Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis--a review.

PURPOSE: The aim of the study was to evaluate frequency and clinical relevance of haemorrhagic events associated with primary angiitis of the central nervous system in childhood (cPACNS), a rare but increasingly recognized disease with varying clinical presentations. METHOD: A systematic literature review from 1990 onwards was conducted to identify reported cases of cPACNS. RESULTS: A total of 110 paediatric patients met the inclusion criteria. The median age was 9.5 years. Seven children (7/110, 6.4 %) demonstrated cerebral haemorrhage. Death occurred only in children with cerebral haemorrhage (4/110, 3.6 %); both a sudden and prolonged course of disease was observed. CONCLUSION: PACNS is a rare disease and the occurrence of haemorrhage with this condition is even rarer; however, the risk of an unfavourable outcome under these circumstances seems to be increased. PACNS adds to the list of differential diagnoses of intracerebral haemorrhage in the paediatric population.

Blood pressure measurements in patients with Takayasu arteritis: a work of caution.

Takayasu arteritis is a large vessel vasculitis among young women that affects the aorta and its branches. Disease-related subclavian or brachial obstructions can lead to hypotensive brachial blood pressure values. By contrast, arterial hypertension is also frequent in this disease, possibly unrecognized in case of solitary brachial blood pressure measurement. We present the case of a 28-year-old woman with cerebral hypoperfusion related to Takayasu arteritis. Despite "pseudo-hypotensive" brachial blood pressure values of 70 mm Hg, Doppler measurement of the ankle revealed a systolic pressure of 220 mm Hg. In Takayasu vasculitis, additional ankle pressure measurement may represent the true cardiac load and blood pressure.

Quantitative analysis of cyclooxygenase 2 in the posterior longitudinal ligament of cervical spondylotic myelopathy.

BACKGROUND: Cervical spondylotic myelopathy (CSM), in part, results from degeneration of the posterior longitudinal ligament (PLL), which mechanically compresses the spinal cord. Much research was done on the ossification of PLL, but not concerning the non-ossifying degeneration of cervical PLL. The degeneration of cervical PLL may be related to inflammation. The aim of this study was to elucidate the pathological features of the PLL and the role of cyclooxygenase 2 (COX-2) in the degeneration of the PLL in CSM. METHODS: A total of 23 PLL specimens were collected during surgery from patients with CSM for the histological and immunohistochemical (type II collagen and Ki-67) study. For the control group 14 cervical PLL autopsy specimens were investigated in the same manner. mRNA expression of COX-2 was quantitatively measured by real-time reverse transcription-polymerase chain reaction (RT-PCR) from 18 PLL specimens of patients with CSM and 18 PLL specimens of autopsy cases. Immunohistochemistry was used to evaluate the cellular location of COX-2 in PLL. RESULTS: A distinct amount of fibrotic area, chondrometaplastic tissue and calcification were found in the PLL of the patient group, compared with the control group. Type II collagen was apparent around chondrometaplastic cells. Ki-67 positive reaction was less than 5%. A COX-2 positive reaction was found in 9 of the patient specimens (39.1%) in which the COX-2 was released from vascular endothelial cells in the PLL. However, such reactions were not found in the control group. Real-time PCR showed that the mRNA expression level of COX-2 in the patient group was significantly higher than that in the control group (P < 0.01). CONCLUSIONS: Chondrometaplastic tissue producing type II collagen was identified as the most predominant pathological feature in the degenerative PLL. The higher expression of COX-2 might be related to degeneration of the PLL in CSM.

Myeloperoxidase and carbonyl proteins: promising markers for non-invasive monitoring of graft rejection after heart transplantation.

