Ocular findings in subacute sclerosing panencephalitis.

PURPOSE: The aim of this study was to evaluate the ocular manifestations of subacute sclerosing panencephalitis (SSPE) and to investigate possible risk factors in a relatively large series of patients. METHODS: In this prospective study, patients who were diagnosed with SSPE and had undergone ophthalmic examination were included. RESULTS: Of the 59 patients with SSPE, 25 (42.4%) had ocular findings. Ocular involvement was bilateral in 22 (88%) patients. The most characteristic fundus findings in our patients were chorioretinitis. No significant difference was found between the two groups in terms of age, gender, clinical stage, and ocular findings. CONCLUSIONS: Although ophthalmological findings do not constitute a part of the diagnostic criteria, and the correlation between the clinical stage of SSPE and the ocular findings is unclear, patients with SSPE should be screened and followed up for ocular involvement.

Tau proteins in the cerebrospinal fluid of patients with subacute sclerosing panencephalitis.

Neurodegenerative diseases characterized by cytoskeletal deformation and neurofibrillary tangles are associated with altered levels of tau and related proteins in cerebrospinal fluid (CSF). Neuronal or glial fibrillary tangles have been shown in 20% of subacute sclerosing panencephalitis (SSPE) patients. We therefore investigated CSF samples from 60 newly diagnosed SSPE and 31 neurological control patients for total tau (t-tau), phosphorylated tau (p-tau), and S100-B levels by ELISA. There was no difference between patient and control groups in t-tau and S100-B levels. p-Tau was lower in the SSPE group (p=0.009). Past history of measles infection, measles immunization status, latent period between measles and onset of SSPE, duration of symptoms, frequency of myoclonia, neurological deficit index, stage and progression rate of the disease, CSF glucose levels and cell counts, CSF and serum measles IgG titer, distribution of lesions on brain magnetic resonance imaging were not related to t-tau, p-tau and S100-B levels. Mental status and age were negatively correlated with t-tau, and male gender and EEG abnormalities were associated with higher t-tau levels. The levels of tau proteins in our patients suggest there is no, or only scarce and immature, neurofibrillary tangle formation in SSPE. Autopsy studies showing neurofibrillary tangles might have examined older patients with longer disease and more parenchymal involvement.

Cerebrospinal fluid nitric oxide levels in subacute sclerosing panencephalitis.

Oxidative damage plays a role in neurodegenerative diseases. Levels of cerebrospinal fluid nitrite and nitrate levels (oxidation products that provide an indirect estimation of nitric oxide) were investigated in relation to clinical and laboratory features in subacute sclerosing panencephalitis (n = 47) and age-matched control (n = 43) groups. Significantly decreased levels of nitrite (median, 4.91 micromol/L) and nitrate (median, 6.14 micromol/L) were found in the patients. Nitrite and nitrate levels did not correlate with clinical or laboratory findings, except for presence of myoclonus. Cerebrospinal fluid nitrite levels of subacute sclerosing panencephalitis patients without myoclonic jerks were significantly higher than in those with myoclonus (median, 15.63 vs 4.34 micromol/L, respectively). The higher levels of nitrite in these patients can be explained by short disease duration and early stages of disease. Nitrate levels in subacute sclerosing panencephalitis patients with myoclonus (median, 9.26 micromol/L) were higher than in those without myoclonus (median, 4.25 micromol/L). Microbleeding resulting in conversion of nitrite to nitrate and increased production of superoxide can be suggested as possible mechanisms underlying these findings.

Paroxysmal tonic upgaze presenting as a clinical isolated syndrome.

We report a 3.5-year-old boy with sudden onset of episodic upward deviation of the eyes that led to diagnosis of paroxysmal tonic upgaze. Cranial magnetic resonance imaging showed right-hand side dominant bilateral hyperintense lesions in the mesencephalon and the thalamus on T2-weighted images. These lesions suggested a demyelinating pathology. Corticosteroid treatment was started. The episodes of upward eye deviation and the lesions on magnetic resonance imaging completely resolved after 3 days and 3 months, respectively. After 2 years of follow-up, he was symptom-free. Our patient with an isolated brain stem syndrome and no mental status changes was diagnosed as having a clinical isolated syndrome.

