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Lege Artis Medicinae

APRIL 01, 2009

[A holistic approach to neuropathic pain]

KISS Gábor

[Neuropathic pain is a chronic pain disorder due to a primary lesion and/or dysfunction of the peripheral or central nervous system. This tormenting condition causes a lot of distress to the patients, impairs their quality of life, and demands significant expenses. Chronic neuropathic pain is frequently under-diagnosed and mistreated. Explanations for these problems are the complex underlying pathomechanism, variability of symptoms, difficulties in diagnosis, and the differences between the treatment of this and other painful disorders. In addition, comorbid conditions such as anxiety, depression, and sleep disorders are often overlooked. Apart from the diagnostic difficulties, also treatment is usually unsatisfactory. Frequently NSAIDs are used, but they are usually not effective. Undoubtedly, even with the use of evidence-based treatment - such as duloxetine and pregabalin - complete pain relief is not always possible. Lack of proper medical education also contributes to problems in diagnosis and treatment. In western countries, diabetes is the most common cause of polyneuropathy. Painful diabetic neuropathy is the most intensely studied neuropathic pain condition; a lot of evidence comes from randomized controlled trials of this type of neuropathy. The same drugs as in the case of other neuropathic pain conditions are used for the symptomatic treatment of painful diabetic neuropathy. Etiological therapy is based on the best achievable glycemic control. A combination of etiological and symptomatic therapy can be a future treatment, but proving this will require further studies.]

Lege Artis Medicinae

FEBRUARY 21, 2004

[LARGE VESSEL MANIFESTATION OF GIANTCELL ARTERITIS]

KOLOSSVÁRY Endre, PINTÉR Hajnalka, ERÉNYI Éva, KOLLÁR Attila, FARKAS Katalin, KISS István, HARCOS Péter, SIMON Károly

[The diagnosis of giant-cell arteritis is a real challenge for clinicians. There are several reasons for the difficulties in establishing the diagnosis. This disease is associated to rare conditions, therefore most physicians lack clinical experience. This condition shows very heterogeneous manifestation, the intensity of the symptoms vary in time. Early diagnosis is of great importance in order to prevent ischemic complications. Among these complications one should emphasise the role of anterior ischemic optic neuropathy that may result in abrupt blindness. In this case report, we show a rare socalled large vessel manifestation of giant-cell arteritis. This form of the disease needs different approach in diagnosis where color duplex ultrasonography may have distinguished importance. The final verification of the diagnosis is based on histology. However the lack of all histological criteria do not exclude the presence of giant-cell arteritis.]

Clinical Neuroscience

JANUARY 30, 2011

[Neurological complications of Fabry-disease]

VASTAGH Ildikó, CONSTANTIN Tamás, KÉRI Anna, RUDAS Gábor, FEKETE György, BERECZKI Dániel

[Background - Fabry-disease (FD) is a rare X-linked lysosomal storage disease. Deficiency of alpha-galactosidase A activity leads to the accumulation of neutral glycosphingolipids, primarily globotriaosylceramide (GL-3) in various tissues, particularly blood vessels, kidneys, myocardium and in ganglions of the peripheral and autonomic nervous system and causes diverse symptoms. The classical phenotype is seen in most males and rarely in females. In women, symptomes start later and the severity is milder. Both peripheral and central nervous system can be both affected. Objectives - Fabry-patiens and gene-carriers in the central region of Hungary are treated in the 2nd Pediatric Department, Semmelweis University. These patients are consulted by an interdisciplinary team. At present, four hemizygous male, four heterozygous female Fabry-patients and three asymptomatic heterozygous gene carriers are followed. Results - After reviewing the neurological complications of FD, we present clinical and neuroimaging data of our patients. Conclusion - We emphasize that neurologists should suspect the rare monogenic FD in the case of acroparaesthesia and heat-cold intolerance in childhood or adolescence; clinical signs of TIA/stroke or unexplained MRI alterations suggesting small vessel disease in young adults. Early diagnosis and introduction of enzyme replacement therapy (ERT) can halt or reverse progression.]

