Thermal imaging and dermoscopy for detecting inflammation in frontal fibrosing alopecia.

BACKGROUND: Frontal fibrosing alopecia (FFA) is an inflammatory condition of the scalp, which leads to scarring and slowly progressive recession of frontotemporal and/or frontoparietal hairline. Choice of FFA treatment is highly dependent on accurate assessment of disease phase, as medical treatments are effective only during the initial inflammatory stage. METHODS: To objectively quantify the activity of the inflammatory process in FFA, 22 female patients were examined by both infrared thermography and optical dermoscopy before tissue sampling. The presence of perifollicular erythema or scaling was considered to be strongly suggestive for appropriate scalp biopsy site. Skin temperature differences in dermoscopy-selected area >0.5°C for temperature of the reference area (lower area of forehead) were considered abnormal. For evaluation of the inflammatory infiltrate, a grading scale was used. RESULTS: The scale classified 14 (64%) subjects as being in the active disease phase of FFA and eight (36%) as in the inactive one. Using the thermography findings, 17 (77%) patients were considered to have the active FFA and five (23%) patients had the inactive one. The clinical assessment of active FFA was most accurate using thermal imaging combined with dermoscopy, with a sensitivity of 64%, a specificity of 88%, a positive predictive value of 90%, and an negative predictive value of 58%. CONCLUSION: We believe that infrared thermography is a noninvasive and accessible imaging modality that may serve as a complementary tool in FFA diagnosis.

Vertebral hemangioma coincident with metastasis of colon adenocarcinoma.

The authors report on colon cancer metastasis to the L-3 vertebra, which had been previously found to be involved by an asymptomatic hemangioma. A 61-year-old female patient was admitted after onset of lumbar axial pain and weakness of the right quadriceps muscle. Her medical history included colon cancer that had been diagnosed 3 years earlier and was treated via a right hemicolectomy followed by chemotherapy. Presurgical imaging revealed an asymptomatic hemangioma in the L-3 vertebral body. Computed tomography and MRI of the spine were performed after admission and revealed a hemangioma in the L-3 vertebral body as well as a soft-tissue mass protruding from the L-3 vertebral body to the spinal canal. Treatment consisted of vertebroplasty of the hemangioma, left L-3 hemilaminectomy, and removal of the pathological mass from the spinal canal and the L-3 vertebral body. Histopathological examination revealed the presence of colon cancer metastasis and a hemangioma in the same vertebra.

Atypical dissemination of lung cancer to the adrenal gland and to the spleen.

Metastases of lung cancer to such organs as the liver, bones or to the central nervous system appear to be a frequent complication of this disease. At the same time, metastases to the adrenal gland are found less frequently. Metastases of lung cancer to the spleen are a great rarity and they are described sporadically. Our report presents a unique case of left lung cancer with simultaneous metastases to the adrenal gland and to the spleen. All the presented lesions were diagnosed by ultrasound guided biopsy and confirmed by histopathological examination. The patient received combined chemoradiotherapy. She was closely monitored over an 18-month observation period following treatment. No new metastases were reported.

Epidermal growth factor receptor gene expression in high grade gliomas.

BACKGROUND: Epidermal growth factor receptor (EGFR) gene amplification and protein expression in malignant gliomas (anaplastic astrocytoma, AA and glioblastoma, GBL) were suggested to be correlated with the degree of malignancy. Large deletions within the EGFR gene occur frequently in glioma patients. The aim of our study was to analyse EGFR gene expression by real-time PCR by three different amplicons located across the gene and relate it to the age of patients and EGFR mutation status. MATERIAL AND METHODS: We analysed EGFR gene expression in 75 patients, median age 58 years (range 28-75), 52% of glioblastomas, 39% of anaplastic astrocytomas and 9% of low grade gliomas. EGFR expression was measured by real-time PCR, three amplicons located at exons 2-3, 13-14, and 17-18 junctions were analysed, gene expression was normalized by 18S RNA expression. EGFRvIII deletion was detected by RT-PCR. RESULTS: EGFR was found to be expressed in 61.8% of brain gliomas, with strongly positive expression in 12.2% of them. We simultaneously analysed by RT-PCR the EGFRvIII status and found the deletion in 21.3% of tumours. In our group EGFRvIII mutation was significantly more frequent in patients older than 50 years of age (48.6%) than in younger patients (23.5%, p < 0.05). When only GBL patients were assessed, none of the patients younger than 50 years of age had EGFRvIII mutation, whereas in the older subgroup they constituted 36.67% of subjects. We observed that younger patients (below 50 yrs) had slightly lower EGFR expression in comparison to older patients, but this difference was not statistically significant. CONCLUSIONS: As nearly 1/3 of high grade gliomas do not demonstrate abnormal gene expression levels, EGFR status should be taken into account in any targeted therapy attempt. The significance of EGFR axis-related differences between young and old glioma patients and their impact on the prognosis warrant further study.

