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Clinical Neuroscience

NOVEMBER 30, 2011

[Common variable immunodeficiency with coexisting central nervous system sarcoidosis. Case report and literature review with implications for diagnosis and pathogenesis]

MAGDALENA Dziadzio, HORTOBÁGYI Tibor, DESMOND Kidd, RONNIE Chee

[We describe a patient with a history of longstanding primary generalised epilepsy, on anticonvulsant therapy, who presented with fever, headache, worsening seizures and hallucinations. Among various investigations, the patient had high CSF protein and ACE levels, leptomeningeal nodular enhancement on MRI brain and non-caseating granulomas in the brain and meninges on the biopsy. The patient was diagnosed with neurosarcoidosis. Subsequently, he was found to be panhypogammaglobulinaemic and was diagnosed with probable common variable immunodeficiency (CVID). The coexistence of common variable immunodeficiency and neurosarcoidosis is rare. Typically, non-caseating granulomas in CVID patients are localised in the lymphatic tissue and solid organs. To our knowledge, there are only five reports of the granulomas of the central nervous system (CNS) in CVID. We discuss the diagnostic difficulties in this case and review the literature.]

Lege Artis Medicinae

FEBRUARY 21, 2004

[EYE DISORDERS ASSOCIATED WITH MUSCULOSKELETAL DISEASES]

VOGT Ferenc

[The diseases of connective tissue and musculoskeletal system frequently associated with typical eye disorders. These can either be mild, recovering fully after treatment or more serious with persisting symptoms and destructive changes resulting in permanent loss of sight Eye symptoms can occur in the following diseases: rheumatoid arthritis, juvenile chronic arthritis, neonatal onset multisystem disease, ankylosing spondylitis, seronegative spondarthrities, Reiter’s syndrome, Behçet’s syndrome, Lyme disease, systemic lupus erythematosus, scleroderma, polyarteritis nodosa, Wegener’s granulomatosis, giant cell arteritis, erythema nodosum, relapsing polychondritis, sarcoidosis, Marfan’s syndrome, osteogenesis imperfecta.]

Clinical Neuroscience

JULY 10, 2004

[Myasthenia in a patient with sarcoidosis and schizophrenia (in English language)]

RÓZSA Csilla, KIS Gábor, KOMOLY Sámuel

[A 44-year-old male patient was hospitalised with paranoid schizophrenia in 1985. Depot neuroleptic treatment was started which successfully prevented further psychotic relapses for the next ten years. His myasthenia gravis started with bulbar signs in 1997 and the symptoms soon became generalized. The diagnosis of myasthenia gravis was confirmed by electromyography, by positive anticholinesterase test and by the detection of anti-acetylcholine receptor antibodies in the serum. Mediastinal CT examination showed enlarged hilar lymph nodes on the left but no thymic pathology was observed. Mediastinoscopy was performed and biopsies were obtained from the affected nodes. Histology revealed sarcoidosis. The patient suffered respiratory crisis following the thoracic intervention (in September 1998). Combined oral corticosteroid (64 mg methylprednisolone/e.o.d.) and azathioprine (150 mg/day) treatment regimen was initiated and complete remission took place in both the myasthenic symptoms and the sarcoidosis. The follow-up CT scans showed no mediastinal pathology (January 2000). During steroid treatment a transient psychotic relapse occured which was successfully managed by supplemental haloperidol medication added to his regular depot neuroleptics. The patient currently takes 150 mg/day azathioprine and receives 40 mg/month flupentixol depot im. His physical and mental status are stable and he has been completely symptome free in the last 24 months. The association of myasthenia gravis and sarcoidosis is very rare. To our best knowledge no case has been reported of a patient suffering from myasthenia gravis, sarcoidosis, and schizophrenia at the same time.]

Lege Artis Medicinae

APRIL 22, 2011

[IgG4-related disease]

ZEHER Margit

[IgG-4-related disease is a clinical entity characterised by significant elevation in serum IgG-4 levels, infiltration of IgG-4+ plasma cells into the involved tissues, enhanced fibrosis, and good therapeutic response to corticosteroids. The IgG-4 associated disease mostly affects two organs. The salivary and lacrimal gland enlargement and inflammation is known as Mikulicz’s disease, which had been previously known as a subtype of Sjögren’s syndrome for a long time. The other commonly involved organ is the pancreas, in which a special form of chronic pancreatitis, namely autoimmune pancreatitis develops. IgG-4 associated disease as a separate disease has been suggested by Japanese authors. Previously published data support the common pathogenesis of autoimmune pancreatitis and Mikulicz’s disease. Besides these two manifestations, similar histological lesions and elevated IgG-4 levels have been demonstrated in many other organs. At present, it is not clearly demonstrated whether IgG-4 syndrome is an autoimmune disorder, and we do not know the exact reason of the elevated IgG-4 levels in patients with this syndrome. IgG-4 is a regulatory immunoglobulin, the main function of which is to decelerate immune responses, thus its pathologic role in tissue destruction is difficult to explain. It is not clear either, whether IgG-4 syndrome is indeed a single disease, or only an example of overlapping symptoms of various diseases. On the basis of the characteristic histological lesions in various organs, IgG-4 syndrome is similar to multi-organ diseases, such as sarcoidosis or vasculitis.]