Clinical Neuroscience

SEPTEMBER 30, 2007

[SIMULTANEOUS CENTRAL AND PERIPHERAL NERVOUS SYSTEM INVOLVEMENT IN SYSTEMIC LUPUS ERYTHEMATOSUS]

ILNICZKY Sándor, KAMONDI Anita, ARÁNYI Zsuzsanna, VÁRALLYAY György, GAAL Barbara, SZIRMAI Imre, NAGY György

[Systemic lupus erythematosus is a frequent autoimmune disease, affecting several organs, including the brain, spinal cord and nerves. Cerebral vasculitis, transverse myelitis and polyneuropathy are the most common neurological manifestations. We report a case of a 46 years old woman who suffered incomplete transverse myelitis in her age of 44. After 2 years the second relapse presented with arthralgias, painful paraesthesias and weakness of the lower limbs. Neurological signs suggested involvement of the central and the peripheral nervous system. Based upon clinical and laboratory findings systemic lupus erythematosus was diagnosed. Magnetic resonance imaging revealed two hyperintense lesions on T2 weighted scans within the cervical spinal cord. The brain scan was normal. Protein content was slightly elevated in the cerebrospinal fluid, with normal cell count. Electrophysiological examinations diagnosed a subacute sensory-motor axonal polyneuropathy. On methylprednisolone treatment her condition improved. Simultaneous development of central and peripheral lesions of the nervous system in cases with systemic lupus erythematosus may lead to a challenge to establish the diagnosis.]

Clinical Neuroscience

JUNE 02, 2009

[Posterior ischaemic optic neuropathy]

BOÓR Krisztina, KOVÁCS Krisztina, RÓZSA Anikó, PÁNCZÉL Gyula, SZILVÁSSY Ildikó, GÁCS Gyula

[Here one case report of the posterior ischaemic optic neuropathy, a rare and underdiagnosed form of the non arteritic ischaemic optic neuropathy is presented, to underline the value of the MRI in the diagnosis. The ischaemic optic neuropathy is the infarction of the optic nerve. Depending on the affected segment of optic nerve (optic nerve head or retrobulbar segment) two subclasses exist: the anterior (AION) and the posterior (PION) ischaemic optic neuropathy. Ischaemic optic neuropathy characterized by sudden, painless, mononuclear loss of vision, and/or visual field defect, that is accompanied by a diagnostic picture of the optic disc fundus only in the case of the AION. The diagnosis of the PION is based on a diagnosis of exclusion described by Hayreh in 1981. The macular and retinal laesions, the etiological role of toxic agent, the compression and the inflammation of the optic nerve all have to be excluded. The differential diagnosis between the PION and the retrobulbar neuritis is more difficult. Nowadays, in addition to the case history and the clinical data the diagnosis of the PION could be confirmed with help of VEP (visual evoked potential) and MRI. In the case of an old woman who had a sudden, painless visual loss of left eye we confirmed the diagnosis of PION with MRI which was presumed after had excluding other etiological factors.]

Lege Artis Medicinae

NOVEMBER 10, 2008

[DISEASES OF THE EXOCRINE PANCREAS AND DIABETES MELLITUS]

CZAKÓ László

[Exocrine and endocrine pancreas constitutes close anatomical and functional links accordingly, any disease affecting one of these sectors will inevitably affect the other. Acute and chronic pancreatitis, pancreatic surgery, cystic fibrosis and pancreatic cancer are those pancreatic conditions that might cause diabetes mellitus. The development of diabetes greatly influences the prognosis and quality of life of patients with exocrine pancreatic diseases. The lack of glucagon and the impaired absorption of nutrients may cause life-threatening complications, such as hypoglycaemia, and the micro- and macrovascular complications may impair the organ functions. Diabetes mellitus is an independent risk factor of mortality in those with exocrine pancreatic diseases. Pancreatic diabetes is a distinct metabolic and clinical form of diabetes, requires special treatment. Diet and pancreatic enzyme replacement therapy may be sufficient in the early stages. Oral antidiabetic drugs are not recommended. If the diet proves inadequate to reach the glycaemic goals, regular insulin treatment is demanded. There are special impairments of the exocrine function and the pancreatic morphology at diabetic patients that resemble to chronic pancreatitis. Atrophy of the exocrine tissue may caused by the lack of trophic insulin. Hyperglycaemia can activate the stellate cells that lead to pancreatic fibrosis. The microangiopathy and neuropathy, as well as the lack of islet hormone action - responsible for the exocrine pancreas regulation - will cause further damage on the pancreas glandular tissue. In the event of a proven impairment of the pancreatic exocrine function in diabetes mellitus, pancreatic enzyme replacement therapy is recommended. This may improve the nutritional condition and decrease the metabolic instability.]

