Gülşen Köse

ACUTE DISSEMINATED ENCEPHALOMYELITIS IN CHILDHOOD : REPORT OF 10 CASES

We report 10 children with the diagnosis of acute disseminated encephalomyelitis. Diagnosis was based on clinical and radiologic findings, and after acute encephalitis was excluded by negative culture and antibody results. The most common presenting symptom was ataxia, followed by optic neuropathy, cranial nerve palsy, convulsions, motor dysfunction, and loss of consciousness. Brain magnetic resonance imaging showing bilateral symmetrical hyper-intense lesions of the same age in brain stem, subcortical white matter, thalamus, basal ganglia, or cerebellum was the mainstay of the diagnosis. The presence of a preceding event (either an infection or vaccination) was present in 8 of 10 patients. Brain computed tomographic scans were abnormal in 3 of 10, and electroencephalogram was normal in all patients. High-dose corticosteroids were given to six patients, one received low-dose steroids, and the other three had symptomatic follow-up. Those who relapsed were mainly from the symptomatic follow-up group. Only one patient (the youngest) receiving high-dose methylprednisolone relapsed. Therefore, early high-dose steroid treatment seems to be the most effective treatment in acute disseminated encephalomyelitis and can prevent relapses. (J Child Neurol 1999;14:198-201).

Tissue inflammatory response in subacute sclerosing panencephalitis (SSPE).

The pattern of inflammatory infiltration was studied in the frontal brain biopsies of 28 cases with subacute sclerosing panencephalitis (SSPE) by immunohistochemistry. Lymphocytic infiltration and gliosis were common pathologic findings. CD4+ T lymphocytes were often observed in perivascular areas and CD8+ lymphocytes in the parenchyma. B lymphocytes were located in large perivascular cuffs associated with longer and slower disease. Major histocompatibility complex antigens, interferon-γ, and tumor necrosis factor-α (TNF-α) were expressed in endothelial and glial cells. The inflammatory lesions in subacute sclerosing panencephalitis consist of various cell subtypes and cytokines localized in particular areas of the brain tissue and show certain associations with clinical course. (J Child Neurol 2001;16:895-900).

β-Interferon Plus Inosiplex in the Treatment of Subacute Sclerosing Panencephalitis

We treated seven patients with subacute sclerosing panencephalitis with β-interferon and oral inosiplex for 2 to 15 months. Stabilization or improvement was observed in three patients. The effect of treatment was equivocal in two other patients who became stable. The disease continued its progression in the remaining two patients who died. Treatment shorter than 2 months was not effective. Changes in electroencephalograms (EEG), magnetic resonance images (MRI), or cerebrospinal fluid measles antibody levels did not have a close correlation with clinical course. These results suggest that β-interferon might be efficient in some patients with subacute sclerosing panencephalitis and justify its trial in larger studies with longer follow-up. (J Child Neurol 1998; 13:557-559).

Brain Single Photon Emission Computed Tomographic Evaluation of Patients With Childhood Absence Epilepsy

This study was performed to determine the utility of 99mTc-hexamethylpropylenamine oxime (HMPAO) brain single photon emission computed tomography (SPECT) in evaluating patients with childhood absence epilepsy. Twenty-three patients (13 female, 10 male), aged 7 to 15 years (mean age 10.3 ± 2.2), were studied. All patients underwent a detailed neurologic examination, interictal and ictal electroencephalography (EEG), computed tomography, and/or magnetic resonance imaging, and SPECT. The baseline study was performed during the interictal period and the activation study was performed on a separate day while the patients were having seizures provoked by hyperventilation. Their EEGs were monitored at the same time. Transaxial, sagittal, and coronal slices were obtained for both studies. The mean counts per pixel were calculated on 11 regions of interest on three representative transaxial slices. Count density was calculated for each region. Region-to-occipital cortex ratios were obtained. For each region, normalized ratios were used to obtain a side-to-side percent asymmetry index between baseline and activation studies. Visual interpretation of the baseline study showed that 10 of the 23 patients had a detectable abnormality in regional cerebral blood flow during the interictal period. These abnormalities consisted of relative hypoperfusion in the frontal lobes that could involve neighboring parietal and temporal regions. The activation study revealed that 13 of 23 patients had relative hyperperfusion in these brain regions that were relatively hypoperfused in the baseline study. These hyperperfused regions occupied larger areas than baseline hypoperfused regions. All patients had global increased perfusion in the ictal study. The side-to-side asymmetry indexes for these visually interpreted regional cerebral blood flow abnormalities ranged from 2 to 6%. The relatively consistent pattern of frontal regional cerebral blood flow alterations suggests that altered frontal lobe functions can be implicated in patients with childhood absence epilepsy. (J Child Neurol 2003;18:542—548).

