic oral graft versus host disease after allogeneic bone marrow transplantation.
A 14-year-old Chinese boy suffering from β–thalassemia major was referred to the dental clinic for the management of rampant dental caries. An oral examination revealed pale conjunctiva, bruising of lips, and depapillation of tongue indicating an underlying condition of anemia. The poor oral condition due to topical and systemic immunosuppressants was seriously aggravated, and rampant caries developed rapidly, affecting all newly erupted, permanent teeth. The teeth were hypersensitive and halitosis was apparent. Strategies for oral health education and diet modification were given to the patient. Xylitol chewing gum was used to stimulate saliva flow to promote remineralization of teeth. Silver diamine fluoride was topically applied to arrest rampant caries and to relieve pain from hypersensitivity. Carious teeth with pulpal involvement were endodontically treated. Stainless steel crowns were provided on molars to restore chewing function, and polycarbonate crowns were placed on premolars, upper canines and incisors.
This case report demonstrates success in treating a young teenager with severe rampant dental decay by contemporary caries control and preventive strategy.
Silver diamine fluoride; Rampant caries; Graft versus host disease
Mucus-producing tumours of the appendix or mucoceles can, if left untreated, lead to dissemination of its contents into the peritoneal cavity causing substantial morbidity to the patient. Symptoms for complicated mucoceles can mimic those of acute appendicitis and the final diagnosis is most likely made intraoperatively. We here present a case that is, to our knowledge, one of only ten described in the literature and the first to characterize torsion of an appendiceal mucocele with abdominal magnetic resonance imaging.
The patient, a 34-year-old Caucasian female presented at the emergency department with acute abdominal pain in the right lower quadrant. Initial diagnostic work-up including ultrasonography and abdominal magnetic resonance imaging showed a large tubular mass at the base of the appendix with indirect signs of torsion. A laparoscopic appendectomy was performed the following day where the finding was confirmed. The patient went on to have an uneventful recovery and was discharged from the hospital on the first postoperative day.
Magnetic resonance imaging is a useful tool in identifying unknown lesions of the appendix and should be considered the primary imaging modality in especially younger patients requiring diagnostic imaging. In this case the preoperative imaging findings aided in choosing the correct timing and treatment option for the patient.
Appendix; Mucocele; Torsion; Ultrasonography; Magnetic resonance imaging; Appendectomy; Laparoscopy
Intraspinal neurocysticercosis is an uncommon manifestation that may present as an isolated lesion. Furthermore, acute hydrocephalus caused by isolated intraspinal neurocysticercosis without concomitant cerebral involvement is extremely rare.
A 64-year-old man presented with a history of severe headache, an unsteady gait, and occasional urinary incontinence. Magnetic resonance imaging of the thoraco-lumbar spine revealed multiple, cystic, contrast-enhancing intraspinal lesions. A computed tomographic scan of the brain showed marked ventricular dilatation but no intraparenchymal lesions or intraventricular cysticercal lesions. This case of acute hydrocephalus was found to be caused by isolated intraspinal neurocysticercosis and was treated by ventriculoperitoneal shunt placement and surgical removal of the intraspinal lesions (which were histologically confirmed as neurocysticercosis), followed by administration of dexamethasone and albendazole.
Isolated spinal neurocysticercosis should be considered in the differential diagnosis of acute hydrocephalus when no explanation is found in the brain, particularly in geographical regions endemic for cysticercosis.
Spinal; Neurocysticercosis; Hydrocephalus
Autoimmune hypophysitis very rarely spreads to nearby organs outside the pituitary tissue, for unknown reasons, with only 5 reported cases of hypophysitis spreading over the cavernous sinus.
Three patients presented with cases of non-infectious hypophysitis spreading outside the pituitary tissue over the cavernous sinus. All three cases were diagnosed with histological confirmation by transsphenoidal surgery, and the patients showed remarkable improvement with postoperative pulse dose steroid therapy, including disappearance of abnormal signal intensities in the bilateral hypothalami on magnetic resonance imaging, resolution of severe stenosis of the internal carotid artery, and normalization of swollen pituitary tissues. Two of 3 cases fulfilled the histological criteria of immunoglobulin G4-related disease, although none of the patients had high serum immunoglobulin G4 level.
The true implications of such unusual spreading of hypophysitis to nearby organs are not fully understood, but the mechanism of occurrence might vary according to the timing of inflammation in this unusual mode of spreading. Pulse dose steroid therapy achieved remarkably good outcomes even in the patient with progressive severe stenosis of the internal carotid artery and rapid visual deterioration.
Hypophysitis; Hypothalamus; Immunoglobulin G4-related disease; Severe internal carotid artery stenosis; Steroid pulse therapy
Cervicomediastinal lymph node carcinoma with an unknown primary site is quite rare, and useful treatment of these diseases has not been established. We report here the case of a patient successfully treated with TS-1 alone after the relapse of cervicomediastinal lymph node carcinoma with an unknown primary site.