Clinical Neuroscience

JANUARY 30, 2011

[Ulcerative carpal tunnel syndrome]

KAMONDI Anita, TEIXEIRA Jose Maria, SZIRMAI Imre

[The carpal tunnel syndrome is the most frequent compression-induced neuropathy. A severe but rare clinical manifestation of this disorder associates with ulceration, acral osteo-lysis and mutilation of the terminal phalanges of the second and third fingers. Recognition of this disorder is difficult, because various dermatological and internal diseases might lead to acral ulcerative lesions, and these patients are seldom referred to neurological and/or electrodiagnostic examination. In this article, we present three cases of this rare clinical form of carpal tunnel syndrome and discuss the electrodiagnostic findings. The early diagnosis is important since decompression of the median nerve in due time might prevent mutilation and could significantly improve the patients’ quality of life.]

Clinical Neuroscience

MARCH 30, 2006

[THE USE OF INTRAVENOUS IMMUNGLOBULIN IN THE TREATMENT OF AUTOIMMUNE NEUROMUSCULAR DISEASES]

MOLNÁR Mária Judit

[Intravenous immunglobulin given in autoimmune neuromuscular disorders modulates the immune system by complex actions, including, 1. the modification of the expression and function of Fc receptors, 2. interference with the activation of the complement and the cytokine network, 3. neutralisation of antiidiotypic antibodies, 4. effects on the activation, differentiation and effector functions of the T and B cells. Controlled trials have shown that intravenous immunglobulin is effective as first-line therapy in patients with Guillain-Barré syndrome and multifocal motor neuropathy. In case of steroid resistance or coexisting diabetes mellitus, intravenous immunglobulin can be the first line therapy in chronic inflammatory demyelinating polyneuropathy as well. As an alternative therapy it can be a second-line choice in dermatomyositis, myasthenia gravis, Lambert-Eaton myasthenic syndrome, and stiff person syndrome. While it has a remarkably good safety record for long term administration the following side effects have been observed: headache, skin rash, thromboembolic events and renal tubular necrosis. In some disorders, the appropriate dose and frequency of infusions that maintain a satisfactory therapeutic response is well defined on the basis of data of evidencebased medicine, whereas in others it still remains to be defined. For the analysis of pharmacoeconomical aspects and the mechanism(s) of response differences in the same disease categories, further studies are necessary.]

Clinical Neuroscience

JULY 22, 2009

[High resolution sonography for the examination of peripheral nerves in vascular neuropathy]

JOSEF Böhm

[High-frequency sonography is a new method for the imaging of the peripheral nerves. In vasculitic neuropathy, pain or axonopathy can often prevent the lesion to be localized during electrophysiological examinations. There are only few informations in the literature about the ability of the sonography for localizing lesions in vasculitic neuropathy. High-frequency sonography helped in our two cases of mononeuritis multiplex and two cases of distal-symmetric polyneuropathy to put the diagnosis of a vasculitic neuropathy and was able to detect focal morphologic lesions which could not be identified electrophysiologically due to the axonopathy.]

Clinical Neuroscience

DECEMBER 20, 2008

[Hereditary neuropathy with liability to pressure palsy in childhood]

GYÖRGY Ilona, BÍRÓ Anna, MECHLER Ferenc, MOLNÁR Mária Judit

[HNPP is an autosomal-dominant inherited disease clinically characterized by painless, episodic, recurrent peripheral palsy often preceded by minor trauma or toxic damage. It generally develops during adolescence and rarely is reported in childhood. We observed two children with this disease. In one of the cases, also the child’s mother is suffering from HNPP. Clinical and genetic characterics of our three patients are summarized in this article.]