Differences in iron deficiency anemia and mean platelet volume between children with simple and complex febrile seizures

OBJECTIVE The relationship between iron deficiency anemia and febrile seizures (FSs) were examined in several studies before. The aim of our study is to find out the differences regarding iron deficiency anemia, demographic characteristics and mean platelet volume (MPV) which is an inflammatory marker between simple and complex febrile seizure groups. METHODS In this study, the authors investigated the recordings of 493 children with a diagnosis of simple and complex febrile seizure, aged between 6 months and 6 years, followed between 2002 and 2010 retrospectively. RESULTS Mean age and male/female ratio were similar in two groups. There was no significant difference regarding with age, gender and family history of FS between two groups. We found significant difference statistically with respect to gestational age, consanguinity, family history of epilepsy and birth weight between two groups. The mean levels of Hb, Htc, MCV were lower and Plt and RDW levels were higher in children with CFS than SFS group, the differences were statistically significant (p: 0.001). A higher proportion of children with CFS (16.2%) had iron deficiency anemia compared to SFS group (12.1%). Mean platelet volume (MPV) of CFS (7.99±0.96fL) were significantly lower than that of SFS group (8.77±0.75) (p<0.001). CONCLUSIONS The results of this study suggests that iron deficiency anemia is more frequently seen among the patients with CFS than the patients with SFS. The lower levels of MPV as an inflammatory marker, supports the idea that CFS is a brain inflammatory disease and the consequence of this inflammatory mechanism is the development of the epilepsy. Further studies are necessary to highlight the relationship between iron metabolism, inflammation and seizures.

Interleukin-1β, Interleukin-1 Receptor Antagonist Levels in Patients With Subacute Sclerosing Panencephalitis and the Effects of Different Treatment Protocols

Subacute sclerosing panencephalitis is a rare progressive inflammatory disease of the central nervous system caused by a persistent aberrant measles virus infection. Cytokines are polypeptides that regulate immune responses and inflammatory reactions. Interleuldn-1β has been implicated as a central mediator of tissue damage and destruction in a number of central nervous system diseases. Interleukin-1 receptor antagonist could function as an important anti-inflammatory cytokine. We studied interleukin-1β and interleukin-1 receptor antagonist levels in the cerebrospinal fluids of patients with subacute sclerosing panencephalitis and evaluated the effects of different treatment protocols on these cytokines. Interleukin-1β and interleukin-1 receptor antagonist levels were measured in 15 patients who had a recent diagnosis of subacute sclerosing panencephalitis (group 1), 6 patients who had been treated with isoprinosine (group 2), 5 patients with intraventricular interferon-α (group 3), and 6 patients with interferon-β (group 4). The results were compared within the groups and also with the results of 10 patients with other neurologic disease (group 5).The interleukin-1β concentrations in cerebrospinal fluid and sera were all below the detection limits (3.9 pg/mL). Interleukin-1 receptor antagonist levels were not statistically different, except for the group treated with intraventricular interferon-α. Interleukin-1 receptor antagonist levels were 170 ± 52, 175 ± 58, 1605 ± 518, 77.5 ± 24, and 108 ± 18 pg/mL in groups 1 to 5, respectively. Interleukin-1 receptor antagonist levels and cerebrospinal fluid serum ratios were significantly increased during interferon-α treatment. In conclusion, interleukin-1 and interleukin-1 receptor antagonist levels were not elevated in the patients with subacute sclerosing panencephalitis. The only treatment protocol that affects interleukin-1 receptor antagonist levels in cerebrospinal fluid was intraventricular interferon-α. Further studies on higher numbers of patients may better document the immunologic status of patients with subacute sclerosing panencephalitis and the effects of different treatment modes. (J Child Neurol 2001;16:417-420).

Guillain-Barré syndrome in children: subtypes and outcome

ObjectiveThis study reviews the clinical features, subtypes, and outcomes of childhood Guillain-Barré syndrome (GBS).MethodsFifty-four children who attended a tertiary care training and research hospital in Turkey were enrolled in the study.ResultsThe mean age was 6.5 ± 4.2 years and 32 patients (59.5%) were male. The most common subtype of GBS was acute inflammatory demyelinating polyneuropathy (AIDP), which was seen in 27 patients (50%). Having antecedent history, especially upper respiratory tract infection was significantly more common in AIDP (P = 0.028). Sensorial symptoms were significantly more frequent in axonal type GBS (P = 0.001). When we compare the demyelinating and axonal forms, all of the groups had favorable outcome.ConclusionThe diagnosis of pediatric GBS can be delayed because of its variable presentation. Early admission to hospital and early treatment are important for decreasing the need for respiratory support and improving the outcome.