BACKGROUND: After heart transplantation (HTx), endomyocardial biopsy (EMB) is currently the standard method to diagnose acute graft rejection. A non-invasive marker of rejection would be desirable as an alternative or to permit more selective use of the costly and invasive EMB. METHODS: In this retrospective study, outcomes of routinely taken EMBs were used to select 28 patients after HTx EMB Grade 0R (8 patients), 1R (9 patients) or 2R (11 patients). For these patients, myeloperoxidase (MPO) and carbonyl proteins (CP) in serum were measured using enzyme-linked immunoassay (ELISA). RESULTS: MPO and CP levels in post-HTx patients with Grade 2R rejection were significantly (MPO: p < 0.01; CP: p < 0.001) elevated at the time of rejection compared with levels 1 month earlier. MPO and CP levels predicted Grade 2R rejection and the best cut-off point was 237.5 µg/l for MPO and 222.5 pmol/mg for CP, respectively. Clinically most important was the marked increase (doubling of basic values within 1 month) of MPO and CP levels in cases of Grade 2R rejection in post-HTx patients. CONCLUSIONS: MPO and CP seem to be appropriate parameters to monitor rejection events non-invasively and to minimize the application of EMBs after HTx.

The Austrian Brain Tumour Registry: a cooperative way to establish a population-based brain tumour registry.

In Austria, registration of malignant brain tumours is legally mandatory, whereas benign and borderline tumours are not reported. The Austrian Brain Tumour Registry (ABTR) was initiated under the auspices of the Austrian Society of Neuropathology for the registration of malignant and non-malignant brain tumours. All Austrian neuropathology units involved in brain tumour diagnostics contribute data on primary brain tumours. Non-microscopically verified cases are added by the Austrian National Cancer Registry to ensure a population-based dataset. In 2005, we registered a total of 1,688 newly diagnosed primary brain tumours in a population of 8.2 million inhabitants with an overall age-adjusted incidence rate of 18.1/100,000 person-years. Non-malignant cases constituted 866 cases (51.3%). The incidence rate was higher in females (18.6/100,000) as compared to males (17.8/100,000), while 95/1,688 (5.6%) cases were diagnosed in children (<18 years). The most common histology was meningioma (n = 504, 29.9%) followed by glioblastoma (n = 340, 20.1%) and pituitary adenoma (n = 151, 8.9%). Comparison with the Central Brain Tumor Registry of the United States (CBTRUS) database showed high congruency of findings. The ABTR model led by neuropathologists in collaboration with epidemiologists and the Austrian National Cancer Registry presents a cooperative way to establish a population-based brain tumour registry with high quality data. This setting links cancer registration to the mission of medical practice and research as defined by the World Medical Association in the Declaration of Helsinki. The continued operation of ABTR will aid in monitoring changes in incidence and in identifying regional disease clusters or geographic variations in brain tumour morbidity/mortality.

Epstein-Barr virus encephalitis presenting with a tumor-like lesion in an immunosuppressed transplant recipient.

Epstein-Barr virus (EBV) associated central nervous system (CNS) infection is a rare disease. We report an atypical manifestation of EBV encephalitis initially presenting with a tumor-like lesion of the optic tract in an immunocompromised patient 8 years after a combined kidney and pancreas transplantation had been performed. Polymerase chain reaction (PCR) in the cerebrospinal fluid (CSF) and antibody testing confirmed the diagnosis of EBV encephalitis, most likely as a consequence of a reactivated persistent EBV infection. After cessation of the immunosuppressive therapy and induction of treatment with ganciclovir, clinical and magnetic resonance imaging (MRI) findings rapidly improved.

Subacute cutaneous lupus erythematosus and interstitial myositis occurring with hepatitis B infection: response to antiviral therapy with lamivudine.

Lupus erythematosus (LE) is a multisystem inflammatory disease resulting from an altered T- and B-cell response. Among various pathogenic factors, viral infections have been implicated in LE exacerbation. We describe a patient with acute onset of subacute cutaneous lupus erythematosus (SCLE) and interstitial myositis with mitochondrial changes in the context of an associated hepatitis B infection and the response of myositis to antiviral treatment with lamivudine. Viral vectors may play an important role in LE. A hitherto undescribed myositis with mitochondrial changes was associated with hepatitis B infection and SCLE and improved with antiviral treatment.

Lymphocytic hypophysitis in the pediatric population.