Rare primary tumours of the hypothalamus in adults: clinical course and surgical treatment.

BACKGROUND AND PURPOSE: The paper presents the operative technique and the results of treatment of adult patients with primary tumours of the hypothalamus, including rare ones. The aim of the study was to show the possibility of safe surgical treatment of rare tumours of the hypothalamus through a bifrontal basal interhemispheric trans-lamina terminalis approach. MATERIAL AND METHODS: Five patients with tumours of the hypothalamus were operated on in the Neurosurgical Clinic in Sosnowiec between 1990 and 2008. There were 2 patients with craniopharyngiomas located exclusively in the third ventricle, and single patients with gemistocytic astrocytoma, Langerhans cell histiocytosis X and hamartoma of the hypothalamus each. The patients were treated surgically with a bi-frontal basal interhemispheric trans-lamina terminalis approach. In two cases, the neuronavigation system with the use of tractography (DTI) was used to determine the location of the lamina terminalis, the posterior surface of the optic chiasm and the optic tracts. RESULTS: All lesions were resected totally, except for partially resected hamartoma of the hypothalamus. The most common postoperative complication was diabetes insipidus, which was transient in two cases. A long-lasting follow-up of all the patients operated on did not reveal regrowth of the lesion. CONCLUSIONS: The bifrontal basal interhemispheric trans-lamina terminalis approach allows for radical resection of primary tumours of the hypothalamus while avoiding serious post-operative deficits. This approach enabled the preservation of the olfactory bulb and tract and prevented damage of the frontal lobes. The use of DTI helped to establish the location and borders of the lamina terminalis, to establish the posterior surface of the optic chiasm and the optic tracts, and to save the anterior and lateral wall of the hypothalamus.

Primary pituitary lymphoma.

Primary CNS lymphoma (PCNSL) is now thought to constitute 3% of all intracranial neoplasms. PCNSL occurrence in the sella turcica region is an extremely rare finding. We present a 37-year-old male with primary pituitary lymphoma treated in our department. The patient, who had had no previous illnesses, was admitted to the hospital because of bilateral blurred vision. Findings on physical examination were normal except for temporal parts of field of vision deficit. No abnormalities were found in his bilateral ocular movement, facial sensory function or motor function. His blood count and biochemical profile were normal. Basic hormonal studies revealed no symptoms of panhypopituitarism. MRI demonstrated a large intrasellar mass with supra- and parasellar extension. MRS revealed decrease in NAA/tCr proportion and increase in Cho/NAA and Cho/tCr proportions. Endoscopic surgery was performed using the transsphenoidal approach. Histopathological examination demonstrated a large B-cell lymphoma. The patient received 6 cycles of CHOP chemotherapy. He was also irradiated with 6 MV photons to the whole brain to a total dose of 40 Gy and then there was a boost to the tumour to a total dose of 50 Gy. Next he was reoperated on with the fronto-temporo-sphenoidal craniotomy approach and subtotal resection of the tumour was performed. After the treatment the visual disturbances significantly decreased. Control MRI revealed a stable remnant of the tumour. Nowadays the patient has 52 months' follow-up and he has only a stable, slight visual field deficit on the upper temporal side of the right eye.

Idiopathic hypertrophic pachymeningitis - case report and literature review.

Idiopathic hypertrophic pachymeningitis (IHPM) is a rare pathological state, with still unclear aetiopathogenesis. We present a case of a 63-year-old woman with cranial variety of that disease. The manifestations of the disease included headaches, paresis of VI, IX, X nerves and cerebellar ataxia. The disease was diagnosed with magnetic resonance imaging (MRI) and histopathological assessment of the pachymeninx biopsy specimen. The MRI revealed significant thickening of the cranial base pachymeninx, compressing the pons and medulla oblongata. MRI examinations could be misinterpreted as extensive meningioma of the skull base. Dura mater biopsy revealed however inflammation with abundant lymphocytic infiltrations. Clinical improvement was obtained after the application of corticosteroids. We noted the subsidence of all symptoms of the disease, as well as radiological improvement, manifested through substantial regression of the described changes in the pachymeninx. The patient has been presented in the context of 65 cases of idiopathic hypertrophic pachymeningitis, described in the literature of English-speaking countries in the last five years. Recently, the importance of the autoimmunogenic background of IHPM has been underlined. In that respect IHPM has become an interdisciplinary problem. Its diagnosis and treatment requires not only radiologists, neurologists, pathomorphologists and neurosurgeons, but also specialists in internal medicine, including immunologists, allergologists and rheumatologists as well - in other words, physicians that rarely take part in the processes of diagnosing and treating intracranial pathologies.

