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Biomedical subjects

Ayse Tosun

Publications and source records attributed to Ayse Tosun.

4 recordsLinked to original sources

Electrodiagnostic pattern approach for childhood polyneuropathies.

Electrophysiologic studies play a key role in the detection and characterization of the pattern in childhood polyneuropathies. In this study, the etiologic profile of 74 children with polyneuropathy was prospectively evaluated based on the electrophysiologic studies. Five electrodiagnostic patterns were identified in the cohort: (1) acute axonal polyneuropathy (n: 32, 43%); (2) chronic axonal polyneuropathy (n: 16, 22%); (3) demyelinating motor and sensory polyneuropathy (n: 13, 17%); (4) pure sensory polyneuropathy (n: 11, 15%); (5) high-low syndrome (n: 2, 3%). Etiologic factors were identified in all of the patients with three electrodiagnostic patterns of polyneuropathy: acute axonal, pure sensory, and high-low syndrome. However, etiologic factors could not be determined in 5 (31%) children with chronic axonal polyneuropathy and in 3 (23%) children with demyelinating sensory and motor polyneuropathy. Among children with the acute axonal pattern, toxic causes were evident in 18 (56%), acute motor axonal neuropathy in 11 (35%), and acute motor sensory axonal polyneuropathy in 3 (9%). Nine (82%) patients with pure sensory polyneuropathy had diabetes mellitus. In conclusion, a thorough history and physical examination in conjunction with specific electrodiagnostic patterns might provide a cost-effective and rational differential diagnosis of childhood polyneuropathies.

Adolescent↗

Central core disease: atypical case with respiratory insufficiency in an intensive care unit.

Central core disease is a rare congenital myopathy characterized by formation of typical cores in myofibrils. We report an atypical case of central core disease with respiratory insufficiency in the late stage of congenital myopathy. A 13-year-old girl was admitted to the intensive care unit with the diagnosis of respiratory distress syndrome. Ventilatory support was initiated. After 2 weeks of follow-up, the Division of Pediatric Neurology was consulted owing to the failure to wean her from the ventilator. Clinical and electromyographic features were in favor of primary muscle disease. Muscle biopsy revealed typical cores in type 1 muscle fibers, which were diagnostic for central core disease. This case was presented to emphasize that patients with respiratory distress who cannot be weaned from the ventilator should be evaluated for central core disease with an atypical presentation.

Adolescent↗

Cerebrospinal fluid interleukin-6 levels in patients with West syndrome.

Elevated cytokine response has been reported in patients with epileptic seizures. The objective of this study was to investigate the possible role of interleukin-6 (IL-6) in the pathogenesis of infantile spasms in West syndrome (WS). We measured IL-6 levels in cerebrospinal fluid (CSF) obtained from the newly diagnosed patients with WS. Twelve patients with WS (Group I) were classified as symptomatic WS (Group IA) in eight and as cryptogenic WS (Group IB) in four. The results were compared with control groups including patients with tonic-clonic seizures associated with two different kind of inflammation of central nervous system; Group IIA (infection): bacterial meningitis/encephalitis and Group IIB (trauma): post-traumatic seizures. There was no statistically significant difference between the mean values of CSF IL-6 levels in patients with WS (2.95 +/- 2.31 pg/ml) and those of subgroups of WS (Group IA: 2.26 +/- 2.01 pg/ml and Group IB: 4.33 +/- 2.52 pg/ml). Both control groups had highly increased IL-6 levels in CSF (Group IIA: 193.05 +/- 185.52 pg/ml and Group IIB: 112.74 +/- 167.44 pg/ml) than those of the patients with WS. Elevated IL-6 response in patients with tonic-clonic seizures associated with inflammation of central nervous system might be due to the seizures themselves or related to the underling etiology (infection or trauma). However, no elevated IL-6 response was found in patients with infantile spasms.

Child, Preschool↗

T-cell subsets and interleukin-6 response in Rasmussen's encephalitis.

To evaluate the immunopathogenesis in Rasmussen's encephalitis, peripheral lymphocyte subsets and interleukin-6 analysis were performed in three patients. Magnetic resonance spectroscopy and diffusion-weighted magnetic resonance imaging were performed to assess neuronal injury in the affected hemisphere. Before initiation of immune therapy, percentage of cytotoxic T cells was found to be increased in peripheral blood obtained from patients compared with a group of age-matched normal control subjects. During follow-up, percentage of cytotoxic T cells returned to the normal ranges only in one patient who had an early functional hemispherectomy. All three patients had significantly increased interleukin-6 concentration in cerebrospinal fluid and serum compared with the mean values of patients with acute viral encephalitis. The magnitude of interleukin-6 response in the patients correlated with the neuronal loss and atrophy on magnetic resonance spectroscopy and diffusion-weighted magnetic resonance imaging studies. The patient, who had a fulminant course and an early hemispherectomy, had higher interleukin-6 concentration in cerebrospinal fluid and serum than those of the other two. Detection of an increased percentage of cytotoxic T cells in peripheral blood supports the presence of a T cell-mediated inflammatory pathogenesis in Rasmussen's encephalitis. However, elevated interleukin-6 response might reflect the magnitude of the inflammatory process in the affected hemisphere.

Adolescent↗