Prevalence of some risk factors in children with epilepsy compared to their controls.

AIM: The goal of this case-control study was to identify the significance of certain risk factors for epilepsy in Turkey. METHOD: A total of 805 cases, aged 1-16 years, followed-up for epilepsy at the Pediatric Neurology Department and a control group consisting of 846 age-matched cases without epilepsy were included in the study. The risk factors examined were gender, neurological impairment, febrile convulsion, head trauma, central nervous system infections, parental consanguinity, family history of epilepsy, prenatal and natal risk and newborn jaundice. Data regarding the investigated epilepsy risk factors were obtained through a questionnaire via personal interviews and the medical records and were assessed using univariate and multivariate analysis. RESULT: Univariate analysis showed an increased risk for epilepsy with a history of atypical febrile seizure (21.97-fold), severe and moderate head injury (27.76- and 7.09-fold respectively), CNS infection (4.76-fold), history of epilepsy in first-, second- or third-degree relatives (6.42-, 3.09- and 2.66-fold, respectively), presence of maternal hypertension (4.31-fold), an apgar score < or =6 at any time (7.78-fold) and neonatal jaundice (3.12-fold). Abnormal neurological signs increased the epilepsy risk 5.92 times in univariate analysis and 30.26 times in multivariate analysis. CONCLUSION: The most important risk factors for epilepsy in this study were neurological impairment, history of atypical febrile seizures, severe head injury and a low apgar score. Other important risk factors were moderate head trauma and a history of epilepsy in the family.

Nerve conduction in children suffering insulin dependent diabetes mellitus.

OBJECTIVE: To determine the incidence of peripheral neuropathy in children suffering Insulin Dependent Diabetes Mellitus (IDDM) as well as to determine the relationship between other criteria of the disease and neuropathy. METHODS: 40 children (17 males, mean age 11.9 years) suffering IDDM and receiving insulin therapy involving two injections a day and 30 healthy children (17 males, mean age 11.7 years) were included in the study. They were inquired about their demographical characteristics as well as the presence of neurological symptoms. Their detailed neurological examinations were conducted. Their glycemic control values (Hb A1C) were recorded, and their nerve conduction studies were performed from right upper and lower extremities. RESULTS: All nerve conduction values of children with IDDM were found to be significantly lower (p<0.0001) as compared to the control group. 60% of diabetic children (n=24) were found to suffer peripheral neuropathy. Statistically significant relationships were found between the glycemic control values and the peroneal, sural, tibial, ulnar and median nerve conduction velocities, and also between the duration of disease and the peroneal, sural, tibial and median nerve conduction velocities. CONCLUSION: The peripheral neuropathy is rather a frequently observed complication in diabetic children. The duration of disease and impaired glycemic control play an important role in the development of neuropathy. The introduction of new methods designed to ensure better glycemic control will reduce the incidence of the complication.

Effects of intranasal midazolam and rectal diazepam on acute convulsions in children: prospective randomized study.

In this study, the effects and side effects of rectal diazepam and intranasal midazolam were compared in the treatment of acute convulsions in children to develop a practical and safe treatment protocol. In the diazepam group, the seizures of 13 (60%) patients terminated in 10 minutes; however, 9 (40%) patients did not respond. In the midazolam group, 20 (87%) patients responded in 10 minutes, but 3 (13%) patients did not respond. Regarding the anticonvulsant effect, midazolam was found to be more effective than diazepam, and the difference was statistically significant (P < .05). The necessity of a second drug for the seizures that did not stop with the first drug was higher in the diazepam group than the midazolam group, and the difference was statistically significant (P < .05). We conclude that as an antiepileptic agent, intranasal midazolam is more effective than rectal diazepam. After administration, we did not observe any serious complications. Further investigations are necessary; however, intranasal administration is easy, so if the nasal drop and spray forms used in some European countries and the United States are available worldwide, it will be very useful for physicians in the emergency room.