Atypical features of dementia in a patient with Creutzfeldt-Jakob disease.

BACKGROUND: This article describes a Polish patient (female, right-handed, age 68 at onset) diagnosed with the Heidenhain variant of Creutzfeldt-Jakob Disease (HvCJD), characterized clinically by isolated visual disturbances with no ocular dysfunction prior to the development of myoclonus and other symptoms of CJD. CASE REPORT: Nothing in the history pointed to iatrogenic or acquired CJD, and genetic testing ruled out familial CJD. The neuroradiological picture (MRI) showed non-specific features of cerebral atrophy (cortical and subcortical). An EEG revealed periodic triphasic sharp waves, particularly in the occipital lobes, and myoclonus occurring synchronically with generalized periodic epileptiform discharges. Comprehensive neuropsychological testing documented rapidly progressive dementia, with dysgraphia and aphasia deteriorating to organic mutism. Post-mortem neuropathological examination confirmed spongiform encephalopathy, especially in occipital cortex, with amyloid plaques but without neurofibrillary tangles. CONCLUSIONS: Over the crucial 6-week period the patient went from "Mild Cognitive Impairment" to a status resembling the final stages of Alzheimer's disease, without any evidence of a CVA. The only aspect of this case that does not fit the usual criteria for the Heidenhain variant is the fact that the patient survived over a year in a persistent vegetative state. Ophthalmologists and family physicians should be aware of the possibility of HvCJD in any patient over 60 presenting with otherwise inexplicable visual disturbances in the absence of significant ocular pathology, even when other symptoms of dementia may not be immediately noticeable.

Use of monoclonal anti-EGFR antibody in the radioimmunotherapy of malignant gliomas in the context of EGFR expression in grade III and IV tumors.

We investigated the putative benefits of simultaneous teleradiotherapy and anti-epidermal growth factor receptor (EGFR) 125I monoclonal antibody (MAb) 425 radioimmunotherapy, when applied after neurosurgery in high-grade gliomas, over teleradiotherapy alone. In comparison to previous studies which have reported good results with this type of radioimmunotherapy, we advanced the adjuvant radioimmunotherapy step, that is, gave it during, not after, teleradiotherapy. The randomized prospective study examined two groups: simultaneous postoperative teleradiotherapy and radioimmunotherapy (TRT + RIT; eight patients) versus teleradiotherapy alone (TRT; 10 patients). Patients who after primary operation of grade III (6 cases) or IV glioma (12 cases), showed no or less than 2 mL of remnant tumor on post-operative magnetic resonance (MR) study and were not treated postoperatively by chemotherapy were enrolled and randomized. Anti-EGFR 125IMAb 425 RIT was started during week 4 of radiotherapy, not later than 8 weeks after neurosurgery, and was repeated three times at 1-week intervals. Total activity given was 5026 + 739 MBq/patient. The tolerance of TRT was good. No immediate side effects of concomitant anti-EGRF 125I RIT were observed. Observation showed a median total survival (as evaluated from the primary neurosurgical treatment) of 14 months (range 3.5-28 months). There was no improvement in disease-free or total survival in the group of patients treated by TRT + RIT after neurosurgery. In addition, an immunohistochemical analysis of EGFR expression in gliomas was performed in a group of 100 cases and was distinctly positive in 50% grade IV gliomas and 68% grade III gliomas. We conclude that simultaneous radiotherapy and radioimmunotherapy with anti-EGFR 125I-MAb 425 is not beneficial over radiotherapy alone in adjuvant treatment of high-grade gliomas after neurosurgery. We also recommend individual confirmation of EGFR expression in further anti-EGFR radioimmunotherapy trials.

The cerebral form of toxocarosis in a seven-year-old patient.

INTRODUCTION: Toxocarosis is a consequence of human infection by Toxocara canis larvae. There are symptomatic (visceral, ocular) and asymptomatic courses of toxocarosis. The cerebral form is very rare. CASE REPORT: We present a seven-year-old patient who developed a cerebral form of toxocarosis. She demonstrated focal neurological symptoms (epilepsy) confirmed by neuro-imaging and histopathological examinations. A positive test for toxocarosis essentially completed the other outcomes. On the basis of the clinical picture and the conducted tests a diagnosis of a cerebral form of toxocarosis was established. Mebendazole was applied in treatment.