A 62-year-old man was referred to our hospital because of cervicomediastinal lymph node swelling and high serum levels of carbohydrate antigen 19-9 and carcinoembryonic antigen. Fluorodeoxyglucose-positron emission tomography/computed tomography revealed an accumulation of fluorodeoxyglucose in the left supraclavicular lymph nodes, mediastinal lymph nodes, and the pelvic cavity. Colonoscopy revealed rectal cancer, which was diagnosed by biopsy as a tubular adenocarcinoma. Because metastases from rectal cancer to the cervicomediastinal lymph nodes are rare, the patient underwent thoracoscopic mediastinal lymphadenectomy. A biopsy specimen from the paraaortic lymph nodes demonstrated papillary adenocarcinoma that was pathologically different from the rectal cancer; therefore, a diagnosis of mediastinal carcinoma with an unknown primary site was established. The patient underwent low anterior resection of the rectum for the rectal cancer, and no abdominal lymph node metastasis (pMP, N0/stage I) was found. Although radiotherapy was performed for the cervicomediastinal lymph nodes, the mediastinal carcinoma relapsed after 6 months. Because the patient desired oral chemotherapy on an outpatient basis, TS-1 was administered at a dosage of 80 mg/day for 2 weeks, followed by a 1-week rest. TS-1 treatment resulted in a decrease in the size of the cervicomediastinal lymph nodes, and the serum tumor marker levels decreased to normal after the fourth course. The patient continued TS-1 treatment without adverse events and is currently alive without recurrence or identification of the primary site at the 32nd month after TS-1 treatment.
This is the first reported case of relapsed cervicomediastinal lymph node carcinoma with an unknown primary site treated by TS-1 alone. TS-1 treatment for the carcinoma with an unknown primary site may be useful in patients who are not candidates for systemic platinum-based chemotherapy.
Mediastinal lymph node; Carcinoma with an unknown primary site; TS-1; Chemotherapy
Patients infected with the human immunodeficiency virus (HIV) are at risk of developing malignancies and have an increased susceptibility to infection. HIV-associated Burkitt lymphoma (BL) is relatively rare in developed countries, but remains prevalent in developing counties and is sometimes compounded by the fact that patients may be unaware that they are HIV-positive.
A 37-year-old Japanese man was referred to our department for diagnosis and management of submandibular swelling. He was unaware that he was HIV-positive at the initial visit. Here, we describe our diagnostic approach, in which we used hematological and immunological investigations, biopsy, fluorescence-activated cell sorting and fluorescence in situ hybridization to confirm the diagnosis of HIV-associated BL. The patient has no risk factors for HIV infection, and the source of infection remains unclear.
In this case, submandibular swelling was the first clinical sign of pathology and the patient’s HIV-positive status only became evident later. It is highly likely that BL was triggered by HIV infection.
Burkitt lymphoma; HIV; Submandibular swelling
Clivus fractures are highly uncommon. The classification by Corradino et al. divides the different lesions in longitudinal, transverse and oblique fractures. Longitudinal types are associated with the highest mortality rate between 67 – 80%. Clivus fractures are often found after high velocity trauma, especially traffic accidents and falls. The risk of neurologic lesions is high, because of the anatomic proximity to neurovascular structures like the brainstem, the vertebrobasilar artery, and the cranial nerves. Longitudinal clivus fractures have a special risk of causing entrapment of the basilar artery and thus ischemia of the brainstem.
This lesion in our patient was a combination-fracture of the craniocervical junction with a transverse clivus fracture. In this case, the primary closed reduction of the clivus fracture and the immobilization with a halo device was the therapy of choice and led to consolidation of the fracture.
Therapy advices and examples in the literature are scarce. We present a patient with a clivus fracture, who could be well treated by a halo device. Through detailed research of the literature a therapy algorithm has been developed.
Clivus fracture; Craniocervical junction; Halo device
Borreliosis is a widely distributed disease. Neuroborreliosis may present with unspecific symptoms and signs and often remains difficult to diagnose in patients with central nervous system symptoms, particularly if the pathognomonic erythema chronica migrans does not develop or is missed. Thus, vigilance is mandatory in cases with atypical presentation of the disease and with potentially severe consequences if not recognized early. We present a patient with neuroborreliosis demonstrating brain stem and vestibular nerve abnormalities on magnetic resonance imaging.
A 28-year-old Caucasian female presented with headaches, neck stiffness, weight loss, nausea, tremor, and gait disturbance. Magnetic resonance imaging showed T2-weighted hyperintense signal alterations in the pons and in the vestibular nerves as well as bilateral post-contrast enhancement of the vestibular nerves. Serologic testing of the cerebrospinal fluid revealed the diagnosis of neuroborreliosis.
Patients infected with neuroborreliosis may present with unspecific neurologic symptoms and magnetic resonance imaging as a noninvasive imaging tool showing signal abnormalities in the brain stem and nerve root enhancement may help in establishing the diagnosis.
Borreliosis; Brain stem; MRI; Neuroborreliosis; Vestibular nerve enhancement
Membranous nephropathy is one of the most common causes of nephrotic syndrome in adults. In contrast, acquired factor V inhibitor is a rare bleeding disorder.
A 62-year-old Asian man with a history of cerebral hemorrhage, purpura, eosinophilia and hyper immunoglobulin E syndrome developed proteinuria. The bleeding disorder was diagnosed with acquired factor V inhibitors. A renal biopsy revealed that he suffered from membranous nephropathy with glomerular endothelial damage which is reported to be involved in another factor disorder. After the steroid administration, the coagulation test and proteinuria were improved.
The presence of factor V inhibitors may have been involved in the development of membranous nephropathy.
Cancerous cells are known to metastasize to different ocular structures. This happens especially to the choroid in males with lung cancer and females with breast cancer. However, we observed two cases of cancerous metastasis to the optic canal region. Both cases showed only a progressive decrease in vision without any other remarkable ophthalmological symptoms or abnormalities in the affected eye.
Two females, a 60-year-old and a 73-year-old, came to our hospital because of progressive loss of vision. These patients showed no remarkable symptoms or signs in their eyes except visual acuity loss. Several ophthalmoscopic examinations, such as slit lamp microscopy and fundoscopy, showed no abnormal changes in their affected eye but magnetic resonance imaging indicated a massive legion around the optic nerve.
It is possible for cancer to metastasize to the optic canal region and the existence of primary tumors should be considered.
Cancer; Metastasis; Optic canal