A Multinational Survey on Actual Diagnostics and Treatment of Subacute Sclerosing Panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system caused by the measles virus (MV). Its prevalence remains high in resource poor countries and is likely to increase in the Northern Europe as vaccination rates decrease. Clinical knowledge of this devastating condition, however, is limited. We therefore conducted this multinational survey summarizing experience obtained from more than 500 patients treated by 24 physicians in seven countries. SSPE should be considered in all patients presenting with otherwise unexplained acquired neurological symptoms. In most patients, the diagnosis will be established by the combination of typical clinical symptoms (characteristic repetitive myoclonic jerks), a strong intrathecal synthesis of antibodies to MV and typical electroencephalogram findings (Radermecker complexes). Whereas the therapeutic use of different antiviral (amantadine, ribavirin) and immunomodulatory drugs (isoprinosine, interferons) and of immunoglobulins has been reported repeatedly, optimum application regimen of these drugs has not been established. This is partly due to the absence of common diagnostic and clinical standards focusing on neurological and psychosocial aspects. Carbamazepine, levetiracetam, and clobazam are the drugs most frequently used to control myoclonic jerks. We have established a consensus on essential laboratory and clinical parameters that should facilitate collaborative studies. Those are urgently needed to improve outcome.

A study on visual evoked responses in children with chronic renal failure.

AIMS OF THE STUDY: Nervous involvement is frequent in patients with renal failure. Early recognition of the condition by electrophysiological tests may provide means for protective measures before irreversible damage of nervous system (NS) structures takes place. This study has two objectives: (1) examining whether pattern-reversal visual evoked potential (PR-VEP) studies may provide information relating to possible subclinical NS involvement in pediatric patients with chronic renal failure (CRF) and (2) looking for a possible relationship between serum parathormone (PTH) and creatinine levels and PR-VEP parameters. METHODS: PR-VEP recordings at low spatial frequencies were performed and peak-to-peak amplitudes and latencies of the P100 component were measured in 19 neurologically asymptomatic children with CRF, 15 of whom were on continuous ambulatory peritoneal dialysis (CAPD) and four on hemodialysis (HD). A similar procedure was applied to 29 healthy, age- and sex-matched, subjects. Patients were sub-grouped according to the serum PTH and creatinine levels. Student's-t and one-way ANOVA tests were used for comparisons within patient and control groups and sub-groups relating to serum PTH and creatinine levels. RESULTS: We did not demonstrate any statistically significant differences in PR-VEP parameters in patients vs. controls. PR-VEP amplitudes were higher in patients with low serum creatinine levels as compared to group with high creatinine values and to controls. No other relationship was found between PR-VEP parameters and serum PTH and creatinine levels in this pediatric population. CONCLUSION: Young patients with CRF and under dialysis do not necessarily show pathologic alterations in PR-VEPs when they are neurologically intact. This fact suggests that either PR-VEPs are not sensitive enough to detect clinically silent NS involvements in such patients, or it could be related to positive effects of the currently improving standards in the management of dialysis and supportive nutrition. Additional factors such as the age of the patient during examination, the latency between dialysis and visual evoked potential (VEP) assessment, or the selected check size may have some impact on the results and justify further studies.

A retrospective analysis of patients with febrile seizures followed by epilepsy.

This study was performed to investigate some clinical parameters of febrile seizures (FSs) in patients with epilepsy, testing any possible correlation between those parameters and the type of subsequent epilepsy. One hundred and nine patients with epilepsy having a history of FSs were evaluated for age at onset of FSs, interval between first FS and first afebrile seizure, recurrence rate, type of FSs, incidence of febrile status, family history for epilepsy and for FSs and the neurological status of the patient. The epilepsy that developed subsequently, were classified as generalised versus partial and also according to their syndromic subgroups. In temporal lobe epilepsy with mesial temporal sclerosis (TLE-MTS), statistical analyses revealed a younger age at onset of FSs, and a high incidence of episodes of febrile status and of complex FSs. Clinical characteristics of FSs followed by partial epilepsies were younger age at onset, presence of focal features and of febrile status, longer interval between the first FS and the first afebrile seizure, and a high incidence of FSs in the family history. In generalised epilepsies, however, a shorter interval between the first FS and the first afebrile seizure, a high incidence of single FS and of a family history of epilepsy were predominating characteristics. Results suggest that certain features of FSs may be predictive of a particular type of subsequent epilepsy.