Prevention of painful neuromas by oblique transection of peripheral nerves.

OBJECT: Neuroma formation often occurs at the proximal stump of the transected nerve, complicating the healing process after gap injuries or nerve biopsies. Most such neuromas cause therapy-resistant neuropathic pain. The purpose of this study was to determine whether oblique transection of the proximal stump of the sciatic nerve can prevent neuroma formation. METHODS: The sciatic nerves of 10 rats were transected unilaterally at an angle of 30 degrees, and the peripheral segments of the nerves were removed. In 10 control animals the sciatic nerves were transected at a perpendicular angle. Twenty weeks after surgery the nerves were reexposed and collected. The presence of neuromas was determined by two board certified pathologists on the basis of histopathological evaluations. CONCLUSIONS: The oblique transection of peripheral nerves, contrary to perpendicularly transected nerves, is rarely followed by classic neuroma development. Moreover, neuropathic pain is significantly reduced compared with that following the traditional method of nerve transection.

Paraganglioma of the lumbar spinal canal -- case report.

We present the case of a 46-year-old female with a lumbar spine paraganglioma. The patient complained of a right-sided lumboischialgia. Preoperative MRI revealed an intradural tumour at the vertebra L3 level, located in the midline, 7 mm in diameter. The tumour was totally removed by laminectomy. Histopathological examination showed nests of chief cells (zellballen), surrounded by reticulin fibres. Immunohistochemistry showed a positive reaction for chromogranin A, neuronal specific protein, synaptophysin and cytokeratin in the chief cells. The sustentacular cells displayed immunopositivity for S-100 protein, single cells were also positive for GFAP. We found no proliferative activity in the tumour cells (Ki-67 index = 0%). In the two years follow-up the patient remains without clinical or radiological signs of recurrence. Spinal paraganglioma is a rare, surgically curable tumour with low proliferative potential. This entity should be taken into consideration in the differential diagnosis of extramedullary spinal lesions.

Chronic calcified subdural empyema occurring 46 years after surgery.

The authors present a case of a 47-year-old female in whom there was diagnosed a chronic calcified subdural empyema 46 years after the removal of an acute subdural empyema resulting from complications after otitis media. The patient had suffered from grand mal convulsions and partial epileptic seizures occurring 3-4 times a month. A large frontotemporoparietal craniotomy was carried out and the subdural empyema filled with numerous brownish-black, uncharacteristic tissue fragments together with the partially calcified and ossified capsule was removed. The empyema mass was found to be sterile for bacteria. After the operation, mental disability symptoms began to withdraw and the number of epileptic seizures decreased.

Cell proliferative activity estimated by histone H2B mRNA level correlates with cytogenetic damage induced by radiation in human glioblastoma cell lines.

We studied the relationship between proliferative activity and radiation-induced DNA damage in human malignant gliomas in vitro. Nine human glioblastoma established cell lines were gamma-irradiated (60Co) over a dose range of 0-10 Gy. H2B and H4 histone mRNA level was assessed with quantitative RT-PCR technique (TaqMan) and histone labeling index (HLI) with in situ hybridization to define proliferation rate, while cytochalasin-block micronucleus assay was performed to measure cytogenetic damage. Micronucleus frequency correlated with H2B mRNA level (Spearman's R up to 0.82 at 8 Gy), HLI, nuclear division index (NDI) and percentage of binucleated cells (%BNC). There was a high correlation between H2B mRNA level and NDI (R = 0.80) as well as %BNC and HLI (R = 0.72). Histone H2B and H4 mRNA level (not significant), HLI, NDI, and %BNC (significant) were higher in cell lines sensitive to DNA damage. Proliferative activity correlates with radiation-induced DNA damage in human glioma cell lines. Histone H2B mRNA level and HLI may be a useful molecular predictor of the tumour response to radiation treatment in gliomas of the same histological grade, however the risk of potentially more rapid tumour-cell repopulation must be considered. Presumed protective activity of histones against radiation-induced DNA damage was not confirmed at the transcript level.

[Surgical treatment of brainstem gliomas and other tumors in adults]. / Leczenie chirurgiczne glejaków i innych guzów pnia mózgu u doroslych.

