Lege Artis Medicinae

[Multiple ectopic tissue in the Meckel diverticulum]

ZARÁND Attila, MERSICH Tamás, BAJTAI Attila, JAKAB Ferenc

MAY 16, 2007

Lege Artis Medicinae - 2007;17(04-05)

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Lege Artis Medicinae

[MEGA (Management of Elevated cholesterol in the primary prevention Group of Adult japanese)]

MATOS Lajos

Lege Artis Medicinae

[An Exhibition Against Charlatanism in 1928]

KÖLNEI Lívia

Lege Artis Medicinae

[For the Beginner, the Advanced and the Fanatic Gábor Winkler: A Walk in the World of Opera, tomes I to IV]

SZÉKELY György

Lege Artis Medicinae

[SEVERE GASTROINTESTINAL AND RENAL MANIFESTATIONS OF HENOCH-SCHÖNLEIN PURPURA]

GECSE Krisztina, ONDRIK Zoltán, KAIZER László, VARGA Erika, LONOVICS János, CZAKÓ László

[INTRODUCTION - Henoch-Schönlein purpura is a systemic small vessel vasculitis characterized by vascular and/or mesangial IgA deposits, primarily affecting the vasculature of the skin, joints, kidneys and gastrointestinal tract. Gastrointestinal findings of various severity occur in 50 to 85% of the cases. We report on a 70-year-old woman who developed ileocaecal invagination and upper gastrointestinal haemorrhage as manifestations of Henoch-Schönlein purpura. CASE REPORT - The patient presented with two days history of palpable purpuric rash localized on the lower extremities. Based on the result of the skin biopsy, which showed leukocytoclastic vasculitis, IgA and C3 deposits, Henoch-Schönlein purpura was suspected. On the second night after admission colicky abdominal pain, vomiting and diarrhoea developed. Radiological examination showed an ileocaecal invagination and since symptoms deteriorated caecum resection and ileo-ascendestomy was performed. On the third postoperative day the patient became oligo-anuric, which was attributed to her Henoch-Schönlein disease, and systemic steroid pulse therapy was given. One month after the admission the patient experienced haematochezia. Emergency upper endoscopy revealed petechiae, haemorrhagic erosions and mucosal oedema, primarily in the descending part of the duodenum. Since these lesions were also considered as presentations of Henoch-Schönlein disease, another bolus of parenteral steroid was administered. Upper endoscopy repeated 3 days later showed remarkable improvement of the gastric and intestinal lesions. CONCLUSION - Gastrointestinal symptoms are common manifestations of Henoch-Schönlein purpura, thus their recognition, in which endoscopy plays a crucial role, is of major importance. Methylprednisolone pulse therapy is an effective therapeutic option not only in the management of severe renal or joint symptoms, but also in the treatment of gastrointestinal manifestations.]

Lege Artis Medicinae

[ERDHEIM-CHESTER DISEASE: A RARE FORM OF HISTIOCYTOSIS]

NÉMETH István, BOGÁTS Gábor, KAISER László, TISZLAVICZ László

[INTRODUCTION - Erdheim-Chester disease is rare non-Langerhans cell histiocytosis with multisystem involvement. The histological hallmark of the disease is CD1a negative histiocyte proliferation. CASE REPORT - Histiocytosis presented in a 57- year-old Caucasian man with exophtalmus, which was after two years followed by progressive pericardial infiltration and effusion leading to cardiac compression and consequent functional disturbance. It was the histological analysis of a surgical sample removed during pericardial fenestration that revealed Erdheim- Chester disease. The patient died from multisystem involvement one year after the initial diagnosis. Postmortem examination showed long bone, orbital cavity and cranial cavity involvement as well as extensive retroperitoneal and mediastinal xanthogranulomatous infiltration. CONCLUSIONS - The clinical diagnosis of Erdheim-Chester disease is difficult because the symptoms are insidious and non-specific. The histological report is often only descriptive. The prognosis of the disease is poor due to the lack of targeted therapy and to the advanced stage at diagnosis.]

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Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

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Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

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Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Clinical Neuroscience

Extraskeletal, intradural, non-metastatic Ewing’s sarcoma. Case report

OTTÓFFY Gábor, KOMÁROMY Hedvig

Intracranial localization of Ewing’s sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing’s sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re­gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima­ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.

Clinical Neuroscience

Isolated hypoglossal nerve palsy due to a jugular foramen schwannoma

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Clinical Neuroscience

Investigation of risk factors, topographic location and stroke mechanisms of unilateral isolated and posterior cerebral ARTERY thalamic infarcts

GÖKCAL Elif, SENGUL Yildizhan, USLU Ilgen Ferda

Aim - In this study, we aimed to examine the risk factors, topographic features and stroke mechanisms of acute ischemic unilateral infarcts of thalamus. Methods - Patient with isolated thalamic infarct and those with posterior cerebral artery (PCA) infarction who were admitted to our hospital between January 2014 and January 2017 with acute unilateral thalamic infarction (TI) were included in this study (isolated thalamic infarction/ isolated TI; thalamic and posterior cerebral artery infarction/PCA+TI). Demographic characteristics and vascular risk factors of the patients were determined. Thalamic infarct areas were recorded topographically as anterior, posteromedial, ventrolateral, posterolateral, more than one area, and variant areas. Stroke mechanism was determined according to the criteria of „Trial of Org 10172 in Acute Stroke Treatment” (TOAST). Patients with isolated TI and PCA TI were compared according to risk factors, stroke mechanism and infarct topography. Results - Forty-three patients with a mean age of 63.3 ± 14.5 years were included in the study. Twenty-eight patients (60.1%) were found to have isolated TI and the remaining 15 patients (34.9%) had PCA+TI. 32.1% of patients with isolated TI had sensory symptoms on presentation, and 60% of patients with PCA-TI had sensorimotor symptoms. The mean age, the mean score on National Institutes of Health Stroke Scale (NIHSS) and the mean frequency of atrial fibrillation were higher in PCA+TI patients than in isolated-TI patients (p: 0.04, p: 0.004, p: 0.02 respectively). 32.6% of the patients had ventrolateral, 30.2% had posteromedial involvement. Ventrolateral topography was seen in 46.7% of the PCA+TI patients, while posteromedial topography was seen in 39.3% of the isolated-TI patients. 53.6% of the isolated-TI had small vessel disease etiology, while 40% of the PCA+TI had cardioembolic etiology, and the other 40% had large artery atherosclerosis. Conclusion - Our study showed that the most ommon stroke mechanism in patients with thalamic infarction is the small vessel disease. Isolated TI and PCA+TI patients differ in terms of etiologic mechanism and infarct topography. Variant territorial involvement and multiple area involvements can be quite common in thalamic infarcts.