Cerebellopontine angle arachnoid cyst associated with mirror movements.

Arachnoid cysts are benign developmental collections of cerebrospinal fluid (CSF). They constitute approximately 1% of intracranial masses. The cerebellopontine angle (CPA) arachnoid cysts are rare and often asymptomatic. The onset of symptoms and signs is usually due to the compression of the brain, cranial nerves and obstruction of CSF circulation. The major clinical symptoms for CPA arachnoid cysts were reported as headache, ataxia and 8(th) cranial nerve palsy. We report a patient with a CPA arachnoid cyst. He presented with cranial nerve palsies and mirror movements found in upper extremities. We postulated that CPA arachnoid cyst compressing the brain stem and the pyramidal decussation may lead to mirror movements. We conclude that mirror movements can be associated with CPA arachnoid cyst.

Can heart rate variability in children with epilepsy be used to predict seizures?

PURPOSE: The aim of this study was to examine interictal, pre-ictal and ictal autonomic system disturbance by comparing heart rate variability in children with uncontrolled epilepsy with that seen in healthy controls and children with controlled epilepsy. METHODS: Our study group included 20 children with refractory epilepsy, our control groups were composed of 20 children with well-controlled epilepsy and 20 healthy children. All subjects were evaluated by Holter ECG monitoring and 12-lead ECG to assess heart rate variability and QTc dispersion. The study group was also evaluated by Holter ECG during seizures. RESULTS: The study group exhibited significantly more pathological QTc dispersion than did the control groups. Heart rate variability was significantly suppressed: reduced parasympathetic activity with lower low frequency (LF) and high frequency (HF) band values were observed in the study group. Findings were similar in the well-controlled epilepsy group and the healthy group but differed from the uncontrolled epilepsy group. The examination of heart rate variability parameters during and before seizures revealed higher nLF and LF/HF ratio and lower nHF values demonstrating increased sympathetic activity. CONCLUSION: We suggest that children with refractory epilepsy have abnormalities of autonomic nervous system functioning which could be linked to the increased risk of sudden unexpected death seen in the patient group. It is possible that a chronically reduced vagal tone predisposes patients to a more dramatic stress response during their seizures. It is possible that heart rate variability parameter arising prior to seizures could be used to predict future seizures.

Association of interleukin 18, interleukin 2, and tumor necrosis factor polymorphisms with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory and degenerative disorder of the central nervous system. The measles virus (MV) and host and environmental factors are involved in the development of SSPE, but the precise mechanism by which the MV causes SSPE is still unknown. Studies have indicated that in SSPE patients, specific polymorphisms of certain genes are most likely involved in impairing the host's ability to eradicate the MV. The purpose of our study was to elucidate the role of polymorphisms in the genes encoding interleukin (IL)-2, IL-18, and tumor necrosis factor alpha (TNF-α) in the development of SSPE. Using the polymerase chain reaction with sequence-specific primers, the single-nucleotide polymorphisms (SNPs) of the promoter regions of IL-2 (-330), TNF-α (-308), and IL-18 (-137 and -607) were studied in 54 patients with SSPE and 72 healthy controls. The frequency of SSPE patients with the AA genotype of IL-18 at position -607 was significantly higher than the frequency of those with the CC genotype (p<0.001, odds ratio [OR]: 5.76), and a significantly higher proportion of patients had the C allele at -137 compared with the controls (p=0.002, OR: 2.72). In a haplotype analysis of two SNPs in the IL-18 gene, the frequency of the CA haplotype was significantly higher in SSPE patients (p<0.001, OR: 3.99) than in the controls. The IL-2 (-330) and TNF-α (-308) polymorphisms revealed no significant differences. In conclusion, these data suggest that the IL-18 gene polymorphisms at position -607 and -137 might be genetic risk factors for the SSPE disease.

PD-1 gene polymorphism in children with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory and degenerative disorder of the central nervous system. Several factors influence the risk of chronic brain infection with the mutant measles virus. However, to date, no pathogenic mechanism that may predispose to SSPE has been determined. Studies have indicated that specific polymorphisms in certain host genes are probably involved in impairing the ability of host immune cells to eradicate the measles virus in SSPE patients. Programmed cell death protein 1 (PD-1), a member of the CD28 family, is a negative regulator of the immune system. The purpose of our study was to investigate whether PD-1 gene polymorphisms affect susceptibility to the development of SSPE in Turkish children. In total, 109 subjects (54 SSPE patients and 55 healthy controls) were genotyped for the PD-1.9 C/T (rs2227982) single-nucleotide polymorphism (SNP). The distributions of T alleles in the PD-1.9 polymorphism in SSPE patients and healthy controls were 2.8 and 10.9%, respectively. There was a statistically significant difference between the groups; the 95% confidence interval (CI) was 0.06 to 0.85 and the odds ratio (OR) was 0.23 (χ(2) test). Thus, we identified an association between SSPE and the PD-1 rs2227982 gene polymorphism; the frequency of T alleles was higher in controls than in SSPE patients.

Treatment of severe amitriptyline intoxication with plasmapheresis.

Tricyclic antidepressant poisoning is one of the most common causes of serious intoxication. Here, we report a 2-year-old girl with severe amitriptyline (70 mg/kg) intoxication. She was in comatose, had generalized tonic clonic seizure, ventricular tachycardia, and wide QRS complexes. Although she did not respond to classical therapies, very good clinical response to plasmapheresis was obtained and she developed no complications. Thus, plasmapheresis may be an effective treatment modality in poisoning with drugs, which bind to plasma proteins with high affinity.

Acute tetraventricular hydrocephalus caused by mumps meningoencephalitis in a child.

Mumps is one of the common causes of childhood aseptic meningitis and encephalitis. Although central nervous system involvement is a common manifestation, hydrocephalus is a very rare complication of mumps, with just a few cases reported in the literature to date. Here we report on an 8-year-old boy with acute tetraventricular hydrocephalus caused by mumps meningoencephalitis and treated by external ventricular drainage and following ventriculoperitoneal shunt.