BACKGROUND AND PURPOSE: After the introduction of MR imaging to the diagnostics of brainstem tumors and after the introduction of microsurgical procedures to their treatment, the successful treatment, particularly of focal and exophytic forms of these tumors has begun all over the world. The objective of this paper is to establish indications for surgical treatment of gliomas and other tumors of brainstem, to determine surgical approaches and to establish the outcome. MATERIAL AND METHODS: Within the last 6 years, 12 patients with focal and exophytic tumors of the brainstem in adults were operated on. 5 tumors were located in the midbrain, 4 in the pons, 2 in the medulla oblongata and 1 was a cervicomedullary one. With one exception, all tumors were approached via the posterior fossa. RESULTS: The total removal was performed in 75% and a subtotal one in 25% of cases. A serious complication in 2 patients was bilateral ptosis after the removal of midbrain tumors. This lesion gradually regressed. A similar dangerous complication is impaired swallowing and coughing reflex loss which, by exposing the patient to the risk of aspiration pneumonia, occurred in 2 patients. One of these patients died after the operation. CONCLUSIONS: Patients with focal and exophytic forms of the brainstem tumors in MR imaging are qualified for surgical treatment. Immediate results of the treatment depend on the localization and size of the neoplasm. A precise operative approach, use of the neurophysiological examination and postoperative care at the Intensive Care Unit are essential conditions to obtain good results of the operative treatment.

[An attempt to use Gore-Tex surgical membrane in lumbar disc surgery]. / Próba zastosowania blony przeciwzrostowej Gore-Tex w operacjach dyskopatii ledzwiowych.

BACKGROUND: Peridural fibrosis developing after lumbar discectomy may be responsible for as much as 20% of all Failed Back Surgery Syndrome. A variety of biological and non-biological materials have been used as a barrier to invasion of fibrous tissue into the vertebral canal. AIM: The purpose of this study was to evaluate the use of expanded polytetrafluoroethylene (ePTFE) surgical membrane (Gore-Tex membrane) to inhibit peridural fibrosis and reduce FBSS symptoms after lumbar discectomy. MATERIAL AND METHODS: In a prospective study we compared postoperative results in 20 patients who had an ePTFE membrane implanted during lumbar discectomy with the results in 20 patients in whom no material was implanted. The outcomes were evaluated using a questionnaire on activities of daily living according to the Low Back Outcome Score, pain grading scale -- Visual Analog Scale, assessment of Lasegue sign and MRI 18-24 months after the operation for all patients. RESULTS: The authors found no evident positive clinical and radiological effects of using ePTFE surgical membrane during lumbar discectomy. CONCLUSIONS: 1. It is impossible to prove that ePTFE membrane used during lumbar discectomy essentially prevents postoperative peridural scar formation. 2. The use of ePTFE membrane does not improve the outcome of the surgical treatment of lumbar disc herniation.

Non-neoplastic, degenerative brain pathologies and fibroses diagnosed on the basis of ultra-small samples obtained by stereotactic biopsy.

Brain biopsy and other stereotactic procedures have evolved over the last decades. Recently, the morbidity and mortality decreased radically along with an increase in the number of successful histopathological diagnoses. Therefore, applications of appropriate treatments in neoplastic brain pathologies are now possible, especially of those located in deep regions. Stereotactic biopsy may also be used as a diagnostic method followed by appropriate management in conditions where a non-neoplastic pathology is suspected. Between December 2000 and February 2004, we performed 116 stereotactic procedures based on the system of stereotactic planning and Brain-Lab treatment, which was equipped with automatic CT/MR image fusion software. In this report, we have focused on 10 cases of non-neoplastic brain pathologies diagnosed on the basis of ultra-small samples obtained from stereotactic biopsy. Among them there were 4 cases of gliosis, 3 cases of brain degenerative disorders, 2 cases of hippocampal fibroses, and 1 case of normal brain tissue. We have presented all these cases in detail by discussing their histology, clinical manifestations, localisation, management and follow-up.

Mutations in the von hippel-lindau tumour suppressor gene in central nervous system hemangioblastomas.

Central nervous system hemangioblastomas (cHAB) are rare tumours which most commonly arise in the cerebellum. Most tumours are sporadic, but as many as one third of cHABs occur in the course of the hereditary disorder - von Hippel-Lindau disease (VHL). In order to diagnose new VHL families in Poland we performed sequencing of the entire VHL gene in archival material (paraffin embedded hemangioblastoma tissues) in a large series of 203 unselected patients with cHAB. VHL gene mutations were detected in 70 (41%) of 171 tumour samples from which DNA of relatively good quality was isolated. We were able to obtain blood samples from 19 of mutation positive cases. Eight (42%) of these harboured germline mutations in persons from distinct undiagnosed VHL families.