BACKGROUND: Lymphocytic hypophysitis (LYH) is a rare inflammatory disease of the pituitary gland that usually affects women in their ante- or immediate postpartum period; males are affected less frequently than females. An autoimmune pathogenesis is suggested. Symptoms comprise anterior and/or posterior pituitary insufficiency of varying degrees. So far, specific characteristics of this rare disease in childhood are not well described. CASE HISTORY: We report the case of a 12-year-and-11-month-old boy with histologically confirmed LYH clinically presenting with diabetes insipidus. A high-dose steroid therapy was administered, in which the therapeutic effect was uncertain. His 6-year follow-up is presented. DISCUSSION: The literature is reviewed for children presenting with LYH; their characteristics, pituitary involvement, and clinical follow-up are listed and discussed. CONCLUSION: Even though magnetic resonance imaging can be highly suspicious for LYH, only surgical exploration can confirm the diagnosis. The efficacy of medical treatment is still controversial; a close follow-up is necessary to control and correct the endocrinological function, if required.

Lymph node metastases arising from uveal melanoma.

Since the eye lacks lymphatic vessels, uveal melanomas primarily metastasize hematogenously. Here we report the case of a patient with ciliary body ring melanoma who developed lymph node metastases after a fistulating glaucoma operation. A 40-year-old female Caucasian patient presented with unilateral pigment dispersion. Pigment dispersion glaucoma was diagnosed and since the intraocular pressure could not be managed with topical medication, transscleral cyclophotocoagulation and two trabeculectomies had to be performed. Due to enlargement of the pigmented iris mass and cell deposits in the chamber angle, a ciliary body ring melanoma was presumed and the eye enucleated. Histology confirmed the diagnosis of "ciliary body ring melanoma". Six months after enucleation the patient presented multiple metastases including ipsilateral preauricular and submandibular lymph node metastases. The patient died two months later. Lymph node metastases arising from ciliary body melanomas are very rare. Tumor seeding through the trabeculectomy site into the bleb and then via conjunctival lymphatic vessels might be the crucial factor for this pathway of metastases. Therefore, in cases of unilateral pigment dispersion, malignancy should be excluded before fistulating operations are performed.

Tendon-to-bone healing of a quadrupled hamstring tendon graft fixed with biodegradable screws in an immature athlete: a radiologic, arthroscopic, histologic, and electromicroscopic investigation.

Abstract Quadrupled hamstring tendon is one of the most commonly used ligamentous substitutes in anterior cruciate ligament reconstruction. We describe a case of a young athlete who sustained a rerupture of a semitendinosus-gracilis autograft fixed with biodegradable screws 2 years after primary surgery. Radiologic, arthroscopic, histologic, and electromicroscopic investigation showed tendon-to-bone healing of the soft tissue graft and partial degradation of the interference screws.

Cytokeratin typing as an aid in the differential diagnosis of primary versus metastatic lung carcinomas, and comparison with normal lung.

Due to more efficient chemotherapy protocols, the number of second and even third primary carcinomas is steadily increasing. To denominate the possible origin of a carcinoma, different markers are available as an aid, e.g. hormones, proteins and lipoproteins, secretion products and cytoskeletal proteins. Cytokeratins (CKs) have gained new popularity; however, they have not been extensively evaluated in lung tumours. In our study we evaluated the staining patterns of CK polypeptides 4-8, 10, 13, 14, and 17-20 and high molecular weight (HMW) CK polypeptides in routinely processed primary lung carcinomas and lung metastases of diverse origin. As expected, immunohistochemical investigation gave no clear-cut results, but, with statistical analysis, lung adenocarcinomas could be separated from metastatic adenocarcinomas using CK 5 and 18 and HMW CK (specificity 92.5%, sensitivity 62.5%). The different origin of the metastases could often be detected using CK 18 and CK 20. Lung clear cell carcinomas and large cell carcinomas with clear cell areas could be distinguished from metastatic renal clear cell carcinomas by the CK 7 staining reaction. Squamous cell carcinomas of the lung and metastatic squamous cell carcinomas of the larynx, pharynx and oesophagus could not reliably be separated in part due to the few number of cases available. CK polypeptide typing is thus an additional aid in the differential diagnosis of lung carcinomas versus carcinomas metastatic to the lung.