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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.]

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 osteolysis 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

SEPTEMBER 30, 2008

[Paraneoplastic chronic demyelinating neuropathy and lambert-eaton myasthenic syndrome associated with multiple anti-neural antibodies and small-cell lung cancer]

RÓZSA Csilla, VINCENT Angela, ARÁNYI Zsuzsanna, KOVÁCS G. Gábor, KOMOLY Sámuel, ILLÉS Zsolt

[Lambert-Eaton myasthenic syndrome (LEMS) developed in a patient with presumed chronic inflammatory demyelinating polyneuropathy (CIDP) and negative chest CT. Since antibodies against both Hu and voltage-gated calcium channel (VGCC) were detected, repeated chest CT was performed, which eventually showed a pulmonary mass lesion. Biopsy revealed small cell lung cancer (SCLC) indicating the importance of repeated chest CT in LEMS even when an existing autoimmune-like disease and negative CT may suggest an autoimmune origin. This is the first report of paraneoplastic CIDP and LEMS associated with anti-Hu, anti-VGCC and SCLC.]