Search results

Clinical Neuroscience

SEPTEMBER 30, 2017

[Current diagnosis and treatment of idiopathic intracranial hypertension]

SALOMVÁRY Bernadett, PÁNCZÉL Gyula, MARKIA Balázs, NAGY Gábor

[Background - Idiopathic intracranial hypertension is cha-racterized by raised intracranial pressure of unknown origin, leading to persisting visual loss if left untreated. Purpose - We assessed timing of surgery, and the efficacy and safety of ventriculo-peritoneal shunt. Methods - Retrospective analysis of 65 patients treated at our Neuro-ophthalmology Clinic between 2009 and 2017. Patients - We treated 15 children and 50 adults, 42 patients conservatively, and 23 surgically. The median age at presentation was 27 years for adults, 88% were obese, and 86% female. The age of children was 5-17 years, 40% were obese, and 53% girl. The commonest presentation symptom was headache in both groups (64%), followed by obscuration (33%), and double vision (22-31%). Subjective visual loss was only experienced in the surgical group (50%). The time until diagnosis was 2 weeks in both groups. However, the conservative group presented to our institute significantly earlier (3 weeks), than the surgical group (8 weeks). The follow-up time was 25 months. Results - In the conservative group papilla edema was 2D, visual acuity ≥0.7, and visual field loss was only mild. Time to cure was 3 months. In the surgical group both preoperative papilla edema (3D), and visual function were significantly worse. Indications for surgery were papilla edema, deteriorating visual function or relapse resistant to conservative treatment. Papilla edema disappeared 3 months after surgery, and visual field deficit improved significantly. We detected significant improvement in all aspects of visual function even at first neuro-ophthalmic control 4 days after surgery. However, visual acuity only improved in cases of preoperative acuity ≥0.3. Shunt revision occurred in 17%, and shunt infection in 8.5%. One patient suffered from persistent visual deterioration after surgery, and asymptomatic complication (epidural hematoma) was found in another patient. There was no surgical mortality. Conclusions - This is a curable condition with early diagnosis and adequate treatment, and persistent visual loss can be prevented. Surgery is effective and safe, close neuro-ophthalmic monitoring is mandatory for its optimal timing. Visual function of all patients can be preserved when operated on in time, whereas severe visual loss appears to be irreversible despite surgery.]

Lege Artis Medicinae

FEBRUARY 15, 2015

[Inflammatory myofibroblastic tumor of abdominal wall arising from uterine myoma]


[INTRODUCTION - The inflammatory pseudotumor appears in various organs. It is well circumscribed, unencapsulated proliferation which simulates malignant tumor clinically as well as radiologically and morphologically. It’s etiology is unknown. The histology prooves inflammatory origin of this process: the nodular structure of the tumor-like mass is composed of fibroblasts, myofibroblasts, plasma cells and lympho­cytes. Recurrence may happen. CASE REPORT - Because of the rapidly expanding uterine myoma of a 40 years old woman laparotomy has been performed. In the lower two-thirds of abdominal wound the myoma penetrated into the abdominal wall. Here the abdominal wall lost it's normal structure, became bacon-like, homogenous in consistency and white-grey in colour. The myoma and the attached part of the abdominal wall was excised together. The part of myoma those in contact with abdominal wall contained proliferating myofibroblasts, fibroblasts and was infiltrated by plasma cells and lymphocytes. These findings could be seen in the abdominal wall too. The rapid proliferation which simulated malignant tumor has been diagnosed as inflammatory myofibroblastic tumor. The patient does well two years after the operation. CONCLUSION - It is important to know about the entity of inflammartory pseudotumor in differential diagnostic point of view, as it mimics malignant tumor. The structure is nodular, the histology is characteristic and the therapy is surgical. The follow up is very important because of possibility of probable recurrence. ]

Hungarian Immunology

JANUARY 22, 2008

[Pseudolymphoma orbitae]