Clinical Neuroscience

JANUARY 20, 2007

[UNCOMMON MANIFESTATION OF CENTRAL NERVOUS SYSTEM SARCOIDOSIS]

RÓZSA Anikó, SZTANKANINECZ Yvette, GÁCS Gyula, MAGYAR Tamás

[Two cases of uncommon manifestation of central nervous system sarcoidosis are reported. A 42 year-old man had a spinal cord sarcoidosis. MRI of the spinal cord showed myelopathy in the cervico-thoracic region, and the T2-weighted image showed increasing signal intensity. Neurological symptoms did not correllate with radiological abnormalities. Neurological manifestation was paucisymptomatic. Half a year later steroid and azatioprin therapy led to almost complet radiological and clinical regression. In the second case we present a 49 year-old woman who had left side internuclear ophthalmoplegia and the brainstem lesion. The patient was proven to have sarcoidosis. In this case no abnormalities were found in brain MRI. Neurological symptoms could not be detected by MRI, probably caused by brainstem parenchymal lesions consisting of microgranulomatosis that is sarcoid "brainstem encephalitis". Neurological symptoms improved after steroid treatment in this case too. In both of the cases pulmonary lymphadenopathy helped to diagnose sarcoidosis. In our cases there were interesting correllations between neurological symptoms and MRI abnormalities. At the spinal cord sarcoidosis the radiological abnormalities were more striking than the clinical manifestation. In the other case we found distinct brainstem symptoms but could not detect MRI abnormalities.]

Hungarian Radiology

DECEMBER 20, 2007

[Sarcoidosis involving liver and spleen, and with hypercalcemia]

TÓTH Mónika, WENINGER Csaba, MORÓ Zsuzsanna, SZOMOR Árpád

[INTRODUCTION - Sarcoidosis is a relatively common multisystemic disorder. Chest involvement is most frequent, but any organ can be involved. In case of abdominal lesions the suspicion of sarcoidosis rarely arises. CASE REPORT - A 53-year old female patient with a history of crural pain unresponsive to medications and weight loss of unknown origin was sent to the internist. Hypercalcemia and bone pain suggested multiple myeloma, however, this diagnosis could not be confirmed. Chest X-ray examination was negative. Splenomegaly and multiple hypodens splenic and hepatic lesions were detected by ultrasound and abdominal computed tomography. Finally, ultrasound guided biopsy of the liver proved stage II sarcoidosis. Steroid therapy was initiated and the splenic and hepatic lesions seen with ultrasound and CT scan disappeared. CONCLUSION - Sarcoidosis is usually suspected following chest X-ray or during chest CT examination. Nevertheless, our case demonstrates that multiple lesions in the spleen or liver may indicate sarcoidosis even in the absence of thoracic lesions. Biopsy taken from the lesions can lead to adequate diagnosis.]

Lege Artis Medicinae

OCTOBER 20, 2004

[TRANSBRONCHIAL NEEDLE ASPIRATION IN THE DIAGNOSIS OF SARCOIDOSIS]

ZSIRAY Miklós, BADÁR Éva, UDUD Katalin, FÜLÖP Andrea, SZABÓ Zsuzsanna, MATESZ István, MARKÓCZY Zsolt

[INTRODUCTION - The histological pattern of sarcoidosis and cytological characteristics are similarly unspecific. Nevertheless, both forms of the diagnosis based on morphology can be taken into consideration if the clinical picture and chest X-ray respectively, are fitted and other diseases are excluded. PATIENTS, METHOD AND RESULTS - Enlargement of hilar lymph nodes is present in 80% of cases of sarcoidosis. In this study, transcarinal needle aspiration combined with rapid on-site cytological staining was performed in the case of 33 patients with I. or II. stage of sarcoidosis. The sensitivity was found to be 76%. CONCLUSION - Since the cytologist can inform the bronchologist fairly soon: during the bronchoscopy, bronchial mucosal biopsies or transbronchial lung biopsies are only necessary to obtain in about quarter of patients.]

Lege Artis Medicinae

JULY 20, 2004

[LATE COMPLICATION OF EXTRANODAL LYMPHOMA FOLLOWING DERMATOMYOSITIS]

VÁNCSA Andrea, PONYI Andrea, CONSTANTIN Tamás, GERGELY Lajos, DANKÓ Katalin

[INTRODUCTION - Intramuscular follicular Bcell lymphoma is a rare entity of malignant lymphomas complicating the disease course of dermato/polymyositis. CASE REPORT - The authors report a female patient who was diagnosed with dermatomyositis in 1963 (age of 36). She was given steroid and hydroxychloroquine therapy. In 1973 she was treated with steroid for Boeck sarcoidosis. In 1999, she was treated for steroid therapy again for reoccurence of dermatomyositis. In 2000, a histological diagnosis of primary intramuscular follicular lymphoma was established from the right thigh region. She was given several courses of polychemotherapy and radiation therapy, but the lymphoma was difficult to control, and she died of progressive disease in 2003. CONCLUSION - It is an interesting case as 37 years elapsed since the diagnosis of dermatomyositis before the lymphoma has started. We could not state a definite relation between the myositis and the lymphoma but the increased frequency of autoimmune diseases is probably due to immune regulatory problems, that have eventually led to the appearance of the rapidly growing indolent lymphoma.]