VÁNCSA Andrea, GERGELY Lajos, NEMES Zoltán, BÍRÓ Edit, ILLÉS Árpád, BAKÓ Gyula

[INTRODUCTION - Pseudolymphoma orbitae is a rare and difficult entity. The cooperation of the pathologist and clinician is needed to properly manage the patient. CASE REPORT - The authors report the case history of a 38 years old male patient. His disease started at the age of 30. He was previously treated with allergic rhinitis. No definitive diagnosis was made for eight years. Several surgical biopsies were made from nasal mucosa, but no specific histologyical diagnosis was applicable. At the age of 30 he developed an unilateral exophthalmus on the left side. Thyroid associated ophthalmopathy was ruled out several times with laboratory analysis. High dose methylprednisone therapy was repeatedly given with limited results. At the age of 34 orbital CT and MRI scan confirmed the pseudotumour orbitae already compressing the optical nerve. Laboratory analysis again ruled out thyroid associated ophthalmopathy. Churg-Strauss syndrome, Wegener’s granulomatosis or Sjögren’s syndrome could be ruled out. A bone marrow trephine biopsy excluded systemic hematological disease as well. A biopsy was performed from the retrobulbar mass again, which confirmed the lymphoid hyperplasia with B-cell dominance. High dose methylprednisone and local irradiation resulted only moderate decrease of the mass, so systemic chemotherapy was started using CVP (cyclophosphamide, vincristin, prednisone) then CHOP (CVP + anthrycycline) polychemotherapy for eight cycles and subcutaneous interferon-α for 20 months. CONCLUSIONS - This resulted a complete regression of the disease, and the patient is well for 48 months now.]

Hungarian Immunology

JUNE 20, 2002

[Ocular myositis]

KISS Emese, FACSKÓ Andrea, DÉVÉNYI Katalin, DANKÓ Katalin, ZEHER Margit

[INTRODUCTION - Dermato-/polymyositis is an autoimmune disorder, which belongs to the idiopathic inflammatory myopaties. It involves skeletal muscles in form of weakness and inflammatory infiltrates. Characteristic skin lesions are present in dermatomyositis. Other organs may also be affected mainly in the presence of myositis specific autoantibodies. The inflammation usually involves the proximal muscles of extremities. CASE REPORT - In the present work we report the case of a 52-year-old woman. In the previous history the removal of rectal adenocarcinoma was remarkable in 1994. After that she received chemotherapy. She complied for severe headache and pain in the right eye in 2000 October, therefore a skull CT was performed, indicating thickening of rectus medalis muscle within orbital cavity. There was an enhancement of contrast material in the muscle. Glaucoma was excluded. Neurologist suspected the presence of myositis and indicated 0.5 mg/kg corticosteroid therapy. Soon after the left eye became painful, but due to the corticosteroid treatment both eyes became painless. A control orbital CT was completely negative in 2000 November. Immunology consultancy revealed a mild proximal muscle atrophy in both lower extremities, but CPK and LDH enzyme levels were normal, EMG was characteristic for mild chronic nerve lesion. The biopsy, taken from the involved proximal muscle of lower extremity, did not show inflammatory infiltration. Complete screening for cancer was negative. Thyroid gland disease could be excluded. Immune laboratory data were negative, autoantibodies, including anti-Jo1, could not be detected. Based on the results a rare disease, ocular myositis was diagnosed. Considering the clinical improvement, the withdrawal of corticosteroid therapy was offered. Stringent immunology and oncology follow-up is required. CONCLUSION - In relation to our case report, we discuss clinical symptoms of orbital myositis, diagnostic procedures to identify the disease and also differential diagnostic considerations.]

Clinical Neuroscience

JANUARY 30, 2011

[Tolosa-Hunt syndrome]


[Both men and women are affected by the rare disease called Tolosa-Hunt syndrome. We don’t know exactly what causes it to evolve. It is usually put into the categories of either idiopathic inflammation or pseudotumor. Its patological feature is a non-specific inflammatory process with fibroblastic, lymphocytic, plasmocytic infiltration, which can be found, for the most part, in the wall of the sinus cavernosus. Granulocytic and giant-cell infiltrations have been described too. The possibility of autoimmune disease has also come up. In our current study we describe the case of a female patient who recovered with the help of a steroid therapy. Through examining her, we also found immunological alterations, which should urge us to thoroughly examine the further observations of this kind.]